title: Incorporating new diagnostic schemas, genetics, and proteinopathy into the evaluation of frontotemporal degeneration.
abstract: OBJECTIVE
  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.
  
  
  RESULTS
  The recent discovery of a novel mutant gene (C9ORF72) and the new nomenclature adopted for subclassification have significantly promoted our understanding of this disorder.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Baycrest Rotman Research Institute, 3560 Bathurst Street, 8th Floor Brain Health Complex, Toronto, ON M6A 2E1, Canada. tchow@research.baycrest.org
authors.forename: Tiffany W
authors.initials: TW
authors.lastname: Chow
authors.forename: Ammar A
authors.initials: AA
authors.lastname: Alobaidy
mesh_terms: D000368:Aged; D018888:Aphasia, Primary Progressive / Q000209:etiology; D001921:Brain / Q000473:pathology; D000073885:C9orf72 Protein; D018450:Disease Progression; D005260:Female; D057174:Frontotemporal Lobar Degeneration / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D005820:Genetic Testing; D006801:Humans; D008297:Male; D001523:Mental Disorders / Q000209:etiology; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D059906:Neuroimaging; D009483:Neuropsychological Tests; D011506:Proteins / Q000235:genetics* / Q000378:metabolism; D016875:tau Proteins / Q000235:genetics
chemical_list: D000073885:C9orf72 Protein; C568651:C9orf72 protein, human; C054369:MAPT protein, human; D011506:Proteins; D016875:tau Proteins
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