CMS Neuro 3 Answers

NEURO FORM 3 by Sergio Angulo1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 E E E C A E E E D L A C K B B F IV ANTIBIOTIC C A C G C E Maybe E, not C A B C B B C E A E E D E E B E 40 41 42 43 44 45 46 47 48 49 50 C D C D G K D C A E E 1. 42 yo woman alcoholic, severe burning paresthesias both feet, decreased vibratory sens both ankles 2 months after ttmt for pulm TBC. Drugs include INH, rifampicin, vit B6, vit B9. Failure to take which drug? E. Vit B6 INH competes with vitamin B6 (pyridoxine) in its action as a cofactor in the synthesis of synaptic neurotransmitters. Resulting dose-related neurologic side effects include peripheral neuropathy, ataxia, and paresthesia. Such side effects are uncommon in the absence of risk factors. Patients at increased risk for INH-induced neurotoxicity include: Older adults Individuals with chronic liver disease Individuals who are malnourished Individuals with HIV infection Individuals with renal failure Diabetics Alcoholics Pregnant or breastfeeding women Children The risk of neurologic side effects can be reduced by concomitant administration of pyridoxine (vitamin B6). The usual dose is 10 mg/day (in patients who do not have highrisk features) and 25 to 50 mg/day (in those with one or more risk factors) [8]. Treatment of INH-induced neuropathies with pyridoxine generally requires higher doses (100 to 200 mg/day). 2. 42 yo woman, confused state for possible 3 days. Hx lithium, haloperidol, clonazepam for psych condition. 99.5F, pulse 85/min, BP 140/90. Pupils reactive 2mm. Coarse tremor upper extremities. Increased deep tendon reflexes. Responds to vigorous stim. Speech slow slurred. Appears sleepy and perplexed. WBC 14000, Urea 60mg/dl, Creat 2.5mg/dl, lithium 3.5 (normal 0.6-1.2). In add to discontinue lithium, next step? E. Begin hemodialysis Lithium is excreted almost entirely by the kidneys and is handled in a manner similar to sodium. Lithium is freely filtered but over 60 percent is then reabsorbed by the proximal tubules. Volume depletion or renal impairment from any cause increases lithium reabsorption. Examples of such conditions include gastrointestinal losses, acute decompensated heart failure, cirrhosis, and the administration of diuretics, nonsteroidal antiinflammatory drugs, or angiotensin converting enzyme inhibitors. With lithium poisoning, it is important to ask about dehydration, medications, and coingestants. Any condition causing dehydration, such as vomiting or diarrhea, fever or heat-related illness, anorexia or infection, may contribute to lithium toxicity. Neurologic findings develop late in acute lithium poisoning because time is required for the drug to be absorbed and to penetrate the central nervous system (CNS). Potential neurologic symptoms and signs include sluggishness, ataxia, confusion or agitation, and neuromuscular excitability, which can manifest as irregular coarse tremors, fasciculations, or myoclonic jerks. Severe lithium intoxication can lead to seizures, nonconvulsive status epilepticus, and encephalopathy. The treatment for lithium poisoning includes intravenous fluids to maintain the glomerular filtration rate and replace losses, gastrointestinal decontamination in selected circumstances (Whole bowel irrigation with polyethylene glycol (PEG), and hemodialysis in cases of severe toxicity. Perform hemodialysis for the following indications: Serum lithium concentration is greater than 4 mEq/L (or mmol/L), regardless of the clinical status of the patient. Serum lithium concentration is greater than 2.5 mEq/L (or mmol/L) and the patient manifests signs of significant lithium toxicity (eg, seizures, depressed mental status), has renal insufficiency or other conditions that limit lithium excretion, or suffers from an illness that would be exacerbated by aggressive IV fluid hydration (eg, heart failure) 3. 47 yo man, alcoholic, ER for confusion. Lives alone, disoriented for 4 hours. Empty container with sweet odor in apt. Creat 1.5mEq/l, HCO3 8mEq/l. ketones (-). Urianalysis, crystals. Cause? F. Ethylene glycol Methanol and ethylene glycol are frequently found in high concentration in automotive antifreeze and de-icing solutions, windshield wiper fluid, solvents, cleaners, fuels, and other industrial products. Few conditions other than methanol and ethylene glycol intoxication cause a profound high anion gap metabolic acidosis (serum bicarbonate less than 8 meq/L (or 8 mmol/L)), and most of these conditions present in a characteristic fashion with a high serum lactate (eg, status epilepticus, profound shock, ischemic bowel) or diabetic ketoacidosis (table 2). Ethylene glycol metabolites target the kidney and lead to reversible oliguric or anuric acute kidney injury (acute renal failure), which in turn slows elimination of ethylene glycol [7]. The renal failure is primarily due to glycolate-induced damage to tubules, although tubule obstruction from precipitated oxalate crystals may contribute [8,9]. Hypocalcemia in ethylene glycol overdose results from calcium oxalate formation. With ingestions of either parent alcohol, a profound anion gap metabolic acidosis develops, which directly correlates with the accumulation of toxic acid metabolites [2,4]. Acidemia increases the ability of the toxic metabolites to penetrate cells, further depressing CNS function and causing a rapid downward spiral of hypoxia and acidemia [10]. Urine testing — Examination of the urine for oxalate crystals and fluorescence is frequently performed in patients with possible ethylene glycol poisoning, but care should be taken not to over-interpret positive or negative results. 4. 27 yo man. Hx 2w neck and left shoulder pain, radiating through lat surface of left arm to thumb and index. Decreased biceps reflex. Strength 4/5 extension left wrist. Sens light touch decreased left thumb and index. Dx? Progressive difficult with activities daily living. Difficult remembering names. Oriented person and place. Which ttmt? a. Indifferent mood and affect. Disoriented in time. C6 radiculopathy 5. Minimental 22/30. No auditory/visual halluc. no serious illness. Acetylcholinisterase inhibitors.C. no drugs. No head trauma. 62 yo man. Hx 3y forgetfulness. Probable AD dementia is a syndrome of dementia defined by the following characteristics: Interference with ability to function at work or at usual activities A decline from a previous level of functioning and performing Not explained by delirium or major psychiatric disorder . Medications metoprolol. Echocard. CT no infarctation or hemorrhage. Next step management? E. glyburide. ER 1h ago with episode of 25 min right facial drop. or using memantine alone in patients who do not tolerate or benefit from a cholinesterase inhibitor (Grade 2B). Hx of HTN. . individual patient tolerance. we suggest adding memantine (10 mg twice daily) to a cholinesterase inhibitor. Controlled with diuretic. thrombus left atrial. Warfarin With the major exception of patients with atrial fibrillation. poor judgment impaired visuospatial abilities impaired language functions changes in personality. we suggest supplementation with vitamin E (2000 IU daily) (Grade 2C). chronic AF. left vent hypertrophy. HTN fllow up. fosinopril. no additional management indicated. Deep tendon reflexes absent in ankles. 7. In patients with mild to moderate Alzheimer dementia (AD) who are interested in seeking therapy with vitamins or who are unable or unwilling to take memantine. BP 170/100. in some patients with advanced dementia it may make sense to discontinue administration of medications to maximize quality of life and patient comfort. Gout. DM2. the risk of major hemorrhage is higher with warfarin than with aspirin. However. BP 140/70. mild stenosis right and left internal carotid art without thrombus or ulcer. we suggest continuing memantine. and objective bedside mental status examination or neuropsychological testing Cognitive impairment involving a minimum of two of the following domains: impaired ability to acquire and remember new information impaired reasoning and handing of complex tasks. given the possibility that memantine may be disease-modifying (Grade 2C). CAD. and physician experience. where anticoagulation is superior to aspirin. mild mitral regurg. Pulse 80/min irregularly irregular. No murmur. behavior or comportment We suggest a treatment trial with a cholinesterase inhibitor for patients with mild to moderate dementia (MMSE 10-26) (Grade 2A). In patients with moderate to advanced dementia (MMSE <17). allopurinol. and galantamine can be based upon cost.Cognitive impairment established by history-taking from the patient and a knowledgeable informant. 77 yo man. The choice between donepezil. resp 18/min. Most appropriate to prevent next episode? E. weakness right arm hand. The benefits of vitamin E are likely to be modest and could be offset by combination therapy with memantine. randomized clinical trials have found no statistically significant difference between aspirin and anticoagulant therapy for reducing the risk of recurrent ischemic stroke [79]. Achilles tendon reflex decreased. 6. In patients with severe dementia (MMSE <10). rivastigmine. Carotid US. 67 yo woman. Vitamin E is not recommended for other forms of dementia or for the prevention of AD. However. as efficacy appears to be similar. Decreased sensation pinprick distal lower extremities. Tight bandlike sensation midabdominal. Started on feet. numbness. The most common clinical presentation of primary progressive MS is a spinal cord syndrome with spastic paraparesis and no clear sensory level. Decrease pinprick up to the umbilicus. These issues are discussed elsewhere. While the benefit outweighs the risk in most patients. or cryptogenic type. nearly all such patients should be treated with an antiplatelet agent. or a spinal cord syndrome (eg. . Aspirin (50 to 100 mg daily). Since antiplatelet agents are effective for the prevention of recurrent ischemic stroke in patients with a history of noncardioembolic ischemic stroke or TIA of atherothrombotic. lacunar (small vessel occlusive). or weakness). the use of anticoagulant therapy is also associated with an increased risk of major bleeding. long tract symptoms/signs (eg. slowly ascending symmetrically to umbilicus. clopidogrel (75 mg daily). Uninary urgency. apixaban. paresthesia. and the combination of aspirin-extended-release dipyridamole (25 mg/200 mg twice a day) are all acceptable options for preventing recurrent noncardioembolic ischemic stroke. felt numbness. a brainstem syndrome (eg. dabigatran. nocturia. careful consideration of the risk to benefit ratio is necessary in those at relatively low risk.8. In addition to atrial fibrillation. Long-term anticoagulation with warfarin. which typically presents in a young adult with a clinically isolated syndrome suggestive of MS such as optic neuritis. internuclear ophthalmoplegia). or rivaroxaban should be considered as prevention for patients with chronic nonvalvular atrial fibrillation who have had an ischemic stroke or transient ischemic attack. frequency. Multiple sclerosis Most patients with MS have relapsing-remitting disease. transverse myelitis). Dx? E. Diffuse hyperreflexia. Slowing left eye adduction during saccadic movements. However. other potential cardiac sources of embolism for which anticoagulation therapy may be indicated for select patients include the following: Mechanical heart valves and a subpopulation of high-risk patients with bioprosthetic valves Left ventricular thrombus Dilated cardiomyopathy Rheumatic valve disease Recent myocardial infarction in high-risk patients 32 yo woman hx 1w progressive sensory loss. Decreased pinprick and light touch in upper extremities. pins-and-needles. tingling both arms. tightness. numbness. and extremities. On testing sensation with a sharp object such as a pin. bladder.[3] and stiffness in the back. 9. Symptoms are commonly described as numbness. coldness.Internuclear ophthalmoplegia (INO) refers to abnormal horizontal ocular movements with lost or delayed adduction and horizontal nystagmus of the abducting eye. Convergence is typically preserved. 72 yo man progressive aching lower extremities during walking for a year. Sensory symptoms are the most common initial feature of MS (table 3) and are present in almost every patient at some time during the course of disease. 4+ in lower extremities. often more critically. can expand and elongate over time. called a syrinx. especially in the hands. These symptoms typically vary depending on the extent and. weakness. The sensory features can reflect spinothalamic. or feels like a mild electric shock. and an atonic dilated bladder that empties by overflow can be the end result. Deep tendon reflex absent in upper extremities. The most common urinary complaints are frequency and urgency. Urinary incontinence becomes more common as the disease progresses. Bowel. Each patient experiences a different combination of symptoms. 4/5 lower extremities. 27 yo woman. hx 1y progressive weakness. A bilateral sensory level is more common than a hemisensory syndrome. The disorder generally leads to a cape-like loss of pain and temperature sensation along the back and arms. Strength 3/5 upper extremities. posterior column. or dorsal root entry zone lesions. It is caused by a lesion of the medial longitudinal fasciculus in the brainstem on the side of diminished adduction. Syringomyelia may also cause a loss of the ability to feel extremes of hot or cold. shoulders. This cyst. Syringomyelia (/sᵻˌrɪŋɡəmaɪˈiːliə. or that the stimulus spreads in a ripple fashion from the point at which it is applied. atrophy forearms and hands bilat. tingling. to the location of the syrinx within the spinal cord. Syringomyelia. The damage may result in pain. paralysis. The extent of sphincter and sexual dysfunction often parallels the degree of motor impairment in the lower extremities. Babinski + bilat. -ɡoʊ-/[1][2]) is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. Pain after 15 . and sexual dysfunction are common in MS. destroying the spinal cord. 10. patients frequently report that the sharp feeling is increased. or swelling of the limbs or trunk. Dx? D. Muscle wasting. In the clinical series described above. Spondylosis. The erect. Pain radiates from buttocks to feet. and weakness in the legs. reflecting involvement of spinal nerve roots within the lumbar spinal canal. leaning forward). Progress to 5-10 min after walking. is the most common cause of lumbar spinal stenosis and typically affects individuals over the age of 60 years [4]. Strenght and sens normal in extremities. Pain in a single nerve root distribution occurred in only 6 percent. relaxing and inward buckling of the ligamentum flavum. The primary symptoms of LSS include discomfort. usually pain. Facet joint arthropathy and osteophyte formation follow. or other factors can lead to disc protrusion and/or loss of disc height with attendant loading of the posterior elements of the spine.min of walking. extended position narrows the lumbar canal by reducing the interlaminar space. along with hypertrophy of the ligamentum flavum. smoking. occurring in 93 percent of patients. Dorsal pedis pulses normal. in some patients with LSS. Obesity may also be a risk factor [5]. No pain with sitting was the most specific finding. In one systematic review of clinical findings in LSS. to be exacerbated with walking. However. especially when intrathecal pressures are elevated Neurogenic (or pseudo) claudication is a hallmark of LSS [1]. trauma. ligamentum flavum hypertrophy. and disc bulging) can encroach on the central canal and the neural foramina. This may explain the onset or persistence of symptoms with prolonged standing. resolve after stop. More than half of patients with LSS also reported relief with sitting or flexion at the waist (squatting. Low back pain in LSS is not necessarily associated with the claudication symptoms. and relieved with sitting or lying [18]. Progressive disc degeneration due to aging. cervical arthritis. The neurologic examination is often normal in patients with LSS. Decreased breath sounds bilat. and/or maintaining certain postures. Symptoms were bilateral in most (68 percent). standing. and usually involved the entire leg rather than just the upper or lower leg (78 versus 15 and 6 percent. Lumbar spinal stenosis.17]. followed by numbness and/or tingling in 63 percent. neck and arm pain. Tendon reflex 1+ knees. but often asymmetrical. neurogenic claudication was moderately sensitive and present in 82 percent of patients [19]. This is the tendency for symptoms. sensory loss. findings that were relatively specific for LSS (84 and 92 percent respectively). but less sensitive. Dx? L. causing overlap of laminar edges of adjoining vertebral bodies. The straight leg raising sign is present only in a minority of patients (10 percent) [18. and after standing of bed. more prolonged or severe nerve root involvement may lead to fixed . pain was the most common symptom. It is also possible that increased metabolic demands on spinal nerve roots during walking may exceed the available microvascular blood flow. Hx of COPD. Babinski (-) bilat. respectively). including the facet joints.20]. All of these processes (facet osteophytes. Many patients with LSS are symptomatic only when active. 2+ upper extremities. Exacerbation by prolonged standing in an erect posture was an even more sensitive but less specific sign. and weakness in 43 percent [18]. Low back pain occurred in 65 percent and was described as mechanical and mild. or degenerative arthritis affecting the spine. and rostral-anterior migration of the superior facets [3. HIV infection. such lesions must be bilateral as these cranial nerve nuclei receive bilateral innervation. 12. Risk factors for spinal epidural abscesses (SEAs) include epidural catheters. In fact. Appears ill. diabetes mellitus. Motor neuron degeneration pseudobulbar palsy describes impairment of function of cranial nerves IX-XII due to upper motor neuron lesions of the corticobulbar tracts in the mid-pons. MRI brain no abnormalities. described as "shooting" or "electric shocks" in the distribution of the affected nerve root. Epidural abscess of the spine. Pinprick decreased below C7. usually for pain control (table 1). muscle strength normal upper extremities. most patients present to healthcare providers multiple times before the diagnosis is made. Oriented and normal affect. and bladder or bowel dysfunction. (See 'Portals of entry' above. an untreated abscess causes symptoms that progress in a typical sequence [5]: ●Back pain. Current. which is often focal and severe. the most common sites of origin are infections of skin and soft tissues and complications of spinal surgery or other invasive procedures. however. it may quickly become irreversible.30. Mechanism of disease? C. only a small proportion of patients have all three components at presentation [26.and/or progressive neurologic deficits.) The initial manifestations of spinal epidural abscess (SEA) are often nonspecific and include such signs and symptoms as fever and malaise. Unable to lift lower extremities against gravity. bacteremia. Babinski (+) bilat. Injection drug abuse is also an important predisposing factor. Pseudobulbar palsy is a medical condition characterized by the inability to control facial movements (such as chewing and speaking) and caused by a variety of neurological disorders. Approximately one-third of patients with SEA have no identifiable source for the infection. 57 yo man progressive weakness arms and legs. Rash started 10 days ago from right foot to calf. 11. and pseudobulbar palsy.31]. and demonstrate . Over time. 77 yo man hospitalized with antibiotic iv for leg rash is unable to urinate. sensory changes. then ●Motor weakness. nuchal rigidity. 1 day ago onset new neck pain. including epidural catheters that are left in place. leading to delayed or missed diagnosis [26]. However.2F. Thus. Hx DM2 treated with glyburide. Tendon reflexes brisk. spinal pain. Patients experience difficulty chewing and swallowing. alcoholism. Admission right lower extremity warm and erythematous. and neurologic deficits. Dx? A. and intravenous drug abuse. fasciculation in hands and right lower extremity. 6 months ago normal strength. then ●Nerve root pain. urgent intervention may be required if progression of weakness or other neurologic findings are detected. Fever may be absent in some patients. and then ●Paralysis Once paralysis develops. have increased reflexes and spasticity in tongue and the bulbar region. Among the two-thirds for whom a portal of entry can be identified. 102. Normal sens and cranial nerves. The classical diagnostic triad consists of fever. For clinically evident dysfunction to occur. Upper motor neuron findings of weakness. Asymmetric limb weakness is the most common presentation of ALS (80 percent). may precede or follow the onset of upper motor neuron and/or lower motor neuron dysfunction in patients with ALS. especially those involving demyelination Multiple sclerosis and other inflammatory disorders High brain stem tumors Metabolic causes: osmotic demyelination syndrome[2] Neurological involvement in Behçet's disease Brain trauma ALS has an age distribution that peaks in the seventh to eighth decades. However.slurred speech (which is often the initial presentation of the disorder). . pupil left eye dilated. Begins to hyperventilate. and fasciculations are a direct consequence of degeneration of lower motor neurons in the brainstem and spinal cord. specifically to the corticobulbar tract (upper motor neuron tract to cranial nerve motor nuclei). The clinical hallmark of amyotrophic lateral sclerosis (ALS) is the combination of upper motor neuron and lower motor neuron signs and symptoms. However. ptosis left eye. sometimes also demonstrating uncontrolled emotional outbursts. 27 yo man ER multiple trauma after motor vehicle collision. ALS can occur in people in their twenties. initially arousable. CADASIL syndrome Progressive supranuclear palsy Amyotrophic lateral sclerosis Parkinson's disease and related multiple system atrophy Various motor neuron diseases. pattern and speed of spread. and the degree of upper and lower motor neuron dysfunction produce a disorder that is remarkably variable between individuals. Cause? K. Frontotemporal dementia may be associated with ALS in 15 to 50 percent of cases. The lower motor neuron findings of weakness. Cognitive impairment. differences in site and segment (cranial. typically related to frontotemporal executive dysfunction. uncal herniation. usually manifested as dysarthria or dysphagia. or lumbosacral) of onset. Examples include: Vascular causes: bilateral hemisphere infarction. is the next most common pattern (20 percent). Pseudobulbar palsy is the result of damage of motor fibers traveling from the cerebral cortex to the lower brain stem.[1] The condition is usually caused by the damage (bilateral degeneration) to the neurons of the brain stem. This damage might arise in the course of a variety of neurological conditions that involve demyelination and bilateral corticobulbar lesions. 13. thoracic. and spasticity result from degeneration of frontal motor neurons. Bulbar onset. hyperreflexia. cervical. Increasing unresponsive next 45 min. atrophy or amyotrophy. which results from compression of the contralateral[7] cerebral crus containing descending . CN VI) and the superior oblique (innervated by trochlear nerve a.a.k. Another important finding is a false localizing sign. which may affect the parasympathetic input to the eye on the side of the affected nerve. Pupillary dilation often precedes the somatic motor effects of CN III compression called oculomotor nerve palsy or third nerve palsy. CN III).The uncus can squeeze the oculomotor nerve (a. CN IV). the so-called Kernohan's notch.k. Compression of the ipsilateral posterior cerebral artery will result in ischemia of the ipsilateral primary visual cortex and contralateral visual field deficits in both eyes (contralateral homonymous hemianopsia). The symptoms occur in this order because the parasympathetic fibers surround the motor fibers of CN III and are hence compressed first.a. This palsy presents as deviation of the eye to a "down and out" position due to loss of innervation to all ocular motility muscles except for the lateral rectus (innervated by abducens nerve (a. causing the pupil of the affected eye to dilate and fail to constrict in response to light as it should.a.k. [1] Downward herniation can stretch branches of the basilar artery (pontine arteries). is present in more than 90 percent of patients at some point during the illness but may be absent at presentation. Since the corticospinal tract predominately innervates flexor muscles. dilated. especially provoked by startle. and deficits involving higher cortical function.corticospinal and some corticobulbar tract fibers. frequent irregular jerk movements upper and lower extremities. protein 45. 14. Death usually occurs within one year of symptom onset Mental deterioration may be manifest as dementia. Mood changes such as apathy and depression are common. fixed pupils with[9] paralysis of upward eye movement giving the characteristic appearance of "sunset eyes". but also insomnia. sCJD should always be considered in a patient with the combination of a rapidly progressive dementia and myoclonus. 67 yo man. CSF glucose 80. MRI vague abnormal T2 signal in basal ganglia. RBC(-). Also found in these patients. Additional finding in CSF? B. are also common.[5][8] Transtentorial herniation can occur when the brain moves either up or down across the tentorium. Normal strength. and anxiety occur less frequently. With disease progression. even when dementia is profound. memory. Myoclonus. Concentration. ataxic gait. downward herniation is characterized by obliteration of the suprasellar cistern from temporal lobe herniation into the tentorial hiatus with associated compression on the cerebral peduncles. can be radiographically characterized by obliteration of the quadrigeminal cistern. and may be a presenting sign [68. euphoria. the diencephalon and parts of the temporal lobes of both of the cerebral hemispheres are squeezed through a notch in the tentorium cerebelli. extension of the leg may also be seen. emotional lability. causing them to tear and bleed. and judgment difficulties are frequent early signs [67]. particularly hypersomnia. Sleep disturbances. behavioral abnormalities. Intracranial hypotension syndrome has been known to mimic downwards transtentorial herniation. visual or . The Centers for Disease Control and Prevention (CDC) outline the following criteria for probable sporadic CJD [111]: Progressive dementia and At least two out of the following four clinical features: myoclonus. Mild ataxia dysarthria. The result is usually fatal. Radiographically. reflexes 3+. Increased 14-3-3 protein concentration. dementia becomes dominant in most patients and can advance rapidly. Upwards herniation. Rapidly progressive mental deterioration and myoclonus are the two cardinal clinical manifestations of sCJD. known as a Duret hemorrhage. hx 6w progressive cognitive changes and twitching movements arms and legs. however descending herniation is much more common. Alert but distractable.[8] Other symptoms of this type of herniation include small. This leads to ipsilateral hemiparesis (as these tracts are above their decussation where they are compressed). often as a terminal complication is the development of diabetes insipidus due to the compression of the pituitary stalk. Minimental 19/30. WBC (-). called ascending and descending transtentorial herniation respectively. In central herniation. on the other hand.69]. peripheral Anti-CV2/CRMP5 neuropathy Anti-Ma proteins¶ (Ma1. myelitis. encephalomyelitis Anti-PCA2 Encephalomyelitis. encephalomyelitis Anti-recoverinΔ Cancer-associated retinopathy Anti-bipolar cells of Melanoma-associated retinopathy the retina◊ Partially-characterized paraneoplastic antibodies* Anti-Zic 4 Cerebellar degeneration Anti-ANNA-3 Sensory neuronopathy. amphiphysin. Antibodies. other Gynecological. brainstem Anti-Hu (ANNA-1) encephalitis. Ma2. These antibodies are surrogate markers of the paraneoplastic disorder. cerebellar degeneration. collapsin response-mediator protein5 (CRMP-5). but in most of these disorders. pyramidal/extrapyramidal dysfunction.cerebellar disturbance. Yo (also known as Purkinje cell cytoplasmic antibody type 1 [PCA-1]). cerebellar dysfunction Anti-AChR Myasthenia gravis Associated cancers SCLC. Antibodies directed against intracellular neuronal proteins (called classical paraneoplastic or onconeuronal antibodies) – These antibodies belong to the category of "well characterized" paraneoplastic antibodies (table 2). akinetic mutism and Atypical electroencephalogram (EEG) during an illness of any duration. thymoma. Examples include Hu (also known as type 1 anti-neuronal nuclear antibody [ANNA-1]). limbic. hypothalamic. Tr (also known as delta/notch-like epidermal growth factor-related receptor [DNER]). cerebellar degeneration Antibodies that occur with and without cancer association Anti-VGCC Lambert-Eaton myasthenic syndrome. cerebellar degeneration. other solid tumors Breast. Ma2) Limbic. and/or a positive 14-3-3 cerebrospinal fluid (CSF) assay with a clinical duration to death less than two years. and/or magnetic resonance imaging (MRI) high signal abnormalities in caudate nucleus and/or putamen on diffusion-weighted imaging (DWI) or fluid attenuated inversion recovery (FLAIR) and Routine investigations should not suggest an alternative diagnosis. SCLC Hodgkin lymphoma SCLC. and/or autonomic dysfunction Anti-Yo (PCA-1) Cerebellar degeneration Cerebellar degeneration. paraneoplastic syndromes and associated cancers Antibody Syndrome Well characterized paraneoplastic antibodies* Encephalomyelitis including cortical. and their detection almost always indicates the presence of an underlying tumor. the pathogenic mechanism is believed to be mediated by cytotoxic T-cells. Ri (also known as type 2 antineuronal nuclear antibody [ANNA-2]). sensory neuronopathy. other Germ-cell tumors of testis. and recoverin. brainstem encephalomyelitis (infrequently cerebellar degeneration) Anti-amphiphysin Stiff-person syndrome. lung cancer SCLC Melanoma SCLC SCLC SCLC SCLC Thymoma . breast Breast. chorea. lung cancer. brainstem encephalitis. opsoclonusAnti-Ri (ANNA-2) myoclonus Anti-Tr (DNER) Cerebellar degeneration Encephalomyelitis. gynecological. 4 months girl. others Often without cancer. Dx? B. seizures. psychosis. SCLC Morvan syndrome and some patients with neuromyotonia Thymoma and variable solid tumors Subacute pandysautonomia Encephalomyelitis with muscle spasms. and . and autonomic Teratoma dysfunction Limbic encephalitis. limbic encephalitis SCLC Limbic encephalitis. dyskinesias. ER b/c drowsiness 3 day hx. 25 th percentile for length. seizures Thymoma. including downward displacement of cerebellar tissue through the foramen magnum (arrow). prosopagnosia. 90th percentile for head circumference. rigidity.Anti-NMDAR Anti-AMPAR Anti-GABA(A) receptor Anti-GABA(B) receptor Anti-LGI1 (previously attributed to VGKC) Anti-Caspr2 (previously attributed to VGKC) Anti-nAChR Anti-GlyR Multistage syndrome with memory and behavioral disturbances. Chiari type 2 malformation A sagittal T1-weighted MRI in a pediatric patient shows several characteristic intracranial findings of the Chiari II malformation. spitting up after feeding. Arrival. responsive to tactile stimuli. hyperekplexia Anti-mGluR5 Limbic encephalitis. 98F. feeding poorly. a small fourth ventricle (arrowhead). myoclonus. Bulging anterior fontanel. Also. resp 24/min. Hyperpigmented macule with hair growth in upper lumbar spine noted at birth. one patient with lung cancer Hodgkin lymphoma§ or no tumor Hodgkin lymphoma or no tumor 15. refractory seizures Thymoma Seizures. BP 100/70. Uncomplicated pregnancy and delivery. psychiatric disturbances Variable solid tumors Encephalopathy. involuntary movements Anti-mGluR1 Cerebellar degeneration SCLC. A reduced volume of the posterior fossa with an enlarged foramen magnum and low torcula with ventral displacement of the tentorium cerebelli are constant features. Chiari II malformation (CM-II). Infarct what territory cerebral artery? F. Hx 2 year AF treated with aspirin. various visual field patterns are seen. Alert and oriented. Chiari I malformation (CM-I) is characterized by abnormally shaped cerebellar tonsils that are displaced below the level of the foramen magnum. BP 110/80. Lesions of the occipital lobe tend to produce an isolated homonymous hemianopia (ie. Almost all patients with a myelomeningocele have CM-II. 16. Left homonimus hemianopia. pyramis and uvula). The malformation obstructs the outflow of cerebrospinal fluid through the posterior fossa. resp 22/min. pulse 95/min. 98F. Depending on the location of the lesion. Denting car while parking.tectal beaking (dashed arrow). not associated with other neurologic deficits). Off-balance. . 3 day hx visual difficulties. 77 yo woman. The Chiari II malformation (CM-II) is characterized by downward displacement of inferior cerebellar vermis (involving the nodulus. Strength and sens normal. and cerebellar tonsils and medulla through the foramen magnum into the upper cervical canal. and a spinal myelomeningocele. in association with a myelomeningocele at the lumbosacral or occasionally a higher level of the spinal cord (image 2). Speech normal. causing hydrocephalus. is characterized by downward displacement of the cerebellar vermis and tonsils. Right posterior Occipital lobe lesions are the most common cause of homonymous hemianopia and are most often vascular in origin [8-10]. a brainstem malformation with beaked midbrain on neuroimaging. Read only words in right side page. also known as Arnold-Chiari malformation. and most have associated hydrocephalus. Muscle fatigability or cramping is common. and opisthotonos. Babinski (-). dry mouth. and are characterized by symptoms such as torticollis. . P/Q-type VGCCs make up more than 95 percent of the functioning receptors at the neuromuscular junction (NMJ) and probably represent the main immunologic target in LEMS. dry mouth was identified in 77 percent. CXR right upper lobe mass. play a central role in the pathophysiology of LEMS. No muscle tenderness. 67 yo woman confused and belligerent one day after cholecystectomy. male sex. An extremely rare dystonia. oculogyric crisis. Other symptoms may include blurred vision and constipation. Reflexes absent. There tends to be relative preservation of distal muscle function. Myoneural junction The clinical features of LEMS were well defined by Lambert and Eaton in Minnesota [29] and by O'Neill and colleagues in England [18]. 1h later present hyperextended neck. Ocular symptoms. Repetitive conjugate upward movement of eyes. and this feature is present in almost all patients at some point in the illness. retrocollis. Risk factors for dystonia include young age. Problem arising from chair and climbing stairs. and a history of acute dystonic reaction. Localization abnormality? C. IM haloperidol administered. Most appropriate treatment? A. use of cocaine. Patients with LEMS and small cell lung cancer typically develop a more rapidly progressive course of proximal and then distal arm muscle weakness than patients who have non-tumor LEMS [30]. Anticholinergic Acute dystonias are involuntary contractions of major muscle groups. weakness hip flex and knee extension. These antibodies interfere with the normal calcium flux required for the release of acetylcholine. dysphagia. Dry mouth from reduced salivation is the most common autonomic symptom and occasionally is the presenting complaint. no medication. while erectile dysfunction is common in men with LEMS. particularly ptosis. Smoking for 40y. CK activity 25U/L. In the largest study of autonomic dysfunction in LEMS involving 30 patients. and impotence was present in 45 percent of all men [31]. Normal sens. Persistent cough. and difficulty chewing have also been occasionally observed. ESR 95. Hx 2y mild dementia. Oropharyngeal symptoms of dysarthria. are the most common cranial nerve manifestation with LEMS. Occasional patients have a subacute or acute presentation. can be life threatening. 57 yo man progressive weakness both legs for 5 months. 19. These are usually rapid in onset and highly disturbing to patients. Antibodies directed against the voltage-gated calcium channel (VGCC). weigh loss. laryngospasm. Symmetrical muscle involvement is the rule.18. Cranial nerve involvement is present in a minority but is typically less severe and less striking than that seen in MG. Upper extremity complaints are usually modest and typically involve proximal muscle function. Patients typically describe an alteration in gait or difficulty arising from a chair or managing stairs. particularly after protracted exercise. Autonomic dysfunction is often present and can be an important clue to the diagnosis. a large transmembrane protein with multiple subunits. Most patients present with complaints of slowly progressive proximal muscle weakness. Alzheimer’s. Many describe aching or stiff muscles. Prophylactic treatment with an anticholinergic agent (benztropine or diphenhydramine) is recommended to prevent an acute dystonic reaction in patients who receive intramuscular haloperidol (eg. especially those with recent or threatened vascular complications such as visual loss. ESR 110. 20/200 right eye. Prophylactic treatment is particularly important in individuals with little prior exposure to antipsychotics. adding an anticholinergic antiparkinson medication such as benztropine 1 to 2 mg by mouth twice daily may be effective. Dx? G. aches morning. sallow waxy complexion. In this setting.Dystonias that are very disturbing can be treated with 1 to 2 mg of benztropine or 50 mg of diphenhydramine daily. 21. but treatment should not be withheld while awaiting the performance or the results of the biopsy. Scant beard. A temporal artery biopsy should be obtained as soon as possible. Impaired vision is the most common symptom that leads a patient with a nonfunctioning . we suggest use of intravenous pulse methylprednisolone. Hct 32%.) The emergence of a dystonia should lead to a reevaluation of the patient's antipsychotic regimen. If changing the antipsychotic is not an option. Pituitary adenoma. 47 yo man hx 8 month progressive severe bifrontal headache. intramuscular haloperidol 5 or 10 mg can be accompanied by intramuscular benztropine 1 or 2 mg. Platelet 300. For patients in whom the diagnostic suspicion of GCA is high. 2h sudden onset dim vision right eye. therapy should be started immediately. the preferred intervention is changing to an antipsychotic with a lower liability for EPS (table 1). 20. This is then followed by oral therapy with 1 mg/kg per day (maximum of 60 mg/day). (See "Classification and evaluation of dystonia". Symptoms started 3w ago generalized stiffness. If there is a strong suspicion of GCA as the cause of visual loss. often even before it is confirmed. Corticosteroid therapy now and temporal artery biopsy within 3 day The diagnosis of giant cell arteritis (GCA) should be considered in a patient over the age of 50 who complains of or is found to have: New headaches Abrupt onset of visual disturbances Symptoms of polymyalgia rheumatica Jaw claudication Unexplained fever or anemia High erythrocyte sedimentation rate (ESR) and/or high serum C-reactive protein (CRP) Glucocorticoid treatment should be instituted promptly once the diagnosis of GCA is suspected strongly. WBC 6000. as recommended above for uncomplicated GCA. Firm tender temporal arteries bilat. 20/40 left eye. bitemporal headache. 72 yo woman. As an example. After acute treatment of the dystonia.000. Bilat temporal hemianopia. Milder dystonias can be treated with oral benztropine 1 to 2 mg once or twice daily. in the treatment of acute agitation or psychosis). Visual acuity normal. the typical dose is 1000 mg intravenously each day for three days. administered intravenously or intramuscularly. Initial step management? C. Most are either intrasellar or suprasellar. Visual impairment is caused by suprasellar extension of the adenoma. BMI 28. presumably caused by expansion of the sella. resulting in hypogonadism in both men and women Craniopharyngiomas are solid or mixed solid-cystic benign tumors that arise from remnants of Rathke's pouch along a line from the nasopharynx to the diencephalon. About 50 percent present clinically during childhood and adolescence. No crossing right index and middle fingers. The major presenting symptoms are growth retardation in children and abnormal vision in adults. to variable degrees. 40. almost 90 percent of men complain of erectile dysfunction. Growth failure. However. Flexion interphalangeal joint right hand strength 5/5. interfere with writing ability. these symptoms are not usually the reason that the patient seeks medical attention. Sens normal. Decreased active abduction small and index fingers. a constellation of neuroophthalmologic findings. At the time of initial presentation with a neurologic symptom. 25 and 25 percent of cases. caused by inferior extension of the adenoma. ●Pituitary apoplexy induced by sudden hemorrhage into the adenoma. some not until age 70 or 80 years. pituitary hormonal deficiencies. leading to compression of the optic chiasm. that result from ectopic pinealomas. respectively [4]. In addition. Other patterns of visual loss can also occur. thyroid stimulating hormone. the other 50 percent present after age 20 years.adenoma. gonadotropin. if both. Other neurologic symptoms that may cause a patient with a sellar mass to seek medical attention include: ●Diplopia. Headaches. One or both eyes may be affected and. an intrasellar lesion should be suspected when there is any unexplained pattern of visual loss. Direct damage to or compression of normal structures can lead to a range of endocrine abnormalities. 52 yo 6-w financial advisor hx progressive right hand weakness. and adrenocorticotropic hormone in an estimated 75. an extremely uncommon presentation. The most common pituitary hormone deficiencies are of gonadotropins. of which over 80 percent are gonadotroph adenomas. causing excruciating headache and diplopia. ●Parinaud syndrome. The quality of the headache is not specific. Diabetes insipidus is frequent when the pituitary stalk is involved. Unable to pinch the plmar apect of the right thumb agains the radial side of the index finger without flexing the interphalangeal joint of the thumb to provide strength. . Frequently observed complications include deficiencies of growth hormone. ●Cerebrospinal fluid rhinorrhea. most often paralysis of upward conjugate gaze. which can be caused by either hypothyroidism or growth hormone deficiency. Diminished visual acuity occurs when the optic chiasm is more severely compressed. The most common complaint is diminished vision in the temporal fields (bitemporal hemianopsia). many patients with sellar masses. is the most common presentation in children. including diabetes insipidus. to seek medical attention. when carefully questioned. admit to symptoms of pituitary hormone deficiencies. Thus. Sexual dysfunction is the most common endocrine manifestation in adults. are common. while most women have amenorrhea. induced by oculomotor nerve compression resulting from lateral extension of the adenoma. 22. . and problems with sleep.[15] An unusual taste in the mouth and personality changes are also early signs. CTS is a clinical diagnosis. Trips walking upstairs. The hallmark of classic CTS is pain or paresthesia (numbness and tingling) in a distribution that includes the median nerve territory. Carpal tunnel syndrome. motor involvement leads to complaints of weakness or clumsiness when using the hands. or opening jar lids [1]. Although the sensory symptoms of CTS are usually limited to the median-innervated fingers. 23. with involvement of the first three digits and the radial half of the fourth digit (figure 1). The symptoms of CTS are typically worse at night and often awaken patients from sleep. there can be significant variability. Feet flop while walking. constipation. Difficultystanding up from low chairs and opening jars. intermittent abdominal pain. such as difficulty holding objects. 3-4 beers/day. Some patients react to these symptoms by shaking or wringing their hands or by placing them under warm running water [1]. but the neck is not affected. Alcohol. Cause? E. The pain and paresthesia may be localized to the wrist or involve the entire hand. Smoke. Clinical signs may include weakness of thumb abduction and opposition. nausea.Next step for dx? c. The most important of these are nocturnal pain or paresthesia in the distribution of the median nerve. This pattern occurs because the palmar sensory cutaneous nerve arises proximal to the wrist and passes over. buttoning clothing. Fixed sensory loss is usually a late finding characterized by a distinctive clinical pattern that involves the median-innervated fingers and spares the thenar eminence. decreased libido.[22] . Electrophysiological studies. Electrodiagnostic testing. fatigue. In more severe cases of CTS. sometimes supplemented with needle electromyography (EMG). and atrophy of the thenar eminence. but are more likely to occur . rather than through.[21] Other early signs in adults include malaise. 6 weeks drinking home-distilled whiskey. It is not uncommon for sensory symptoms to radiate proximally into the forearm. loss of appetite. 47yo man with 2 week progressive weakness. the carpal tunnel. is a standard part of the evaluation for CTS because it has a high sensitivity and specificity for confirming the diagnosis. Deep tendon reflexes 1+. Lead poisoning Early symptoms of lead poisoning in adults are commonly nonspecific and include depression. turning keys or doorknobs. Atrophy of intrinsic muscles hand and feet. The diagnosis is suspected when the characteristic symptoms and signs are present. Constipation but no changes in bladder/sexual function. primarily with nerve conduction studies (NCS). and less frequently to radiate above the elbow to the shoulder. diarrhea. Electrodiagnostic testing can be helpful to confirm or exclude CTS when the clinical diagnosis is uncertain [5]. symptoms can occur at levels above 40 μg/dL. Sens normal. It is also useful to gauge severity of nerve compression and to aid in decisions regarding surgical intervention. Babinski (-). Microcytic anemia and hyperuricemia. Strength 4/5. in adults. and muscle pain. At blood lead levels between 25 and 60 μg/dL. Lead affects the peripheral nervous system (especially motor nerves) and the central nervous system. such as those that accompany increased pressure within the skull. may appear at blood lead levels greater than 80 μg/dL. abdominal colic. coma. as well as slowed motor nerve conduction and headache can occur.[25] Anemia may appear at blood lead levels higher than 50 μg/dL.[16] Signs that occur in adults at blood lead levels exceeding 100 μg/dL include wrist drop and foot drop. This is seen less frequently since governmental regulations have resulted in lower lead exposures at work. and difficulty concentrating. seizures. neuropsychiatric effects such as delayed reaction times. In adults.[18] . and headache. and signs of encephalopathy (a condition characterized by brain swelling). irritability. A peripheral neuropathy that frequently manifests with extensor weakness or "wrist/ankle drop" due to an axonal degeneration that primarily affects motor nerves [54]. tremor has also been reported.only above 50–60 μg/dL. delirium. involving paroxysms of pain. In rare situations of high-level lead poisoning.[18] Peripheral nervous system effects are more prominent in adults and central nervous system effects are more prominent in children.[24] Lead causes the axons of nerve cells to degenerate and lose their myelin coats. cannot read. CT normal. 32 yo woman ER progressive visual dimming right eye since awakening 8h ago. over right eye before going to bed. Headache is different than previous usual migraines treated with propranolol. Optic neuritis is the presenting feature of MS in 15 to 20 percent of patients and occurs in 50 percent at some time during the course of their illness. Dx? Maybe E. Optic neuritis Optic neuritis is an inflammatory. Symptoms started with a mild headache. It is highly associated with multiple sclerosis (MS). usually monocular. demyelinating condition that causes acute. varely count fingers in right eye. visual loss.24. . most percent improve achieve 20/40 or by three or more better lines Equal Disc hyperemia but no swelling. (See "Amaurosis fugax (transient monocular or binocular visual loss)".Clinical features of more common optic neuropathies* Non-arteritic Optic neuritis ischemic optic neuropathy 20 to 50 years >50 years Age Gender 2:1 female Equal Pain Present in >90 percent Present in <10 percent Onset Hours to days Unilateral or bilateral Usually unilateral Funduscopic examination Papillitis present in one-third Visual field defect Central scotoma Magnetic resonance imaging: optic nerve Prognosis Sudden Usually unilateral.) Irreversible visual loss may be a complication of retinal migraine. exudates Central or Variable cecocentral defect May show enhancement Normal Variably abnormal Poor once vision loss has occurred. although the incidence is uncertain. associated with or followed by headache. low chance may recur in other eye years later Arteritic ischemic optic neuropathy Leber's hereditary Neuroretinitis optic neuropathy >70 years 25 to 40 years 80 to 90 percent male Children Not present Variable 3. since both retinal and ciliary circulations may be involved [97].Retinal migraine is a rare condition that is characterized by repeated attacks of monocular scotomata or blindness lasting less than one hour. may cause rapid blindness untreated One-third achieve Most recover some fully improvement differential dx Not C. but ocular migraine has been suggested as a more precise term. In one of the largest studies to date that reported 6 new . scalp tenderness. only 40 four weeks. fundus may also be Papillitis present normal (posterior in most ischemic optic neuropathy . Occasionally the onset may be abrupt and difficult to distinguish from amaurosis fugax [79].indicates giant cell arteritis) Altitudinal Altitudinal or (usually generalized inferior) defect constriction Inflammation of optic nerve in most (onethird to one-half will Often normal have other demyelinating lesions) Over several Begins within two to months. peripapillary telangiectasia Papillitis.but May occur in both presentation often Often bilateral eyes in rapid sequence unilateral Pale swelling of disc.5:1 female Headache. The International Headache Society prefers the term retinal migraine [76]. macular edema. jaw claudication Sudden Weeks to months Hours to days Bilateral . include the following [1. or tics during exacerbations The proposed model for pathogenesis of PANDAS suggests that group A streptococcal (GAS) infection in a susceptible host causes an abnormal immune response with resultant central nervous system manifestations. It is one of the major clinical manifestations of acute rheumatic fever (ARF). choreiform movements. emotional lability. FSHD or FSH)—originally named Landouzy-Dejerine[2]—is a usually autosomal dominant inherited form of muscular . which are discussed in greater detail below. reporting bias). generalized involuntary movements of face trunk . The investigators also identified a subset of children with OCD or tic disorders following group A streptococcal (GAS) infection who did not meet criteria for Sydenham chorea [4. since it is likely that cases with such a major complication are more apt to be identified and to be reported (ie. extremities.cases and reviewed 40 from the literature. and extremities. such as motoric hyperactivity. Investigators at these centers noted an association between Sydenham chorea and OCD [5-7]. Examination. The hypothesized association between PANDAS and GAS is controversial. The diagnostic criteria for PANDAS. Autoinmune disorder of the basal ganglia Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci (PANDAS) is a term used to describe a subset of children whose symptoms of obsessive compulsive disorder (OCD) or tic disorders are exacerbated by group A streptococcal (GAS) infection [1]. she has been very anxious irritable. If this hypothesis is correct. permanent visual loss was eventually present in 20 patients (43 percent) [98]. permanent visual loss may be less frequent than suggested by these data. as is the limitation of the diagnosis exclusively within the pediatric age group Most of the knowledge about PANDAS has been obtained by studying patients with a known tic disorder or long-standing obsessive-compulsive disorder (OCD) in research facilities and referral centers [1-4]. No predictors of irreversible visual loss could be identified. trunk. 25.8. No previous serious illness. Sydenham chorea is a movement disorder characterized by chorea. and no consistent pattern of visual loss was observed among these patients.10]: ●OCD and/or tic disorder (Tourette disorder. the hypothesis is as yet unproven. restless appear. and hypotonia. chronic motor or vocal tic disorder) ●Pediatric onset (between three years and onset of puberty) ●Abrupt onset and episodic course of symptoms ●Temporal relation between GAS infection and onset and/or exacerbation ●Neurologic abnormalities. However. This subset is described by the acronym PANDAS [1]. and headaches. except migratory joint pain. Cause? A. Facioscapulohumeral muscular dystrophy (FSHMD. However. During episodes. 10 yo after Thankgiving. She has been worrying obsessively.9]. with hx 4-day rapid irregular involuntary movements of her face. there might be a role for prophylactic antibiotics and/or immune modulating therapies in the prevention and treatment of PANDAS. biceps. Younger children lack the capacity to accurately verbally report symptoms and emotional distress. Triceps. Several laboratory findings are characteristic of dermatomyositis (DM) and polymyositis (PM). Muscle weakness is the most common feature of DM and PM. A progressive skeletal muscle weakness usually develops in other areas of the body as well. including antinuclear antibodies. Electromyography and nerve conduction studies. Normal urianalysis and blood count. and these criteria generally are extended to children. glutei. with gradual worsening over a period of several months before medical attention is sought. Next step in Dx? B.54] ●Autoantibodies. However. patients present with focal myositis that usually but not always progresses to the typical generalized form over time [14].dystrophy (MD)[3] that initially affects the skeletal muscles of the face (facio). Diagnostic criteria were established for adults predominantly. and they occasionally mistakenly ascribe weakness to the joint involvement. Hypothyroidism treated with thyroxine. carrying heavy groceries. Affected muscles typically include the deltoids and the hip flexors. Typically mild myalgias and muscle tenderness occur in 25 to 50 percent of cases. 26. Distal muscle weakness. Weakness of the neck flexors is also common. cuadriceps. consideration of a somatic symptom group of disorders can be considered.) The distribution of weakness is characteristically symmetric and proximal in both PM and DM. often the weakness is asymmetrical. scapula (scapulo) and upper arms (humeral). if present. Conversion disorder. getting up from a chair. or picking up their children due to the proximal muscle involvement. FSHD is the third most common genetic disease of skeletal muscle. cutaneous manifestations often precede or accompany weakness. However. tends to be mild and usually does not cause significant functional impairment. Patients usually report a history of the insidious or subacute development of the muscle weakness. psoas. an acute onset of weakness is occasionally reported. and stiffness is not a prominent complaint. if present. ESR 85. Pain is mild. There are no separate child-specific criteria. Normal vital signs. Patients may describe increasing difficulty climbing stairs. CK 2456. the disease is associated with contraction of the D4Z4 repeat in the 4q35 subtelomeric region of Chromosome 4.10]. However. in up to 80 percent of . In more than 95% of known cases. over 90 percent of patients with PM present with muscle weakness [9]. diagnoses in children and adolescents are more difficult because the expression of emotional distress in the form of physical complaints is developmentally appropriate in younger children. They may notice joint pain and swelling. Hx 3-month gradually progressive weakness shoulder and hip. when physical symptoms are persistent and a child's functioning deteriorates. which is found at presentation in only 50 to 60 percent of patients with DM [9. Generally. Non-muscular symptoms frequently associated with FSHD include subclinical sensorineural hearing loss and retinal telangiectasia. Rarely. 57 yo woman. These include: ●Elevated levels of muscle enzymes [9. if present. (See 'Skin findings' below. Moderate bilat weakness deltoid. positive sharp waves. in patients in whom the initial evaluation is consistent with possible DM or PM.000 international units/L). while others have symptoms consistent with a brachial plexopathy [6. myositis-specific antibodies.patients with DM and PM [9. and higher levels are sometimes seen. up to 10. EMG findings are not specific for either DM or PM. Arm involvement occurs in up to one-third of patients and may be in the form of mononeuropathies of the ulnar and median nerves. EMG shows evidence of muscle membrane irritability. spontaneous fibrillations. the following observations were . and the absence of another explanation for the findings. Some have pain or weakness involving the chest or abdominal wall. Numbness in T-12 distibution. In one retrospective series of 85 patients diagnosed with diabetic cervical radiculoplexus neuropathy who presented with pain. However. In severe cases. depend upon the clinical presentation. and other features characteristic of the antisynthetase syndrome. including muscle or skin biopsy. Additional diagnostic studies. the serum CK concentration may be elevated more than 50-fold or even 100-fold (ie. In untreated patients with active muscle disease. as well as in patients with findings of a typical rash. even in patients with active muscle disease [5]. 27. Most upper limb symptoms occur in association with lumbosacral plexus involvement. Hx of chroniclow back pain. The level of serum CK can vary widely.10]. brachial plexus.56-58]. Treated with 10day corticosteroids for asthma 3 weeks ago.000 to 20.54. and the EMG is normal in approximately 10 percent of patients. paraesthesia or weakness involving the cervical nerve roots. myositis-specific autoantibodies. Diabetic radiculopathy Upper limb and thoracic involvement has also been observed as part of the syndrome of diabetic amyotrophy [6. or may affect more proximal sites in the brachial plexus [10. asthma treated with beclomethasone and albuterol inhalers. at least 2000 to 3000 international units/L).55]. 72 yo man 7-day hx increasing severe pain around waist.11]. and magnetic resonance imaging (MRI) of skeletal muscle. suggesting a thoracic radiculopathy. in at least 30 to 40 percent of patients [37. usually including increased insertional activity. We diagnose DM without a biopsy in patients with symmetrical proximal muscle weakness.60+ The erythrocyte sedimentation rate (ESR) is often normal or is only mildly elevated. electromyography (EMG). it is usually more than 10-fold the upper limit of normal (ie. This information can be valuable in selecting a site for muscle biopsy. a rash that is relatively specific for DM (such as Gottron’s papules or a heliotrope rash). Most likely explanation? C. and complex repetitive discharges.10. feels like a burning belt from the left midback to the umbilicus. EMG findings are helpful in confirming the presence of a myopathic process and in indicating which muscle groups are most involved. especially in patients with overlap syndromes ●Elevated levels of serum and urine myoglobin *59. DM2 treated with glyburide. and myositis-associated autoantibodies. elevated muscle enzymes.10]. further studies should include testing for antinuclear and myositis-specific antibodies (MSA) and a chest radiograph. or upper extremity nerves. were noted in 15 percent. Systemic therapy may be beneficial in patients with chemosensitive tumors. brought to ER 12-hour hx severe pelvic pain and numbness. 28. Decreased rectal sphincter tone. Several studies have suggested that lower doses can be effective but they have not been assessed in randomized trials. ●Neurophysiologic testing showed a predominantly axonal neuropathy. Strength 4/5 lower extremities. For patients with ESCC and either neurologic symptoms or substantial thecal sac compression by imaging. middle. The most frequent symptoms at evaluation were weakness. and pan plexopathy was present in 30 percent. respectively. the most common cerebrospinal fluid abnormality was elevated protein. or anterior interosseus nerves. and numbness in 99. 81. paraparesis or paraplegia). pain. ●Weight loss >10 lbs was recorded in 35 percent. BP 152/70. ●The most common symptom at onset was pain in 62 percent. and 66 percent. such as orthostatic dizziness and changes in sweating. pulse 104/min. (See 'Glucocorticoids' above.16.17]. followed by surgery. Most of the remaining patients had impaired fasting glucose. gestational diabetes.made ●The median age was 62 years (range 32 to 83 years). 99F. Decreased sens light touch in saddle distribution. or stereotactic body radiotherapy (SBRT). we recommend glucocorticoids as an integral component of the initial management (Grade 1B). resolves pain but no numbeness. Autonomic symptoms. Catheter yields to 800 mL of urine. 32 yo woman breast CA metastasis to bone. suprascapular. with eventual involvement of the contralateral side in 35 percent. long thoracic. Next step management? B. ●Among 28 patients who had lumbar puncture. ●Type 2 and type 1 diabetes mellitus were present in 79 and 7 present. and lower brachial plexus was approximately equal. Some patients had isolated or superimposed involvement of the phrenic. external beam radiation therapy (EBRT). Onset was unilateral in 81 percent. MRI shows tumor on L4-S1 intrathecal. Medications hydrocodone. ●Involvement of the upper. dexamethasone 96 mg intravenously followed by 24 mg four times daily for three days and then . axillary. High-dose corticosteroid therapy is generally considered to be part of the standard regimen for ESCC.) ●For patients with severe neurologic deficits (eg. Suprapubic fullness. Involvement of lumbosacral and thoracic regions was noted in 24 and 19 percent. nerve biopsy. was interpreted as showing mainly ischemic injury from microvasculitis. IV administration of high dose Dexamethasone Management of patients with Neoplastic epidural spinal cord compression (ESCC) includes the immediate administration of glucocorticoids in nearly all patients. with each occurring in 50 to 60 percent. resp 18/min. we suggest high-dose glucocorticoid therapy (eg. despite limited documented evidence of benefit and a significant risk of serious side effects [1. or steroid-induced diabetes. obtained in 11 patients. we suggest moderate doses of glucocorticoids (eg. is complete spinal fusion with hyperkyphosis. pulse 90/min. Located behind left eye and improve after sleep. 30. 98. 25 yo man. entheses. Occasional dysuria. and digits. The male preponderance persists until puberty. Decreased forward flexion of the spine and tenderness over the lumbosacral area and at the insertion site of the achiles tendon. Almost all patients with AS report back pain. AS typically develops in young adults. but not invariably. usually without much swelling. more females are affected. which occurs in only a subset.tapered over 10 days) (Grade 2C). shoulders. Dx? B. a form of spondyloarthritis (SpA). affected boys outnumber girls. Enthesitis manifests as pain. enthesitis. abdominal pain. may also be seen in patients with AS and other forms of SpA. and relief with sleep. by 10 to 14 years of age. 13 yo girl headaches accompanied by nausea and vomiting over past 2 weeks. Headaches started during final examinations. The headache of migraine tends to have a throbbing or pulsatile quality. inflammation of the enthesis. Pain relieved by stretching. who generally are unable to voice complaints about headache. peripheral joints. with a peak age of onset between 20 and 30 years. the symptoms of migraine vary with age. especially as the intensity increases. Inflammatory back pain typically exhibits at least four of the following five features: ●Age of onset <40 years ●Insidious onset ●Improvement with exercise ●No improvement with rest ●Pain at night (with improvement upon arising) The enthesis is the region of attachment of tendons and ligaments to bone. which frequently. In toddlers. missing 2-3 days at school. it can also involve the hips. The endpoint. has characteristics suggesting an inflammatory etiology. BP 95/60. symptoms appreciated . Extraarticular manifestations. Thus. The onset of migraine tends to be earlier in boys than girls (7 versus 10 years of age) [3]. is a chronic inflammatory disease of the axial skeleton manifested by back pain and progressive stiffness of the spine. ●For patients with minimal neurologic symptoms. stiffness and tenderness of insertions. Migraine Migraine is the most common acute and recurrent headache syndrome in children. dexamethasone as a bolus of 10 mg intravenously followed by 16 mg daily orally in divided doses) (Grade 2C). However. including uveitis. vomiting.6F. before age seven. resp 20/min. No trauma Hx. It is characterized by periodic episodes of paroxysmal headache accompanied by nausea. Ankylosing spondylitis Ankylosing spondylitis (AS). although swelling is a prominent feature at the Achilles tendon attachment. ●We suggest that glucocorticoids be omitted in patients with small epidural lesions and a normal neurologic examination 29. Dx? C. hx 4-month increasing low back most severe on awakening in the morning. is a classic feature of AS and other spondyloarthritides. the diagnosis of migraine should be made [1]. Recall 0/5 objects after 5 min. 31. while bitemporal headache is more common in the early teenage years [46]. in adolescents and young adults. unusual decreased activity. Symptom Migraine headache cluster headache) Commonly bilateral in young children. Labile affect. Weightloss. Associated phonophobia*. and can last from 30 minutes to 7 days (table 2B). mild to moderate severity. Many children have vomiting that occurs once or repetitively. eye. photophobia*. photophobia or phonophobia but usually is not accompanied by vomiting [1]. Noncompliant ttmt with antiretrovirals. pallor. Always unilateral. may have aura (usually None sweating. usually begins around Location Bilateral unilateral in 60 to 70% and bifrontal or the eye or temple global in 30% Pressure or Gradual in onset. 1-month Hx of forgetfulness and inapropiate behavior. quiet remain active or Patient remains active appearance room may need to rest Duration 2 to 72 hours Variable 30 minutes to 3 hours Ipsilateral lacrimation and redness of the Nausea. Buying numerous items on internet. reaches a crescendo tightness that Characteristics pulsating. 3rd edition (beta version) (ICHD-3b) specifies that migraine diagnosis takes priority over the diagnosis of tension-type headache. stuffy nose. waxes and aggravated by routine physical activity excruciating. Diarrhea. 52 yo man HIV (+). CT shows atrophy cortex and demyelination in subcortical white matter. pain is deep. non-throbbing. or generalized than unilateral [42]. The overlap of some of these symptoms with those of migraine can make differentiation between the two headache types difficult [24]. bifrontal headache is more common in younger children. Thrush. Dx? E. sensitivity to cause speech or motor deficits) alcohol The most common primary headaches in children are migraine and tension-type headache (table 1). Tension-type headaches in children are discussed separately. continuous. 101. Progressive multifiocal leukoencephalopathy. the headache is more often bifrontal. so when in doubt between the two. but can involve other senses or neurologic symptoms rare. . Characteristics of common headache syndromes in children and adolescents Tension-type Trigeminal autonomic cephalagia (eg.3F. crescendo pattern. focal symptoms visual. and do not worsen with activity (although the child may not wish to participate in activity). Tension-type headache may be associated with nausea.by caregivers include episodic pallor. BP 110/60. Horner syndrome. In children. bitemporal. BMI 15. moderate or severe intensity. within minutes.45]. The migraine attack is often accompanied by nausea and sensitivity to light and noise. resp 16/min. and vomiting [2. Pain begins quickly. and explosive in quality wanes Patient may Patient Patient prefers to rest in a dark. vomiting. Trigeminal autonomic cephalalgias (including cluster headaches) are rare in children younger than 10 years. Tension-type headaches are characterized by headaches that are diffuse in location. The International Classification of Headache Disorders. rhinorrhea. reports of PML affecting patients who have conditions associated with minimal or occult immunosuppression. JC virus remains latent in kidneys and lymphoid organs. and induce a lytic infection of oligodendrocytes. but. in the context of profound cellular immunosuppression.Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system that is caused by reactivation of the polyomavirus JC (JC virus) [1-3]. spread to the brain. There are only isolated case reports of PML in patients without apparent immunosuppression [5-9]. Progressive multifocal leukoencephalopathy (PML) occurs almost exclusively in immunosuppressed individuals. JC virus can reactivate. Asymptomatic primary infection with JC virus occurs in childhood and antibodies can be found in 86 percent of adults [4]. There are. For patients with clinical and neuroimaging features of PML who have a negative PCR . However. however. the diagnosis of progressive multifocal leukoencephalopathy (PML) is established by demonstrating the presence of JC virus DNA in the cerebrospinal fluid (CSF) using polymerase chain reaction (PCR) [76]. brain biopsy remains the gold standard for the diagnosis. In most individuals. The typical appearance of PML on neuroimaging studies consists of symmetric or asymmetric multifocal areas of white matter demyelination that do not conform to cerebrovascular territories and exhibit neither mass effect nor contrast enhancement. such as hepatic cirrhosis and renal failure. which are the CNS myelin-producing cells. In patients with compatible clinical and neuroimaging features. 20 yo college student follow up 2-days after head injury playing football. ●A more conservative approach is probably appropriate for children and adolescents. swimming or stationary cycling keeping intensity exercise <70 percent MPHR. participate in normal training practice activities Objective of each stage Recovery Increase HR Add movement Exercise. An emergency department evaluation is indicated for any athlete who suffers loss of consciousness [1. He asks if he can plays football tomorrow.) ●Individuals with a history of multiple concussions should undergo a more detailed evaluation regarding neurobehavioral symptoms. but not remember the rest of the game. (See "Minor head trauma in infants and children: Evaluation". (See 'Acute evaluation and management' above. they should be referred for neurologic and neuropsychological assessments [186]. They also suggest that a more conservative approach be followed for adolescents and children Graduated return to play protocol Rehabilitation stage Functional exercise at each stage of rehabilitation 1.for JC virus in the CSF. Patients with persistent neurobehavioral complaints or objective deficits should be counseled about the risk of chronic traumatic encephalopathy and possible retirement from contact sports. is evaluated for symptoms. No activity Complete physical and cognitive rest 2. If symptoms occur. our suggested approach is to evaluate for other neurologic disorders (see 'Differential diagnosis' below) and repeat the PCR for CSF JC virus (algorithm 1). He did not lose consciousness. if these are present. international group and proposes a six-day graduated return to play protocol in which the athlete makes a stepwise increase in functional activity. Based on these concerns. coordination. it is recommended that ●Athletes suspected of having a concussion should be removed from play and evaluated by a licensed health professional. The most recently issued 2012 Consensus Statement on Concussion in Sport was written by a multi-disciplinary.) ●Athletes with diagnosed concussion should be removed from play or practice (contact-risk activity) until symptoms have resolved off medication. no head exercise impact activities Progression to more complex training drills. Sport-specific Skating drills in ice hockey. and cognitive load Restore confidence and assess functional skills by coaching staff . running drills in soccer. vomiting. Recommendation? A. While these guidelines further suggest that a more rapid return to play may be possible for asymptomatic adult athletes. 32. eg. Evaluated 1h after the game in ER. No nausea. same day return to play is not recommended.185]. Brain biopsy is indicated if this evaluation and repeat PCR is unrevealing. Non-contact drills in football and ice hockey. CT no abnormalities. Discharged. then the patient should drop back to the previous asymptomatic level and reattempt progression after 24 hours. memory problems. No contact sport pending evaluation in 1 week. Today present with headache improves with acetaminophen. Full contact Following medical clearance. no resistance training 3. Light aerobic Walking. may start progressive training drills resistance training 5. passing 4. and is allowed to progress to the next stage each successive day if asymptomatic (table 4) [6]. rather than augmenting the SSRI with a second drug (Grade 2B). In addition. For depressed patients with substantial benefit from SSRI therapy but severe sexual dysfunction. 33. and is allowed to progress to the next stage each successive day if asymptomatic. Neurotransmitter responsible for symptons? E. reasonable alternatives include agomelatine. including decreased libido. fumbling movements hands. moclobemide. if female sexual dysfunction is limited to delayed orgasm or anorgasmia. we suggest switching antidepressants. 60 yo woman with 2-min episodes rising sensation abdomen followed by loss arousal. is evaluated for symptoms. Ttmt with sertraline. two to eight weeks) for spontaneous remission of the sexual impairment. rather than bupropion (Grade 1B). Our initial approach for patients who respond to SSRIs but suffer sexual dysfunction is to wait (eg. anorgasmia in women. 27 yo man. We typically switch to bupropion or mirtazapine. Normal physical examination. and selegiline. as well as a different SSRI. Location lesion? E. low modd. Among patients receiving SSRIs. we recommend adjunctive treatment with a phosphodiesterase-5 inhibitor. For women with sexual dysfunction. Each day the athlete makes a stepwise increase in functional activity. the estimated incidence of sexual dysfunction is approximately 50 percent. For men with erectile dysfunction. however. However. if the impairment persists. Past 3 months.6. and increased ejaculation latency in men. e-month hx decreased libido and delayed eyaculation. a reasonable alternative to augmentation for both men and women is switching antidepressants. 34. a phosphodiesterase-5 inhibitor is a reasonable option. Patients treated with SSRIs who obtain substantial relief from the depressive syndrome and suffer only moderate sexual dysfunction are typically managed by augmenting the SSRI with a second drug. Patients with SSRI induced sexual dysfunction often do not respond to watchful waiting or lower doses. Return to play Normal game play Six-day return to play protocol. loss interest and productivity. decreased arousal. we typically suggest add-on bupropion at higher doses rather than a phosphodiesterase-5 inhibitor (Grade 2C). During episode. makes chewing movements. Right superior quadrantanopsia without macular sparing. we decrease the dose within the therapeutic dose range. Temporal lobe . 4 month ago. or with modest benefit from SSRI therapy and moderate sexual dysfunction. Not problems in the past. stares. such as phosphodiesterase-5 inhibitors or bupropion. Detrimental in relationship. Post episode confusion. Serotonin Selective serotonin reuptake inhibitors (SSRIs) can cause sexual dysfunction. hemiparesis Receptive aphasia (dominant hemisphere) Hemisensory loss. Over time. hemianesthesia. ER 1-hour after falling 25 feet. 18 yo man. Babisnky (+) bilat. level. a number of problems can result: ●Bladder or detrusor hyperactivity produces reflexive bladder emptying. Patients may be troubled by bladder spasms as well as urgency and frequency. depending on lateralization: hemineglect Gerstmann's syndrome Isolated symptom Isolated if occipital lobe Often isolated symptom Isolated symptom Anton's syndrome 35. Compression of the thoracic spinal cord. X-ray. Explanation urnary incontinence? D. Paraplegic and urinary incontinence. often with incontinence. and completeness of the spinal cord lesion. this can lead to decreased capacity of the . Depending on the acuity. signs Optic atrophy.Localizing retrochiasmal visual field defects Defect Localization Homonymous sectoranopia Lateral geniculate body Anterior temporal lobe Homonymous pie-in-the-sky Posterior optic radiations. visual Homonymous quadrantanopia cortex Homonymous paracentral Posterior primary visual cortex scotomas Macular-sparing homonymous Primary visual cortex (sparing hemianopia posterior portion) Temporal crescent only Far anterior primary visual cortex Temporal crescent-sparing Primary visual cortex (sparing far homonymous hemianopia anterior portion) Bilateral homonymous Bilateral posterior optic radiations hemianopia or primary visual cortex Associated symptoms. fracture dislocation of T10. Lower extremities with absent reflexes and flaccid tone. jaw claudication Ischemia of optic nerve Headache. Metoprolol. Next step dx? E. motor symptoms Epileptic discharge Postictal 20 minutes." meaning dark. other embolic source 1 to 10 minutes Monocular. a combination of detrusor and sphincter hyperactivity. Hx HTN and angina. headache Spreading cortical Usually followed by depression. Etiology Typical duration Pattern of visual loss Monocular ischemia. diplopia Elevated intracranial pressure Transient retinal arterial Vasospasm narrowing. altitudinal onset hemispheric symptoms Giant Cell arteritis Variable Usually monocular Papilledema Seconds Idiopathic retinal vasospasm 5 to 60 minutes Migraine 10 to 30 minutes Vertebrobasilar ischemia 1 to 10 minutes Homonymous hemianopia Seizure: Ictal 3 to 5 minutes Binocular. can lead to bladder contractions against a closed sphincter. Pulse 72/min. and the Latin "fugax. Carotid doplex ultrasonography. This leads to chronic urinary retention with overflow incontinence and incomplete emptying. leading to elevated bladder pressures and vesicoureteral reflux. positive symptoms with spread Associated symptoms and signs Mechanism Retinal embolism (usually) Headache. brought 3-hour after episode 5-min of visual loss left eye. BP 150/92. 67 yo woman. Amaurosis fugax (from the Greek "amaurosis. positive phenomenon common Altered consciousness. Thromboembolic events from carotid disease or elsewhere generally last 1 to 15 minutes and only rarely an hour or more [6-8]. rapid onset. Funduscopic no abnormalities. lateralized. Ischemia is the most common cause of transient monocular visual loss (TMVL) [5]. and ocular disease are other causes of TMVL. Hollenhorst plaque.bladder. possibly migraine headache retinal vasospasm Isolated or accompanied by other brainstem Embolic deficits Transient visual obscurations due to papilledema typically last seconds. this in itself can have diverse etiologies. No focal neurological sign. neck pain. optic neuropathy. longer Binocular visual field loss Preceding ictus Cortical inhibition Monocular graying or blurring Monocular positive or negative symptoms Usually binocular. ●Bladder flaccidity is produced in lower motor neuron injuries affecting the cauda equina or conus medullaris as well as with acute upper motor neuron injuries (spinal shock). In contrast to transient ischemic attacks (TIAs) involving the cerebral hemispheres. Medications lisinopril. 36. ●Detrusor sphincter dyssynergia. Migraine aura typically lasts 10 to 30 minutes. 3/6 holosystolic murmur at apex." meaning fleeting) refers to a transient loss of vision in one or both eyes [1]. . ●Sphincter hyperactivity can impair complete emptying of the bladder. carotid disease. Papilledema. Pharmacotherapy? E. Lower extremities strength 1/5. It was bilateral in 27 to 35 percent of these. pulse 78/min. hyperlipidemia) who have experienced TMVL. 98. Immune globulins In most cases. Physical examination reveals symmetric weakness with diminished or absent reflexes. and swept into the same distal vascular bed. and an elevated protein concentration (>45 mg/dL). whereby particulates of uniform size are consistently deposited into one lamina.38]. Most patients reach the nadir of their function within two to four weeks. Flu-like illness past week. Magnetic resonance imaging (MRI) should be performed in a patient of any age with TMVL who has a history suggestive of carotid artery dissection GCA most often presents as acute sustained unilateral visual loss with a swollen optic disc [36]. Common accompanying symptoms include jaw claudication and headache (each in about half of patients). However. WBC 4. these small particles may be more prone to obstruct the smaller retinal arterioles. characteristic cerebrospinal fluid findings include normal pressure. BP 115/70. the facial nerve is most commonly affected. Erythrocyte sedimentation rate and C-reactive protein All older patients (>50 years) with transient monocular or binocular vision loss should have a sedimentation rate and C-reactive protein to exclude giant cell arteritis (GCA). Treatment should not await pathology results. With lumbar puncture. decreased sens pinprick.6F. or computed tomographic angiography should be ordered in all older patients (>50 years) and in younger patients with vascular risk factors (diabetes. patients with occult giant cell arteritis may have no other symptoms [37]. 37. followed by return of function occurring slowly over the course of weeks to months. This may represent a streaming effect of laminar blood flow. or if the history is very suggestive. In two large series (each >150 patients) of biopsy-confirmed GCA. The main modalities of therapy for GBS are intravenous immune globulin (IVIG) and plasma exchange (also called plasmapheresis).retinal ischemia is more commonly associated with emboli originating from carotid stenosis rather than heart disease [5. neurologic symptoms develop two to four weeks after having what appears to be a benign febrile respiratory or gastrointestinal infection. magnetic resonance angiography. decreased patellar and achilles reflexes. 11 yo girl.28-30]. IVIG and plasma exchange for children with GBS should be reserved for those with any . fewer than 10 cells (typically mononuclear). If these are elevated. CSF protein 80. patients should proceed to a confirmatory temporal artery biopsy. resulting in bilateral facial weakness. hypertension. Small emboli from carotid disease may be more likely to drift to the edge of the bloodstream and enter the ophthalmic artery. ER 2-day hx increasing weakness and prickling sensation in her legs. TVL was the presenting symptom in just 10 to 15 percent [36. resp 20/min. Lower extremity symmetric weakness may ascend over hours to days to involve the arms and the muscles of respiration in severe cases. Also. Carotid imaging — Carotid duplex ultrasound. In those with cranial neuropathy. Autonomic dysfunction occurs in approximately one-half of children with GBS. The predominant symptoms of GBS at presentation in children are pain and gait difficulty. No signs trauma. generalized tonic clonic seizures. Glucocorticoids are not beneficial for GBS and have no role in its treatment. resp 15/min. Neither treatment is recommended for ambulatory children with GBS who have mild disease or for children whose symptoms have stabilized. 25 yo woman. Problem sustaining . Intubated. IV dextrose started. Tonic stiffness trunk and synchronous jerking of extremities.of the following indications: ●Progressing weakness ●Worsening respiratory status or need for mechanical ventilation ●Significant bulbar weakness ●Inability to walk unaided We prefer IVIG to plasma exchange for the treatment of children because of its relative safety and ease of administration. However. Found having continuous. BP 140/70. 32 yo man. ER 20 min after found in bathroom bus station. 38. 99F. children who have rapid progression followed by stabilization of symptoms within the first or second week of GBS onset may still be considered candidates for treatment. Flushed in diaphoretic. and supplemental O2. Pupils 4mm reactive. Next step ttmt? B. Lorazepam 39. although it has not been shown to have better results. 6-month hx worsening excessive daytime sleepiness. pulse 110/min. Normal hearing. There are several nonpharmacologic interventions that may benefit the patient with narcolepsy: ●Avoiding certain drugs – Some drugs should be avoided by patients with narcolepsy. Moldafinil therapy. prazosin and other alpha-1 antagonists can worsen cataplexy. No vomiting. Episodes are provoked by specific types of head movements. they may have difficulty meeting economic and social responsibilities [6]. Nearly half of patients with narcolepsy report that their sleepiness and cataplexy substantially interfere with their daily lives. ●Psychosocial support – Patients with narcolepsy face various psychosocial and work-related problems throughout their lives. but queasy. are that sleep attacks and cataplexy (emotionally triggered muscle paralysis resulting in partial or complete collapse) are manifestations of poor motivation. and rolling over in bed. a brief nap at work or school is often helpful. Many patients will benefit from these nonpharmacologic approaches. Initial step management. One or two well-timed 20 minute naps will often improve sleepiness for one to three hours though some patients only benefit from long naps [4]. Modafinil has become a first line pharmacologic therapy because it provides good control of sleepiness. jobs. BP 120/76. including school. but most also require medications that reduce sleepiness and cataplexy. but it may increase dopaminergic signaling. 3 month hx of spinning sensation. Otolith Patients with benign paroxysmal positional vertigo (BPPV) present with recurrent episodes of vertigo that last one minute or less. BMI 22. 68 yo woman. lying down or getting up from bed." its mechanism of action is not well understood. In addition. A nonamphetamine "wakefulness promoting agent. is generally well tolerated. resp 12/min. even among medical caregivers. such as looking up while standing or sitting. resolves in 1 min. or social lives [1. impair work productivity. Occasional muscles go limp and falls suddenly. Normal ocular movements if sitting. or avoidance. patients often benefit from participation in support groups that focus on coping skills and identification of community resources to assist with administrative and medical issues. marriages. The mainstays of therapy are brief daytime naps and pharmacologic therapy. as a result. Thus. and illicit use is rare.2]. Drugs that can worsen daytime sleepiness include benzodiazepines. opiates. Other medications such as theophylline or excessive caffeine can cause insomnia.attention. Common misconceptions. The vertigo may be associated with nausea and . If it can be arranged. They also have the additional burden of coping with misperceptions about the causes and the involuntary nature of their symptoms. 40. which can worsen daytime sleepiness. Pulse 85/min. E. and alcohol. but coarse rotatory nystagmus to one side if head is hanging from examination table. antipsychotics. ●Napping/sleep hygiene – Behavioral interventions are often helpful for optimal control of narcolepsy. Feeling room spinning when she tries to turn over in bed. denial. Abnormality in which site? C. Sleep deprivation may worsen narcolepsy symptoms and therefore patients should be counseled to maintain a regular and adequate sleep schedule [5]. Diagnostic criteria employing the Dix-Hallpike maneuver have been proposed for posterior canal BPPV (figure 1) [18]: ●Nystagmus and vertigo usually appear with a latency of a few seconds and last less than 30 seconds ●It has a typical trajectory. 41. The patient is kept in this position until 30 seconds has passed if no nystagmus occurs. ie. A video demonstrating this maneuver can be viewed at http://www. This debris likely represents loose otoconia (calcium carbonate crystals) within the utricular sac. The semicircular canals normally detect angular head accelerations. BP 150/90. The patient is then placed supine rapidly. With the patient sitting. the intensity and duration of nystagmus will diminish Benign paroxysmal positional vertigo (BPPV) is commonly attributed to canalithiasis. ER after 1-hour generalized tonic-clonic seizure lasted 5 min.org/content/70/22/2067/suppl/DC2.8F. with each repetition. and causes the erroneous sensation of spinning when the head shifts with respect to gravity. Immigrated from Central America 6 months ago. The patient is then returned to upright. No medications. Dx? D. pulse 80/min.vomiting. Hearing loss or symptoms are typically absent [14]. resp 18/min. Neurocysticercosis . calcium debris within the semicircular canal [3]. Heavy debris in the canal causes inappropriate movement of the endolymph with linear accelerations. 98. beating upward and torsionally. so that the head hangs over the edge of the bed. Patients with BPPV typically have no other neurologic complaints.neurology. and the maneuver is repeated with the head turned to the other side. the neck is extended and turned to one side. observed for another 30 seconds for nystagmus. the nystagmus will recur but in the opposite direction ●The patient should then have the maneuver repeated to the same side. such as gravity. with the upper poles of the eyes beating toward the ground ●After it stops and the patient sits up. CT head multiple enhancing lesions. Drowsy but cooperative. 24 yo woman. Toxycology (-). tachycardia. Intoxication for which substance? C. Pupils 5mm bilat.10. Urea nitrogen 40. CNS stimulation) are often seen in MDMA toxicity. especially in patients with calcified lesions [10]. Shaking for 1 minute. although such infection accounts for a minority of cases in the United States. Users . 42. particularly among young party-goers at electronic dance music venues. including dance clubs and large music festivals. and euphoria. then silent and staring. PT 16s. Unresponsive. Ecstasy MDMA is a commonly abused drug. When present. which had a higher proportion of immigrant Hispanic patients than the other hospitals. Seizures may result from inflammation. perhaps from intermittent antigen release [6. parenchymal cysts are associated with seizures and headache. but it can be delayed for >30 years [14. altered vision.9]. and disinhibition. Na 114. the cystic lesions either resolve or form a calcified granuloma.16. Studies have highlighted a potential role of matrix metalloproteinases in the pathogenesis of seizures in neurocysticercosis. sympathomimetic amphetamine that causes release of endogenous catecholamines. Typical effects include feelings of euphoria. wakefulness. ER for seizure at dormitory party on campus. Other less common manifestations include mass effect. Profuse diaphoresis and piloerection.12-15].7]. The clinical manifestations depend upon whether the cysts are localized to the brain parenchyma. SIADH) may be seen as well. Restless and grinds her teeth. although the mechanism of seizures associated with calcified lesions is not completely clear. Creat 2. while extraparenchymal cysts are associated with symptoms of elevated intracranial pressure (eg. serotonergic toxicity (serotonin syndrome. and meningitis. CK 1200. Although typical findings of amphetamine toxicity (hypertension. ALT 90. reduces fatigue. and vomiting) and may be accompanied by altered mental status. MDMA differs somewhat from traditional amphetamines in that it is structurally similar to serotonin. This results in a toxicity profile for MDMA that is different from that of traditional amphetamines. focal neurologic signs. MDMA (3.3F.11]. and Albuquerque (5. In general. the calcifications are associated with recurrent seizures.Neurocysticercosis was observed more frequently in emergency departments of Los Angeles. and leads to feelings of increased physical and mental powers. or both.17]. pulse 90/min. Neurologic examination usually does not demonstrate focal signs in the absence of mass effect or stroke. This difference likely accounts for an increased release of serotonin and inhibition of serotonin reuptake [4. Parenchymal cystic or enhancing lesions are the most common form of neurocysticercosis (NCC) in hospitalbased series and is present in >60 percent of these patients [3. altered mental status. 19 yo woman.4-methylenedioxymethamphetamine) increases alertness. changes in brain plasticity and scarring (eg. nausea. Fever is not typically present. Later onset may be due to calcified lesions. The onset of symptomatic NCC has been estimated to peak at three to five years after infection. Alternatively. Travelers to endemic areas represent another source of cysticercosis.7 percent). Drinking more water than usual. sexual arousal. hippocampal sclerosis) may result in epileptogenic foci [8. headache. intimacy. Phoenix. resp 24/min BP 150/95. hyperthermia. 103. the extraparenchymal tissues. Ultimately. and hypertension. Individuals who use MDMA in . Psychomotor agitation may be associated with hyperthermia as well as rhabdomyolysis. Hyperthermia can lead to disseminated intravascular coagulation and rhabdomyolysis. ataxia. Even in the absence of severe hyperthermia or disseminated intravascular coagulation. hepatitis.) Marked and often acute reductions in serum sodium can lead to serious neurologic manifestations including confusion. dancing all night at a "rave"). The serum sodium in such patients is usually below 120 meq/L (120 mmol/L). aspartate transaminase (AST). Hyperthermia and related effects — Hyperthermia may result from drug effects on the central nervous system (CNS). Hepatotoxicity — Hepatotoxicity caused by MDMA poisoning is well recognized. blurry vision. Cardiovascular toxicity can include hypertensive emergencies. diaphoresis. has been reported [22. and hepatic fibrosis may result from MDMA abuse [25. Young women appear to be at increased risk for both symptomatic hyponatremia and residual neurologic injury. Clinical findings are similar to other forms of toxin-induced hepatic injury and may include jaundice. including intracranial hemorrhage and posterior spinal artery aneurysm. and even delirium [24]. prolonged physical exertion (eg.typically begin to experience the desired effects of MDMA approximately one hour following oral administration [10]. and altered mental status. These effects are usually selflimited and resolve within hours [14]. and dysrhythmia [15-21]. such as agitation. In addition. Vital signs — MDMA can cause hypertension. and death.24]. and environmental conditions (eg. (See 'Hyperthermia' below. nausea. Serotonin syndrome findings — Serotonin syndrome is a potentially life-threatening condition characterized by the triad of autonomic dysfunction. Cardiovascular stimulation — Life-threatening increases in heart rate and blood pressure may occur. cerebral herniation. hyperactivity. tachycardia. bruxism (grinding teeth). More serious effects are uncommon and described below.26]. The mechanisms that lead to hyponatremia are described separately. centrilobular necrosis. myocardial infarction. abdominal pain. aortic dissection. abnormal neuromuscular activity. dancing in a densely populated. Both the stimulant effect of amphetamines and serotonin syndrome may contribute to severe hyperthermia in these patients [19. MDMA use can cause serotonin syndrome. can also occur at typical MDMA doses. and alanine transaminase (ALT) may be present. (See "Causes of hyponatremia in adults". persistent secretion of antidiuretic hormone that slows the rate of water excretion.) Manifestations of hyponatremia — MDMA use can lead to hyponatremia due to a marked increase in fluid intake and in some patients. and hyperthermia. Minor adverse reactions. and vomiting. The belief among some MDMA users that they can avoid hyperthermia by drinking large amounts of water is the reason for increased fluid intake in many cases. hot room). Elevations of bilirubin. presumably via stimulation of massive serotonin release. tachycardia. section on 'Ecstasy (MDMA) intoxication'.23]. cerebral edema. Neurologic — Stimulation of the CNS is common and can manifest as agitation. anxiety. catastrophic central neurologic hemorrhage. seizures. Seizures and status epilepticus can occur. and secondarily generalized have been eliminated. Simple partial (motor) seizure with generalization. and they may be asymmetric. which can be subcortical or cortical structures. patients frequently complain of facial swelling or head fullness.30]. The clinical diagnosis of superior vena cava (SVC) syndrome is made on the basis of characteristic signs and symptoms of central venous obstruction. or possibly coma. elbow. BP 142/90. which may be exacerbated by bending forward or lying down. Papilledema. or lithium) are at greater risk of developing serotonin syndrome [27-30]. New Classification: In the 2010 proposal.combination with selective serotonin reuptake inhibitors (SSRIs). Find himself in floor after. 52 yo man episodes of loss consciousness preceded by twitching of the right thumb. or dysphagia. mode of onset is subdivided into generalized. the terms simple partial. Spread duration of 20 seconds. Bites tongue and urinates during episodes. and facial plethora are less frequent. No neurological local findings. chest pain. Regardless of etiology. dusky reddish-purple skin color with venous distention over head and neck. Generalized — Generalized seizures are conceptualized as those that originate at some point within. Importantly. cyanosis. G. both external compression and thrombosis coexist. the most common findings are facial edema and distension of the veins in the neck and on the chest wall (picture 1).9. however. 37 yo man. focal. On physical examination. complex partial. or by thrombosis of blood within the SVC. monoamine oxidase inhibitors (MAOIs). Er with 10-day hx increasingly severe headaches. then hand. since they were difficult to define pragmatically and were often used incorrectly. and unknown. or other drugs that increase 5-HT1A receptor activity (such as meperidine. Pain is severe in pounding. and rapidly engage bilaterally distributed networks. In addition. the proposal uses various descriptors of focal seizures to describe an event more precisely. Pulse 70/min. resp 16/min. the latter of which includes spasms. confusion. In addition. and does not exclude the possibility of a surgical remedy. lymph nodes. and other mediastinal structures. a generalized presentation can still arise from a focal lesion. Obstruction of venous return to the heart Superior vena cava (SVC) syndrome results from any condition that leads to obstruction of blood flow through the SVC. Patients with cerebral edema may have headaches. Obstruction can be caused by invasion or external compression of the SVC by an adjacent pathologic process involving the right lung. Focal — The term focal has replaced partial to describe seizures that originate in networks limited to one hemisphere [3]. Arm edema. wrist. Unremitting pain for 18h. dyspnea is the most common symptom [8. 43. and shoulder. Instead. Generalized seizures do not need to necessarily include the entire cortex. Most likely cause of intracranial HTN? D. Examples of preferred descriptors include: . Mild swelling. Other symptoms include arm swelling. Focal seizures may arise from either subcortical structures or neocortex. 44. tryptophan. cough. In some cases. or dyscognitive ●Evolving to a bilateral convulsive seizure Unknown — The term unknown mode of onset is used for seizure types where it remains unclear whether onset is focal. concerning which there has traditionally been controversy. No warming symptons. old classification: 45. suddenly . A key example is epileptic spasms. 72 yo man episodes loss of consciousness past 2 years. and current knowledge is inadequate to specify mode of onset as either focal or generalized.●Without impairment of consciousness or awareness ●Involving subjective sensory or psychic phenomena ●With impairment of consciousness or awareness. generalized. or perhaps either. ILAE Classification of seizures Generalized seizures Tonic-clonic (in any combination) Absence Typical Atypical Absence with special features Myoclonic absence Eyelid myoclonia Myoclonic Myoclonic Myoclonic atonic Myoclonic tonic Clonic Tonic Atonic Focal seizures Unknown Epileptic spasms ILAE: International League Against Epilepsy. Fracture arm and bruised. He recovers completely and is not confused at the end of episodes. Jerking or twitching movements of the trunk and extremities for several seconds during episode. K. Syncope Imitators of epilepsy: Nonepileptic paroxysmal disorders Neonates Apnea Jitteriness Benign neonatal sleep myoclonus Hyperkplexia Infants Breath-holding spells Benign myoclonus of infancy Shuddering attacks Sandifer syndrome Benign torticollis in infancy Abnormal eye movements (eg. opsoclonus-myoclonus) Rhythmic movement disorder (head banging) Children Breath-holding spells Vasovagal syncope Migraine Benign paroxysmal vertigo Staring spells Tic disorders and stereotypies Rhythmic movement disorder Parasomnias Adolescents and young adults Vasovagal syncope Narcolepsy Periodic limb movements of sleep Sleep starts Paroxysmal dyskinesia Tic disorders Hemifacial spasm Stiff person syndrome Migraine Psychogenic nonepileptic pseudoseizures Hallucinations Older adults Cardiogenic syncope Transient ischemic attack Drop attacks Transient global amnesia Delirium or toxic-metabolic encephalopathy Rapid eye movement sleep behavior disorder . During episodes becomes pale and sweaty. spasmus nutans.find on the floor. Hx of 2 MI. struggling or Usually atonic. including Purposeful movements Absent Absent avoidance Biting Tongue. there is a period of transition from the ictal state back to the individual's normal level of awareness and function. common possible. weak if Pulse Rapid. rare Regains consciousness in 2 to 3 Postictal Tired. strong Normal vasodepressor. possibly sudden if Onset Sudden Gradual cardiogenic Sequence of symptoms Stereotyped Variable Stereotyped Spontaneous or induced by Termination Spontaneous supraorbital pressure. heat. or both >20 seconds: tonic. that of arrhythmias if cardiogenic Timing Usual duration 1 to 5 min 5 to 60 min 1 to 2 min Gradual. alert but tired Following the end of a seizure. if syncope lasts Generalized motor Tonic. injury. mild. then clonic Partial flexion or Tonic posture Opisthotonic straight Head movements To one side or none Side to side Clonus/limb jerks Bilaterally synchronous Asynchronous Bilaterally synchronous Occasional. confused. if any During course None Location of motor Proximal limb Proximal limb None component (if present) Tonic. flashing Emotion if cardiogenic lights Presence of others Variable Usual Variable Motor phenomena Vocalization At onset. any position if Situation Awake or sleep Awake cardiogenic Sleep loss. alcohol Emotion. scalp. but multiple bruises Injury If sudden onset face. other people Tongue biting rare Babinski's sign Present Absent Absent Autonomic features Micturition Frequent Rare Occasional Eyes Open Closed Open Dilated or hippus during Pupils Normal Dilated attacks Colour Cyanotic or grey Rubor or normal Pale Slow if vasovagal. none Precipitating factors withdrawal. crowds. scalp. Rare. Rapid suggestion Sequelae Frequent. face. inside mouth Lips. sleepy Alert. then clonic thrashing. flailing. This interval is referred to as .Differentiation of generalized tonic-clonic seizures from pseudoseizures and syncope Generalized seizure Characteristic Pseudoseizure Syncope tonic-clonic Circumstances Usually upright. arms. emotional outburst min. 47. chiasm. Difficulty maintaining her balance past 3 months. BP 180/130. 48. Need constant stimulation to stay awake. whether the individual was on antiseizure drugs. In addition to hypertensive encephalopathy. If a person had a focal seizure with impairment of consciousness or a convulsion.54]. Unable to take medication corticosteroids and antihypertensive. 46. focal neurologic deficits may also be present. resp 40/min.the "postictal period" and signifies the recovery period for the brain. if present. and/or seizures. This pathology is caused by cerebrovascular endothelium breakdown secondary to failure of cerebral autoregulation. particularly if there is underlying brain dysfunction [27]. while elderly patients with secondarily generalized seizures may have postictal confusion and sleepiness that persists for as long as several days to a week. ESR 12. Osseous lesions. Manifestations typically include confusion and suppressed alertness. They rarely occur in older children and adults [53. As an example. Malignant transformation of tumors may also occur in childhood. 12 yo girl with SLE in ER b/c difficulty awakening this morning. Scattered nodules on skin. young adults with focal seizures of frontal lobe origin may have postictal states that last only several seconds. axillary and/or inguinal freckling. Dx? C. pulse 60/min. and age. 48 yo woman. and neurofibromas [32]. 98. Hypertensive encephalopathy Hypertensive emergencies in children usually manifest as hypertensive encephalopathy: severe BP elevation with cerebral edema and neurological symptoms of lethargy. OPGs are typically low-grade pilocytic astrocytomas [55]. Greatest risk for? D. Tendon reflexes brisk symmetric. Other tumors and neurologic complications typically begin to appear after the first year of life. coma. The postictal state may last from seconds to minutes to hours. flu-like symptoms and vomiting. They can arise anywhere along the anterior visual pathway to the optic radiations and involve the optic nerves. Hypertension may occur in childhood. buccal mucosa. and postchiasmal optic tracts. optic nerve glioma The typical order of appearance of clinical manifestations is café-au-lait macules. the length of the seizure.6F. 25 yo woman. Bilat papilledema with flame hemorrhages. and symptomatic optic pathway glioma (OPG) usually occurs by the time the patient is three years of age (table 1). Funduscopic examination is of particular importance since papilledema and retinal hemorrhage or exudates may be the only sign of a hypertensive emergency. visual changes may also result from retinal hemorrhage or exudates that involve the macula (central vision) or cause noticeable defects in peripheral vision. limbs. usually appear during the patient's first year after birth. 6 months ago episode of nephrolithiasis complicated with UTI and require antibiotic. Decreased left nasolabial fold and slight perioral droop when . OPGs occur in 15 percent of children younger than six years of age with NF1 [52]. tongue. depending upon several factors including which part(s) of the brain were affected by the seizure. Obtunded but moves extremities to painfull stimuli. Past 2-day. Nodules in lips. growths in her lips and tongue for years. Lisch nodules (iris hamartomas). his or her level of awareness gradually improves during the postictal period. much like a person waking up from anesthesia after an operation. but more often occurs in adolescence and adulthood. hx 6-month gradual hearing loss left ear. Café au lait spot in trunk. Childhood exposure to low-dose radiation for benign conditions of the head and neck has been associated with an increased risk of vestibular schwannoma. vestibular schwannomas can present with sudden sensorineural hearing loss. The two major symptoms were hearing loss and tinnitus. The most common symptoms were facial numbness (paresthesia). hypesthesia. Forehead normal. The functions of the lower cranial nerves can also become impaired. labile blood pressure for 3 hours. Gait normal. Affected patients frequently acknowledged having unsteadiness while walking. the central vestibular system can often compensate for the gradual loss of input from one side. The average duration of symptoms was 1. affecting the right and left sides with equal frequency. 5 days after hemicolectomy in ICU. diaphoresis. cerebellar tonsil herniation.3 years. True spinning vertigo was uncommon because these slow growing tumors cause gradual rather than acute asymmetries in vestibular function. Clinical manifestations in this series included the following: ●Cochlear nerve — Symptomatic cochlear nerve involvement occurred in 95 percent of patients [18]. The tumors are unilateral in more than 90 percent of cases [5]. leading to dysarthria. The incidence of tinnitus was higher in hearing than in deaf patients but was also present in 46 percent of deaf patients. the symptoms usually occurred after hearing loss had been present for more than two years and vestibular symptoms for more than one year. In this setting. but difficulty with tamden walking. Bilateral vestibular schwannomas are primarily limited to patients with neurofibromatosis type 2. Hearing loss was present in 95 percent but only two-thirds of these patients were aware of this limitation. aspiration. Brainstem compression.smiles. and pain. acoustic neuroma (vestibular schwannoma) The median age at diagnosis is approximately 50 years [2]. Medication morphine. less often. ●Trigeminal nerve — Trigeminal nerve disturbances occurred in 17 percent of patients [18]. 49. ●Vestibular nerve — Involvement of the vestibular nerve occurred in 61 percent of patients [18]. Tinnitus was present in 63 percent with an average duration of three years [18]. The hearing loss was usually chronic. The clinical presentations of these tumors are illustrated by a series of 1000 vestibular schwannomas treated at a single institution [18]. High frequency hearing loss left with impaired speech discrimination. Dx? A. ●Tumor progression — Other presenting signs can be the result of tumor progression. The primary symptoms were facial paresis and. Very large tumors can press on the cerebellum or brainstem and result in ataxia. . with an average duration of about four years. and hoarseness. hydrocephalus and death can occur in untreated cases. leading to pressure on adjacent posterior fossa structures. Post-op is complicated with fever. Haloperidol nightly (2mg) Hx of Bipolar. which was typically mild to moderate in nature and frequently fluctuated in severity. dysphagia. present with 105F. lithium. 62 yo man. lethargy. Occasionally. ●Facial nerve — The facial nerve was involved in 6 percent of patients [18]. nocturnal agitation since day 2. taste disturbances. 105F.11]. Temperatures of more than 38ºC are typical (87 percent). Typical symptoms — The tetrad of NMS symptoms typically evolves over one to three days. It is not surprising. Evolution to profound encephalopathy with stupor and eventual coma is typical [15]. Lying in pool of sweat and drooling. 70 percent of patients followed a typical course of mental status changes appearing first.clonazepam. Urea nitrogen 38. Neutro 80%. Other motor abnormalities include tremor (seen in 45 to 92 percent). Lymphs 10%. given the usual psychiatric comorbidity of the typical patient. Dysrhythmias may occur. no erythema. Muscular rigidity is generalized and is often extreme. Diaphoresis is often profuse. bands 5%.6. clozapine. WBC 20000 .4]. but even higher temperatures. chlorpromazine) and the newer "atypical" antipsychotic drugs (eg. and tachypnea (in 73 percent) [2.19]. and other reports do not necessarily substantiate a typical course. promethazine) [4. haloperidol. pulse 140/min. Autonomic instability typically takes the form of tachycardia (in 88 percent). Wound clean and dry. and autonomic dysfunction [35]. Wet skin and dry mucosa. Dx? E. NMS is defined by its association with a class of medications that block dopamine transmission and a tetrad of distinctive clinical features: fever. including the low potency (eg. dysarthria. then hyperthermia. There is substantial variability in the presentation of NMS. and other dyskinesias [2. This often takes the form of an agitated delirium with confusion rather than psychosis. Bowel sounds hyperactive. risperidone. trismus. leading to initial diagnostic confusion [2]. every class of neuroleptic drug has been implicated. chorea. BP 180/min. Labs Hb 15. Catatonic signs and mutism can be prominent. Babinski (-). that its significance is often underappreciated. and dysphagia. Patients can also have prominent sialorrhea. and autonomic instability [3. Hyperthermia is a defining symptom according to many diagnostic criteria. Creat 1. dystonia. Herpetic lesion lower lip. Each feature is present in 97 to 100 percent of patients: Mental status change is the initial symptom in 82 percent of patients [35]. no erythema. metoclopramide. labile or high blood pressure (in 61 to 77 percent). Some case reports document delay in the appearance of fever of more than 24 hours. However. greater than 40ºC. fluphenazine) [8-10]. In an analysis of 340 cases. opisthotonus. Reflexes hyperactive and symmetric. Neurological shows masked facies and intention tremor. and less commonly. Urianalysis and CXR normal.34]. mono 5%. followed by rigidity. mental status changes. Superimposed tremor may lead to a ratcheting quality or a cogwheel phenomenon. CK 1000. are common (40 percent) [4]. Neuroleptic malignant syndrome. NMS is most often seen with the "typical" high potency neuroleptic agents (eg. rigidity. Neck supple and IV catherer insertion site is non-tender. Drugs that can cause neuroleptic malignant syndrome Neuroleptic agents Antiemetic agents Aripiprazole Domperidone . The increased tone can be demonstrated by moving the extremities and is characterized by "lead pipe rigidity" or stable resistance through all ranges of movement. olanzapine) as well as antiemetic drugs (eg. sensory neuropathy. although there is a strong association with myasthenia gravis and other paraneoplastic syndromes. Thymomas account for about 20 percent of mediastinal neoplasms (table 1). There are no known risk factors. neuromyelitis Neurologic and optica. rheumatoid arthritis. panhypopituitarism. and fatigue. scleroderma. Greatest risk for? E. Symmetrical muscle involvement is the rule. Many describe aching or stiff muscles. dysphagia. nephrotic syndrome. Most patients present with complaints of slowly progressive proximal muscle weakness. pemphigus. Occasional patients have a subacute or acute presentation. Most thymoma patients are between 40 and 60 years of age. hepatitis. stiff person syndrome. Patients with LEMS and small cell lung cancer typically develop a more rapidly progressive course of proximal and . hemolytic anemia. There tends to be relative preservation of distal muscle function. Pupillary reflexes normal. Bilateral ptosis. and there is a similar incidence in men and women [1]. 62 yo woman. Upper extremity complaints are usually modest and typically involve proximal muscle function. vitiligo Dermatologic Addison's disease. week hx intermittent drooping of his left eyelid and double vision. Patients typically describe an alteration in gait or difficulty arising from a chair or managing stairs. myocarditis. Cushing syndrome. agranulocytosis. particularly after protracted exercise. pernicious anemia Hematologic Alopecia areata. thyroiditis Endocrine Acquired hypogammaglobulinemia. Paraneoplastic syndromes associated with thymic neoplasms Myasthenia gravis. gastrointestinal pseudoobstruction. Common symptoms include diplopia. ulcerative colitis The clinical features of Lambert-Eaton myasthenic syndrome LEMS were well defined by Lambert and Eaton in Minnesota [29] and by O'Neill and colleagues in England [18]. weakness. hemichorea neuromuscular Pure red cell aplasia. ptosis. polymyositis. Eaton Lambert syndrome. thymomas and thymic carcinomas are the most common neoplasms arising in the thymus. Isaac's syndrome (neuromyotonia). more prominent on left. The most common paraneoplastic syndrome associated with thymoma is myasthenia gravis. Muscle fatigability or cramping is common. and this feature is present in almost all patients at some point in the illness. Thymoma In adults. Myasthenia gravis is an autoimmune disorder caused by interference with acetylcholine receptors of voluntary muscle at the neuromuscular junction. oral lichen planus.Chlorpromazine Clozapine Fluphenazine Haloperidol Olanzapine Paliperidone Perphenazine Quetiapine Risperidone Thioridazine Ziprasidone Droperidol Metoclopromide Prochlorperazine Promethazine 50. Weaknees on left eye abduction/adduction and right abduction. Miscellaneous sarcoidosis. In the largest study of autonomic dysfunction in LEMS involving 30 patients. . Dry mouth from reduced salivation is the most common autonomic symptom and occasionally is the presenting complaint.then distal arm muscle weakness than patients who have non-tumor LEMS Autonomic dysfunction is often present and can be an important clue to the diagnosis. Other symptoms may include blurred vision and constipation. dry mouth was identified in 77 percent. while erectile dysfunction is common in men with LEMS. and impotence was present in 45 percent of all men [31].