White Matter Disease

Question 1

MRI appearance of white matter injury

Answer 1

T2 prolongation

tumefactive demyelination: mass like/enhance

Question 2

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

Answer 2

chronic microvascular dx (older adults)

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

Question 3

Virchow robin spaces

Answer 3

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

Question 4

enlarged virchow robin spaces and J shaped sella

Answer 4

mucopolysaccharidoses

Question 5

ependymitis granularis

Answer 5

frontal horn periventricular hyperintensity due to interstitial CSF backup

Question 6

autoimmune/idiopathic/inflammatory white matter disease

Answer 6

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

Question 7

most common demyelinating disease

Answer 7

Multiple sclerosis

Question 8

Multiple sclerosis population, cause, presentations

Answer 8

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

Question 9

Relapsing/remitting MS

Answer 9

partial/complete resolution of each acute attack

most common type

Question 10

Progressive MS

Answer 10

no resolution between acute attacks

primary progressive: slow onset, without discrete exacerbations

secondary progressive: less complete resolution between attacks/ progressive disability

Question 11

first sign of MS

Answer 11

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

Question 12

McDonald Criteria 2010

Answer 12

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

Question 13

MS imaging findings

Answer 13

• 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

Question 14

concentric/balo sclerosis

Answer 14

rare variant of MS

pathognomonic alternating concentric bands of normal/abnormal myelin

often seen in younger pts

Question 15

Marburg variant

Answer 15

fulminant manifestation of MS, death in a few months

Question 16

Devic disease/neuromyelitis optica

Answer 16

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

Question 17

toxic/metabolic white matter diseases

Answer 17

osmotic demyleination, marchiafava-bignami, wernicke encephalopathy

Question 18

osmotic demyelination

Answer 18

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

Question 19

marchiafava bignami

Answer 19

fulminant demyelination disease of corpus callosum; male alcoholics

Question 20

Wernicke encephalopathy

Answer 20

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

T2 prolongation/enhancement of mammillary bodies/medial thalamus

nonalcoholic form may also affect cortex

Question 21

vascular white matter disease

Answer 21

PRES, CADASIL, vasculitis, microangiopathy

Question 22

posterior reversible encephalopathy syndrome

Answer 22

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

Question 23

CADASIL

Answer 23

cerebral autosomal dominant arteriopathy with subcortical infarcts/leukoencephalopathy

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

Question 24

CNS vasculitis

Answer 24

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

Question 25

microangiopathy

Answer 25

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

Question 26

Binswanger disease

Answer 26

dementia + severe microangiopathy

Question 27

infectious white matter disease

Answer 27

progressive multifocal leukoencephaly (PML), subacute sclerosing panencephalitis

Question 28

progressive multifocal leukoencephalopathy

Answer 28

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

Question 29

MRI imaging of PML

Answer 29

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

Question 30

ddx for PML

Answer 30

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

Question 31

subacute sclerosing panencephalitis (SSPE)

Answer 31

demyelinating disease from measles reactivation periventricular white matter lesions; some surrounding edema and mass effect

Question 32

acute disseminated encephalomyelitis (ADEM)

Answer 32

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

Question 33

Hurst variant (acute hemorrhagic leukoencephalitis)

Answer 33

fulminant form of ADEM; dead in days multifocal T2 prolongation and white matter hemorrhage >> confluent hematomas

Question 34

iatrogenic white matter disease

Answer 34

radiation and chemotherapy related disease

Question 35

radiation injury white matter disease

Answer 35

small vessel arteritis/secondary ischemia >> diffuse T2 white matter prolongation early phase may show mass effect, edema, enhancement additional complications include meningiomas, capillary telangiectasia, cavernous malformations, moya moya

Question 36

chemotherapy related white matter disease

Answer 36

focal, multifocal, diffuse white matter disease