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Behavioral variant of frontotemporal dementia mimicking Huntington's disease

Published online by Cambridge University Press:  22 February 2010

T. Rune Nielsen ,
Peter Bruhn ,
Jørgen E. Nielsen  and
Lena E. Hjermind
T. Rune Nielsen*
Affiliation:
Memory Disorders Research Group, Neuroscience Centre, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark
Peter Bruhn
Affiliation:
Department of Neurology, Copenhagen University Hospital Glostrup, Denmark
Jørgen E. Nielsen
Affiliation:
Memory Disorders Research Group, Neuroscience Centre, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark Section of Neurogenetics, Institute of Cellular and Molecular Medicine, University of Copenhagen, Copenhagen, Denmark
Lena E. Hjermind
Affiliation:
Memory Disorders Research Group, Neuroscience Centre, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark Section of Neurogenetics, Institute of Cellular and Molecular Medicine, University of Copenhagen, Copenhagen, Denmark
*
Correspondence should be addressed to: T. Rune Nielsen, Memory Disorders Research Group, Section 7661, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen Ø, Denmark. Phone: +45 3545 8759; Fax: +45 3545 5323. Email: rune.nielsen@rh.regionh.dk.
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Abstract

Behavioral changes and cognitive decline are the core clinical manifestations in the behavioral variant of frontotemporal dementia (bv-FTD). The behavioral changes may include characteristic stereotypic movements. These movements, although without clear purpose, are not involuntary. Involuntary movements are usually not seen in FTD.

Two patients with involuntary choreoathetoid movements but otherwise presenting a bv-FTD-phenotype were referred and Huntington's disease (HD) was suspected. The diagnoses of bv-FTD were made after comprehensive assessment and exclusion of other diagnoses, including HD and Huntington's disease-like (HDL) phenotypes. Although a definite diagnosis will require neuropathological confirmation, we conclude that a HDL phenotype may be part of the clinical spectrum of the bv-FTD phenotype.

Keywords

frontotemporal dementiafrontotemporal lobar degenerationchoreahereditary dementiaHuntington's diseaseHuntington's disease-like phenotype
Type
Case Report
Information
International Psychogeriatrics , Volume 22 , Issue 4 , June 2010 , pp. 674 - 677
Copyright
Copyright © International Psychogeriatric Association 2010

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