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DISEASES
By
Dr. Deepti Patil
Dept. of Dravyaguna
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.
05 reflex
Plantar reflex Abnormal Absent
Clinical
(Babinski’s Sign)
06 Deep reflex Exaggerated Lost
07 Clonus Present Lost
08 Electrical Normal Absent
Conformation
Clinical
09 activity
Muscles Group of muscles Individual muscle
affected affected affected
10 Fascicular Absent Present
twitch in
07/28/10 MOTOR NEURON DISEASES 8
E.M.G
DEFINITION
• Motor Neuron Diseases (MND) are
a group of neurological disorders
that selectively affect motor
neurons.
• Cells that control voluntary muscle
activity including speaking,
walking, breathing, swallowing and
general movement of the body.
07/28/10 MOTOR NEURON DISEASES 9
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,”
07/28/10 MOTOR NEURON DISEASES 10
CLASSIFICATION
• Amyotrophic Lateral Sclerosis (ALS)
• Primary Lateral Sclerosis (PLS)
• Progressive Muscular Atrophy (PMA)
• Monomelic Amyotrophy
• Postpolio syndrome
• Spinal Muscular Atrophy
• Bulbar
– Pseudobulbar palsy - spastic
– Progressive bulbar palsy - spastic and
flaccid
07/28/10 MOTOR NEURON DISEASES 11
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
• Oxidative stress
• Hypoxia responsiveness
07/28/10 MOTOR NEURON DISEASES 12
RISK FACTORS
• Family history
• Smoking[probable risk factor]
• Riluzole: Antiglutamate
• SE: Vomiting, Weakness, Headache,
Vertigo, Pain, Deranged LFTS.
• Improves survival by 3 months