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Myasthenia gravis

BY

Prof.Dr/ Ahmed
Gamal AZab
Definition

A chronic disease with tendency to remit and


to relapse , characterized by abnormal
muscular fatigability .
Pathogenesis

 Itis due to an autoimmune process in which


antibodies against acetylcholine receptors
cause a disordered conduction in myoneural
junction.

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Pathology

 Characteristicclusters of lymphocytes
( lymphorrhages) in between the muscle
fibres.
 Thymus gland : may show hyperplasia or a
thymic tumor (thymoma) .

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Incidence

 Female : male = 2 : 1
 Mean age of onset : 26y in F, 30y in M.
 Close relationship w/thyrotoxicosis, SLE,
rheumatoid arthritis, biliary cirrhosis, M.S,
and cancer.

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Clinical picture

 Gradual onset , progressive course


w/remissions.
 It has a characteristic descending march
course ,affecting occular muscles followed by
facial, bulbar then skeletal muscles { upper
before lower limb, proximal > distal }.
 No muscle wasting, smooth muscles are not
involved.
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Fluctuations in myasthenia

1- On action : eating, talking, walking.


2- Diurnal variation.
3- Spontaneous remission & relapse.

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Diagnosis
1- Clinical fatigue tests : inducing fatigue by asking the
patient to count to 100 { > dysarthria } or maintain
looking up { > ptosis } .
2- Prostigmine test { 1.5mg prostigmine + 0.5mg
atropine IM }.
3- Tensilon test { edrophonium HCL} : 2mg iv
followed,if no idiosyncrasy ,by 8mg > improve
myasthenia after a minute.
4- EMG : repititive stimulation test >diminished
amplitude after repeated stimulation .
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Diagnosis { cont..}

5- Detection of anti Ach R antibodies : the most


sensitive test.
6- Chest tomogram : to detect thymoma or
thymic hyperplasia .
7- Thyroid function test.
8- Tests for collagen disorders,R/O malignancy
Treatment
1- Anticholine esterases : mestinon , prostigmine :
titrate until adequate response.
2- Prednisolone : Initially 50 mg daily,tapered gradually
to a maintenance of 5 mg/day.
3- Imuran { azathioprine } : 2mg/Kg/day.
4- Plasmapheresis : to get rid of harmful antibodies.
5- Thymectomy : obligatory for thymoma or thymic
hyperplasia.
6- IVIG : 0.4 gm/Kg/day for 5 succesive days.

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Crisis in Myasthenia

It is a severe deterioration in a myasthenic patient .


Types:
1- Myasthenic crisis : exacerbated disease process.
C/P :pupillary dilatation, flushed face, dry mouth, dry
skin,constipation and tachycardia.
2- cholinergic crisis (drug overdosage ) : pupillary
constriction, pallor, excessive salivation, sweating,
diarrhea and bradycardia .
Management of Crisis
- ICU admission .
- Mechanical ventillation.
- Stop all medications.
- Tensilon test for differentiation.
- Plasmapheresis or IVIG for myasthenic crisis.
- Antibiotics, naso-gastric tube feeding.
- Avoid : phenytoins, aminoglycosides, ampicillin,
propranolol, morphia, barbiturates { has a
neuromuscular blocking effect } .
- Later on : re-introduce drugs in a proper dosage.
Neonatal myasthenia

 Seen in infants from a myasthenic mother ,


recovers spontaneously between 1week – 3
months after delivery.
 It is due to antibodies from the mother
serum.

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Lambert-Eaton(myasthenic-myopathic)
syndrome

 Often associated w/ bronchogenic carcinoma


or other malignancies.
 Proximal weakness, wasting with myasthenic
fatigability.
 No Ach R antibodies.
 No significant response to prostigmine , but
respond to guanidine HCL 20mg/Kg/day or
prednisolone + imuran.

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