Location via proxy:   [ UP ]  
[Report a bug]   [Manage cookies]                

Myasthenia Gravis

Download as pptx, pdf, or txt
Download as pptx, pdf, or txt
You are on page 1of 17

KEPANITERAAN ILMU PENYAKIT SARAF RS PGI CIKINI

PERIODE 24 Juni 2013 20 Juli 2013


FAKULTAS KEDOKTERAN UNIVERSITAS KRISTEN INDONESIA
JAKARTA

Dosen Pembimbing :
dr. Hophoptua N. Manurung, Sp.S

Disusun oleh :
Bintari Anindhita (09-58)

Myasthenia gravis (MG) is a neuromuscular disorder
characterized by weakness and fatigability of
skeletal muscles.

The underlying defect..

antibody-mediated
autoimmune attack
acetylcholine receptors
(AChRs) at neuromuscular
junctions

The neuromuscular abnormalities in MG are
caused by an autoimmune response
mediated by specific anti-AChR antibodies.

There are 3 distinct mechanisms:
Rapid endocytosis of the receptors
Blockade of the active site of the achr
Damage to the postsynaptic muscle membrane
The thymus appears to play a role in autoimune
response in MG
The thymus is abnormal in ~75% of patients with MG
In ~65% the thymus is hyperplastic, with the presence of
active germinal centers detected histologically
An additional 10% of patients have thymic tumors
(thymomas).
Muscle-like cells within the thymus (myoid cells),
which bear AChRs on their surface, may serve as a
source of autoantigen and trigger the autoimmune
reaction within the thymus gland.
Number of
available ACHRs
The
postsynaptic
folds are
atrophic
Efficiency of
neuromuscular
transmission
Fail to trigger muscle
action
Potentials
Weakness of muscle
contraction
Normally..
The amount of ACh released per impulse declines
on repeated activity (termed presynaptic
rundown).
In the myasthenic patient..
Efficiency of
neuromuscular
transmission
Normal
rundown
Activation of fewer and
fewer muscle fibers by
successive nerve
impulses
Increasing
weakness, or
myasthenic
fatigue
The cardinal features weakness and
fatigability of muscles.
The weakness increases during repeated use
(fatigue) and may improve following rest or
sleep.
Exacerbations and remissions may occur,
particularly during the first few years after
the onset of the disease.
Ocular muscles affected first in about 40% of patients
and are ultimately involved in about 85% (Ocular MG)
Ptosis
Diplopia
Weakness of facial or oropharyngeal muscles, resulting in:
dysarthria,
dysphagia, and
limitation of facial movements.
Together, oropharyngeal and ocular weakness cause
symptoms in virtually all patients with acquired MG.
In ~85% of patients, the weakness becomes generalized,
affecting the limb muscles as well
often proximal and
may be asymmetric


Generalized
MG
A. Ptosis
B. Attempted gaze to
the right.
Only right eye
abducts incompletely.
C. Demonstrates
proximal
weakness upon
attempt to
raise the arms.
D. Holding the arms
and fingers extended
the extensor muscles
weaken and finger
drop occurs
Crisis occur in patients with oropharyngeal
or respiratory muscle weakness.
Provoked by..
respiratory infection
surgical procedures
emotional stress
systemic illness
Anticholinestrase test
Acetylcholinesterase Inhibitors
First line of therapy
Neostigmine bromide (Pyridostigmine)
Edrophonium chloride (Tensilon)
Immunosuppressive Therapy
Prednisone
Azathioprine


Plasmapheresis
Immunoglobulin Therapy
Thymectomy

Drachman, D. B. (2010). Myasthenia Gravis and Other Diseases of
the Neuromuscular Junction. In S. L. Hauser, & S. A. Josephson,
Harrison's Neurology in Clinical Medicine (2 ed., pp. 559-566). The
Mcgraw-Hill Companies.

Goldenberg, W. D., & Shah, A. K. (2013, February 11). Myasthenia
Gravis. (N. Lorenzo, Editor) Retrieved July 10, 2013, from
Medscape Drugs, Diseases & Procedures:
http://emedicine.medscape.com/article/1171206-overview

Penn, A. S., & Rowland, L. P. (2005). Myasthenia Gravis. In L. P.
Rowland, Merritt's Neurology (11 ed.). Lippincott Williams &
Wilkins.

You might also like