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ABPP > White matter, MS, & disconnection syndromes > Flashcards

White matter, MS, & disconnection syndromes Flashcards

1
Q

What is the most prominent artery supplying the cerebral white matter?

A

Lenticulostriate

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2
Q

Factors of MS that are related to cognitive impairment

A

Duration of illness, atrophy, total lesion volume, physical impairment, enlarged cerebral ventricles, CC atrophy, cerebral hypometabolism

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3
Q

Corona radiata

A

Rich mass of white matter within each hemisphere above the internal capsule

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3
Q

Central pontine myelinolysis (CPM)

A

Related to too rapid correction of sodium deficiency; leads to longstanding dementia or death

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4
Q

Psychiatric symptoms of white matter dementia

A

Irritability, depression, apathy, psychosis

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5
Q

Adrenoleukodystrophy

A

Generalized loss of myelin in the brain & adrenal insufficiency; posterior pattern of affected white matter

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5
Q

White matter disease is associated with what type of memory disturbance?

A

Poor retrieval but intact encoding

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5
Q

What type of aphasia is most closely associated with white matter disease?

A

Conduction aphasia

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5
Q

How has white matter damage been implicated in schizophrenia?

A

Research suggests that schizophrenia may result from disrupted frontal lobe connectivity related to oligodendriaglial dysfx and death resulting in abnormalities in myelin laying down & maintainence

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6
Q

Relapsing/remitting course of MS

A

Specific periods of relapse followed by periods of remission; most common type in the early years

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6
Q

Secondary progressive course of MS

A

Initially relapsing/remitting course followed by progression with or without occasional relapses, minor remissions, & plateaus

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6
Q

Typical areas of preserved functioning in MS

A

Memory storage, encoding, recognition, simple auditory span, motor skill & implicit learning, language skills (other than fluency), basic visuospatial functions

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7
Q

Lhermitte’s sign

A

Electrical sensation radiating down extremities or back upon cervical flexion; seen in MS

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8
Q

How are MS lesions related to cognition?

A

Extent of plaques & total lesion area is related to cognitive function, but, in general, location of plaques is not predictive

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9
Q

What toxin is associated with 2-3 weeks delayed demyelination?

A

Carbon monoxide

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10
Q

Impairments associated with white matter dementia (Filley)

A

Slowness of cognition, poor sustained attention/vigilance, visual-spatial deficits, memory retrieval deficits, exec dysfx

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11
Q

Factors associated with better MS prognosis

A

Young age (

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12
Q

Metachromatic leukodystrophy

A

Disorder of myelin metabolism that usually begins in infancy

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13
Q

White matter is most prominent in which lobe of the brain?

A

Frontal lobe

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14
Q

Sympathetic apraxia in Broca’s aphasia

A

Apraxia of command mvmts of the LH, due to disconnection of L language area & motor assoc. cortices from R motor association cortices

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15
Q

Cognitive impact of commissurotomy

A

L neglect, right-ear dominancy in dichotomous listening tasks, inability to name objects in L hand but can pick up with L hand if named, R constructional apraxia, spatial acalculia, cannot cross-replicate hand postures, difficulty naming points touched on L side of body, R-sided verbal anosmia, double hemianopia, hemialexia

17
Q

What cognitive domains are spared in white matter dementia?

A

Language (word finding deficits but not aphasic), no extrapyramidal symptoms, normal procedural memory

18
Q

Primary progressive course of MS

A

Continued decline

19
Q

What are the symptoms of Hallervorden-Spatz syndrome?

A

Progressive rigidity beginning in the legs, choreathetoid movements, dysarthria, dystonia, emotional & mental deterioration (visuospatial & memory)

20
Q

Marchiafava-Bignami disease

A

Rapidly progressing degeneration/demylination of CC that is associated with cognitive deterioration, emotional disturbances, confusion, hallucinations, tremor, rigidity, & seizures Associated with excessive consumption of red wine

21
Q

Progressive relapsing course of MS

A

Progressive disease from onset with clear acute relapses, periods between relapses are characterized by continuing progression

21
Q

What is the most common & debilitating complaint among patients with MS?

A

Fatigue - affects up to 90%

22
Q

Charcot’s triad

A

Nystagmus, dysarthria, tremor (associated with cerebellar dysfx)

23
Q

Pathology of MS

A

Multifocal areas of CNS white matter inflammation, demyelination with loss of oligodendrocytes & astrogliosis

23
Q

Pure word mutism

A

Lose capacity to speak but oral & reading comprehension & writing intact; due to separation of Broca’s area from subcortical motor centers

25
Q

Risk factors for central pontine myelinolysis

A

Alcoholism, liver disease, malnutrition

27
Q

3 major groups of white matter pathways

A

Projection, commissural, association

29
Q

1st degree relatives of patients with MS are ____x more at risk than the general population?

A

6-8x

31
Q

Transsynaptic degeneration

A

Neuronal loss & reactive gliosis in neurons deprived of synaptic input by lesions in adjacent neurons

32
Q

Common affective/behavioral problems in MS

A

Depression, personality changes, anxiety

33
Q

Most common cognitive deficits in MS

A

Memory, complex attention/speed of info processing, exec fx, verbal fluency

34
Q

Disconnection syndrome (Geschwind)

A

Any disorder in which cortical areas that normally work in conjunction become isolated, involving interruption of info transfer

35
Q

Outcomes in childhood-onset MS

A

Increased risk of having deficits in at least 1 area of cognition, increased rate of need for educational assistance, 1/2 had psych diagnosis, little evidence of physical disability

37
Q

Proposed explanations for MS

A

Slow-acting virus, delayed reaction to a common virus, autoimmune reaction in which body attacks its own tissues

39
Q

How does MS risk vary by race/ethnicity?

A

Predilection for whites; Asian & black populations have low risk

40
Q

Transverse myelitis

A

Acutely evolving inflammatory-demyelinative lesion of the spinal cord, often an expression of MS

41
Q

Visual changes seen in MS

A

Optic neuritis, intranuclear ophthalmoplegia, Marcus Gunn pupil

43
Q

Which hemisphere has a greater proportion of white matter?

A

Right

44
Q

Overall prevalence of cognitive impairment in MS

A

40-70%; of those with impairment, 80% are mild

45
Q

How does immigration from a high-risk to a low-risk latitude affect MS risk?

A

Individuals carry with them some of the risk from their place of origin if they move after age 15

46
Q

How does childhood-onset MS differ from adult-onset?

A

Higher lesion burden on initial MRI, esp. in brainstem & cerebellum; however, T2-bright foci in younger children can vanish with repeat scans

47
Q

Describe the steps of myelogenesis (i.e., which areas of the brain are myelinated 1st, 2nd, etc.)

A

1) Primordial (‘premature’) fields are myelinated before birth (somesthetic cortex, primary visual, primary auditory) 2) Intermediate (‘postmature’) fields myelinate during first 3 monts (secondary association areas) 3) Terminal fields myelinate b/t 4th month and 14 yrs of age (classical association areas)

48
Q

Multiple sclerosis

A

Multifocal demyelinating disease that causes destruction of myelin sheath of nerve fibers along with sclerotic plaques that form in areas where demyelination has occurred & block or distort the normal transmission of nerve impulses

49
Q

Retrobulbar neuritis

A

Optic neuritis that occurs far enough behind the optic disk that no early changes of the optic disk are visible by ophthalmascope

50
Q

How do prevalence rates of MS change relative to the Equator?

A

Prevalence decreases systematically as latitude of habitation nears Equator

ABPP (34 decks)

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