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Neurology > Demyelination & Dementia > Flashcards

Demyelination & Dementia Flashcards

1
Q

what 2 structures are damaged during the process of demyelination?

A

myelin sheath itself

oligodendrocytes / schwann cells which form myelin sheaths

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2
Q

what is the primary demyelinating disease process in adults?

A

MS

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3
Q

what primary demyelinating diseases are more common in children?

A

acute disseminated encephalomyelitis (self limiting)

acute haemorrhagic leukoencephalitis (rapidly fatal)

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4
Q

what are secondary causes of demyelination?

A

viral eg progressive multifocal leukoencephalopathy (PML)

metabolic eg central pontine myelinosis

toxic agents eg carbon monoxide

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5
Q

why does MS present as hyperintense lesions in the white matter on MRI?

A

most axons are concentrated in the white matter

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6
Q

what CSF sign is indicative of MS?

A

IgG oligoclonal bands in CSF

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7
Q

what focal neurological signs do patients with MS present if they have optic nerve lesions?

A

optic neuritis - unilateral visual impairment

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8
Q

what focal neurological signs do MS patients present with if they have lesions in spinal cord?

A

motor or sensory deficit in trunk and limbs
spasticity
bladder dysfunction

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9
Q

what focal neurological signs are present in MS patients with brainstem lesions?

A

cranial nerve signs
ataxia
nystagmus
internuclear ophthalmoplegia

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10
Q

describe the appearance of plaques in MS?

A 
well circumscribed 
irregular shaped 
glassy / translucent experience
vary in size 
non-anatomical distribution
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11
Q

what areas of the brain are plaques normally found in?

A 
adjacent to lateral ventricles 
corpus callosum 
optic nerves 
brainstem 
cerebellum
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12
Q

explain the histological findings in an acute active MS lesion?

A

microglia (digest myelin like macrophage)
inflammatory cells around blood vessels
plaques grow in a perivascular distribution

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13
Q

describe the histological findings in a chronic or inactive MS lesion?

A

gliosis (proliferation of glial cells)

less oligodendrocytes and myelinated axons present

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14
Q

what is the difference in the macroscopic appearance of active and inactive MS lesions?

A

active = ill defined edge

inactive = well demarcated

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15
Q

give examples of degenerative diseases of the cerebral cortex?

A

alzheimer’s disease

CJD

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16
Q

what neurodegenerative diseases originate in the basal ganglia and brainstem?

A

parkinson disease
progressive supranuclear palsy
huntington disease

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17
Q

where else can neurodegenerative disease present aside from the cerebral cortex, brainstem and basal ganglia?

A

cerebellum (freidrich’s ataxia)

motor neurones (MND)

18
Q

dementia is an ageing process and not pathological - true or false?

A

false

dementia is not part of the normal ageing process

19
Q

give examples of primary dementia?

A

alzheimer’s disease

lewy body dementia

20
Q

give examples of causes of secondary dementia?

A

multi infarct (vascular) dementia
infection (HIV, syphillis)
trauma
metabolic

21
Q

what symptoms can alzheimers progress to show in the later stages?

A

disorientation
worsened memory loss
aphasia
profound disability/immobility

22
Q

patients with alzheimers usually die of the disease - true or false?

A

false

death usually occurs due to secondary cause ie bronchopneumonia

23
Q

describe the macroscopic appearance of the brain in alzheimers dementia?

A

cortical atrophy in frontal, temporal and parietal lobes (occipital, brainstem and cerebellum spared)

widening of sulci / narrowing of gyri

dilation of ventricles

24
Q

what microscopic features are typical of alzheimers disease?

A

neuronal loss (+ astrocyte proliferation = gliosis)

neurofibrillary tangles (in hippocampus and temporal lobe)

neuritic plaques due to amyloid angiopathy

25
Q

why is alzheimers disease thought to be more common in those with down syndrome (trisomy 21)?

A

genetically have more of amyloid protein precursor (on chromosome 21) = more at risk of amyloid angiopathy

26
Q

describe the microscopic appearance of amyloid angiopathy?

A

extracellular eosinophils
polymerised beta pleated sheats
stains with congo red
disrupts BBB - local oedema

27
Q

what is lewy body dementia?

A 
progressive dementia 
hallucinations 
fluctuating levels of attention / cognition 
fluctuation on day to day basis 
features of parkinsonism
28
Q

what are the clinical features of parkinsons disease?

A 
loss of facial expression 
stooping 
shuffling gait 
slow initiation of movements 
stiffness
pill rolling tremor
29
Q

where in the brain does lewy body dementia cause degeneration?

A

in the substantia nigra (similar to parkinsons)

30
Q

describe how lewy body dementia appears microscopically?

A

loss of pigmented neurones
reactive gliosis
remaining neurones may show lewy bodies

31
Q

when are patients with huntingtons disease usually diagnosed and when does dementia develop?

A

usually diagnosed between 35-50

dementia presents later in disease process

32
Q

how does huntington’s dementia appear macroscopically in brain?

A

atrophy of basal ganglia - caudate nucleus and putamen

cortical atrophy occurs later

33
Q

how does huntingtons disease appear microscopically in the brain?

A

atrophy of striatal neurones in basal ganglia

astrocytic gliosis

34
Q

what condition is responsible for fronto-temporal dementia??

A

pick’s disease

35
Q

when does pick’s disease usually present?

A

commencing in middle life (usually between 50 and 60 years)

36
Q

what symptoms do patients with pick’s disease get (relating to damage of frontal / temporal lobes)?

A

personality and behavioural change
speech and communication problems
changes in eating habits
reduced attention span

37
Q

picks disease is naturally progressive - how many years do patients normally live with this?

A

2-10 years (average = 7)

38
Q

how does pick’s disease appear macroscopically?

A

extreme atrophy of cerebral cortex in frontal and later in temporal lobes

brain weight <1kg

39
Q

how does pick’s disease appear microscopically and what are hallmarks of disease?

A

neuronal loss and gliosis

hallmarks - picks cells (swollen neurones), intracytoplasmic filamentous inclusions (picks bodies)

40
Q

what groups of patients are most likely to get multi infarct dementia?

A

men > women

>60 most common but also seen in middle aged hypertensives

41
Q

what clues can help to distinguish multi-infarct dementia from alzheimers disease?

A

abrupt onset
stepwise progression
history or hypertension / stroke
evidence of stroke on CT / MRI

Neurology (32 decks)

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