White Matter Disease Flashcards

1
Q

MRI appearance of white matter injury

A

T2 prolongation

tumefactive demyelination: mass like/enhance

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2
Q

scattered foci of T2 prolongation (hyperintense) in the subcorical, deep, periventricular white matter

A

chronic microvascular dx (older adults)

chronic migraine headaches (younger pts); from prior infection, inflammatory disease

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3
Q

Virchow robin spaces

A

tiny perivascular spaces that follow deep penetrating vessels into subarachnoidd space; follows CSF space

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4
Q

enlarged virchow robin spaces and J shaped sella

A

mucopolysaccharidoses

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5
Q

ependymitis granularis

A

frontal horn periventricular hyperintensity due to interstitial CSF backup

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6
Q

autoimmune/idiopathic/inflammatory white matter disease

A

MS, Concentric/balo sclerosis, Marburg variant, Devic disease/Neuromyelitis optica

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7
Q

most common demyelinating disease

A

Multiple sclerosis

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8
Q

Multiple sclerosis population, cause, presentations

A

population: middle aged caucasian females from northern latitdes
cause: autoimmune; idiopathic inflammatory destruction of CNS axons in brain/spinal cord; lymphocytes attack oligodendrocytes (make CNS myelin)
presentations: relapsing/remitting (most common) and progressive

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9
Q

Relapsing/remitting MS

A

partial/complete resolution of each acute attack

most common type

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10
Q

Progressive MS

A

no resolution between acute attacks

primary progressive: slow onset, without discrete exacerbations

secondary progressive: less complete resolution between attacks/ progressive disability

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11
Q

first sign of MS

A

optic neuritis; MRI brain used to look for other lesions which may be clinically silent

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12
Q

McDonald Criteria 2010

A

new lesions separate in space (areas of CNS) and time (new lesions across scans)

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13
Q

MS imaging findings

A
  • periventricular ovoid T2 prolongation foci that point toward ventricles (Dawson fingers), usually in corpus callosum
  • enhancing = active demyelination
  • T2 dark lesions: black holes associated with severe demyelination/axonal loss
  • chronic: cortical atrophy, thinning corpus callosum
  • MRI spectroscopy: decrease NAA, increase choline, lipids, lactate
  • tumefactive MS: ringe enhancement, mass like appearance of active plaque
  • spine: short segment/unilateral
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14
Q

concentric/balo sclerosis

A

rare variant of MS

pathognomonic alternating concentric bands of normal/abnormal myelin

often seen in younger pts

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15
Q

Marburg variant

A

fulminant manifestation of MS, death in a few months

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16
Q

Devic disease/neuromyelitis optica

A

involves optic nerves/spinal cord; worse prognosis than MS

NMO-IgG antibody to aquaporin 4 is specific for NMO

MS type lesions in optic tracts and spinal cord

17
Q

toxic/metabolic white matter diseases

A

osmotic demyleination, marchiafava-bignami, wernicke encephalopathy

18
Q

osmotic demyelination

A

rapid shift in extracellular osmolality (corrrecting hyponatremia); typically seen in pons/brainstem/deep gray nuclei

bilateral central T2 prolongation

seen in poor nutritional status, alcoholics, chronic lung disease, liver transplant patients

19
Q

marchiafava bignami

A

fulminant demyelination disease of corpus callosum; male alcoholics

20
Q

Wernicke encephalopathy

A

ataxia, confusion, oculomotor dysfunction – alcoholism/metabolic disturbances (bariatric surgery)

T2 prolongation/enhancement of mammillary bodies/medial thalamus

nonalcoholic form may also affect cortex

21
Q

vascular white matter disease

A

PRES, CADASIL, vasculitis, microangiopathy

22
Q

posterior reversible encephalopathy syndrome

A

vasogenic edema of posterior circulation due to failed autoregulation/hyperperfusion (acute hypertension); symmetric subcortical white matter changes, mild mass effect/enhancement

associated with eclampsia, sepsis, autoimmune disorders, multidrug chemo, solid/stem cell transplantation

23
Q

CADASIL

A

cerebral autosomal dominant arteriopathy with subcortical infarcts/leukoencephalopathy

inherited disease of small vessel arteriopathy; recurrent stroke, migraine, subcortical dementia, pseudobulbar palsy

24
Q

CNS vasculitis

A

typically affects leptomeningeal and small parenchymal vessels
lupus, polyarteritis nodosa, giant cell arteritis, sjogren disease

multiple small focal areas of T2 prolongation in subcortical/deep white matter; foci of hemorrhage are also seen (not seen in MS)

beaded vessels on vascular imaging

25
Q

microangiopathy

A

age related chronic axonal loss, gliosis, ischemic changes seen in elderly; NEVER involves corpus callosum

26
Q

Binswanger disease

A

dementia + severe microangiopathy

27
Q

infectious white matter disease

A

progressive multifocal leukoencephaly (PML), subacute sclerosing panencephalitis

28
Q

progressive multifocal leukoencephalopathy

A

demyelinating disease of immunocompromised patients caused by reactive JC virus

AIDS defining illness, but also seen in malignancy, status post organ transplant, autoimmune disorders, and MS patients on natalizumab

diagnosis spinal tap with PCR for JC virus in CSF

29
Q

MRI imaging of PML

A

asymmetric multifocal white matter lesions that may become confluent; arcuate/subcortical U fibers typically involved

30
Q

ddx for PML

A

HIV encephalitis, which is usually bilateral and spares subcortical white matter; cerebral atrophy

31
Q

subacute sclerosing panencephalitis (SSPE)

A

demyelinating disease from measles reactivation

periventricular white matter lesions; some surrounding edema and mass effect

32
Q

acute disseminated encephalomyelitis (ADEM)

A

monophasic demyelinating disorder in kids after viral infection/vaccination

most recover, but some have permanent neurologic sequelae; imaging findings similar to MS involve brain, brainstem, spinal cord

33
Q

Hurst variant (acute hemorrhagic leukoencephalitis)

A

fulminant form of ADEM; dead in days

multifocal T2 prolongation and white matter hemorrhage&raquo_space; confluent hematomas

34
Q

iatrogenic white matter disease

A

radiation and chemotherapy related disease

35
Q

radiation injury white matter disease

A

small vessel arteritis/secondary ischemia&raquo_space; diffuse T2 white matter prolongation

early phase may show mass effect, edema, enhancement

additional complications include meningiomas, capillary telangiectasia, cavernous malformations, moya moya

36
Q

chemotherapy related white matter disease

A

focal, multifocal, diffuse white matter disease