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MOTOR NEURON

DISEASES

By
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.

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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 Click to edit Master text styles
Second level
● Third level
functional unit of ● Fourth level
● Fifth level

the nervous
system, also called
as nerve cells.

Based on function,
has
4/8/11 two types:
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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 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
paralysis paralysis
03 Muscle Absent Occurs
Observation

wastage
04 Superficial Lost Lost
Clinical

reflex
05 Plantar reflex Abnormal Absent
(Babinski’s Sign)
06 Deep reflex Exaggerated Lost
Conformatio

07 Clonus Present Lost


Clinical

08 Electrical Normal Absent


n

activity
09 Muscles Group of muscles Individual muscle
affected affected affected
10 Fascicular Absent Present
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twitch in
DEFINITION
Motor Neuron Diseases are group of
progressive neurological disorders that
destroy motor neurons.

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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
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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
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RISK FACTORS

Family history

Smoking[probable risk factor]

Beware: Artificial sweeteners and


flavor enhancers can destroy
nerve cells 

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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,
4/8/11 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,

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:
Conti…
Spasticity
Baclofen, Tizanidine
Cramping & fasciculation
Quinine sulphate
Salivation & drooling
Amitriptyline,Scopolamine,
irradiation
Pseudobulbar affect
4/8/11 Amitriptyline
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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
4/8/11 Stephen Hawking
MOTOR NEURON a person 2121
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-
bound or bedridden, including

Decubitus ulcers and

Skin infections (While rare in patients


with ALS,

these complications can emerge if


appropriate
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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
4/8/11
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)
Slow progressive degeneration of only
lower motor neurons

Largely affects men

Weakness typically seen first in hands


& then spreads into the lower body

Trunk and respiratory muscles affected


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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
Ø
PALSY
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

4/8/11 MOTOR NEURON 3333


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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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
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Second level
● Third level

● Fourth level

● Fifth level

THANK YOU
4/8/11 MOTOR NEURON 3838

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