Universitas udayana

Faculty of medicine

Myasthenia gravis

Name:Gythrie Karthikason
Nim:1102005209

Myasthenia gravis
Definition
Myasthenia gravis is a chronic autoimmune neuromuscular disease
characterized by varying degrees of weakness of the skeletal muscles of
the body. The hallmark of myasthenia gravis is muscle weakness that
increases during periods of activity and improves after periods of rest.
The muscles that control breathing and neck and limb movements may
also be affected.

Epidemiology
Frequency
United States
Myasthenia gravis is uncommon. Estimated annual incidence is 2 per
1,000,000.
Mortality/Morbidity
Recent advances in treatment and care of critically ill patients have
resulted in marked decrease in the mortality rate. The rate is now 3-4%,
with principal risk factors being age older than 40 years, short history of
severe disease, and thymoma. Previously, the mortality rate was as high
as 30-40%.
Sex
The female-to-male ratio is said classically to be 6:4, but as the
population has aged, the incidence is now equal in males and females.
Age
Myasthenia gravis presents at any age. Female incidence peaks in the
third decade of life, whereas male incidence peaks in the sixth or seventh
decade. Mean age of onset is 28 years in females and 42 years in males.

Causes and Risk Factors for Myasthenia Gravis

MG usually is caused by a malfunction of the immune system. The
causative factor is unknown, but the disorder may have a genetic link.
Causes include a genetic defect, which results in congenital MG, and the
circulation of maternal antibodies through the placenta, which result in
transient neonatal MG.
Acetylcholine (ACh) is a neurotransmitter that is involved in the transfer
of information to muscle tissue. In myasthenia gravis, cells that bind to
other cells to neutralize or destroy them (called antibodies) destroy
acetylcholine receptor sites (AChR) in areas of muscle tissue that receive
nerve impulses (called neuromuscular junctions), preventing nerve
impulses from reaching the muscles. This results in weakness and rapid
fatigue in affected muscles.

Pathophysiology of myasthenia gravis.

Myasthenia gravis (MG) is arguably the best understood autoimmune
disease, and its study has also led to fundamental appreciation of
mechanisms of neuromuscular transmission. MG is caused by antibodies
against the acetylcholine receptor (AChR), which produce a compromise
in the end-plate potential, reducing the safety factor for effective synaptic
transmission. It is clear that AChR antibody destruction of the
postsynaptic surface is dependent on complement activation. A musclespecific kinase has been recently found to be an antigenic target in MG
patients without antibodies against the AChR. Autoantibody production in
MG is a T-cell-dependent process, but how a breakdown in tolerance
occurs is not known. In MG there is an interesting differential involvement
of muscle groups, in particular, the extraocular muscles. This article
reviews normal neuromuscular transmission, mechanisms of the
autoimmune process of MG, and differential susceptibility of eye muscles
to MG.

Symptoms

Onset of the disorder may be sudden

Affect any voluntary muscle
The facial expression and swallowing are most frequently affected
Weakness of muscles that control eye and eyelid movement causing
blurred or double vision (diplopia)
Difficulty in swallowing and slurred speech may be the first signs
Drooping of one or both eyelids (ptosis),
Unstable or waddling gait, weakness in arms, hands, fingers, legs,
and neck,
Shortness of breath
Impaired speech (dysarthria).

Diagnosis
MG can be a difficult diagnosis, as the symptoms can be subtle and hard
to distinguish from both normal variants and other neurological disorders.
A thorough physical examination can reveal easy fatigability, with the
weakness improving after rest and worsening again on repeat of the
exertion testing. Applying ice to weak muscle groups characteristically
leads to improvement in strength of those muscles. Additional tests are
often performed, as mentioned below. Furthermore, a good response to
medication can also be considered a sign of autoimmune pathology.

Treatment
Anticholinesterase agents such as neostigmine and pyridostigmine, which
help improve neuromuscular transmission and increase muscle strength.
mmunosuppressive drugs such as prednisone, cyclosporine, and
azathioprine which help improve muscle strength by suppressing the
production of abnormal antibodies.
Thymectomy
Plasmapheresis a procedure in which abnormal antibodies are removed
from the blood, and high-dose intravenous immune globulin, which
temporarily modifies the immune system and provides the body with
normal antibodies from donated blood.