International Psychogeriatrics

Table of Contents - 01 June 2010 - Volume 22, Issue 04  

Case Report

Behavioral variant of frontotemporal dementia mimicking Huntington's disease

T. Rune Nielsena1 c1, Peter Bruhna2, Jørgen E. Nielsena1a3 and Lena E. Hjerminda1a3

a1 Memory Disorders Research Group, Neuroscience Centre, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark

a2 Department of Neurology, Copenhagen University Hospital Glostrup, Denmark

a3 Section of Neurogenetics, Institute of Cellular and Molecular Medicine, University of Copenhagen, Copenhagen, Denmark

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.

(Received November 04 2009)

(Revised November 27 2009)

(Revised January 05 2010)

(Accepted January 07 2010)

(Online publication February 22 2010)

Key words:

Correspondence:

c1 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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