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Clinical Features of One Histopathological Abnormality Underlying Frontotemporal Lobar Degeneration

Alison K. Godbolt, MRCP; Keith A. Josephs, MST, MD; Tamas Revesz, MD, FRCPath; Elizabeth K. Warrington, PhD, DSc; Peter Lantos, MD, PhD, DSc, FRCPath; Andrew King, MRCPath; Nick C. Fox, MD, FRCP; Safa Al Sarraj, FRCPath; Janice Holton, PhD, MRCPath; Lisa Cipolotti, PhD; M. Nadeem Khan, PhD; Martin N. Rossor, MD, FRCP

Arch Neurol. 2005;62:1097-1101.

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.

Author Affiliations: Dementia Research Centre (Drs Godbolt, Josephs, Warrington, Fox, and Rossor) and Department of Neuropathology (Drs Revesz and Holton), Institute of Neurology, University College London, London, England; Department of Neurology, Mayo Clinic, Rochester, Minn (Dr Josephs); Department of Neuropathology, Institute of Psychiatry, King’s College, London, England (Drs Lantos, King, Al Sarraj, and Khan); Department of Neuropsychology, National Hospital for Neurology and Neurosurgery, London, England (Dr Cipolotti).

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