Kilgo- Demyelinating Disorders Flashcards
only real primary demyelinating disorder is ____
MS
4 principles to diagnose MS:
demyelinating event
dissemination in space
dissemination in time
other explanations ruled out
CNS demyelination that is separated in time and space
Multiple Sclerosis
= >1 clinical event by history or presence of both active and inactive plaques simultaneously on MRI
MS separation in time
=exam deficits referable to >1 CNS lesion, or >1 MRI lesion
MS separation in space
MS plaques are active for ___ weeks
6 weeks
painful vision loss in young person
optic neuritis
Optic neuritis
Transverse myelitis
Brainstem syndromes
common presenting symptoms of MS
(MLF attacked and +nystagmus in unaffected eye and the affected eye cannot adduct); heavily myelinated structure in CNS so look out for demyelination
Internuclear opthalmoplegia
Cognitive presentations (dementia)
Tumefactive presentations (tumor-like)
Isolated neurogenic bladder/bowel (can’t pee/poop)
less common presentations of MS
Inflammation of one or both of the optic nerves
May be the first indication of multiple sclerosis (MS)
optic neuritis
mimickers of MS
NMOSD and MOG Ab disease
Symptoms may include pain with extraocular movement, visual field loss, flashing lights and loss of red/green color vision
optic neuritis
usually in ____ only one optic nerve affected
MS
in ____ and ____ both optic nerves are affected and can be at different points in time
NMOSD
MOG Ab disease
Peak age of onset is 20-40 years
MS
Most common cause of non-traumatic disability in young adults
MS
Vision loss (optic neuritis)
Double vision (brainstem lesion)
Paresthesias/weakness (spinal cord lesion)
Appendicular or Gait Ataxia
MS
supporting (not diagnostic) paraclinical test for MS
Oligoclonal immunoglobin bands in CSF
on section, areas of well-demarcated discoloration seen in white matter
MS
Often present in the optic nerves (20% of cases) and at the lateral angles of the lateral ventricles but can be anywhere in white matter
MS
MS
have myelin and their cell bodies are in the retina
see areas of demyelination with relative axonal sparing
optic nerve
on Luxol Fast Blue
MS
this MRI has subtracting out T2 and looks like T1 again except any white matter lesion will be accentuated
FLAIR
(multiple scars) areas of gliosis that are seen from prior inflammation
MS
MRI T2
MS
demyelination of periventricular region
MS
MRI T2
MS
hypercellular with parenchymal and perivascular lymphocytes and macrophages
preservation of axons
active plaques
hypocellular and gliotic with loss of oligodendrocytes and variable loss of axons
inactive plaques
areas of remyelination, often within larger zones of demyelination
shadow plaques
active plaques
active plaques
green: active plaques
red: shadow plaques
Rare multifocal inflammatory white matter disease
viral cause
progressive multifocal leukoencephalopathy (PML)
caused by reactivation of JC virus (john cunningham) mainly in immunosuppressed patients
PML
being on ______ and _____ are most common causes of PML in multiple sclerosis patients
Natalizumab and Fingolimab
PML (progressive multifocal leukoencephalopathy)
how to diagnose progressive multifocal leukoencephalopathy
(+) JCV DNA PCR in CSF
PML
PML
Classically associated with rapid overcorrection of hyponatremia, osmotic demyelination
Central Pontine Myelinolysis
Central Pontine Myelinolysis can be seen in other conditions with normal sodium level, ___ and ___
AIDS
alcohol
Older patient who came in hyponatremic; blood Na+ down in the 120s and gets way to rapidly corrected overnight to 140
central pontine myelinolysis or even locked-in syndrome
lesion to the pons—–basilar a. stroke affecting the whole pons
locked-in syndrome
central pontine myelinolysis
myelin gone, but axons remain
central pontine myelinolysis
Cardinal symptom: encephalopathy
Neurological symptoms may be preceded (days to weeks) by a viral syndrome
acute disseminated encephalomyelitis (ADEM)
decreased consciousness
most often monophasis event (compared to MS which is multiple events)
ADEM (acute disseminated encephalomyelitis)
perivascular inflammation and “sleeve like” demyelination
ADEM
Good prognosis in children, worse in adults
(+) MOG antibody in serum is seen in high percentage of pediatric cases
ADEM
Will look like hypertensive encephalopathy (confused, cant see)
Rx: lower bp
See this on MRI (affects occipital part of brain)
Dialysis patients; patients with eclampsia
posterior reversible encephalopathy syndrome (PRES)
inability of posterior circulation to autoregulate
PRES (posterior reversible encephalopathy syndrome)
Genetic cause of peripheral neuropathy
FOOT DROP
PMP-22 involved
Charcot-Marie-Tooth
family history important
foot drop
high foot arch
decreased tendon reflex
Charcot-Marie-Tooth
“onion bulb”
Charcot-Marie-Tooth
Immune system attack on peripheral myelin
Usually doesn’t happen again and again (unlike MS in CNS)
guillain-barre syndrome
can happen after Infection: campylobacter or mycoplasma
Guillain-Barre syndrome
Weakness and tingling or loss of sensation starting in the feet and legs then spreading to the upper body and arms (ascending)
Guillain-Barre syndrome
main treatment for guillain-barre syndrome
IVIG
peripheral nerve
guillain-barre syndrome
