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Incorporating New Diagnostic Schemas, Genetics, and Proteinopathy into the Evaluation of Frontotemporal Degeneration

Chow, Tiffany W. MD; Alobaidy, Ammar A. MD

CONTINUUM: Lifelong Learning in Neurology: April 2013 - Volume 19 - Issue 2, Dementia - p 438–456
doi: 10.1212/01.CON.0000429173.35439.9c
Review Articles

Purpose of Review: Within the continuously growing body of knowledge in the field of dementia, frontotemporal degeneration stands out in importance as the second most common cause of early-onset dementia after Alzheimer disease. Neurologists, neuropsychologists, and speech pathologists are particularly involved in the diagnosis and recognition of etiologies for patients with deficits in frontal lobe function and language.

Recent Findings: The recent discovery of a novel mutant gene (C9ORF72) and the new nomenclature adopted for subclassification have significantly promoted our understanding of this disorder.

Summary: This article relates the most recent consensus criteria for diagnosis of the two forms of frontotemporal degeneration (ie, behavioral and primary progressive aphasia variants) to basic neurologic principles and remind clinicians of the neuropsychiatric and neuroradiologic components that clarify frontotemporal degeneration diagnoses and guide management.

Address correspondence to Dr Tiffany W. Chow, Baycrest Rotman Research Institute, 3560 Bathurst Street, 8th Floor Brain Health Complex, Toronto, ON M6A 2E1, Canada,

Relationship Disclosure: Dr Chow has served as an independent medical examiner and medicolegal consultant to Lesser & Associates. Dr Alobaidy reports no disclosure.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Chow and Alobaidy discuss the use of medications for the treatment of behavioral and psychiatric symptoms of dementia, none of which carry indications from the US Food and Drug Administration. The text cites reviews of clinical trials in frontotemporal degeneration as applicable, as well as case reports, but clinicians are urged to specify that any such use of medications should be openly discussed with patients and substitute decision makers as off-label prescriptions.

© 2013 American Academy of Neurology
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