MYASTHENIA GRAVIS

By: Winda Diah Nugraheni

Lecturer Adviser: Dr. Donny H. Hamid SpS

Definition
Myasthenia gravis is a disorder of neuromuscular
transmission, characterised by weakness and fatiguing of
some or all muscle groups. Weakness worsening on
sustained repeated exercise and relieved by rest. This
condition is a consequence of an autoimmune destruction
of the post synaptic reseptor for acetylcholine.

Anatomy of
Neuromuscular Transmission

Physiology of Neuromuscular
Transmission

Epidemiology
The prevalence of autoimmune MG is estimated at 1
case in 10.00020.000 people
Women are affected more often in the second and third
decades of life, and men more often in the fifth and sixth
decades.
The peak age of onset is between 20 and 30 years in
women and between 50 and 60 years in men.
Associated autoimmune diseases are present in
approximately 5% of patients, and comorbid thyroid
disease occurs in more than 10%.

Pathogenesis

Pathogenesis

Clinical Manifestations

Classifications

I. Ocular myasthenia (15 to 20 percent)

II. A. Mild generalized myasthenia with slow progression, no crises, drugresponsive (30 percent)
II. B. Moderately severe generalized myasthenia; severe skeletal and bulbar
involvement but no crises, drug response less than satisfactory (25
percent)
III. Acute fulminant myasthenia; rapid progression of severe symptoms
with respiratory crises and poor drug response, high incidence of
thymoma, high mortality (15 percent).
IV. Late severe myasthenia; symptoms same as III, but resulting from
steady progression over 2 years from class I to class II (10 percent).

Diagnosis
Physical Examination
1. Cranial nerve signs and symptoms:
Ocular involvement produces ptosis
and muscle paresis.
Weakness of jaw muscles allows the
mouth to hang open
Weakness of facial muscles results in
expressionless appearance
On smiling, buccinator weakness
produces a characteristics smile
(myasthenia snarl)

2. Bulbar involvement may

result in:
Dysarthric, dysphonic
speech and dysphagia
Nasal regurgitation of fluids
or nasal quality to speech

3. The demonstration of
fatiguing :
- Simpson test
- Cogans lid twitch sign
- Blowing out cheeks
against pressure
- A counting test 100:
Counting as far as
possible in one breathe

Simpson test

Cogans lid twitch sign

4. Limb and trunk signs and symptoms

Weakness of neck muscles may result in lolling of
the head. Proximal limb muscles are preferentially
affected. Fatigue may be demonstrated by
movement against a constant resistance.
Limb reflexes are often hyperactive and fatigue on
repeated testing.
Muscle wasting occurs in 15% of cases.

Diagnostic Studies

Differential Diagnosis
For generalized MG the differential diagnosis includes
Lambert-Eaton myasthenic syndrome, botulism, and
myopathy
For ocular myasthenia alternative diagnoses include
progressive external ophthalmoplegia, thyroid disease,
and oculopharyngeal muscular dystrophy
For bulbar predominant myasthenia gravis Motor
neuron disease, brainstem stroke, diphtheria, and
botulism

Treatment

The treatment of this disease involves the careful use

of two groups of drug-anticholinesterases and
immunosuppresants including corticosteroids and in
special acute circumstances, plasma exchange and
intravenous immunoglobulin, an elective thymectomy is
appropriate in many patients as discussed below

1. Anticholinesterase Drugs
The two drugs that give the best results in
ameliorating myasthenia weakness are
neostigmine and pyridostigmine
The usual dose of pyridostigmine is 30 to 90
mg given every 6 h (typically a 60 mg pill is
tried first)
The oral dose of neostigmine ranges from 7.5
to 45mg given every 2 to 6 h

2. Corticosteroids
The usual form of corticosteroid therapy is
prednisone (or corresponding doses of
prednisone), beginning with 15 to 20 mg/d and
increasing the dose gradually until a statisfactory
clinical response is obtained or until a daily dose
of 50 to 60 mg is reached.

3. Plasma Exchange and Intervenous Immune Globuline

Striking temporary remissins ( 2 to 8 weeks) may be

obtained by the use of plasma exchange. This form
of treatment may be life saving during a myasthenic
crisis.