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Created by
Sara Fuad
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Cards (21)
Myasthenia
gravis
Autoimmune disorder of neuromuscular junction (
type II hypersensitivity
)
Myasthenia gravis
Anti-acetylcholine
receptor antibodies at the postsynaptic membrane of
NMJ
Can present at any age, peaks in the
third
(females) and
sixth
(males) decade, more in women
Associated with thymic hyperplasia (70%), thymoma (10%) or other autoimmune disorders ex:
thyroid
,
rheumatoid
, and pernicious anemia (must screen)
Clinical
manifestations of condition
Weakness and fatigability of ocular, bulbar, and proximal limb weakness
Weakness is
descending
(starts from the
top
and moves down)
Muscle
fatigue after sustained/repetitive action, better with
rest
Ocular
muscles -
ptosis
and diplopia, better with sleep/icepack
Difficulty
swallowing
,
chewing
, and talking (nasal speech)
Respiratory
difficulties
Diurnal
variation (symptoms worse at night)
Bedside
tests
1.
Worse
when counting to
20 quickly
2.
Upward gaze
- ptosis; relieved by
icepacks
3.
Outstretched arms
-
slow downward drift
Normal
reflexes
Anti
-AChR antibodies
Most
specific
Single
fiber electromyography
Most
sensitive
Nerve
stimulation test
Decrement in
evoked
potential
Anti-MuSK antibodies
Seronegative
disease; mostly
ocular
manifestations
CT
Thymoma
/
thymic hyperplasia
Tensilon
test
1. Giving
IV anticholinesterase
2.
Temporary
rapid improvement
Differential
diagnosis of MG
Thyroid
ophthalmopathy
Myotonic
dystrophy
Brainstem
cranial nerve lesions
Motor
neuron disease
Lambert-Eaton
myasthenic syndrome
Lambert
-Eaton myasthenic syndrome
Associated with small cell
lung
cancer
Autoantibodies against
presynaptic
calcium channels
Presents with
proximal
muscle weakness and hyporeflexia
Symptoms improve with
repeated
muscle stimulation
Botulism
History of consumption of
contaminated
food (clostridium botulinum: anaerobic spore forming
bacteria→neurotoxin
production)
Acute onset of bilateral cranial neuropathies,
pupillary
paralysis, hyporeflexia, symmetrical descending weakness,
anticholinergic
symptoms
Anticholinesterases (pyridostigmine)
↑ availability of
Ach
at
NMJ
Immunosuppressants
steroids
azathioprine
If no response/relapse on Tx
Use
immunosuppressants
Thymectomy
Indicated if thymoma is seen on CT (malignant potential)
Indicated if no thymoma but <
50
years old & anti
AchR antibodies
+ve
Acute
Myasthenic Crisis
Life-threatening
condition
Profound
weakness
Respiratory
/
bulbar
involvement
Triggers
for
Acute Myasthenic Crisis
Drugs
(aminoglycosides, macrolides, fluoroquinolones, phenytoin, BBs)
Upper respiratory tract
infection
Surgery
Pregnancy
/
postpartum
Treatment for Acute Myasthenic Crisis
1.
Plasmapheresis
2.
IVIg