White Matter Disease

Question 1

What is the worldwide leading cause of nontraumatic neurologic disability in young and middle-aged adults?

Answer 1

Multiple Sclerosis

Question 2

What criteria is used to diagnose MS?

Answer 2

Updated International Panel Criteria (McDonald Criteria)

Question 3

Lab findings in MS?

Answer 3

Approximately 70% of patients with MS have elevated
cerebrospinal fluid (CSF) levels of IgG index and approximately 90% have elevated oligoclonal bands.

Question 4

Types of “normal” MS

Answer 4

Replapsing remitting (85% initially)
Secondary progressive (50% within 10 years)
Primary progressive (5-10% initially, older usually)
Benign MS
Malignanat MS

Question 5

% of MS patients with optic neuritis at some point?

Answer 5

80%

Question 6

Very low signal MS lesions on T1 may be referred to as what?

Answer 6

black holes

Question 7

MC areas for lesions in MS?

Answer 7

periventricular, corpus callosum, subcortical, optic nerves and visual pathways, posterior fossa, cervical spinal cord…. but really, anywhere in the brain white matter

Question 8

typical shape of MS lesions?

Answer 8

ovoid.

Question 9

Where do the ovoid lesions of MS typically occur histologically, and what are they called? (eponym)

Answer 9

Their morphology has been attributed to inflammatory changes around the long axis of a medullary vein - Dawson’s fingers

Question 10

Is perfusion in MS lesions normal, increased or decreased compared to tumors? Do lesiosn affect the course of veins through the brain?

Answer 10

Perfusion in tumors is usually increased and in MS it is normally not. Veins are displaced by neoplasms but course through MS lesions.

Question 11

Other diseases in the differential both clinically and radiographically for MS?

Answer 11

primary angiitis of the central nervous system, polyarteritis nodosa, Behçet’s disease, syphilis, Wegener’s granulomatosis, Sjogren syndrome, and lupus

Question 12

Typical appearance of MS in the spinal cord?

Answer 12

Multifocal, nonexpansile, single vertebral level (typically less than 3 levels in length), enhancing, and nonenhancing lesions

Question 13

MS-like diseases? (3)

Answer 13

neuromyelitis optica (Devic disease) - transverse myelitis and bilateral optic neuritis
Balo disease (concentric sclerosis) - concentric rings of demyelination
Diffuse sclerosis (schilder disease) - bilateral symmetric demyelination, acute and rapidly progressive

Question 14

What are Virchow-Robin Spaces?

Answer 14

perivascular spaces. May be dilated and should not be confused with pathology such as MS. Typically seen in basal gangila, corona radiata, centrum semiovale, peri-insular region, center of brain stem.

Question 15

Where are high-signal T2 lesions seen in migraine?

Answer 15

subcortical, NOT periventricular, favoring the frontal and parietal lobes

Question 16

What does ADEM stand for?

Answer 16

Acute Disseminated Encephalomyelitis

Question 17

Typical history in ADEM?

Answer 17

The usual history is of a recent viral or respiratory infection, vaccination, or exanthematous disease of childhood. ADEM has been identified most frequently with antecedent measles, varicella, mumps, and rubella infection/vaccinations, but is not limited to these viruses.

Question 18

Distribution of findings in ADEM on MR?

Answer 18

As opposed to MS, ADEM favors the subcortical and deep white matter regions with no resemblance to Dawson’s fingers. There usually is at least one large dominant lesion, unlike MS. No new lesions should appear on MR 6 months from the start of the disease.
There may, however, be incomplete resolution of lesions.
ADEM can enlarge the spinal cord or brain stem (and appear as a mass lesion), but it usually is seen in the cerebrum. Symmetric deep gray matter lesions can also be identified and help dispel the inclination toward calling it MS.

Question 19

Lab findings in ADEM?

Answer 19

CSF may demonstrate an increase in white cells with a lymphocytic predominance and increased
myelin basic protein.

Question 20

Extreme end of ADEM is known as what?

Answer 20

Although rare, at the fulminant end of the spectrum of ADEM is acute hemorrhagic leukoencephalitis (Hurst disease)

Question 21

What does PML stand for?

Answer 21

Progressive multifocal leukoencephalopathy (PML)

Question 22

What causes PML?

Answer 22

is a demyelinating disease with a known viral etiology. It is caused by a JC virus infecting the oligodendrocyte and is associated with the immunosuppressed state.

Question 23

Does PML enhance, and if so in what pattern?

Answer 23

It typically does not enhance

Question 24

Angiographic findings in PML?

Answer 24

parenchymal blush in the early to midarterial phase with persistence into the venous stage associated with arteriovenous shunting. This correlates with neoangiogenesis associated with microvascular inflammatory disease.

Question 25

AKA for Biswanger disease

Answer 25

Subcortical Arteriosclerotic Encephalopathy

Question 26

What is Biswanger disease caused by?

Answer 26

demyelinating disease equally affecting men and women generally older than age 55 years. It is associated with hypertension (approximately 98% of patients) and lacunar infarction.

Question 27

MR findings in Biswanger disease?

Answer 27

MR reveals broad regions of high-intensity abnormalities

in the white matter of the frontal-parietal-occipital regions into the centrum semiovale

Question 28

What does CADASIL stand for?

Answer 28

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

Question 29

MRI findings in demyelination related to severe alcoholism/nutritional deficiencies?

Answer 29

demyelination and central necrosis of the corpus callosum

Question 30

What causes Wernicke Encephalopathy?

Answer 30

thiamine deficiency, often secondary to nutritional deficiency, gastric bypassbariatric surgery, or alcoholism causes

Question 31

MRI findings in Wernike encephalopahty?

Answer 31

atrophy of the mamillary bodies and may be high intensity in the mamillothalamic tracts, periaqueductal gray matter, medial thalami, and forniceal columns.

Question 32

Adrenoleukodystrophy pathophysiology?

Answer 32

X-linked or autosomal recessive (neonatal) peroxisomal disorder associated with cerebral degeneration degeneration and adrenal cortical insufficiency.

Question 33

Adrenoleukodystrophy imaging findings?

Answer 33

starts in the parieto-occipital region and progresses
anteriorly to involve the temporal and frontal lobes together with the corpus callosum. The disease can also progress from anterior to posterior. The advancing edge of the lesion represents the region of active demyelination and enhances