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Pick's disease (PiD)

Fronto-Temporal Degeneration


by André Delacourte

General outline

Pick's disease (PiD) is a neuro-degenerative disorder that belongs to the group of fronto-temporal dementia. This is a rare type of presenile dementia, with sometimes a familial character.

PiD is characterised by specific lesions named Pick bodies that are found in the hippocampus and in the neocortex. Pick bodies are made up of tau proteins.

Pick's disease is a form of dementia characterised by a slow deterioration of social skills and changes in personality, along with impairment of intellect, memory, and language.

Synonym

Dementia with Lobar Atrophy and Neuronal Cytoplasmic Inclusions , Diffuse Degenerative Cerebral Disease , Lobar Atrophy of the Brain, Pick Disease of the Brain

Symptoms and course

One of the first and most important warning signs of FTD is insensitivity to other people. This could be linked to difficulty in identifying emotions shown by their relatives.

Although the disease varies greatly in the way it affects individuals, there is a common core of symptoms among patients, which may be present at different stages of the disease. These symptoms include loss of memory, lack of spontaneity, difficulty in thinking or concentrating, and disturbances of speech. Other symptoms include gradual emotional dullness, loss of moral judgment, and progressive dementia.

Pick's disease usually has its onset between the ages of 40 and 60 years, but extreme cases have been reported with onset as early as 21 and as late as 80. In an analysis of 18 cases of Pick's disease average survival was reported to be 6.3 years for men and 8.4 year for women.

Causes and risk factors

A specific neurofibrillary degeneration, revealed by the presence of Pick bodies in the hippocampus and fronto-temporal cortex and an abnormal processing of tau proteins, is associated to this pathology.

Frequency

PiD defined as a disease with Pick bodies is extremely rare. This disease occurs sporadically. But some FTDP-17 with specific mutations have also Pick bodies, showing that there are probably two subsets for this rare disease.

Diagnostic procedures

Clinical with the specific features of fronto-temporal impairment. Imaging reveals a fronto-temporal atrophy and hyperperfusion.

Care and treatment

Symptomatic for disinhibition and behavioural problems. Antidepressants for apathy. Cholinesterase inhibitors are not useful. Trazodone for agitation. No prevention.

Ongoing research / clinical trials

Like most fronto-temporal dementia, tau proteins seem to be involved in the aetiology of the disease. Many laboratories are working on the molecular pathology of tau proteins.


Available services

The Pick's Disease Support Group Brooksby Close Oadby Leicester LE2 5AB Tel : 0116 271 1414 Fax : 0870 706 0958 carol@pdsg.org.uk www.pdsg.org.uk


References

  • Dickson, D. W. (2001). "Neuropathology of Pick's disease." Neurology 56(11 Suppl 4): S16-20.
  • Lebert F, Stekke W, Hasenbroekx Ch, Pasquier F.Fronto-temporal dementia. A randomized, controlled trial with trazodone. Dem Cogn Disord. (in press)

 

 
 

Last Updated: Friday 09 October 2009

 

 
  • Acknowledgements

    This information was gathered in the framework of the European Commission financed project "Rare forms of dementia". Neither the European Commission nor any person acting on its behalf is responsible for any use that might be made of the following information.
  • European Union
 
 

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