MOTOR NEURON DISEASESBy Dr. Deepti Patil Dept. of Dravyaguna INTRODUCTION Deadly disease with sporadic cause Hampers all the motor activities of the person in a very short duration. No everlasting treatment. 4/8/11 MOTOR NEURON 22 INCIDENCE Incidence of MND is approximately 1–5 out of 100,000 people. Men have a slightly higher incidence rate than women. Approximately 5,600 cases are diagnosed in the U.S. every year. M:F -1.5:1, Affects young and middle aged adults. 4/8/11 MOTOR NEURON 33 NEURONS Structural and functional unit of the nervous system, also called as nerve cells. Based on function, 4/8/11 MOTOR NEURON has two types: 44 Click to edit Master text styles Second level ● Third level ● Fourth level ● Fifth level Conti…. Centrosome is absent in nucleus of the nerve cell body. Dendrites transmit impulses towards the nerve cell body. Axons are covered by myelin sheath which is responsible for white colour of the nerve fibers. Myelin sheath-responsible for faster conduction of impulse through nerve cell 4/8/11 MOTOR NEURON 55 Motor nerve Motor- that which produces motion or movement Nerve which supplies muscles, an exocrine glands also fibers. Higher part of the brain to lower parts or spinal cord. All motor nerves are axons of the corresponding nerve cell body. 4/8/11 MOTOR NEURON 66 UPPER MOTOR NEURON Neurons in higher center of brain which control the lower motor neurons. Three types: 1. 2. Motor neuron in the cerebral cortex Neuron in the basal ganglia & nuclei in brainstem Neuron in the cerebellum 3. Effect of UMN lesion depends upon the type of neuron involved. 4/8/11 MOTOR NEURON 77 LOWER MOTOR NEURON Anterior gray horn cells in the spinal cord & motor neurons of cranial nerve nuclei situated in brain stem. Effect of LMN lesion are loss of muscle tone & flaccid paralysis. 4/8/11 MOTOR NEURON 88 DIFFERENCE Sl.No 01 02 EFFECTS Muscle Tone Paralysis Muscle wastage Superficial reflex Plantar reflex Deep reflex Clonus Electrical activity Muscles affected Fascicular twitch in UMN Lesion Hypertonia Spastic type of paralysis Absent Lost Abnormal (Babinski’s Sign) Exaggerated Present Normal Group of muscles affected Absent MOTOR NEURON LMN Lesion Hypotonic Flaccid type of paralysis Occurs Lost Absent Lost Lost Absent Individual muscle affected Present 99 Clinical Observation Clinical Conformatio n 4/8/11 03 04 05 06 07 08 09 10 DEFINITION Motor Neuron Diseases are group of progressive neurological disorders that destroy motor neurons. 4/8/11 MOTOR NEURON 1010 ALTERNATE NAMES Amyotrophic Lateral Sclerosis (ALS), or Lou Gehrig's disease. Maladie de Charcot (Charcot's disease) Sclerose laterale amyotrophique (SLA) A- "no", myo-"muscle", and Trophic-"nourishment"; Amyotrophic- "no muscle nourishment,” 4/8/11 MOTOR NEURON 1111 CLASSIFICATION Amyotrophic Lateral Sclerosis (ALS) Primary Lateral Sclerosis (PLS) Progressive Muscular Atrophy (PMA) Monomelic Amyotrophy Postpolio syndrome Spinal Muscular Atrophy Bulbar Pseudobulbar palsy - spastic 4/8/11 MOTOR NEURON 1212 CAUSES About 90% of cases of MND are "sporadic”. Approximately 10% of cases are "familial MND”. Neurofilament disruption Neurotransmitter system disruption • Neurotrophic factors • Heavy metals 4/8/11 MOTOR NEURON 1313 RISK FACTORS Family history Smoking[probable risk factor] Beware: Artificial sweeteners and flavor enhancers can destroy nerve cells 4/8/11 MOTOR NEURON 1414 PATHOLOGY Degeneration of the upper motor neurons, loss of myelinated fibers in the corticospinal tract Occasionally there is atrophy of the pre-central gyrus 4/8/11 MOTOR NEURON 1515 SIGNS & SYMPTOMS Progressive weakness Muscle wasting Muscle fasciculations Spasticity or stiffness in the arms and legs Overactive tendon reflexes Dragging foot Unilateral muscle wasting in the hands, or slurred speech 4/8/11 MOTOR NEURON 1616 DIAGNOSIS Blood tests Electrophysiological studies Imaging Invasive 4/8/11 MOTOR NEURON 1717 TREATMENT Multidisciplinary approach Neurologist, Palliative Nurse, Dietician Speech Therapist, Early intervention is favored 4/8/11 MOTOR NEURON 1818 Conti…. Disease modifying therapy: Riluzole: Antiglutamate SE: Vomiting, Weakness, Headache, Vertigo, Pain, Deranged LFTS. Improves survival by 3 months 4/8/11 MOTOR NEURON 1919 Symptomatic management: Spasticity Baclofen, Tizanidine Cramping & fasciculation Quinine sulphate Salivation & drooling Conti… Amitriptyline,Scopolamine, irradiation Pseudobulbar affect 4/8/11 MOTOR Amitriptyline NEURON 2020 PROGNOSIS MND progress quite quickly, Decline occurs over the course of months. Fatal within 2–5 years. Around 50% die within 14 months of diagnosis. 1 in 5 patients survive for 5 years, and 1 in 10 patients survive 10 years. 4/8/11 MOTOR NEURON Professor Stephen Hawking a person 2121 COMPLICATIONS Progressive inability to perform activities of daily living Deterioration of ambulation Aspiration pneumonia Respiratory insufficiency 4/8/11 MOTOR NEURON 2222 Conti… Complications from being wheelchairbound or bedridden, including Decubitus ulcers and Skin infections (While rare in patients with ALS, these complications can emerge if appropriate 4/8/11 MOTOR NEURON 2323 Both upper and lower motor neurons are affected. 75% of people ALS develop weakness and wasting of the bulbar muscles First noticed in the arms and hands, legs, or swallowing muscles. 4/8/11 MOTOR NEURON Speech becomes slurred or nasal. 2424 Amyotrophic Lateral Sclerosis (ALS) Ø Difficulty with balance PRIMARY LATERAL SCLEROSIS (PLS) Ø Weakness and stiffness in the legs Clumsiness, spasticity in the legs which produces slowness and stiffness of movement, dragging of the feet Ø Ø Facial involvement resulting in dysarthria (poorly articulated speech) MOTOR NEURON 2525 4/8/11 PROGRESSIVE MUSCULAR ATROPHY (PMA) of only Slow progressive degeneration lower motor neurons Largely affects men Weakness typically seen first in hands & then spreads into the lower body Trunk and respiratory muscles affected 4/8/11 MOTOR NEURON 2626 SPINAL MUSCULAR ATROPHY Hereditary disease affecting the lower motor neurons. Weakness is often more severe in the legs than in the arms. 4/8/11 MOTOR NEURON 2727 Ø PROGRESSIVE BULBAR PALSY Pharyngeal muscle weakness (involved with swallowing) Weak jaw and facial muscles Progressive loss of speech and tongue muscle atrophy Ø Ø Ø Emotional liability MOTOR NEURON 2828 4/8/11 PSEUDOBULBAR PALSY Expressionless face Tongue may become immobile and unable to protrude from the mouth Emotional lability 4/8/11 MOTOR NEURON 2929 POST POLIO SYNDROME Ø Fatigue Slowly Progressive Muscle Weakness Muscle Atrophy Fasciculations Cold Intolerance Muscle & Joint Pain. Etc MOTOR NEURON 3030 Ø Ø Ø Ø Ø 4/8/11 MONOMELIC AMYOTROPHY Weakness & wasting of a single limb Weak & wasted hand muscles Weak & wasted lower arm muscles Fine motor control problems Weak grip Clawed hand Hand tremors 4/8/11 MOTOR NEURON 3131 IN AYURVEDA 4/8/11 MOTOR NEURON 3232 DIFFERENTIAL DIAGNOSIS Mamsagata vata Majjagata vata Sarvanga vata Ardhanga vata Pakshaghata 4/8/11 MOTOR NEURON 3333 MANAGMENT Vatavyadhi chikitsa. Sneha dravya prayoga, Abhyanga, swedana Basti chikitsa Bruhmana dravya upayoga 4/8/11 MOTOR NEURON 3434 Conti…. Bruhmana nasya Bruhmana Basti Rasayana 4/8/11 MOTOR NEURON 3535 PATHYA Madhura, Amla, Lavana Rasayukta Aahara Purana shaali, Masha, Godhuma, Mrudu, Sthira, Usna, Sthira Gunayukta Dravyas. Mamsa(except all aquatic animals), Sura, Asava, Usna Jala. 4/8/11 MOTOR NEURON 3636 CONCLUSION Deadly disease with sporadic cause. Debilitating condition. No everlasting treatment. Ayurveda has multiple approaches to the condition. Multiple treatment modalities adopted. 4/8/11 MOTOR NEURON 3737 Click to edit Master text styles Second level ● Third level ● Fourth level ● Fifth level THANK YOU 4/8/11 MOTOR NEURON 3838