Hostname: page-component-7c8c6479df-r7xzm Total loading time: 0 Render date: 2024-03-27T02:40:22.176Z Has data issue: false hasContentIssue false

Behavioral variant of frontotemporal dementia mimicking Huntington's disease

Published online by Cambridge University Press:  22 February 2010

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.

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.

Type
Case Report
Copyright
Copyright © International Psychogeriatric Association 2010

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Brunnström, H., Gustafson, L., Passant, U. and Englund, E. (2009). Prevalence of dementia subtypes: a 30-year retrospective survey of neuropathological reports. Archives of Gerontology and Geriatrics, 49, 146149.CrossRefGoogle ScholarPubMed
Cairns, N. J. et al. (2007). Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathologica, 114, 522.CrossRefGoogle Scholar
Dubois, B., Slachevsky, A., Pillon, B., Beato, R., Villalponda, J. M. and Litvan, I. (2005). Applause sign helps to discriminate PSP from FTD and PD. Neurology, 64, 21322133.CrossRefGoogle ScholarPubMed
Kertesz, A., Blair, M., McMonagle, P. and Munoz, D. G. (2007). The diagnosis and clinical course of frontotemporal dementia. Alzheimer's Disease and Associated Disorders, 21, 155163.CrossRefGoogle ScholarPubMed
Larner, A. J. (2006). Frontal variant Alzheimer's disease: a reappraisal. Clinical Neurology and Neurosurgery, 108, 705708.CrossRefGoogle ScholarPubMed
Lindquist, S. G., Brændgaard, H., Svenstrup, K., Isaacs, A. M. and Nielsen, J. E., on behalf of the FReJA Consortium. (2008). Frontotemporal dementia linked to chromosome 3 (FTD-3) – current concepts and the detection of a previously unknown branch of the Danish FTD-3 family. European Journal of Neurology, 15, 667670.CrossRefGoogle ScholarPubMed
Lindquist, S. G., Schwartz, M., Batbayli, M., Waldemar, G. and Nielsen, J. E. (2009). Genetic testing in familial AD and FTD: mutation and phenotype spectrum in a Danish cohort. Clinical Genetics, 76, 205209.CrossRefGoogle Scholar
Saddichha, S. and Pandey, V. (2008). Alzheimer's and non-Alzheimer's dementia: a critical review of pharmacological and nonpharmacological strategies. American Journal of Alzheimer's Disease and Other Dementias, 23, 150161.CrossRefGoogle ScholarPubMed
Shigenobu, K. et al. (2002). The Stereotypy Rating Inventory for frontotemporal lobar degeneration. Psychiatry Research, 110, 175187.CrossRefGoogle ScholarPubMed
Wu, L. J., Sitburana, O., Davidson, A. and Jankovic, J. (2008). Applause sign in Parkinsonian disorders and Huntington's disease. Movement Disorders, 23, 23072311.CrossRefGoogle ScholarPubMed