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MOTOR NEURON

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.

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NEURONS
• Structural and functional
unit of the nervous
system, also called as
nerve cells.
• Based on function, has
two types:
1. Sensory Neurons &
2. Motor Neurons

• Made up of Nerve cell


body, Dendrite, &
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Axon.
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
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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.

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UPPER MOTOR NEURON
• Neurons in higher center of brain
which control the lower motor
neurons.
• Three types:
1.Motor neuron in the cerebral cortex
2.Neuron in the basal ganglia & nuclei
in brainstem
3.Neuron in the cerebellum
• Effect of UMN lesion depends upon
the type of neuron involved.
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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.

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DIFFERENCE
Sl.No EFFECTS UMN Lesion LMN Lesion
01 Muscle Tone Hypertonia Hypotonic
02 Paralysis Spastic type of Flaccid type of
03 Muscle paralysis
Absent paralysis
Occurs
04 wastage
Superficial Lost Lost
Observation

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
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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.
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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,”
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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
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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
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RISK FACTORS
• Family history
• Smoking[probable risk factor]

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PATHOLOGY

• Degeneration 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 & 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

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DIAGNOSIS
• Blood tests
• Electrophysiological studies
• Imaging
• Invasive

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TREATMENT
• Multidisciplinary approach

• Neurologist, Palliative Nurse, Dietician

• Speech Therapist & Social Services

• Has been shown to improve survival by


6 months

• 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 months

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Symptomatic management:
• Spasticity
 Baclofen, Tizanidine
• Cramping & fasciculation
 Quinine sulphate
• Salivation & drooling
 Amitriptyline,Scopolamine,
irradiation
• Pseudobulbar affect
 Amitriptyline
• Artificial ventilation
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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.
• Professor Stephen Hawking a person
with MND, and has lived for more than
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COMPLICATIONS
• Progressive inability to perform
activities of daily living
• Deterioration of ambulation
• Aspiration pneumonia
• Respiratory insufficiency

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Conti…
• Complications from being wheelchair-
boundor bedridden, including
– Decubitus ulcers and
– Skin infections (While rare in
patients with ALS,
 these complications can emerge if
appropriate
 padding is not used.)
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– Deep vein thromboses and
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Amyotrophic Lateral
Sclerosis (ALS)
• 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.
• Speech becomes slurred or nasal.

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PRIMARY LATERAL
SCLEROSIS (PLS)
Ø Difficulty with balance
Ø Weakness and stiffness in the legs
Ø Clumsiness, spasticity in the legs which
produces slowness and stiffness of
movement, dragging of the feet
Ø Facial involvement resulting in dysarthria
(poorly articulated speech)

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PROGRESSIVE MUSCULAR
ATROPHY (PMA)
• Pharyngeal muscle weakness
• Limb weakness with both LMN &UMN
signs but less prominent.
• Affected persons have outbursts of
laughing or crying (called emotional
lability).

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SPINAL MUSCULAR
ATROPHY
• Hereditary 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
Ø Pharyngeal muscle weakness
(involved with swallowing)
Ø Weak jaw and facial muscles
Ø Progressive loss of speech and
tongue muscle atrophy
Ø Emotional liability

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PSEUDOBULBAR PALSY
• Expressionless face
• Tongue may become immobile and
unable to protrude from the mouth
• Emotional lability

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POST POLIO SYNDROME
Ø Fatigue
Ø Slowly Progressive Muscle Weakness
Ø Muscle Atrophy
Ø Fasciculations
Ø Cold Intolerance
Ø Muscle & Joint Pain. Etc

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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
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IN
AYURVEDA

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DIFFERENTIAL DIAGNOSIS
Mamsagata vata
Majjagata vata
Sarvanga vata
Ardhanga vata
Pakshaghata
Kampavata
Ardita

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MANAGMENT
Vatavyadhi 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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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.
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THANK YOU
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