MOTOR NEURON DISEASESBy Dr. Deepti Patil Dept. of Dravyaguna

INTRODUCTIONDeadly disease with sporadic cause Hampers all the motor activities of the person in a very short duration. No everlasting treatment.

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INCIDENCEIncidence of MND is approximately 15 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

NEURONSStructural and functional unit of the nervous system, also called as nerve cells. Based on function,4/8/11 MOTOR NEURON has two types: 44

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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 cell4/8/11 MOTOR NEURON 55

Motor nerveMotor- 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 NEURONNeurons 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 NEURONAnterior 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.

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DIFFERENCESl.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 (Babinskis 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 ObservationClinical Conformatio n4/8/11

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DEFINITIONMotor Neuron Diseases are group of progressive neurological disorders that destroy motor neurons.

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ALTERNATE NAMESAmyotrophic 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

CLASSIFICATIONAmyotrophic Lateral Sclerosis (ALS) Primary Lateral Sclerosis (PLS) Progressive Muscular Atrophy (PMA) Monomelic Amyotrophy Postpolio syndrome Spinal Muscular Atrophy Bulbar Pseudobulbar palsy - spastic4/8/11 MOTOR NEURON 1212

CAUSESAbout 90% of cases of MND are "sporadic. Approximately 10% of cases are "familial MND. Neurofilament disruption Neurotransmitter system disruption Neurotrophic factors Heavy metals4/8/11 MOTOR NEURON 1313

RISK FACTORSFamily history Smoking[probable risk factor] Beware: Artificial sweeteners and flavor enhancers can destroy nerve cells

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PATHOLOGYDegeneration of the upper motor neurons, loss of myelinated fibers in the corticospinal tract Occasionally there is atrophy of the pre-central gyrus

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SIGNS & SYMPTOMSProgressive 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 NEURON1616

DIAGNOSISBlood tests Electrophysiological studies Imaging Invasive

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TREATMENTMultidisciplinary approach Neurologist, Palliative Nurse, Dietician Speech Therapist, Early intervention is favored

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Conti.Disease modifying therapy: Riluzole: Antiglutamate SE: Vomiting, Weakness, Headache, Vertigo, Pain, Deranged LFTS. Improves survival by 3 months4/8/11 MOTOR NEURON 1919

Symptomatic management: Spasticity Baclofen, Tizanidine Cramping & fasciculation Quinine sulphate Salivation & drooling

Conti

Amitriptyline,Scopolamine, irradiation Pseudobulbar affect4/8/11 MOTOR Amitriptyline NEURON 2020

PROGNOSISMND progress quite quickly, Decline occurs over the course of months. Fatal within 25 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

COMPLICATIONSProgressive inability to perform activities of daily living Deterioration of ambulation Aspiration pneumonia Respiratory insufficiency4/8/11 MOTOR NEURON 2222

ContiComplications from being wheelchairbound or bedridden, including Decubitus ulcers and Skin infections (While rare in patients with ALS, these complications can emerge if appropriate4/8/11 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)

Difficulty with balance

PRIMARY LATERAL SCLEROSIS (PLS)

Weakness and stiffness in the legs Clumsiness, spasticity in the legs which produces slowness and stiffness of movement, dragging of the feet

Facial involvement resulting in dysarthria (poorly articulated speech)MOTOR NEURON 2525

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PROGRESSIVE MUSCULAR ATROPHY (PMA) of only Slow progressive degenerationlower motor neurons Largely affects men Weakness typically seen first in hands & then spreads into the lower body Trunk and respiratory muscles affected4/8/11 MOTOR NEURON 2626

SPINAL MUSCULAR ATROPHYHereditary disease affecting the lower motor neurons. Weakness is often more severe in the legs than in the arms.

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PROGRESSIVE BULBAR PALSY Pharyngeal muscle weakness(involved with swallowing) Weak jaw and facial muscles Progressive loss of speech and tongue muscle atrophy

Emotional liabilityMOTOR NEURON 2828

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PSEUDOBULBAR PALSYExpressionless face Tongue may become immobile and unable to protrude from the mouth Emotional lability

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POST POLIO SYNDROME

Fatigue Slowly Progressive Muscle Weakness Muscle Atrophy Fasciculations Cold Intolerance Muscle & Joint Pain. EtcMOTOR NEURON 3030

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MONOMELIC AMYOTROPHYWeakness & wasting of a single limb Weak & wasted hand muscles Weak & wasted lower arm muscles Fine motor control problems Weak grip Clawed hand Hand tremors4/8/11 MOTOR NEURON 3131

IN AYURVEDA4/8/11 MOTOR NEURON 3232

DIFFERENTIAL DIAGNOSISMamsagata vata Majjagata vata Sarvanga vata Ardhanga vata Pakshaghata4/8/11 MOTOR NEURON 3333

MANAGMENTVatavyadhi chikitsa.Sneha dravya prayoga, Abhyanga, swedana Basti chikitsa Bruhmana dravya upayoga

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Conti.Bruhmana nasya Bruhmana Basti Rasayana

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PATHYAMadhura, 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

CONCLUSIONDeadly 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

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THANK YOU4/8/11 MOTOR NEURON 3838