Sporadic and familial dementia with ubiquitin-positive tau-negative inclusions: Clinical features of one histopathological abnormality underlying frontotemporal lobar degeneration

Alison K. Godbolt, Keith A. Josephs, Tamas Revesz, Elizabeth K. Warrington, Peter Lantos, Andrew King, Nick C. Fox, Safa Al Sarraj, Janice Holton, Lisa Cipolotti, M. Nadeem Khan, Martin N. Rossor

Research output: Contribution to journalReview article

27 Scopus citations

Abstract

Background: Frontotemporal lobar degeneration comprises a group of diseases with clinical presentations and underlying histopathologies that overlap. Familial disease occurs in up to 50% of frontotemporal lobar degeneration cases. One of several underlying histopathological abnormalities is of ubiquitin-positive tau-negative inclusions, similar to those in motor neuron disease. Objective: To compare clinical features of familial and sporadic cases in this pathological subgroup. Design and Patients: Case note review of dementia patients with ubiquitin-positive tau-negative inclusion pathological abnormalities proven by autopsy. Setting: United Kingdom tertiary referral center. Main Outcome Measures: Analysis of clinical features. Results: Eleven familial cases (autosomal dominant) and 18 sporadic cases were identified. Most familial case patients presented with behavioral disturbances similar to those seen in sporadic behavioral cases. Semantic dementia was only seen in sporadic cases. Atypical features occurred in a minority. Sporadic and familial behavioral cases showed no differences in age at onset or disease duration. Neuropsychological test results revealed frontal or temporal deficits in most, but unexpected early parietal deficits in 1. Conclusions: Behavioral features in familial and sporadic cases were similar, but semantic dementia only occurred in sporadic cases. Diagnostic confusion with Alzheimer disease and corticobasal degeneration occurred in some cases.

Original languageEnglish (US)
Pages (from-to)1097-1101
Number of pages5
JournalArchives of neurology
Volume62
Issue number7
DOIs
StatePublished - Jul 1 2005

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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    Godbolt, A. K., Josephs, K. A., Revesz, T., Warrington, E. K., Lantos, P., King, A., Fox, N. C., Al Sarraj, S., Holton, J., Cipolotti, L., Khan, M. N., & Rossor, M. N. (2005). Sporadic and familial dementia with ubiquitin-positive tau-negative inclusions: Clinical features of one histopathological abnormality underlying frontotemporal lobar degeneration. Archives of neurology, 62(7), 1097-1101. https://doi.org/10.1001/archneur.62.7.1097