Behavioral variant of frontotemporal dementia mimicking Huntington's disease.
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.
Nielsen
T Rune
TR
Memory Disorders Research Group, Neuroscience Centre, Copenhagen University Hospital Rigshospitalet, DK-2100 Copenhagen, Denmark. rune.nielsen@rh.regionh.dk
Bruhn
Peter
P
Nielsen
Jørgen E
JE
Hjermind
Lena E
LE
D000368:Aged; D003072:Cognition Disorders / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D057180:Frontotemporal Dementia / Q000175:diagnosis*; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis*; D008875:Middle Aged; D009483:Neuropsychological Tests; D010641:Phenotype; D012720:Severity of Illness Index; D012919:Social Behavior; D019956:Stereotypic Movement Disorder / Q000175:diagnosis*