demyelinating diseases Flashcards
autoimmune inflammatory demyelination of peripheral nerves at Schwann cells (due to molecular mimicry)
Guillain-Barre syndrome
infections that cause Guillain-Barre syndrome
campylobacter jejuni (most common) GI illness in last month (20% with GBS) respiratory tract infection in last month: CMV, EBV, mycoplasma pneumoniae HIV stress idiopathic
ASCENDING SYMMETRIC muscle weakness from feet → legs → hands → trunk → may involve bilateral facial paralysis (50% cases)
respiratory muscle weakness (10-30%, requires ventilation)
no sensory deficits
occurs over days-wks
recovery: wks to mo
Guillain-Barre syndrome
bilateral facial paralysis/weakness (Bell’s palsy)
Guillain-Barre syndrome
Lyme disease
diagnosis of Guillain-Barre syndrome
↓ nerve conduction studies
treatment of Guillain-Barre syndrome
supportive care
if caught early - decrease Ab with plasmaphoresis
IV Ig
demyelination of CNS due to destruction of oligodendrocytes (vs GBS - schwann cells)
progressive multifocal leukoencephalopathy
associated with JC virus (seen in AIDS)
progressive multifocal leukoencephalopathy (leuko = white matter)
associated with campylobacter jejuni (diarrhea illness)
guillain-barre syndrome
inflammation and demyelination after a viral infection or vaccine
acute disseminated post-infectious encephalomyelitis
associated with viral infections: chickenpox, measles, or vaccine
acute disseminated post-infectious encephalomyelitis
AR lysosomal storage disease due to deficiency of arylsulfatase A → destroys myelin
metachromatic leukodystrophy (white matter)
hereditary motor + sensory neuropathy
mutations cause defects in neuronal proteins (in neurons, myelin sheaths, etc.)
progressive loss of muscle tissue + touch sensation across various parts of body
Charcot-Marie-Tooth disease