Muscular Dystrophy & Myasthenia Gravis - PPT By Prof. Dr. R. R. Deshpande • This PPT is based on the – Syllabus of CCIM ( 2014) for4th BAMS – Kayachikitsa subject – Paper 2 Part A Point No 5 . • Contents of PPT are – Causes ,Symptoms & Treatment of Muscular Dystrophy & Myasthenia Gravis • Visit – www.ayurvedicfriend.com • Phone – 922 68 10 630

1. Muscular Dystrophy &
Myasthenia Gravis &
• Presented By –
Prof.Dr.R.R.Deshpande
(M.D in Ayurvdic
Medicine & M.D. in
Ayurvedic Physiology)
• www.ayurvedicfriend.c
om
• Mobile – 922 68 10 630
• professordeshpande@g
mail.com
12/19/2016 1Prof.Dr.R.R.Deshpande

2. 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
12/19/2016 2Prof.Dr.R.R.Deshpande

3. 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
12/19/2016 3Prof.Dr.R.R.Deshpande

4. Muscular Dystrophy
12/19/2016 4Prof.Dr.R.R.Deshpande

5. Muscular Dystrophy
• Several inherited disorders are included
• Characterized by progressive degeneration of
groups of muscles
• But no involvement of the nervous system
12/19/2016 5Prof.Dr.R.R.Deshpande

6. 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)
12/19/2016 6Prof.Dr.R.R.Deshpande

7. Types of Muscular Dystrophy
12/19/2016 7Prof.Dr.R.R.Deshpande

8. Duchenne type or Pseudo
hypertrophic type
12/19/2016 8Prof.Dr.R.R.Deshpande

9. 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
12/19/2016 9Prof.Dr.R.R.Deshpande

10. Muscular Dystrophy -Inheritance
12/19/2016 10Prof.Dr.R.R.Deshpande

11. 1. Duchenne type or Pseudo hypertrophic type
• 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 .
12/19/2016 11Prof.Dr.R.R.Deshpande

12. 1. Duchenne type or Pseudo hypertrophic type
• 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
12/19/2016 12Prof.Dr.R.R.Deshpande

13. 1. Duchenne type or Pseudo hypertrophic type
• In due course of time, all muscles, showing
pseudoatrophy ,also become atrophic
• Then contractures are seen
12/19/2016 13Prof.Dr.R.R.Deshpande

14. Symptoms of Muscular Dystrophy
12/19/2016 14Prof.Dr.R.R.Deshpande

15. 1. Duchenne type or Pseudo hypertrophic type
• 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
12/19/2016 15Prof.Dr.R.R.Deshpande

16. Gait in Muscular Dystrophy
12/19/2016 16Prof.Dr.R.R.Deshpande

17. 1. Duchenne type or Pseudo hypertrophic type
• 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
12/19/2016 17Prof.Dr.R.R.Deshpande

18. Muscular Dystrophy –Gait
12/19/2016 18Prof.Dr.R.R.Deshpande

19. 1. Duchenne type or Pseudo hypertrophic type
• 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
12/19/2016 19Prof.Dr.R.R.Deshpande

20. MD – Gower’s sign
12/19/2016 20Prof.Dr.R.R.Deshpande

21. 1. Duchenne type or Pseudo hypertrophic type
• 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
12/19/2016 21Prof.Dr.R.R.Deshpande

22. Mnemonics for Muscular Dystrophy
12/19/2016 22Prof.Dr.R.R.Deshpande

23. 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 .
12/19/2016 23Prof.Dr.R.R.Deshpande

24. Differential Diagnosis –
of Muscular Dystrophy
• Based on –
• Age at onset
• Distribution of affected muscles
• pattern of inheritance
12/19/2016 24Prof.Dr.R.R.Deshpande

25. Investigations - of Muscular Dystrophy
• Diagnosis can be confirmed by EMG & muscle
Biopsy
• In Duchenne muscular dystrophy, Creatine
Kinase is markedly elevated
12/19/2016 25Prof.Dr.R.R.Deshpande

26. 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
12/19/2016 26Prof.Dr.R.R.Deshpande

27. Dystrophia myotonica
• 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
12/19/2016 27Prof.Dr.R.R.Deshpande

28. Dystrophia myotonica
• 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

29. 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
12/19/2016 29Prof.Dr.R.R.Deshpande

30. Treatment for Muscular Dystrophy
12/19/2016 30Prof.Dr.R.R.Deshpande

31. 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
12/19/2016 31Prof.Dr.R.R.Deshpande

32. 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
12/19/2016 32Prof.Dr.R.R.Deshpande

33. 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

34. 3 .Facioscapulohumeral Form
( Landouzy Dejerine Form)
• Facial weakness ( Myopathic facies )
exist for many years ,before the
development of weakness anywhere
• In advanced cases, Lordosis &Scoliosis
develop
• Disease progress very slowly
12/19/2016 34Prof.Dr.R.R.Deshpande

35. 3 .Facioscapulohumeral Form
( Landouzy Dejerine Form)
• 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
12/19/2016 35Prof.Dr.R.R.Deshpande

36. 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
12/19/2016 36Prof.Dr.R.R.Deshpande

37. 4 Distal Form (Gower’s)
• Disease starts between 30 & 50 years
• Progress of the disease is slow
• Patient usually disabled within a few
decades
12/19/2016 37Prof.Dr.R.R.Deshpande

38. 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)
12/19/2016 38Prof.Dr.R.R.Deshpande

39. Investigations for Muscular Dystrophy
• Specific change is increase in Creatin
Kinase in the blood
• Muscle Biopsy & Electromyography are
Confirmatory
12/19/2016 39Prof.Dr.R.R.Deshpande

40. 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
12/19/2016 40Prof.Dr.R.R.Deshpande

41. Differential Diagnosis
of Muscular Dystrophy
• 2. Residual Poliomyelitis –
• There will be history of fever ,acute
onset asymmetrical ,non progressive
findings are seen
12/19/2016 41Prof.Dr.R.R.Deshpande

42. Differential Diagnosis
of Muscular Dystrophy
• 3. Acquired Myopathies –
• Develop from various endocrinal
disorders carcinomatosis &
dermatomyositis.
• These cases may improve with steroid
therapy
• Also there may be spontaneous recovery
12/19/2016 42Prof.Dr.R.R.Deshpande

43. Treatment of Muscular Dystrophy
• No Effective treatment is available
• General measures to improve health
• Physiotherapy & orthopedic measures
can help to overcome deformities &
contracture
12/19/2016 43Prof.Dr.R.R.Deshpande

44. Myasthenia Gravis
• Abnormal fatigue of striated muscles --
• Due to deficiency or abnormal behaviour
of Ach at myoneural junction
12/19/2016 44Prof.Dr.R.R.Deshpande

45. 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
12/19/2016 45Prof.Dr.R.R.Deshpande

46. 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
12/19/2016 46Prof.Dr.R.R.Deshpande

47. 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
12/19/2016 47Prof.Dr.R.R.Deshpande

48. Pathology in Myasthenia Gravis
12/19/2016 48Prof.Dr.R.R.Deshpande

49. 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
12/19/2016 49Prof.Dr.R.R.Deshpande

50. Symptoms of Myasthenia Gravis
12/19/2016 50Prof.Dr.R.R.Deshpande

51. Myasthenia Gravis - Clinical Features
• Muscles most commonly affected are –
• Extra ocular, bulbar, neck & shoulder
girdle muscles
• Rarely the respiratory & pelvic girdle
muscles are affected
12/19/2016 51Prof.Dr.R.R.Deshpande

52. Myasthenia Gravis - Clinical Features
• 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
12/19/2016 52Prof.Dr.R.R.Deshpande

53. Earliest Symptom in Myasthenia Gravis
12/19/2016 53Prof.Dr.R.R.Deshpande

54. Myasthenia Gravis - Clinical Features
• 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
12/19/2016 54Prof.Dr.R.R.Deshpande

55. Symptoms of Myasthenia Gravis
12/19/2016 55Prof.Dr.R.R.Deshpande

56. 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
12/19/2016 56Prof.Dr.R.R.Deshpande

57. 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
12/19/2016 57Prof.Dr.R.R.Deshpande

58. Bedside Test for Myasthenia Gravis
12/19/2016 58Prof.Dr.R.R.Deshpande

59. Test for Myasthenia Gravis
12/19/2016 59Prof.Dr.R.R.Deshpande

60. 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
12/19/2016 60Prof.Dr.R.R.Deshpande

61. Myasthenia Gravis – Treatment
• Pyridostigmine is an alternative which needs
less frequent dosing
• If Intolerable muscarinic side effects are
produced, atropine can be added to block
them.
12/19/2016 61Prof.Dr.R.R.Deshpande

62. Myasthenia Gravis – Treatment
• Corticosteroids afford considerable improvement in
such cases by their immunosuppressant action.
• They inhibit production of NR-antibodies and may
increase synthesis of NRs.
12/19/2016 62Prof.Dr.R.R.Deshpande

63. Myasthenia Gravis – Treatment
• 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
12/19/2016 63Prof.Dr.R.R.Deshpande

64. Treatment for Myasthenia Gravis
12/19/2016 64Prof.Dr.R.R.Deshpande

65. Myasthenia Gravis – Treatment
• 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
12/19/2016 65Prof.Dr.R.R.Deshpande

66. Myasthenia Gravis – Treatment
• 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
12/19/2016 66Prof.Dr.R.R.Deshpande

67. Myasthenia Gravis – Treatment
• 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
12/19/2016 67Prof.Dr.R.R.Deshpande

68. Myasthenia Crisis
12/19/2016 68Prof.Dr.R.R.Deshpande

69. Myasthenia Gravis – Treatment
• 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
12/19/2016 69Prof.Dr.R.R.Deshpande

70. Myasthenia Gravis – Treatment
• 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.
12/19/2016 70Prof.Dr.R.R.Deshpande

71. Myasthenia Gravis & Ayurved
• This is Bala Kshaya ( Loss of strength) +
• Glani +
• Dhatu Kshatajanya Vata Prakop in Mansa
Dhatu
• Cause – Auto Immune Disease
12/19/2016 71Prof.Dr.R.R.Deshpande

72. Myasthenia Gravis & Ayurved
• Important Clinical features –
• Abnormal fatigue & initially weakness of
selected muscles i.e Eye, mouth, throat
• Facial expressions are affected
12/19/2016 72Prof.Dr.R.R.Deshpande

73. Myasthenia Gravis & Ayurved
• 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
12/19/2016 73Prof.Dr.R.R.Deshpande

74. Myasthenia Gravis & Ayurved
• 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
12/19/2016 74Prof.Dr.R.R.Deshpande

75. Myasthenia Gravis & Ayurved
• Nerve conduction study is done
• CT scan can be used to detect abnormal
Thymus gland or the presence of
Thymone
12/19/2016 75Prof.Dr.R.R.Deshpande

76. Myasthenia Gravis – Ayurved Treatment
• Avaranaghna chikitsa
• Dhatu Kshayajanya vata prakop chikitsa
• Mansa Kshaya chikitsa
• Bruhan ,Shramahar chikitsa
• Ushna chikitsa
12/19/2016 76Prof.Dr.R.R.Deshpande

77. Myasthenia Gravis & Ayurved
• 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
12/19/2016 77Prof.Dr.R.R.Deshpande

78. Myasthenia Gravis & Ayurved
• External –
• Abhyana – Dhanvantaram Kuzambu or
Maha masha oil in the morning +
Alternate day Abhyanga with Sahachar
oil
• Basti – Mustadi Rajyapan Basti
• Nasya – Sahacharadi oil
12/19/2016 78Prof.Dr.R.R.Deshpande

79. Almond oil for Myasthenia Gravis
12/19/2016 79Prof.Dr.R.R.Deshpande

80. 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

81. 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.
12/19/2016 81Prof.Dr.R.R.Deshpande

82. Myasthenia Gravis & Ayurved
• Eat – Milk , Almond, Garlic
• Avoid – Vata vruddhi kar Diet & Life style
• Excess dry & cold food
• Avoid -- Hectic Life style
12/19/2016 82Prof.Dr.R.R.Deshpande

83. Prof.Dr.R.R.Deshpande
• Sharing of Knowledge
• FOR
• Propagating Ayurved
12/19/2016 83Prof.Dr.R.R.Deshpande