Pick’s disease
Definition and pathology
Pick’s disease is a clinicopathological subtype of frontotemporal dementia (FTD). Neuropathologically it is a tauopathy characterised by neuronal accumulation of abnormal tau protein forming spherical, silver‑staining inclusions called Pick bodies, with predominant degeneration of the frontal and anterior temporal lobes.
Epidemiology and genetics
- Typical onset is in mid‑life, commonly between 40 and 60 years, although earlier or later presentations occur.
- A proportion of FTD cases are familial. Examples of implicated genes include MAPT, GRN and C9orf72.
- Consider referral to clinical genetics when there is a strong family history of early dementia, very early onset, or other features suggesting hereditary FTD.
Clinical features
The behavioural variant (bvFTD) phenotype is most typical in Pick’s disease. Early and prominent personality and social changes help distinguish it from Alzheimer’s disease.
Common features:
- Disinhibition, tactlessness, impulsivity, altered moral judgement and reduced empathy.
- Apathy, social withdrawal, loss of motivation, or compulsive/ritualised behaviours such as pacing and wandering.
- Executive dysfunction, including poor planning, impaired abstraction, set‑shifting difficulties and reduced concentration.
- Language problems and global cognitive decline emerge as the disease progresses.
- Episodic memory is often relatively preserved early on; lack of insight (anosognosia) is common.