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Brant - 5th Edition > Chapter 7 - White Matter and Neurodegenerative Disease > Flashcards

Chapter 7 - White Matter and Neurodegenerative Disease Flashcards

1
Q

Cerebral white matter diseases are classified into two broad categories which are?

A
  1. Demyelinating

2. Dysmyelinating

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2
Q

This is an acquired disorder that affects normal myelin.

A

Demyelination

The vast majority of white matter diseases.

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3
Q

This is an inherited disorder affecting the formation or maintenance of myelin, and thus is typically encountered in the pediatric population.

A

Dysmyelination

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4
Q

Demyelinating disease can be divided into four main categories on the basis of etiology.

A
  1. Primary/immune-mediated
  2. Ischemic
  3. Infectious
  4. Toxic and metabolic
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5
Q

This is the classic example of a primary or immune- mediated demyelinating disease and is the most common nontraumatic cause of neurologic disability in young adults.

A

Multiple sclerosis

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6
Q

This is an autoimmune disorder affecting the central nervous system and is a disease characterized by immune dysfunction with the production of abnormal immunoglobulins and T cells, which are activated against myelin and mediate the damage associated with the disease.

A

Multiple sclerosis

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7
Q

MRI sequence that provides the best visualization of supratentorial white matter lesions.

A

FLAIR

However, the FLAIR sequence may have mild limitations when imaging the posterior fossa and spine, partly because of pulsation artifacts.

In these anatomic regions, both proton density and short tau inversion recovery (STIR) imaging are valuable.

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8
Q

Characteristics and locations of MS plaques.

A

MS plaques are typically round or ovoid, with a periventricular, juxtacortical, infratentorial, and spinal cord location.

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9
Q

These findings are indicative of acute lesions with active inflammation/demyelination.

A

Contrast- enhancing lesions and lesions with restricted diffusion on diffusion-weighted imaging (DWI) are indicative of acute lesions with active demyelination and disruption of the blood–brain barrier.

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10
Q

In MS, lesions are bright on T2WI would indicate what?

A. Acute
B. Chronic
D. Both

A

B. Both

It reflects acute lesions with active inflammation/demyelination or chronic lesions with gliotic scarring.

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11
Q

Within the CNS, cells can mount only a limited response to neuronal injury.

This scarring typically manifests as a focal proliferation of astroglia at the site of injury. What is this called?

A

Gliosis

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12
Q

Dark lesions of MS are seen in what cases?

A

Severe cases
- actual loss of neuronal tissue may occur and the white matter lesions may have dark signal on T1WIs

These lesions are prognostically significant because they reflect actual loss of underlying neuronal tissue rather than simple demyelination and are in keeping with a more advanced stage of this disease.

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13
Q

Diffuse loss of deep cerebral white matter, with associated thinning of the corpus callosum and ex vacuo ventriculomegaly are seen in what stage of MS?

A

Chronic cases

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14
Q

Imaging pattern the is suggestive of MS

A
  1. Lesions that are periependymal (abutting the ependymal, ventricular surface)
  2. Juxtacortical (gray-white matter junction)
  3. Lesion involving the posterior fossa structures
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15
Q

Structure that is excluded in MS.

A

Pons

Because most pontine lesions are either ischemic in nature or the result of osmotic demyelination

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16
Q

The periventricular lesions suggestive of MS are often ovoid and aligned perpendicular to the long axis of the ventricles.

What is the reason?

A

This pattern is the result of the alignment of the lesions along the perivenular spaces

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17
Q

Additional characteristic features of MS include:

Lesions along the callosal–septal interface.

Confluent lesions are also seen. What are the sizes of these lesions?
(periventricular or juxtacortical location)

A

Greater than 6 mm

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18
Q

Other location of MS in addition to periventricular white matter. (5)

A
  1. Cerebellar peduncle
  2. Cerebral peduncle
  3. Corpus callosum
  4. Medilla
  5. Spinal cord
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19
Q

What are some key differences between ishcemia and demyelinating disease.

A
  1. Ischemic changes rarely involvees the medulla and cerebeller/celebral penduncles.
  2. MS brainstem lesions are peripheral in location, ischemic changes tend to be centrally located.
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20
Q

These MS lesions may also present as a large, conglomerate, deep white matter mass that can be mistaken for a neoplasm.

A

Tumefactive MS or tumefactive demyelinating lesions

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21
Q

Difference between tumefactive MS from neoplasm.

A
  1. MS often demonstrate a peripheral crescentic rim of contrast enhancement, which represents the advancing leading edge of active demyelination.
  2. Paucity of perilesional edema as well as relative lack of mass effect for lesion size.
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22
Q

Characteristic of a spinal cord MS plaques.

A
  1. Well defined
  2. Less than one to two vertebral segments in the CC dimension
  3. Less than 50% of the cross sectional area of the spinal cord often affecting the peripheral white matter.
  4. Majority of spinal cord Ms lesions (70% to 80%) will have plaques in the brain.
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23
Q

This consist of an area of high signal on T2WI along the tips of the frontal horns. This is a normal anatomic finding that may mimic pathology.

A

Ependymitis granularis.

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24
Q

This may mimic deep white matter or lacunar infacts. As blood vessels penetrate into the brain parenchyma, they are enveloped by the CSF and a thin sheath of pia.

A

Prominent perivascular spaces

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25
Q

This is an inheritable condition relating to a Notch 3 mutation on chromosome 19.

A

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

The presence of subcortical anterior temporal and medial frontal lesions is relatively specific for this condition.

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26
Q

These represents a heterogeneous group of disorders which are associated with inflammation of blood vessels leading to a variety of ischemic manifestations ranging from ischemic brain lesions, cerebral perfusion deficits, intracerebral or subarachnoid hemorrhage to vessel stenosis.

A

CNS vasculitis

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27
Q

This is an idiopathic vasculitis limited to the brain and spinal cord typically observed in the fifth and sixth decades of life. This condition is associated with an elevation in inflammatory markers such as ESR.

A

Primary Angiitis of the Central Nervous System (PACNS)

28
Q

This is an autoimmune disorder which presents with white matter lesions (75% of patients) as well as a spectrum of neurologic and neuropsychiatric manifestations such psychosis, stroke, headaches, and neurocognitive deficits.

A

Systemic Lupus Erythematosus (SLE)

29
Q

Japanese for “puff of smoke” is a progressive occlusive condition of the supraclinoid or terminal aspects of the internal cerebral arteries.

A

Moyamoya disease

The term “moyamoya disease” is used in idiopathic or familial conditions, whereas “moyamoya syndrome” is used when there is a known cause such as radiation vasculitis, sickle cell anemia, and Down or Marfan syndrome.

30
Q

Characteristic imaging findings of Moyamoya disease

A

Marked narrowing and occlusion of the terminal internal cerebral arteries (usually bilateral, but may be asymmetric and occasionally unilateral), with formation of a diffuse extensive network of tiny collateral vessels throughout the deep gray matter structures.

On angiography - the enhancement of these collateral vessels results in a dense blush of contrast, giving rise to the term “puff of smoke.”

31
Q

This is a disorder that results in characteristic demyelination of the central pons.

This is most commonly seen in patients with electrolyte abnormalities, particularly involving hyponatremia, that are rapidly corrected, giving rise to the term “osmotic demyelination syndrome.”

A

Central pontine myelinolysis (CPM)

32
Q

Imaging finding pf central pontine myelinolysis

A

MRI : abnormal high signal on T2WI, corresponding to the regions of central pontine demyelination.

In addition, extrapontine sites of involvement have been described in this condition, including the white matter of the cerebellum, thalamus, globus pallidus, putamen, and lateral geniculatebody, giving rise to the term extrapontine myelinolysis.

33
Q

This is a condition characterized by signal changes within the brain parenchyma, primarily involving the posterior vascular distribution.

It has also been referred to as reversible posterior leukoencephalopathy syndrome (RPLE)

A

Posterior reversible encephalopathy syndrome (PRES)

34
Q

Imaging pattern of PRES

A

MRI revealing relatively symmetric areas of bilateral subcortical and cortical vasogenic edema within the parietooccipital lobes

35
Q

This is a rare form of demyelination seen most frequently in alcoholics.

The disease is characterized by demyelination involving the central fibers (medial zone) of the corpus callosum, although other white matter tracts may be involved, including the anterior and posterior commissures, the centrum semiovale, and the middle cerebral peduncles.

A

Marchiafava-Bignami disease

36
Q

Most common symptom of Marchiafava-Bignami disease

A

Nonspecific dementia

37
Q

These are metabolic disorders caused by thiamine (B1 vitamin) deficiency secondary to poor oral intake in severe chronic alcoholics (most common association), hematologic malignancies, or recurrent vomiting in pregnant patients.

A

Wernicke encephalopathy and Korsakoff syndrome

38
Q

This is characterized by a clinical triad of acute onset of ocular movement abnormalities, ataxia, and confusion.

A

Wernicke encephalopathy

39
Q

Imaging pattern of Wernicke encephalopathy

A

Acute stage - MRI may reveal T2 hyperintensity or contrast enhancement of the mammillary bodies, basal ganglia, thalamus, and brainstem, with periaqueductal involvement.

Chronic stage - may show atrophy of the mammillary bodies, midbrain tegmentum, as well as dilatation of the third ventricle.

40
Q

Radiation may result in damage to the white matter secondary to a radiation-induced vasculopathy.

These usually follows a cumulative dose in excess of 40 Gy delivered to the brain and occurs 6 to 9 months after treatment.

A

Radiation leukoencephalitis

41
Q

Image finding of radiation lekoencephalitis

A

Abnormal high signal on T2WIs, typically involving confluent areas of white matter extending to involve the subcortical U fibers in the distribution of the irradiated brain.

This represents an indirect effect of radiation on the brain and results from an arteritis (endothelial hypertrophy, medial hyalinization, and fibrosis) involving small arteries and arterioles.

42
Q

These are major hazards related to CNS radiation.

A

Radiation necrosis and radiation arteritis

43
Q

This may occur several weeks to years after radiation, but it most commonly occurs between 6 and 24 months after radiation.

A

Radiation necrosis

Radiation necrosis is rarely noted at less than 6 months after treatment unless gamma knife is employed.

44
Q

Radiation may induce what vascular anomaly?

A

Telangiectasia

  • Which may appear similar to cryptic vascular malformations
45
Q

Large vessels included within the radiation port may undergo what processes?

A
  1. Radiation induced endothelial hypertrophy
  2. Medial hyalinization
  3. Fibrosis

Net result is progressive vascular narrowing that may be obliterative in nature

46
Q

Two radiation related conditions associated with children being treated with leukemia.

A
  1. Mineralizing microangiopathy

2. Necrotizing leukoencephalopathy

47
Q

During treatment of methotrexate and radiation therapy:
This results in diffuse destructive changes to the brain characterized by symmetric corticomedullary junction and basal ganglia calcifications.

A

Mineralizing microangiopathy

48
Q

This process results in widespread damage to the white matter, consisting of demyelination, necrosis, and gliosis.

It is a more serious but less common complication of combined radiation and methotrexate therapy

A

Necrotizing leukoencephalopathy

49
Q

Also referred to as leukodystrophies.

These disorders in which myelin is abnormally formed or cannot be maintained in its normal state because of an inherited enzymatic or metabolic disorder.

A

Dysmyelinating conditions

50
Q

This is the most common of the leukodystrophies.

A

Metachromatic leukodystrophy

51
Q

It is transmitted by an autosomal recessive pattern and is the result of a deficiency of the enzyme arylsulfatase.

A

Metachromatic leukodystrophy

  • The most common type is an infantile form that becomes apparent at approximately 2 years of age with gait disorder and mental deterioration.
52
Q

This is a sex-linked recessive condition (peroxisomal enzyme deficiency) occurring only in boys.

Has a striking predilection for the visual and auditory pathways, presenting with symmetric involvement of the periatrial white matter with extension into the splenium of the corpus callosum.

A

Adrenal leukodystrophy

  • as the name implies, these patients often have symptoms related to the adrenal gland, such as adrenal insufficiency or abnormal skin pigmentation.

The predilection for periatrial involvement results in early extension to the medial and lateral geniculate nuclei, which represent relays for the auditory and visual pathways, respectively.

53
Q

This is a mitochondrial enzyme defect that commonly manifests in infancy or childhood (usually younger than 5 years).

Also known as subacute necrotizing encephalomyelopathy

A

Leigh disease

54
Q

A characteristic finding of Leigh disease is involvement of the what structure?

A

Periaqueductal gray matter.

In contrast to Wernicke encephalopathy, there is sparing of the mammillary bodies.

55
Q

These are the rarest of the leukodystrophies and may appear as early as the first few weeks of life. (2)

Patients often have an enlarged brain and have macrocephaly on examination. Typically, these patients present with seizures, spasticity, and delayed developmental milestones.

A

Alexander and Canavan diseases

56
Q

Difference between Alexander and Canavan disease.

A

In Alexander disease, white matter lesions often begin in the frontal white matter and progress posteriorly.

Canavan disease is caused by a deficiency of the enzyme aspartoacylase, which leads to the buildup of NAA in the brain and subsequent myelin destruction.
This results in a pathognomonic MRI spectra consisting of a giant NAA peak.

57
Q

This is the most common neurodegenerative disease and the most common cause of dementia.

A

Alzheimer disease

58
Q

Imaging pattern of patients with Alzheimer disease

A
  1. Diffuse atrophy with predilection for the hippocampal formation, temporal lobes, and parietotemporal cortices
  2. Enlargemet of the temoral horns, suprasellar cisterns, and Sylvian fissures
59
Q

This is the most common basal ganglia disorder and one of the leading causes of neurologic disability in individuals older than age 60.

This disease is characterized clinically by tremor, muscular rigidity, and loss of postural reflexes.

A

Parkinson disease

60
Q

Imaging pattern of Parkinson disease

A

MRI - occasionally reveal thinning of the pars compacta.

The substantia nigra is made of the pars compacta (high signal intensity band on T2WIs) posteriorly, which is sandwiched between the pars reticulata anteriorly and the red nuclei posteriorly.

With thinning of the pars compacta, the high signal intensity band between the pars reticularis and the red nuclei is lost.

61
Q

This is a progressive hereditary disorder that appears in the fourth and fifth decades of life.

This disease is characterized by a movement disorder (typically choreoathetosis), dementia, and emotional disturbance.

A

Huntington disease

62
Q

Imaging pattern of Huntington disease

A

neuroimaging studies demonstrate diffuse cortical atrophy, the caudate nucleus and putamen are most severely affected. Atrophy of the caudate nucleus results in characteristic enlargement of the frontal horns, which take on a heart-shaped configuration.

63
Q

This is an inborn error of copper metabolism that is associated with hepatic cirrhosis and degenerative changes of the basal ganglia.

A

Wilson disease

also known as hepatolenticular degeneration

64
Q

This is virtually diagnostic of the Wilson disease when present (75% of cases)

A

Kayser-Fleischer ring

65
Q

Imaging finding of Wilson disease

A

MRI findings include diffuse atrophy with signal abnormalities involving the deep gray matter nuclei and deep white matter.

Brant - 5th Edition (8 decks)

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