MYASTENA

GRAVS
(Myasthenia gravis)
Anwar Wardy*

Neurologist, DFM

anwar wardy.FKK-UMJ

Myasthenia Gravis
Autoimmune
Incidence < 1/100,000
Prevalence 1/7500
Females 20s - 30s
Males 70s - 80s

Neurologist, DFM

anwar wardy.FKK-UMJ

Myasthenia Gravis
Fatigue, Weakness
Proximal Muscles
Bulbar muscles
ptosis, diplopia, weakness, dysphagia, dysarthria

worst as day progresses, prolonged activity

(reading, chewing)

Neurologist, DFM

anwar wardy.FKK-UMJ

Myasthenia Gravis Diagnosis

Edrophonium
Antibodies
EMG

Neurologist, DFM

anwar wardy.FKK-UMJ

Myasthenic Crisis
Respiratory failure due to severity of symptoms
FVC < 1L - consider intubation
Ensure the known myasthenic patient receives
their regular dosing while in the ED (ie.
Pyridostigmine 60mg q4h)

Neurologist, DFM

anwar wardy.FKK-UMJ

Myasthenic Crisis Versus

Cholinergic Crisis
Myasthenic : inadequate drug delivery
Cholinergic : excessive drug delivery

Neurologist, DFM

anwar wardy.FKK-UMJ

Myasthenic Crisis Versus

Cholinergic Crisis
Edrophonium (Tensilon) 1-2mg IV (1/10th dose)
resolution (total/partial) suggests myasthenic crisis
give rest of dose (8mg) for full effect
follow with neostigmine IM/SC 0.5 - 2mg doses q4h

Neurologist, DFM

anwar wardy.FKK-UMJ

Myasthenic Crisis Versus

Cholinergic Crisis
Exacerbation, fasiculations, respiratory depression
suggests cholinergic crisis
beware possible need for intubation at this point

further Tensilon contraindicated effects wear off

quickly

Neurologist, DFM

anwar wardy.FKK-UMJ

Tensilon
Cholinergic (muscarinic) agent
Atropine to treat bradycardia, AV block, cardiac
arrest
Muscle paralysis (nicotinic) atropine not effective

Unpleasant side effects

Neurologist, DFM

anwar wardy.FKK-UMJ

Treatment of MG
Neostigmine / Physostigmine
Plasmapheresis
Steroids
Thymectomy

Neurologist, DFM

anwar wardy.FKK-UMJ

Myasthenia gravis
neuromuscular junction disorder
weakness and fatigue of skeletal
muscles

Neurologist, DFM

anwar wardy.FKK-UMJ

Normal and myasthenic

neuromuscular junctions

Neurologist, DFM

anwar wardy.FKK-UMJ

Immunopathogenesis of MG
antigen: acetylcholine receptor
antibody: IgG to Ach receptor (80% of cases)
passive transfer: injection of patients IgG into mice
reproduces disease
immunization with antigen reproduces disease
good correlation between antibody levels and clinical
disability in the same patient

Neurologist, DFM

anwar wardy.FKK-UMJ

Origin of the autoimmune

response in myasthenia gravis
antibodies produced by B cells
T cell clones reactive to Ach receptors in blood and
thymuses of MG patients
75% of patients have thymus hyperplasia or thymoma
thymectomy improves clinical condition
thymus gland contains muscle-like cells with surface
Ach receptors
Neurologist, DFM

anwar wardy.FKK-UMJ

MuSK antibodies in MG((Myasthenia

gravis)
70% of patients with former seronegative MG
antibody to the muscle specific tyrosin kinase (agrin
receptor)
Agrin mediates clustering of Ach receptors during synapse
formation

MuSK

Neurologist, DFM

anwar wardy.FKK-UMJ

Acquired presynaptic
NMJ disorders
LEMS:
idiopathic or paraneoplastic antibodies to
voltage-gated calcium channels on the presynaptic
membrane
Botulism: toxin inhibits release of Ach
from presynaptic vesicles
therapeutic use for muscle
overactivity disorders

Neurologist, DFM

anwar wardy.FKK-UMJ

Congenital myasthenic disorders

presynaptic
Paucity of synaptic vesicles
Choline acetyltransferase def
basal lamina
synaptic
Ach-esterase deficiency
post-synaptic
Neurologist, DFM

Ach receptor deficiency

anwar wardy.FKK-UMJ
Rapsyn deficiency

MG istirahat

Neurologist, DFM

anwar wardy.FKK-UMJ

EMG
Normal

anormal
anwar wardy.FKK-UMJ

Neurologist, DFM

Wassalam, Wr,Wbr
Thank U

anwar wardy.FKK-UMJ

Neurologist, DFM