Download pdf - 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• [email protected]

11/17/2016 1Prof.Dr.R.R.Deshpande

http://www.ayurvedicfriend.com/

mailto:[email protected]

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 breathing11/17/2016 9Prof.Dr.R.R.Deshpande

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 saliva11/17/2016 13Prof.Dr.R.R.Deshpande

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• [email protected]


http://www.ayurvedicfriend.com/

mailto:[email protected]

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 neck11/17/2016 47Prof.Dr.R.R.Deshpande



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. 11/17/2016 82Prof.Dr.R.R.Deshpande

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 regions11/17/2016 87Prof.Dr.R.R.Deshpande

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. 11/17/2016 92Prof.Dr.R.R.Deshpande

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 survival11/17/2016 106Prof.Dr.R.R.Deshpande

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
