Frontotemporal Degeneration Flashcards

1
Q

definition of frontotemporal dementia

A

characterised by progressive degeneration of the frontal and/or temporal lobe

  • sparing of parietal and occipital lobe
  • degeneration of t
    he striatum
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2
Q

three main types of Frontotemporal lobar degeneration

A
  1. Frontotemporal dementia - pick’s disease
  2. Progressive non fluent aphasia - slow onset of language deficit overtime / difficulty with speech production
  3. Semantic dementia - progressive neurological disorder characterized by loss of semantic memory (
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3
Q

Presenting picture in Pick’s disease?

A
  1. personality change and social conduction problems - apathy, social inhibition and sexual inhibition
  2. increased appetite
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4
Q

macroscopic changes of Pick’s disease

A
  1. atrophy of frontal and temporal lobes
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5
Q

microscopic changes of Pick’s disease

A
  1. Pick bodies - spherical aggregations of tau protein
  2. Gliosis - non specific reactive change of glial cells in response to cns damage
  3. Neurofibrillary tangles
  4. Senile plaques - microscopic mass of fragmented and decaying nerve terminals
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6
Q

management of frontotemporal dementia

A

NICE do not recommend that AChE inhibitors or memantine are used in people with frontotemporal dementia

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7
Q

CPA

A

Here the chief factor is non fluent speech. They make short utterances that are agrammatic. Comprehension is relatively preserved.

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8
Q

Semantic dementia

A

Here the patient has a fluent progressive aphasia.

The speech is fluent but empty and conveys little meaning.

Unlike in Alzheimer’s memory is better for recent rather than remote events

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9
Q

What is preserved in frontotemporal lobar dementia?

A

Relatively preserved memory and visuospatial skills

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10
Q

Describe the onset of frontotemporal lobar dementia

A

Insidious onset

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11
Q

Age of onset of fronto temporal dementia?

A

Onset before 65

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12
Q

Main feature of frontotemporal lobar dementia

A

Personality change and social conduct problems

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13
Q

What are pick’s bodies

A

spherical aggregations of tau protein (silver-staining)

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