Guillain barre syndrome ,neuralgia,als

Guillain-Barre Syndrome, Neuralgia, ALS
• Presented By –
Prof.Dr.R.R.Deshpande (M.D in
Ayurvdic Medicine & M.D. in
Ayurvedic Physiology)
• www.ayurvedicfriend.com
• Mobile – 922 68 10 630
• professordeshpande@gmail.com
11/17/2016 1Prof.Dr.R.R.Deshpande

Kayachikitsa -- Paper 2 Part A Point 5
• Nidana and Chikitsa of Urusthambha
• Gullian Barrie syndrome
• Muscular Dystrophy
• Myasthenia Gravis
• Motor Neuron Diseases and Neuralgia
• Neuralgia

Guillain Barre Syndrome –Introduction
• Guillain-Barre  ( G B ) syndrome  --- rare disorder
• The syndrome is named after the French neurologists Guillain &
Barre,who described it in 1916
• Body's immune system attacks on  nerves
• Weakness and tingling in  extremities are usually the first
symptoms
• These sensations can quickly spread
• Eventually paralyzing  whole body
• Most severe form Guillain-Barre syndrome is a medical
emergency & must be hospitalized

• The cause is unknown.
• Auto Immune disorder .Body’s own immune system attacks
the peripheral nerves & damage their myelin insulation
• Immune dysfunction can be triggered by an
Infection,Surgery,Vaccination

• Diagnosis is made by signs & symptoms --- with exclusion of
other causes
•
• Supported by Tests like Nerve Conduction Studies ,Exam of
CSF
• There are sub types based on areas of weakness ,results of
Nerve conduction studies ,presence of Antibodies
• Called also as Acute Poly Neuropathy

Guillain Barre Syndrome –Causes
• Cause  --- Idiopathic  ( Exact cause not Known)
• Often preceded by an infectious illness such as a respiratory
infection
• There is  no known cure for Guillain-Barre syndrome,
• Treatments can ease symptoms and reduce the duration of the illness
• Most people recover from Guillain-Barre syndrome
• Some may experience lingering effects from it like  weakness,
numbness or fatigue.

Guillain Barre Syndrome –Causes
• The disorder usually appears days or weeks after a
respiratory or digestive tract infection
• Rarely, recent surgery or immunization can trigger Guillain-
Barre syndrome

Clinical Features of GB syndrome
• Begins with tingling and weakness starting in feet and legs
and spreading to upper body and arms
• In about 10 percent of people with the disorder, symptoms
begin in the arms or face
• As Guillain-Barre syndrome progresses, muscle weakness
can evolve into paralysis.

G B Syndrome – Clinical Features
• Prickling, "pins and needles" sensations in fingers, toes, ankles or
wrists
• Weakness in legs that spreads to upper body
• Unsteady walking or inability to walk or climb stairs
• Difficulty with eye or facial movements, including speaking, chewing
or swallowing
• Severe pain that may feel achy or cramp-like and may be worse at
night
• Difficulty with bladder control or bowel function
• Rapid heart rate ,Low or high blood pressure ,Difficulty breathing

G B Syndrome – Clinical Features
•Patient experiences most significant weakness within
two to four weeks after symptoms begin
• Recovery usually begins two to four weeks after
weakness plateaus

Types of GB Syndrome
• Acute Inflammatory Demyelinating Polyradiculo
neuropathy (AIDP) --- most common form in the U.S.
• The most common sign of AIDP is muscle weakness which
starts in the lower part of the body and spreads upward
• Miller Fisher syndrome (MFS) -- in which paralysis starts in
the eyes & is also associated with unsteady gait

Types of GB Syndrome
• MFS occurs in about 5 percent of people with Guillain-Barre
syndrome in the U.S. but is more common in Asia
• Acute Motor Axonal Neuropathy (AMAN)and Acute Motor-
Sensory Axonal Neuropathy (AMSAN) ---- are less common
in the U.S. but more frequent in China, Japan and Mexico

Warning signals –
must consult Doctor
• Tingling that started in the feet or toes and is now moving
up the body
• Tingling or weakness that's spreading rapidly
• Difficulty catching your breath or shortness of breath when
lying flat
• Choking on saliva

Why Hospitalization ?
• Guillain-Barre syndrome is a serious condition that requires
immediate hospitalization because it can worsen rapidly.
• The sooner appropriate treatment is started, the better the
chance of a good outcome.

GB Syndrome Pathology & Effect
• In Guillain-Barre syndrome, own  immune system — which usually
attacks only invading organisms — attacks on the nerves
• In AIDP, the most common form of Guillain-Barre syndrome in the
U.S., the nerves' protective covering (myelin sheath) is damaged.
• This  damage prevents nerves from transmitting signals to your
brain, causing weakness, numbness or paralysis

GB Syndrome Pathology

GB Syndrome Cause & Symptoms

GB Syndrome –Risk factors
• Guillain-Barre syndrome can affect all age groups.
• But greater risk is seen in --
• Older adult
• Guillain-Barre syndrome may be triggered by --
• Infection with campylo bacter, a type of bacteria often found in
undercooked poultry ,Influenza virus ,Epstein-Barr virus, HIV,
Mycoplasma pneumonia , Surgery Hodgkin's lymphoma , Rarely,
influenza vaccinations or childhood vaccinations

ARS Campylobacter jejuni Triger GB

GB Syndrome
Risk factors & symptoms

GB Syndrome – Complications
• Breathing difficulties --- The weakness or paralysis can spread to the
muscles that control your breathing, a potentially fatal complication.
• These patients will need Ventilator
• Blood pressure fluctuations and irregular heart rhythms (cardiac
arrhythmias) are common side effects
• Up to half of people with Guillain-Barre syndrome experience severe
nerve pain, which may be eased with medication.
• Bowel and bladder function problems -- Sluggish bowel function and
urine retention may result from Guillain-Barre syndrome.

Hand Held Spirometry in GB

• People who are immobile due to Guillain-Barre syndrome are at risk
of developing blood clots.
• Until patient is  able to walk independently, taking blood thinners and
wearing support stockings are  recommended.
• Due to immobility puts a  risk of developing bedsores (pressure
sores). Frequent repositioning may help avoid this problem.
• Up to 5 percent of people with Guillain-Barre syndrome experience a
relapse.

• Severe, early symptoms of Guillain-Barre syndrome
significantly increase the risk of serious long-term
complications.
• Rarely, death may occur from complications such as
respiratory distress syndrome and heart attack.

GB Syndrome – Diagnosis
• Diagnosis is mostly done with a medical history and thorough
physical examination.
• Lumbar puncture – CSF is tested for a change , that
commonly occurs in people who have Guillain-Barre
syndrome.
• Electromyography -- The electrodes measure nerve activity
in the muscles.
• Nerve conduction studies -- to measure the speed of nerve
signals.

GB Syndrome – Treatment
• There's no cure for Guillain-Barre syndrome.
• But two types of treatments can speed recovery and reduce the
severity of the illness:
• i) Plasma exchange (plasmapheresis) --
• Plasma is removed and separated from blood cells. The blood cells
are then put back into the body, which manufactures more plasma to
make up for what was removed. Plasmapheresis works  by ridding
plasma of certain antibodies which  contribute to the immune
system's attack on the peripheral nerves.

• ii) Immunoglobulin therapy ---
• Immunoglobulin containing healthy antibodies from blood
donors is given intravenously.
• High doses of immunoglobulin can block the damaging
antibodies which contribute to Guillain-Barre syndrome.

• Both treatments , plasmapheresis & Immunoglobulin
therapy are equally effective.
• Mixing them or administering one after the other is no more
effective than using either method alone.
• Medication is given to relieve pain ( which can be severe ) ,
• Medicines are given to prevent blood clots, which can
develop while the patient is immobile

GB Syndrome – Physiotherapy
• People with Guillain-Barre syndrome need Physiotherapy during
recovery
• Movement of your arms and legs ,before recovery  help to keep
muscles flexible and strong
• Physical therapy during recovery  help to  regain strength and proper
movement
• Training with adaptive devices, such as a wheelchair or braces give
mobility and self-care skills
• Exercise therapy help  to cope with fatigue

GB Syndrome –Recovery
• Some people can take months and even years to recover
• After the first signs and symptoms, the condition tends to
progressively worsen for about two weeks
• Symptoms reach a plateau within four weeks
• Recovery usually lasts for six to 12 months
• But for some people it could take as long as three years

GB Syndrome – Recovery
• About 80 percent can walk independently six months after diagnosis
• About 60 percent fully recover motor strength one year after
diagnosis
• About 5 to 10 percent have very delayed and incomplete recovery
• Children, who rarely develop Guillain-Barre syndrome, generally
recover more completely than adults.

Support to patients of GB Syndrome
• A diagnosis of Guillain-Barre syndrome can be emotional shock
• Although most people eventually recover fully, the condition is generally
painful and requires hospitalization and months of rehabilitation.
• The patient has to adjust to limited mobility and fatigue.
• How to manage the stress of recovery from Guillain-Barre syndrome ?
• Patient has to maintain a strong support system of friends and family
• Patient has to contact a support group, for oneself or for family members
• Patient can discuss feelings and concerns with a counsellor


GB Syndrome & Ayurved
• This condition can be correlated with  Sarvanga gata Vata Vyadhi
• Hence, the choice of treatment is santarpaṇa  Chikitsa (nourishing
treatment).
• Nourishing External Treatment – Abyanga with Chandanbala Lakshadi
taila + Shalishastik Pinda sweda  + Karma Basti ( Pittaghna dravya
cooked in milk ) + Internal Medicine with Bruhatvatchintamani Kalpa (
Bruhatvata Chitamani ras + Guduchi Sattva + Rajat bhasma +
Sutshekhar ras )

• Massage with Ashwagandha + Bala + Shatavari Pinda Sweda ( Rice
cooked with milk & decoction of these plants )
• All substances are Santarpana qualities ( Nourishing + Anti Oxidant)
•
• All substances are predominant in Prithvi + Jala Mahabhut
• Action – Balya ,Bruhan,Vata Pacification

• Abhyanga pacifies Vata Dosha .This is Pushtikar + Jara har (
Strengthing + Anti ageing)
• Abhyanga with Chandan Bala Lakshadi Tailam –with Anulom gati (
because Dhatu Kshayajanya Vata prakop – Reduction in Chala guna (
Inability to transmit Nerve Impulse)
• Massage movements with Anuloman Gati
• Shadti Shali helps to open the blocks in nerve conduction

• Basti is an effective treatment for Vata . It  brings
about anulomana of  vata
• In short, Vata  + Pittaghna Treatment  is important in treatment of
autoimmune disorders
• Various  Vata + Pittaghna medicines  can be used according to sāmatā
or nirāmatā in the treatment of autoimmune disorders

Neuralgia
• Presented By –
Prof.Dr.R.R.Deshpande (M.D in
Ayurvdic Medicine & M.D. in
Ayurvedic Physiology)
• www.ayurvedicfriend.com
• Mobile – 922 68 10 630
• professordeshpande@gmail.com

What is Neuralgia ?
• Neuralgia ---
• Neuron = nerve + & algos = pain
• Pain in the distribution of a nerve or nerves

• Intercostal neuralgia,
• Trigeminal neuralgia
• Glossopharyngeal neuralgia

Trigeminal Nerve –
Neuralgia Pain Distribution

Different Types of Neuralgia
• Trigeminal Neuralgia ( TN)
• Atypical Trigeminal Neuralgia ( ATN)
• Occipital Neuralgia
• Glossopharyngeal Neuralgia
• Post Herpetic Neuralgia ( Shingles or Herpers)
• Sciatica pain & Pain due to Brachial Plexopathy

Atypical Trigeminal Neuralgia ( ATN)
• Mostly misdiagnosed
• Symptoms are confused with other problems like
• Migraine
• Dental Problems
• Arthritis of Temporomandibular joint
• Musculo skeletal problems
• Hypochondriasis

Symptoms of ATN
• Pain intensity varies from mild to moderate like crushing or burning
or may be extreme unbearable pain
• Pain type --- Heavy ,aching ,burning
• Patient complains -----
• constant migraine-like headache
• Pain in all three trigeminal nerve branches. (aching teeth, ear aches,
feeling of fullness in sinuses, cheek pain, pain in forehead and
temples, jaw pain, pain around eyes )
• Occasional electric shock-like stabs.

ATN pain
• Normal Function of Trigeminal Nerve -- Responsible for
sensations like Touch ,Temperature & Pressure in the facial
area from jaw to forehead
• Pain in the back of the scalp and neck also
• Pain is worsened  with talking, facial expressions, chewing,
and with cool breeze
• short episodes of excruciating pain ,which lasts  usually for
less than two minutes and  only one side of the face

Causes of ATN
• Vascular compression of the Trigeminal Nerve
• Infections of the teeth or sinuses
• Physical trauma
• Post viral infections

ATN Pain
• Which can Trigger the attack of pain ?
• Simple stimuli—such as eating, talking, making facial
expressions, washing the face or any light touch  or even the
sensation of a cool breeze.
• Attacks may be only one time , clusters of attacks, or
constant episodes.
• There may be muscle spasm  (original term is  TN of "tic
douloureux“ in French ----  tic = Spasm  and douloureux =
painful )

Glossopharyngeal Neuralgia
• Recurring attacks of severe pain in the back of the throat,
near the tonsils, the back of the tongue, and part of the ear
• The pain is due to malfunction of the Glossopharyngeal
Nerve .9th Cranial Nerve and carries sensory information
from the throat, tonsils, and tongue to the brain

• This is a rare disorder,
• Begins after age 40 and occurs more often in men.
• Cause is unknown.
• Glossopharyngeal neuralgia sometimes results from an abnormally
positioned artery which compresses the glossopharyngeal nerve
where it exits the brain stem.
• Rarely, the cause is a tumor in the brain or neck

Occipital Neuralgia
• Known as C2 neuralgia or Arnold's neuralgia
•
• Clinical Features -- chronic pain in the upper neck, back of
the head and behind the eyes.
• Mechanism ---
• There are neuroplastic changes after nerve damage . Then
hyperexcitability in the nervous system causes neuropathic
pain

Occipital Neuralgia

Peripheral Nerve Injury
• Neuronal damage depends on severity of injury
• Seddon’s Classification --- Nerve Injury is explained as follows –
• Neurapraxia
• Axonotmesis
• Neurotmesis
• After trauma to the nerve, a short onset of afferent impulses i.e
injury discharge occurs. This lasts only minutes .This is responsible for
onset of neuropathic pain

Pathology – in Neuronal Damage
• When an  Axon is damaged the segment of the axon distal to
the cut degenerates and is absorbed by  Schwann cells
• The proximal segment fuses, retracts, and swells, forming a
retraction bulb
• The  Synaptic Terminal function is lost because Axoplasmic
transport ceases and no Neurotransmitters  are created.

• The nucleus of the damaged axon undergoes Chromatolysis
preparation for axon regeneration
• Schwann cells in the distal stump of the nerve and Basal lamina help
to stimulate regeneration
• The regenerating axon must connect to the appropriate receptors to
make an effective regeneration
• If proper connections to the appropriate receptors are not
established, aberrant reinnervation occur
• If the regenerating axon is halted by damaged tissue, neurofibrils
may create a mass known as a Neuroma

• If  an injured neuron degenerates or does not regenerate
properly, the neuron loses its function or may not function
properly.
• Neuron trauma is not an separate  event and  cause
degenerative changes in surrounding neurons
• When one or more neurons lose their function or begin to
malfunction, abnormal signals sent to the brain  & called as
painful signals

Central Neuronal Injury
• Neuronal injury ---- in the Central Nervous System leads to --
-- local degeneration of the nerve axon and myelin sheath.
• Axonal debris in the CNS is eliminated by Macrophages
• Trauma to neurons in the CNS also causes a proliferation
of Glial cells that form a Glial scar.

Effects of Glial scar
• Inhibit regeneration of central neural connections
• The damaged nerve terminal begins to swell and glial cells
push the defective terminal away from connections to other
neurons
• Aberrant sprouting of damaged CNS neurons, specifically
sensory neurons ------------- results in Neuralgia

Neuralgia – Diagnosis
• Diagnosis  is based on locating the damaged nerve by identifying
missing sensory or motor function. This may involve tests such as
an EMG test or a nerve conduction test using Microneurography  in
which, the peripheral nerve is stimulated and recordings are taken
from a sensory portion of the nerve
• Neuralgia is a Chronic  pain  which is  difficult to diagnose
• Post herpetic neuralgia is easy  to diagnose because it follows an
obvious cause i.e Herpes Infection with shingles

Post Herpetic Neuralgia

• Proper History taking is important , to find the underlying mechanism
• History of the pain, description of pain, Physical examination,
experimental examination are required
• Physical examinations involve -- testing responses to stimuli such as
touch, temperature, and vibration
• Since pain is subjective --- it is important to use a pain assessment
scale, such as the Visual Analogue Scale or Mc Gill Pain
Questionnaire
• Qualifying the severity of the pain is essential in evaluating the
effectiveness of the treatment.

Classification of Neuralgia
• This classification is based on response to stimuli ---
• Mechanical
• Thermal
• Chemical
• Response to the course of treatment will determine the
mechanism of the pain

•In Multiple Sclerosis nerve damage --- Neuralgia
occurs . In these cases ,family history will help to
diagnose.
• Nothing unusual can be seen in brain scans, so
diagnosis is usually based on the description of the
symptoms and the response to the medication or
procedures

• Laser evoked potentials
• Neuropathic pain is the result of a lesion in Spinothalamic
pathways
• Laser evoked potentials (LEPs) are measurements of
cortical responses using lasers to selectively stimulate
thermonociceptors in the skin
• Lasers can emit a radiant-heat pulse stimulus to selectively
activate A-delta and C free nerve endings.

Laser Evoked Potentials ( LEP)
• By specifically targeting pain and temperature pathways and
measuring cortical responses, doctor can identify even
minute lesions in the spinothalamic pathways
• LEP abnormalities are strongly indicative of neuropathic
pain, while a normal LEP is often more ambiguous
• LEPs have high sensitivity and are very reliable in assessing
damage to both central and peripheral nervous systems

Neuralgia Diagnosis by QST
• Quantitative sensory testing
• Another method for testing the proper function of a nerve is
Quantitative sensory testing (QST)
• QST do the analysis of a patient's response to external
stimuli of controlled intensity.
• A stimulus is applied to the skin of the nerve area being
tested in ascending and descending orders of magnitude

Quantification of Tactile sensation
• Quantification of the tactile stimulus is done by von Frey hairs  or
Semmes-Weinstein monofilaments
• Also, weighted needles can be used --- to measure pin-prick sensation
• Electronic vibrameter is used ---  to measure vibration sensitivity
• Thermal stimuli are quantified by -- using a probe that operates on
the Peltier principle
• Problems with QST --QST abnormalities may be observed in non-
neuralgia pains & then Diagnosis become difficult
• QST is very time consuming and needs  an expensive equipments

• Punch skin biopsy
• Recently, skin biopsy has been used to investigate Mechanoreceptors
and their myelinated afferents.
• Available in only a few research centers
• skin punch biopsy is an easy procedure and is minimally invasive
• Punch skin biopsy is used -- to quantify nerve fibers C fibers and A-
delta nerve fibers through measurement of the density of intra-
epidermal nerve fibers (IENF).
• Loss of IENF has been observed in several cases of neuropathic pain

Neuralgia -- Treatment
• Medicines and surgery.
• Neuralgia is difficult to treat than other types of pain
because -- it does not respond well to normal NSAIDS
• Special medications --- membrane stabilizing drugs or Anti
Depressants
• The antiepileptic medication(AED) pregabalin was
developed specifically for neuralgia and other neuropathic
pain as a successor to gabapentin

Neuralgia -- Treatment
•High doses of anticonvulsant medicines are used to
block nerve firing and tricyclic antidepressants are
generally effective in treating neuralgia
• If medication fails to relieve pain or produces
intolerable side effects, surgical treatment is
recommended

Neuralgia – Surgical
•Neural augmentative surgeries are used --- to
stimulate the affected nerve.
•By stimulating the nerve the brain can be "fooled" into
thinking it is receiving normal input.
•Electrodes are carefully placed in the dorsal root and
subcutaneous nerve stimulation is used to stimulate
the targeted nerve pathway

Neuralgia & Surgery
• Facial numbness may occur  after most of these surgical procedures
• Neuralgia might return despite the procedure's initial success.
• Surgical risks ---  hearing loss, balance problems, infection, and stroke
• Surgeries ---
• Rhizotomy (select nerve fibers are destroyed to block pain)
• Microvascular Decompression  (surgeon moves the vessels which
are compressing the nerve away from it and  soft cushion is placed
between the nerve and the vessels)

Post Herpetic Neuralgia & Ayurved
• Can be compared with Visarpa Upadrav ,Vata + Pitta vruddhi in
Rasa,Rakta,Twak
• Cause – Varicella Zoster Virus
• Diagnostic features – Chronic burning ,stabbing pain after attack of
Herpes Zoster ,Altered sensation
• Diagnosis is based on history & clinical examination

Post Herpetic Neuralgia & Ayurved
• Treatment like – Pitta Jvara
• Guduchydi Kashay + Tiktakam Kashay  each 4 tsf + 8 tsf water BD
• Mahatiktak Ghee or Shatavari Lehya 20 ml BD before food
• Praval Pisti vati  or Chandanadi vati or Chandrakala ras 3 BD
• At night – Avipatikar Churna 10 Gm with water
• External –Shatadhauta Ghruta
• Basti –Guduchyadi Kshir Basti 500 ml ,alternate day – 5 Basti
• Avoid – Pitta prakopak  Diet & Life style

Trigeminal Neuralgia & Ayurved
• Clinical Diagnosis ,MRI head to r/o Tumor etc
• Treatment – Ushna Chikitsa
• Ekangavir rasa 2 + Purna Chadrodaya ras 1 –BD with Maha rasnadi
kadha + Mashabala Atma guptadi Kashay ,each 4 tsf + 8 tsf water –
BD
• OR
• Vasanta kusumakar ras 1 BD – with Ashtavarga Kashay +
Bhadradarvadi Kashay ,each 4 tsf + water 8 tsf –BD
• Abhyanga & Nasya with – Karpasyadi Tail ( Massage in opposite
direction)

• Can be compared with Ananta vat,Kapha vat ,Vata vruddhi in Kapha
site ,Shita vruddhi, Kaphavrut vat
• Causes – Multiple Sclerosis ,Pressure on Trigeminal Nerve from a
swollen vessel or a tumour ,Sometimes Idiopathic
• Diagnostic Features – Very painful, sharp ,electric shock like spasms
,which usually occur for few seconds or minutes, Pain is unilateral
,around eyes ,cheek, lower part of face
• Pain may be stimulated by Touch, sounds, brushing teeth, eating,
drinking, shaving

• Do – Massage in opposite direction ( Pratilom)
• Fomentation with Medicated oils like Dashamul, Eranda, Nirgundi,
Rasna
• Put cotton in ears while travelling ,rather whole face from cold breeze
• Avoid cold shower
• Avoid Vata & Kapha prakopak Diet & Life style

ALS = Amyotrophic lateral sclerosis
Hawkings
Joost van der Westhuizen
sauser player

ALS – Meaning of word
• Amyotrophic comes from the Greek word Amyotrophia
• A = No ,Myo = Muscle ,Trophia = Nourishment
• No muscle Nourishment = Atrophy
• Lateral – Indicates area in spinal cord ,where affected
portions of nerve cells are located
• Sclerosis = Scarring or Hardening ( Due to Degeneration)
• In Commonwealth countries ALS is called as Motor Neurone
Disease ( MND)

Amyotrophic lateral sclerosis
(Lou Gehrig's disease, Charcot disease)
• This disease causes death of neurons which control voluntary
muscles
• This disease is called as Motor Neuron Disease ( Group of conditions)
• ALS is the most common condition
• Clinical features of ALS –
• Stiff muscles ,Muscle Twitching ,
• Muscle weakness increases due to decrease in muscle size
• Gradually difficulty in speaking swallowing & breathing

Normal Neuron

ALS – Nerve & Atrophied Muscle

ALS – causes
• Not known in ---  90% to 95% of cases.
• About 5–10% of cases are inherited from a person's parents
• About half of these genetic cases are due to one of two
specific genes
• The diagnosis is based on  -- a person's signs and symptoms
• But  testing done to rule out other potential causes

ALS – Treatment
• No cure for ALS is known
• A medication called Riluzol may extend life by about two to three
months .
• Non Invasive Ventilation improve quality and length of life
• The disease usually starts around the age of 60 and in inherited cases
around the age of 50
• The average survival from onset to death is two to four years
• About 10% survive longer than 10 years.
• Most die from respiratory failure.

History of ALS
• Descriptions of the disease is found in 1824 ,given by  Charles
Bell
• In 1869, Jean Martin Charcot described  the connection
between the symptoms and the underlying neurological
problems
• In 1874  the term amyotrophic lateral sclerosis was
established
• In 1939 the baseball player  Lou Gehrig was affected by ALS
& then it was known in USA

Motor Neuron Disease Group --
• ALS is Motor neurone disease
• Motor Neurone Disease is a group of Neurological Disorders ,which affect
Motor Neurons ,which control voluntary Muscles
• This Group includes –
• 1) ALS
• 2) Primary Lateral Sclerosis
• 3)Progressive Muscular Atrophy
• 4) Progressive Bulbar Palsy
• 5) Pseudobulbar Palsy
• 6) Spinal Muscular Atrophy

Multiple Sclerosis

MND

Classification of ALS
• How fast the disease progresses  ? -- slow or  fast progressors
• whether it is inherited or sporadic ?
• where it starts ?
• In 70 % cases ----- the limbs are affected first - in this case UMN & LMN are
dying .This is  called "limb onset".
• In 25% of cases ---  muscles in the face, mouth, and throat are affected first
because motor neurons in the part of the brain stem called Medulla
Oblongata or bulb start to die first along with lower motor neurons . This is
called "bulbar onset".
• In  5% of cases ----  muscles in the trunk of the body are affected first.
• In all cases the disease spreads and affects other regions

3 Types of Motor Neurone Disease

Clinical Features of ALS
• Muscle weakness and Atrophy  throughout the body due to
the degeneration of UMN & LMN
• Patient ultimately  lose the ability to initiate and control all
voluntary movement
• Only  bladder and bowel function and the Mucles responsible
for eye movement  are spared until the final stages of the
disorder

Clinical Features of ALS
• Cognitive and/or behavioural dysfunction is present in 50 % patients
• 10 - 15% will show signs of Fronto temporal dementia
• Behavioral features of ALS – Repeating phrases ,Apathy ,Loss of
inhibition
• Cognitive symptoms in ALS --- Language dysfunction ,Executive
dysfunction ,problems with social cognition & verbal memory
• 50% patient suffer from Emotional Lability – So they cry or laugh for
no reason
• There is no relation in dysfunction & disease severity

ALS --- No problem in sensory system
•In ALS –Sensory Nerves & ANS – are not affected
•So patient has normal perception of Touch, Hearing,
vision, Taste, Smell

ALS – Symptoms in the beginning
• Start of the disease is very subtle & may not get noticed
• Earliest symptoms of ALS  ---  muscle weakness and/or
muscle atrophy.
• Other symptoms ---
• Difficulty in  swallowing or breathing
• Cramping, or stiffness of affected muscles
• Muscle weakness affecting an arm or a leg
• Slurred and nasal speech.

ALS – Problems in hands & legs
• First experience awkwardness when walking , running ,
tripping over ,stumbling -------
•
• Often this is marked by walking with a dropped foot ,which
drags gently on the ground
• In arm-onset -- difficulty with tasks requiring manual
dexterity such as buttoning a shirt, writing, or turning a key
in a lock

ALS --- Bulbar onset
• Difficulty speaking clearly or swallowing
• Speech may become slurred, nasal in character, or quieter
• Difficulty in swallowing and loss of tongue mobility.
• Some people experience "respiratory-onset" ALS, in which
the Intercostal Muscles ,which support breathing are
affected first

ALS – Symptoms will progress
• Increasing difficulty in moving, swallowing i.e Dysphagia
,Speaking or forming words i.e Dysarthria
• In UMN involvement --- Tight and stiff muscles i.e Spasticity ,
exaggerated reflexes , including an overactive gag reflex or
positive Babinski’s sign

ALS – Symptoms of LMN
• Muscle weakness and atrophy
• Muscle cramps
• Fleeting twitches of muscles that can be seen under the skin
(fasciculations) although
• Twitching is not a diagnostic symptom ( Rather side effect )
• Twitching would either occur after or accompany weakness
and atrophy

How ALS will progress ?
• The disease eventually spreads to unaffected regions and the
affected regions become more affected
• Most people eventually become ----
• Unable to walk or use their hands and arms
• Lose the ability to speak and swallow food and own saliva
• Lose the ability to cough and to breathe on their own

ALS – Rate of Progression
• Measured  by using an outcome measure called the "ALS
Functional Rating Scale Revised (ALSFRS-R)
• 12-item instrument administered as a clinical interview or
self-reported questionnaire which  produces a score
between 48 (normal function) and 0 (severe disability)
• This is the most commonly used outcome measure in clinical
trials and is used by doctors to record  disease progression

ALS – Rate of Progression
• Slower in people who are younger than 40 at onset, mildly
obese, disorder restricted only to one limb, and with
primarily UMN symptoms
• progression is faster and prognosis poorer in people with
bulbar-onset disorder, respiratory-onset disorder, and
frontotemporal dementia

ALS --- Late stages
• Difficulty in chewing and swallowing  ---  eating very difficult and
increases the risk  of aspirating food into the lungs.
• In later stages Aspiration Pneumonia  can develop
• Maintaining a healthy weight can become a  problem which will
require the insertion of a feeding tube.
• When the diaphragm , Intercostal muscles ,rib cage ,which support
breathing weaken Lung Function Tests show lower
• In respiratory-onset ALS, this may occur before significant limb
weakness is noted
• Most people with ALS die of respiratory failure or Pneumonia

ALS – Prognosis
• Most people with ALS die between 2 and four years after the diagnosis.
• Around half of people with ALS die within 30 months of their symptoms
beginning
• About 20% of people with ALS live between 5 years and 10 years after
symptoms begin
• Guitarist Jason Becker has lived since 1989 with the disorder & physicist
Stephen Hawking has survived for more than 50 years. But these are
exceptions .
• Most people with ALS die in their own home, with stopping of breath in
sleep

ALS – Causes
•Genetics --
• 5–10% of cases are directly inherited from a person's
parents
•Overall, First degree relatives of an individual with
ALS have a 1% risk of developing ALS

ALS – Genetic cause
• A defect on  Chromosome 21  is associated with about 20%
of familial cases of ALS, or about 2% of ALS cases overall
• This mutation is  transmitted in an  Autosomal Dominant
.The most common ALS-causing mutation is a mutant SOD
1 gene, seen in North America; which  is characterized by an
exceptionally rapid progression from onset to death.

ALS & Genetic Mutations
• A number of genetic mutations have been associated with
various types of ALS. The common  known associations are:
• ALS1 -- The most common form of familial ALS –Gene is
SOD1-Autosomal Dominant
• ALS2 -- Juvenile-onset – Gene is  ALS2 –Autosomal recessive

ALS – Causes
•Head injury -- moderate to severe -- Traumatic Brain
Injury is a risk for ALS
•90% of cases – no cause is known for ALS
• Possible associations for which evidence is
inconclusive include -- military service, frequent drug
use, and participation in contact sports

ALS –Cause – Glutamate
• Role of   Glutamate -- in motor neuron degeneration
• Glutamate is one of the Neurotransmitter  in the brain
• Scientists have found, people with ALS have higher levels of
glutamate in blood   with compare to normal healthy
people
• Riluzole --  is currently the only FDA-approved drug for ALS
and targets glutamate transporters.
• It only has a modest effect on survival

Cause of ALS in Athletes
• Relation  between sporadic ALS (specifically in athletes) and a diet
enriched with Branched chain aminoacid   is found
• Branched chain aminoacid is  a common dietary supplement among
athletes
• This type of diet  cause cell hyperexcitability which is usually seen  in
ALS.
• Cell hyper excitability results in increased calcium absorption by the
cell which brings about cell death of neuronal cells, ( because
Neuronal cells have particularly low calcium buffering capabilities)

ALS – Pathology
• ALS  lesion is found in the motor system in areas like fronto temporal
lobes
• Lesions in these areas often show signs of early deficit, which can be
used to predict the loss of Motor function ,which is followed by
spread of ALS
• The pathology  of ALS is present long before any signs or symptoms
are seen
• When  muscular atrophy becomes apparent during ALS, nearly  one
-third of the motor neurons  must be destroyed already

ALS – Risk factors
•Chemical exposure
•Electromagnetic field exposure
•Physical trauma, and electric shock
•Exposure to a number of pesticides like
organochlorine insecticides  Aldrin  ,dieldrin, DDT &
Toxaphene

ALS – Patho physiology
•In ALS ---- There is the death of both UMN & LMN in
the Motor Cortex of the brain, the brain stem, and
the spinal cord.
•Before their destruction, motor neurons develop
protein-rich inclusions in their cell bodies and axons
• This is due to defects in protein degradation

ALS – Investigations
• MRI (axial flair ) shows  increased  T2 signals within the Posterior part
of internal capsule
• No test can provide a definite diagnosis of ALS
• The presence of upper and lower motor neuron signs in a single limb
is strongly suggestive of ALS
• The diagnosis of ALS is primarily based on the symptoms and signs
.But  a series of tests are carried out to rule out other diseases
• Certain EMG findings can support the diagnosis of ALS

ALS – Diagnosis
• Medical History is important
• Periodical Neurological Examination to know ,whether any of
the following symptoms are present & worsening -- ?
• Muscle weakness
• Atrophy of muscles
• Hyperreflexia
• spasticity

ALS – Investigations
• Specific abnormalities in the Test Nerve Conduction Velocity ( NCV) can
suggest Peripheral Neuropathy or Myopathy ,rather than ALS
• MRI is normal in early stage of ALS .Rather then MRI will rule out
conditions like Spinal cord Tumour,Multiple Sclerosis,Prolapsed
Intervertebral Disk ,Syringomyelia ,Cervical Spondylosis
• Routine Haematological & Urine Tests are performed to rule out other
diseases
• If Myopathy is suspected rather than ALS --- Better do Muscle Biopsy

Differential Diagnosis for ALS
• Viral infections diseases like HIV ,Human T cell Leukaemia virus ( HTLV)
,Lyme disease ,Syphilis ,Tick borne Encephalitis
• Neurological diseases like –
• Multiple Sclerosis
• Post Polio Syndrome
• Multifocal Motor Neuropathy
• CIDP
• Spinal Muscular Atrophy
• Bulbar Muscular Atrophy
• Myasthenia Gravis can mimic ALS (MG is treatable & ALS is not treatable)

Myasthenia Gravis – Ptosis

ALS – Treatment
•To give symptomatic relief & extend life expectancy
•Supportive care by Multidisciplinary Team of Doctors -
-- to provide maximum mobility & comfort to patient

ALS --- Treatment
•Medicines – Riluzole – Can improve survival for
several months
•Graeater benefit for ALS with Bulbar onset
•This drug can not reverse damage already happened
with Motor Neuron
•LFT should be done periodically ,while giving this drug

ALS --- Treatment
• Baclofen & Diazepam – to control spasticity of ALS
• Amitryptaline – to reduce trouble swallowing saliva ,in ALS
patients
• Medicines are given for – To reduce fatigue, muscle cramps
,control spasticity, reduce excess saliva ,reduce pain,
depression, sleep disturbances, dysphagia, constipation

ALS & Ventilator
• In ALS when respiratory muscles weaken & breathlessness starts
following measures are taken –
• Intermittent Positive pressure Ventilation
• Bilevel positive airway pressure ( BiPAP)
• Biphasic cuirass ventilation ( BCV)
• These devises are used continuously to maintain oxygen & carbon
dioxide levels in blood
• Some times Tracheostomy is needed
• Although ventilation support can ease problems with breathing and
prolong survival, it does not affect the progression of ALS

ALS & Physiotherapy
• Physiotherapy is very useful role in rehabilitation for individuals
with ALS
• Physical & Occupational Therapists can help as follows –
• Delaying loss of strength
• Maintaining endurance
• Limiting pain
• Preventing complications
• Promoting functional independence

ALS & Physiotherapy
• Gentle, low-impact aerobic exercises like   performing activities of
daily living, walking, swimming, and stationary bicycling  -- strengthen
unaffected muscles, improve cardiovascular health, and help patients
to  fight fatigue and depression
• Range of motion and stretching exercises can help – To  prevent
painful spasticity and shortening (contracture) of muscles.
• Physical and occupational therapists  recommend exercises that
provide these benefits without overworking muscles

ALS & Speech problem
• ALS patients develop speaking problems .Then what to do ?
• Speech Language Pathologist can help
• They teach patient of ALS ,some adaptive strategies such as
techniques to help them speak louder and more clearly.
• As ALS progresses, speech-language pathologists can recommend
the use augmentive & alternative communication like such as voice
amplifiers, speech-generating devices (or voice output
communication devices) and/or low tech communication techniques
such as head mounted laser pointers ,Alphabe boards ,Yes or No
signals

ALS & Nutrition
• Care taker of ALS patient can consult with dieticians about ----
• How to plan and prepare numerous small meals throughout the day
that can provide enough calories, fiber and fluid ?
• How to avoid foods that are difficult to swallow?
• Care taker can use suction devices to remove excess fluids or saliva
and prevent choking
• Occupational therapists can give  recommendations for adaptive
equipment to ease the physical task of self-feeding
• Speech-language pathologists give  food choice recommendations
which  are more conducive to patient’s  unique deficits and abilities.

ALS & Nutrition
• When patient  with ALS can not  get enough nourishment
from eating, doctors may advise Ryle’s tube feeding of liquid
diet
• The use of Ryle’s  tube  reduces the risk of choking and
Aspiration Pneumonia
• The tube is not painful and does not prevent patient  from
eating food orally if they wish

ALS – Last stage care
• Family members relatives, Social workers ,Nursing staff all
can work together for managing the medical, emotional, and
financial challenges of coping, particularly during the final
stages of the disease
• Social workers provide support like assistance in obtaining
financial aid, arranging durable Power of attorney , preparing
a living will and finding support groups for patients and
caregivers

ALS & Home Nursing staff
• Home nurses provide medical care & also teach family
members about maintaining respirators, giving feedings, and
moving patient in bed to avoid painful bed sores and
contractures
• Nurses work as per instructions of physicians to give proper
medication, pain control and other care affecting the
quality of life

Motor Neuron Disease & Ayurved
• Can be compared with – Sarvang Vat ,Kshaya ,Vat –
Aavaran
• Causes --- Genetic ,Environmental ,Viral ,Toxic
• Clinical Features – Progressive muscle weakness, wasting,
Fasciculations, slurred speech, Impaired gait ,Dysphagia,
Facial weakness, Muscle cramps ,Difficult breathing,
Salivation,Emotional Lability

• Investigations – EMG, Nerve Conduction study
• Treatment – Vata hara ,Avaran vat Chikitsa,Dhatu Kshaya chikitsa,
Majja gata vat Chikitsa, Medhya Chikitsa, Rasayan Chikitsa
• In the beginning – Rasonadi vati 2 with Gandharva Hastyadi Kashayam
4 tsf with equal quantity water BD --- 2 months
• Then – Tapyadi Loha vati 3 tab + Ashvagandharishta + Balarishta
,each 4 tsf + 8 tsf water BD --- 4 months
• Followed by Bruhatvatchintamani 1 tab with 2 tsf Chavanprash in
the morning ----- 6 months

• External – Abhyanga with Dhanvantarum Kuzambu ,followed by
Steam fomentation with Nirgundi
• Mustadi Yapan Basti
• Shali shastik Pinda sweda
• Shirodhara with Kshir Bala oil
• Natsya with Panchendriya Vardhan tail

Prof.Dr.R.R.Deshpande
• Sharing of Knowledge
• FOR
• Propagating Ayurved

Guillain barre syndrome ,neuralgia,als

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