title: The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants.
abstract: Primary progressive aphasia (PPA), typically resulting from a neurodegenerative disease such as frontotemporal lobar degeneration or Alzheimer's disease, is characterized by a progressive loss of specific language functions with relative sparing of other cognitive domains. Three variants of PPA are now recognized: semantic variant, logopenic variant, and nonfluent/agrammatic variant. We discuss recent work characterizing the neurolinguistic, neuropsychological, imaging and pathologic profiles associated with these variants. Improved reliability of diagnoses will be increasingly important as trials for etiology-specific treatments become available. We also discuss the implications of these syndromes for theories of language function.
authors.affiliation: Department of Neurology, 2 Gibson, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA. michafra@mail.med.upenn.edu
authors.forename: Michael F
authors.initials: MF
authors.lastname: Bonner
authors.forename: Sharon
authors.initials: S
authors.lastname: Ash
authors.forename: Murray
authors.initials: M
authors.lastname: Grossman
mesh_terms: D001039:Aphasia, Broca / Q000175:diagnosis; D018888:Aphasia, Primary Progressive / Q000145:classification* / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D006801:Humans; D012660:Semantics; D013060:Speech
references.citation: Neurology. 2007 Jan 30;68(5):369-75
references.pmid: 17261685
references.citation: Ann Neurol. 2003 Jan;53(1):35-49
references.pmid: 12509846
references.citation: Brain. 2010 Jan;133(Pt 1):286-99
references.pmid: 19759202
references.citation: Arch Neurol. 2010 Apr;67(4):483-9
references.pmid: 20385916
references.citation: Arch Neurol. 2011 Feb;68(2):248-50
references.pmid: 21320991
references.citation: Brain. 2009 Sep;132(Pt 9):2553-65
references.pmid: 19506067
references.citation: Brain. 2006 Jun;129(Pt 6):1385-98
references.pmid: 16613895
references.citation: Neuropsychology. 2006 Sep;20(5):529-38
references.pmid: 16938015
references.citation: Neurology. 2010 Aug 17;75(7):595-602
references.pmid: 20713948
references.citation: Arch Neurol. 2009 Dec;66(12):1545-51
references.pmid: 20008661
references.citation: N Engl J Med. 2003 Oct 16;349(16):1535-42
references.pmid: 14561797
references.citation: Brain. 2009 Oct;132(Pt 10):2734-46
references.pmid: 19696033
references.citation: Brain Lang. 2010 Apr;113(1):13-20
references.pmid: 20074786
references.citation: Neurology. 2008 Jan 1;70(1):25-34
references.pmid: 18166704
references.citation: Neurology. 2003 Oct 28;61(8):1108-13
references.pmid: 14581673
references.citation: Annu Rev Psychol. 2007;58:25-45
references.pmid: 16968210
references.citation: Cogn Neuropsychol. 2009 Sep;26(6):568-79
references.pmid: 20183015
references.citation: Nat Rev Neurol. 2010 Feb;6(2):88-97
references.pmid: 20139998
references.citation: J Int Neuropsychol Soc. 2003 Jul;9(5):710-9
references.pmid: 12901777
references.citation: J Neurolinguistics. 2009 Jul 1;22(4):370-383
references.pmid: 22180700
references.citation: J Neurol Neurosurg Psychiatry. 2009 Jul;80(7):808-9
references.pmid: 19531690
references.citation: Neuropsychology. 2007 Jan;21(1):9-19
references.pmid: 17201526
references.citation: Ann Neurol. 2001 Apr;49(4):425-32
references.pmid: 11310619
references.citation: Arch Neurol. 1990 Dec;47(12):1329-35
references.pmid: 2252450
references.citation: J Neurolinguistics. 2008 Sep;21(5):418-432
references.pmid: 19727332
references.citation: Neurology. 2010 Aug 17;75(7):588-94
references.pmid: 20713947
references.citation: Neuropsychology. 2009 Jul;23(4):492-9
references.pmid: 19586212
references.citation: Neuroimage. 2010 Jan 1;49(1):984-93
references.pmid: 19679189
references.citation: Nat Rev Neurosci. 2007 Dec;8(12):976-87
references.pmid: 18026167
references.citation: Brain. 2000 Mar;123 Pt 3:484-98
references.pmid: 10686172
references.citation: Lancet Neurol. 2007 Nov;6(11):1004-14
references.pmid: 17945154
references.citation: Neurology. 2007 Jan 16;68(3):238-9
references.pmid: 17224582
references.citation: Ann Neurol. 2006 Jan;59(1):156-65
references.pmid: 16374817
references.citation: Neurology. 2008 Oct 28;71(18):1445-51
references.pmid: 18955688
references.citation: Brain. 2004 Jul;127(Pt 7):1479-87
references.pmid: 15090478
references.citation: Neurology. 2008 Oct 14;71(16):1227-34
references.pmid: 18633132
references.citation: Brain. 2006 Nov;129(Pt 11):3066-80
references.pmid: 17071925
references.citation: J Neurolinguistics. 2007 Nov;20(6):482-494
references.pmid: 18978927
references.citation: Neurocase. 2004 Feb;10(1):3-18
references.pmid: 15849155
references.citation: Nature. 1996 Nov 14;384(6605):159-61
references.pmid: 8906789
references.citation: Med Image Comput Comput Assist Interv. 2007;10(Pt 2):303-10
references.pmid: 18044582
references.citation: Alzheimer Dis Assoc Disord. 2007 Oct-Dec;21(4):S23-30
references.pmid: 18090419
references.citation: Ann Neurol. 2004 Mar;55(3):335-46
references.pmid: 14991811
references.citation: Neurology. 2009 May 5;72(18):1562-9
references.pmid: 19414722
references.citation: Brain Lang. 2009 Sep;110(3):121-34
references.pmid: 19477502
references.citation: Arch Neurol. 2007 Nov;64(11):1601-9
references.pmid: 17998442
references.citation: Neurology. 2004 Apr 13;62(7):1163-9
references.pmid: 15079017