White matter, MS, & disconnection syndromes Flashcards Preview

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Flashcards in White matter, MS, & disconnection syndromes Deck (50):
1

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

Lenticulostriate

2

Factors of MS that are related to cognitive impairment

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

3

Corona radiata

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

3

Central pontine myelinolysis (CPM)

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

4

Psychiatric symptoms of white matter dementia

Irritability, depression, apathy, psychosis

5

Adrenoleukodystrophy

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

5

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

Poor retrieval but intact encoding

5

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

Conduction aphasia

5

How has white matter damage been implicated in schizophrenia?

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

6

Relapsing/remitting course of MS

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

6

Secondary progressive course of MS

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

6

Typical areas of preserved functioning in MS

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

7

Lhermitte's sign

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

8

How are MS lesions related to cognition?

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

9

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

Carbon monoxide

10

Impairments associated with white matter dementia (Filley)

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

11

Factors associated with better MS prognosis

Young age (

12

Metachromatic leukodystrophy

Disorder of myelin metabolism that usually begins in infancy

13

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

Frontal lobe

14

Sympathetic apraxia in Broca's aphasia

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

15

Cognitive impact of commissurotomy

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

What cognitive domains are spared in white matter dementia?

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

18

Primary progressive course of MS

Continued decline

19

What are the symptoms of Hallervorden-Spatz syndrome?

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

20

Marchiafava-Bignami disease

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

Progressive relapsing course of MS

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

21

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

Fatigue - affects up to 90%

22

Charcot's triad

Nystagmus, dysarthria, tremor (associated with cerebellar dysfx)

23

Pathology of MS

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

23

Pure word mutism

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

25

Risk factors for central pontine myelinolysis

Alcoholism, liver disease, malnutrition

27

3 major groups of white matter pathways

Projection, commissural, association

29

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

6-8x

31

Transsynaptic degeneration

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

32

Common affective/behavioral problems in MS

Depression, personality changes, anxiety

33

Most common cognitive deficits in MS

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

34

Disconnection syndrome (Geschwind)

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

35

Outcomes in childhood-onset MS

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

Proposed explanations for MS

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

39

How does MS risk vary by race/ethnicity?

Predilection for whites; Asian & black populations have low risk

40

Transverse myelitis

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

41

Visual changes seen in MS

Optic neuritis, intranuclear ophthalmoplegia, Marcus Gunn pupil

43

Which hemisphere has a greater proportion of white matter?

Right

44

Overall prevalence of cognitive impairment in MS

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

45

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

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

46

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

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

47

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

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

Multiple sclerosis

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

Retrobulbar neuritis

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

50

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

Prevalence decreases systematically as latitude of habitation nears Equator