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Clinical course of neuropathologically confirmed frontal-variant Alzheimer's disease

Abstract

Background A 66-year-old man presented with a 3-year history of personality changes marked by increasing apathy, social withdrawal and deficits in complex attention, and a 1-year history of progressive memory problems and difficulties in planning and carrying out complex tasks.

Investigations Three neuropsychological examinations over 2 years, neurological examination, routine laboratory tests, brain MRI, single-photon emission CT scan, genetic analyses, and neuropathological examination.

Diagnosis A clinical diagnosis of frontal-variant frontotemporal dementia was superseded by postmortem neuropathological evidence, which established a diagnosis of frontal-variant Alzheimer's disease.

Management The patient and his spouse were referred for counseling, and the patient was referred for follow-up examinations.

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Figure 1: Clinical course of a patient with neuropathologically diagnosed frontal-variant Alzheimer's disease.
Figure 2: Neuropathological findings of frontal-variant Alzheimer's disease.
Figure 3: Neurofibrillary tangle and neuritic plaque load in various brain regions of a patient with frontal-variant Alzheimer's disease.
Figure 4: Selected key clinical features of a patient with autopsy confirmed fvAD that overlap with those features typical of AD and fvFTD.

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Acknowledgements

The authors thank Prof Dr Stefan Kneifel for the SPECT analysis and Professor Christophe Büla for the helpful discussion. KI Taylor was supported by the Swiss Foundation for Ageing Research (financed by the Loterie Romande) and the Swiss Alzheimer's Association. Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Correspondence to Markus Tolnay.

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The authors declare no competing financial interests.

Supplementary information

Supplementary Table 1

Clinical assessment of the patient's behavior as rated by his spouse on the Nurses' Observation Scale for Geriatric Patients. (DOC 34 kb)

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Taylor, K., Probst, A., Miserez, A. et al. Clinical course of neuropathologically confirmed frontal-variant Alzheimer's disease. Nat Rev Neurol 4, 226–232 (2008). https://doi.org/10.1038/ncpneuro0746

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