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Basic Neurosciences · Paper A

Pick's disease

The idea

A 55-year-old man becomes tactless, impulsive and emotionally cold over two years, while his bedside memory testing still looks respectable — the personality change arrived first, and that order is the whole clue. When personality, inhibition or language fails before memory does, the disease is pointing toward the frontal and anterior temporal lobesThe part of the brain by the ears, involved in hearing, language and memory.Read more →, not the hippocampusA brain structure central to forming new memories.Read more →.

How it works

Frontotemporal degeneration progressively damages frontal and anterior temporal networks that regulate social conduct, motivation, empathy and language. Pick disease is a specific pathological form in which abnormal tau (a protein that normally stabilises the internal scaffolding of a neuron) accumulates in intraneuronal Pick bodies (rounded clumps of misfolded protein that build up inside the neuron's own cell body, rather than outside it); the broader clinical syndrome is frontotemporal dementia, which can also present as a primary progressive aphasia (a gradual loss of language ability, arising before any other cognitive domain is affected).

How to spot it

Look for onset in midlife, progressive disinhibition, apathy, loss of empathy, stereotyped behaviour, dietary change or a primary progressive language syndrome.

Tell it apart

  • Frontotemporal dementia vs Alzheimer disease: early behaviour or language change contrasts with early episodic-memory failure.
  • Frontotemporal dementia vs Lewy body dementia: disinhibition contrasts with recurrent visual hallucinationsSeeing, hearing, or otherwise sensing things that are not really there.Read more →, fluctuations and parkinsonism.
  • Pick disease pathology vs clinical FTD, Pick bodies name a specific pathology, whereas FTD is the broader clinical syndrome.

Watch for

Trap, using Pick disease as an exact synonym for every frontotemporal dementia. Resolution: the historical labels overlap, but strict modern usage reserves Pick disease for characteristic Pick-body pathology.

Trap, excluding dementia because memory is initially preserved. Resolution: frontotemporal syndromes can begin with behaviour or language.

Use it again

Identify the first cognitive domain to fail, then match pathology: behaviour/language points frontotemporal; memory points Alzheimer; hallucinationsSeeing, hearing, or otherwise sensing things that are not really there.Read more → and parkinsonism point Lewy body disease.

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