WELCOME

Dr MD AMIR HOSSAIN
MBBS FCPS MD (Cardiology)

Associate Professor of Cardiology

BMCH

Myasthenia Gravis
This

condition is characterized by progressive fatigable weakness, particularly of the ocular, neck, facial and bulbar muscles.

Aetiologya and pathology

The disease is most commonly caused by autoantibodies to actylcholine receptors in the post-junctional postmembrane of the neuromuscular junction. These antiboies block neuromuscular transmission and initiate a complementmedated inflammatory response which reduces the number of acetyicholine receptors and demages the end plate.

About 15% patients have a thymoma and the majority of the remainder have thymic follicular hyperplasia. There is an increased incidence of certain HLA haplotypes. Nothing is known about factors which trigger the disease itself, but penicillimine can cause an antibodyantibodymediated myasthenic syndrom which may persist even after drugs, especoally aminoglycosides and ciprofloxacin, may excerbate the neuromuscular blockade and should be avoided in patients with mysthenia.

Clinical feature
 The

disease usually Present between the age of 15 and 50 years, with women affected more then men in the younger age groups and the reverse at older ages. It tends to run a relapsing and remitting course, especially during the early years.  The cardinal symptom is abnormal fatigable weakness of the muscles movement is initially strong but rapidly weakens.

Worsening of symptoms towards the end of the day or following exercise is characteristic. There are no sensory signs or sings of involvement of the central nervous system although weakness of the oculomotor muscles may mimic a central eye movement disorder. The first symptoms are usually intermittent ptosis or diplopia, but weakness of chewing, swallowing, speaking or limp movement also occurs. Any limbs muscle may be affected, most commonly those of the shoulder girdle; the patient unable to undertake task above shoulder level, such a combing the hair, without frequent rests.

Respiratory muscles may be involved, and respiratory failure in a not uncommon cause of death. Aspiratoion may occur if the cough is ineffectual. Sudden weakness from a cholinergic or myasthenic crisis may require ventilatory support.

Investigation:
 The

Tensilon test. Endrophonium Bromide 2 mg I V initially if not 8mg further improvement musle power.  EMG.  Anty-acctylcholine receptor antibody. Anty Positive anti-skeletel muscle antiantibody.  Autoimmune disorder screening eg thyroid.

Management:


Principles of treatment: a) To miximise the activity of remaining acetycholine receptors in N-M junction. Nb) To limit or abolish immunological attack endplates. Anticholineterase drug-pyrodostigmine. 30drug30120mg orally 6 hourly. Increases the action of acetycholine by inhibiting its hydrolysing enzyme acetylcholinestarse.

Immunological Treatment of Myasthenia

Thymectomy: Should be performed as soon as feasible in any antibody-positive antibodypatient under 45 years with symptoms not confined to extracular muscles, unless the disease has been established for more than 7 years.

Plasma exchange
Removing antibody from the blood may produce marked improvement but, as this is usually brief, such therapy is normally resserved for mysthenic crisis or for pre-operative prepreparation. Intervenenous immunoglobulin: An olternative to plasma exchange in the treatment of severe myasthnia.

Corticosteroid treatment
 Improvement

is commonly preceded by marked exacerbation of mysastenic symptoms and treatment should be initiated in hospital. is usually necessary to continue treatment for months or year

 It

Other immunosuppressant treatment

 Treatment

with azathioprine 2.5 mg/kg daily is of value in reducing the doses of steroids necessary and may allow steroids to be withdrawn.  The effect of treatment on clinical disease is often delayed for several months.