muscular dystrophy & myasthenia gravis ppt

Muscular Dystrophy & Myasthenia Gravis &

• 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]

12/19/2016 1Prof.Dr.R.R.Deshpande

http://www.ayurvedicfriend.com/

http://www.ayurvedicfriend.com/

mailto:[email protected]

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


Myopathies

• Group of conditions having primary affections of muscles

• Characterized by –• Involvement of proximal muscle groups, Ultimate wasting, Absence of fibrillation bilateral involvement ,hereditary transmission, no involvement of nervous system ,downhill course, absence of remission


Muscular Dystrophy


Muscular Dystrophy

• Several inherited disorders are included

• Characterized by progressive degeneration of groups of muscles

• But no involvement of the nervous system


Muscular Dystrophy-Clinical Types

• 1. Duchenne type or Pseudo hypertrophic type

• 2.Juvenile Scapulohumeral type of ERB

• 3 .Facioscapulohumeral Form ( Landouzy Dejerine Form)

• 4 Distal Form (Gower’s)


Types of Muscular Dystrophy


Duchenne type or Pseudo hypertrophic type


1. Duchenne type or Pseudo hypertrophic type

• Only in males • Transmitted by a sex linked recessive gene • Females carry the disease through gene but males are victims

• Disease starts in the first few years of life • First pelvic girdle muscles are affected & lastly there is involvement of shoulder girdle muscles


Muscular Dystrophy -Inheritance



• In many cases ,to start with Pseudo hypertrophy is seen in Gluteal region, Calf, Quadriceps, Deltoids, Infra spinatus muscles

• Muscles look bulkier from outside but Motor power ,tone are less & jerks are dull .



• On voluntary contraction, the affected muscles do not become hard & globular .The firm fleshy feel is lost

• At the same time of this Pseudohypertrophy some muscles also show atrophy. For example, Pectoralis major ,becomes so thin like parchment paper



• In due course of time, all muscles, showing pseudoatrophy ,also become atrophic

• Then contractures are seen


Symptoms of Muscular Dystrophy



• Due to weakness of the affected muscle group, patient cannot walk in a normal manner

• He stands on a broad base, with a lordosis of lumbar spine .Whole chest is thrown in a forward plane .During stepping patient swings this way & that ,simulating a duck


Gait in Muscular Dystrophy



• In the early stages, instead of this gait, patient may show, just a limping gait

• In the terminal stages ,when all the muscles are wasted ,patient can not stand erect .In an attempt to do ,this patient crawls on the ground .This is called as Frog like gait


Muscular Dystrophy –Gait



• When lying down position, when patient tries to get up ,he rolls over body

• Then supports on the ground ,then on his feet & knees & then stands up ,as if he is climbing up his own body

• • This is called as Rising Test or Gower’s sign

This is characteristic feature of this disease


MD – Gower’s sign



• Due to weakness of shoulder girdle muscle, patient cannot hang by the support of his axillae & falls down

• Death occurs usually in the second decade from inanition or respiratory infection

• Histologically, the affected muscles show ,few islands of muscle fibrils in a vast ocean of fibrofatty tissue


Mnemonics for Muscular Dystrophy


Clinical features of Muscular Dystrophy

• symmetrical wasting & weakness • No fasciculation • No sensory loss

• Tendon reflexes are preserved ( Except in Dystrophia Myotonica)

• Tendon reflexes are preserved until a late stage .


Differential Diagnosis – of Muscular Dystrophy

• Based on –

• Age at onset

• Distribution of affected muscles

• pattern of inheritance


Investigations - of Muscular Dystrophy

• Diagnosis can be confirmed by EMG & muscle Biopsy

• In Duchenne muscular dystrophy, Creatine Kinase is markedly elevated


Dystrophia myotonica

• Dystrophia myotonica is diagnosed clinically by the distribution of muscle weakness & other features like myotonia (slow relaxation of muscle) ,cataract, Ptosis, frontal baldness Gonadal atrophy

• This is caused by expansion of a trinucleotide repeat on chromosome 19 .Diagnosis is possible by measuring the number of repeats



• The genetic defects of Duchenne dystrophy & facioscapulohumeral dystrophy -- can be mapped to chromosomes Xp 21 & 4q 35, respectively

• DNA analysis may help in early diagnosis

• Pre natal testing in Dystrophia myotonica can also help



• Treatment –

• No specific Therapy

• Physiotherapy & Occupational Therapy help the patient to cope with disability

• Genetic counseling is important 12/19/2016 28Prof.Dr.R.R.Deshpande

Prognosis of Duchenne Dystrophy

• Most patients with Duchenne Dystrophy die within 10 years of diagnosis

• Life span of Limb girdle & facioscapulohumeral dystrophies is normal

• Premature death occurs due to respiratory or cardiac failure in early middle age occurs in Dystrophia myotonica


Treatment for Muscular Dystrophy


2.Juvenile Scapulohumeral type of ERB

• This disease affects both genders

• It is inherited as an Autosomal recessive gene • This is seen in 2nd & 3rd decade of life • But may occur at any age ,after first 5 years


2.Juvenile Scapulohumeral type of ERB

• Symptoms may begin in upper arms, in the shoulder girdle or in the pelvic girdle muscles • Most patients are disabled within 20

years .Do not survive up to the middle age


3 .Facioscapulohumeral Form ( Landouzy Dejerine Form)

• This affects both genders • Inherited as an Autosomal dominant

gene • The muscles involved earliest are of face • Then the disease progresses centrifugally to shoulder girdle & upper arms muscles• Disablement occurs during puberty 12/19/2016 33Prof.Dr.R.R.Deshpande


• Facial weakness ( Myopathic facies ) exist for many years ,before the development of weakness anywhere

• In advanced cases, Lordosis &Scoliosis develop• Disease progress very slowly



• Most patients remain ambulatory until middle or later life • Contracture & Pseudo hypertrophy is absent • Sometimes Juvenile Scapulohumeral type & Facioscapulohumeral Form are together classified as Limb Girdle type


4 Distal Form (Gower’s)

• This is comparatively rare• Distal muscles of the extremities are

first affected • Then the disease proceeds centripetally

• Common in Scandinavia


4 Distal Form (Gower’s)

• Disease starts between 30 & 50 years

• Progress of the disease is slow

• Patient usually disabled within a few decades


Investigations for Muscular Dystrophy

• Aminoaciduria ,Pentosuria ,Creatinuria are seen .But not specific

• Aldolase , Phosphohexoisomerase Transminases are increased in blood ( But doubtful value in the diagnosis)


Investigations for Muscular Dystrophy

• Specific change is increase in Creatin Kinase in the blood

• Muscle Biopsy & Electromyography are Confirmatory


Differential Diagnosis of Muscular Dystrophy

• 1.Chronic Motor neurone disease –

• In this condition fibrillation, muscular wasting ,non symmetrical findings negative family history ,evidences of pyramidal lesion are present



• 2. Residual Poliomyelitis –

• There will be history of fever ,acute onset asymmetrical ,non progressive findings are seen



• 3. Acquired Myopathies – • Develop from various endocrinal disorders carcinomatosis & dermatomyositis.• These cases may improve with steroid therapy• Also there may be spontaneous recovery


Treatment of Muscular Dystrophy

• No Effective treatment is available

• General measures to improve health

• Physiotherapy & orthopedic measures can help to overcome deformities & contracture


Myasthenia Gravis

• Abnormal fatigue of striated muscles --

• Due to deficiency or abnormal behaviour of Ach at myoneural junction


Myasthenia Gravis -Etiology

• Common at age 20 to 30 years .But occur exceptionally in new born .Females are more prone

• This condition may be associated with – Thyrotoxicosis, Hashimoto’s disease, Rheumatoid arthritis, Carcinoma


Myasthenia Gravis - Etiology

• Exact cause of the disease not known

• In 15 % vases ,Thymic Tumour is present

• The behaviour of Acetyl choline at the myoneural junction is seen abnormal


Myasthenia Gravis - Etiology

• Auto immune disorder • Affecting 1 in 10,000 population .• Due to development of antibodies directed to

nicotine receptors ( NR) at the muscle endplate • Reduction in number of free N-M cholinoreceptors to 1/3rd of normal

• Structural damage to neuromuscular junction• Weakness & easy fatigability on repeated activity • Recovery after rest


Pathology in Myasthenia Gravis


Myasthenia Gravis - Clinical Features

• Characteristic symptom –

• Abnormal fatigue of skeletal muscles • They become rapidly tired • This tiredness is prominent at the end of the day


Symptoms of Myasthenia Gravis



• Muscles most commonly affected are – • Extra ocular, bulbar, neck & shoulder

girdle muscles

• Rarely the respiratory & pelvic girdle muscles are affected



• Earliest symptom is Ptosis & diplopia which may be intermittent • There may be tiredness during chewing, swallowing, speaking & during movement of limbs • All symptoms may wax and wane from time to time


Earliest Symptom in Myasthenia Gravis



• Relapses are common after emotional upsets severe muscular exercises, pregnancy or in generalized infections • Paralysis of respiratory muscles can cause asphyxia & death • In chronic cases there may be atrophy of

involved muscles


Symptoms of Myasthenia Gravis


Myasthenia Gravis - Diagnosis

• Ameliorative test –

• Edrophonium 2–10 mg injected slowly i.v. improves muscle strength only in myasthenia gravis and not in other muscular dystrophies


Myasthenia Gravis - Diagnosis

• Ask the patient to look upwards continuously when gradually the upper eyelids will droop down

• Injection Prostigmin 2.5 mgm IM or Edropheonium hydrochloride 10 mgm IV can give quick relief to this fatigue


Bedside Test for Myasthenia Gravis


Test for Myasthenia Gravis


Myasthenia Gravis – Treatment

• Neostigmine & it’s congeners improve muscle contraction, by allowing Ach released from prejunctional endings to accumulate & act on receptors over a larger area & by directly depolarizing the end plate

• Treatment – Neostigmine 15 mg orally 6 hourly .Dose & frequency is then adjusted according to response.

• However, the dosage requirement may fluctuate from time to time



• Pyridostigmine is an alternative which needs less frequent dosing

• If Intolerable muscarinic side effects are produced, atropine can be added to block them.



• Corticosteroids afford considerable improvement in such cases by their immunosuppressant action.

• They inhibit production of NR-antibodies and may increase synthesis of NRs.



• Prednisolone 30–60 mg/day induces remission in about 80% of the advanced cases; 10 mg daily or on alternate days can be used for maintenance therapy.

• Other immunosuppressants have also been used with benefit in advanced cases.

• Both azathioprine and cyclosporine also inhibit NR-antibody synthesis by affecting T-cells


Treatment for Myasthenia Gravis



• Advice – Avoid fatigue ,Excess work, Exercises

• Tab Prostigmin orally 15 to 45 mgm every 2 to 4 hours • In severe cases – Inj Prostigmin 2.5 mgm IM may require



• To minimize the effects this drug Inj Atropine 0.6 mgm can be given ,20 min before or --• Propantheline bromide 15 mgm BD is given • Long acting drugs like Pyridostigmin ,may be given



• Very large doses of Prostigmin may cause ‘Cholinergic crisis’• The symptoms are sweating, pallor, fasciculations, small pupil ,salivation

• In this condition Inj Atropine must be given immediately


Myasthenia Crisis



• In respiratory distress ,Artificial Respiration should be given

• Surgical Thymectomy ,can give permanent cure ( specially in young females & when disease is not more than 6 years duration

• In patients with Thymoma ,x ray radiation is given ,before operation



• Thymectomy produces gradual improvement in majority of cases

• Thymus may contain modified muscle cells with NRs on their surface, which may be the source of the antigen for production of anti-NR antibodies in myasthenic patients.


Myasthenia Gravis & Ayurved

• This is Bala Kshaya ( Loss of strength) + • Glani + • Dhatu Kshatajanya Vata Prakop in Mansa Dhatu

• Cause – Auto Immune Disease



• Important Clinical features –

• Abnormal fatigue & initially weakness of selected muscles i.e Eye, mouth, throat

• Facial expressions are affected



• Muscle weakness worsens with activity & improves with rest • Ptosis, Double vision ,Dysarthria,

Dysphagia• Muscles temporarily paralysed • Breathing becomes difficult ,due to weakness of chest wall muscles



• Investigations – • Ach receptor antibody in blood • Edrophonium test – IV Endrophonium chloride or Tensilon is given .This blocks degradation of Ach & temporarily increase the level of Ach at the neuromuscular junction

• In the patient who have involved the eye muscles Endrophonium chloride will partially relieve weakness



• Nerve conduction study is done

• CT scan can be used to detect abnormal Thymus gland or the presence of Thymone


Myasthenia Gravis – Ayurved Treatment

• Avaranaghna chikitsa • Dhatu Kshayajanya vata prakop chikitsa• Mansa Kshaya chikitsa

• Bruhan ,Shramahar chikitsa• Ushna chikitsa



• Tab Bruhatvat chitamani – 1 in morning with 2 tsf Chavanprash • Tab Bala Ghana vati 3 TDS with

Ashwagandharishta 4 tsf with equal quantity of water • Kshirbala tail 10 ml + Prasaranyadi Tail 10 ml – with warm milk at night



• External – • Abhyana – Dhanvantaram Kuzambu or Maha masha oil in the morning + Alternate day Abhyanga with Sahachar oil • Basti – Mustadi Rajyapan Basti • Nasya – Sahacharadi oil


Almond oil for Myasthenia Gravis


Rajyapan Basti-Indications

• It is recommended in dry allergic cough or in• COPDs like emphysema, bronchiectasis,• chronic fever, gouty arthritis, irritable bowel• syndrome, Knee joint osteoarthritis etc.• It is used in Auto immune conditions like• Ankylosing spondylosis & RA.• Rajyapan Basti increases the strength, it• increases the sexual drive, improves the• digestion and Vital sap.12/19/2016 80Prof.Dr.R.R.Deshpande

Rajyapan Basti-Ingrediants•

• Decoction of Ashwagandha, Shatavari,• Erandamool, Bala in a dose of 240 ml.• Honey - 20 ml.• Rock salt - 5 Gm• Sesame oil - 40 ml• Ghee - 40 ml.• Meat soup – 160 ml & Cow Milk – 160 ml.



• Eat – Milk , Almond, Garlic

• Avoid – Vata vruddhi kar Diet & Life style • Excess dry & cold food

• Avoid -- Hectic Life style


Prof.Dr.R.R.Deshpande

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