disorders of NMJ Sachen Flashcards
MG what HLA haplotypes and what other diseases
HLA B8 and DR3
seen with other autoimmune disorders like SLE, RA, and thyroid disorders
what is lambert eaton Myasthenic syndrome usualy associated with
cancer (SCCL)
sarin and VX clinical features (nerve gas)
inhibit acetylcholineesterase at NM junction to cause end organ overstimulation (choinergic crisis)
onset within minutes (inhalation) or minutes to hours with contact
DUMMBELLS
can cause direct lung damage if inhaled
death by resp failure
which antibiotics can exacerbate or unmask MG (most significant)
aminoglycosides
clinical presentation of lambert eaton
proximal weakness, loss of DTRs**, myalgias, dry mouth, impotence
oropharyngeal and ocular muscles if involved then only mild symptoms
treatment of nerve gases
resp support
atropine
pralidoxime chloride
seizures respond to benzos
treatment MG
which for pts in crisis or prior to surgery
Acetlycholinesterase inhibitors like mestinon
predinose
other immunosupp agents
IVIg for pts in crisis or prior to sugery
thymectomy
treatment for MUSK syndrome
rituximab is best
clinical presentation of botulism
dry, sore mouth and throat, blurred vision, diplopia, nausea, vomiting
toxin that blocks presynaptic mech for release of ACh
botulism
emg finding lambert eaton
low amp motor response that increase after brief period of exercise
incremental response on fast repetitive stim
antibody negative MG
syndrome with it
40% of these have MUSK Abs MUSK syndrome mainly in women: -oculopharyngeal weakness -neck, shoulder, resp weakness -indistinguishable from antibody positive MG
treatment lambert eaton
first look for malig and treat acetylcholinesterase inhibs 3-4 diaminopyridine guanidine hydrochloride (bone marrow supp AE) immunosupp IVIg
DAGI
antibodies in MG
AChR Abs
MUSK antibodies
if someone has lambert eaton and HAs dont give
mg oxide
