The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants.
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.
Bonner
Michael F
MF
Department of Neurology, 2 Gibson, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA. michafra@mail.med.upenn.edu
Ash
Sharon
S
Grossman
Murray
M
D001039:Aphasia, Broca / Q000175:diagnosis; D018888:Aphasia, Primary Progressive / Q000145:classification* / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D006801:Humans; D012660:Semantics; D013060:Speech
Neurology. 2007 Jan 30;68(5):369-75
17261685
Ann Neurol. 2003 Jan;53(1):35-49
12509846
Brain. 2010 Jan;133(Pt 1):286-99
19759202
Arch Neurol. 2010 Apr;67(4):483-9
20385916
Arch Neurol. 2011 Feb;68(2):248-50
21320991
Brain. 2009 Sep;132(Pt 9):2553-65
19506067
Brain. 2006 Jun;129(Pt 6):1385-98
16613895
Neuropsychology. 2006 Sep;20(5):529-38
16938015
Neurology. 2010 Aug 17;75(7):595-602
20713948
Arch Neurol. 2009 Dec;66(12):1545-51
20008661
N Engl J Med. 2003 Oct 16;349(16):1535-42
14561797
Brain. 2009 Oct;132(Pt 10):2734-46
19696033
Brain Lang. 2010 Apr;113(1):13-20
20074786
Neurology. 2008 Jan 1;70(1):25-34
18166704
Neurology. 2003 Oct 28;61(8):1108-13
14581673
Annu Rev Psychol. 2007;58:25-45
16968210
Cogn Neuropsychol. 2009 Sep;26(6):568-79
20183015
Nat Rev Neurol. 2010 Feb;6(2):88-97
20139998
J Int Neuropsychol Soc. 2003 Jul;9(5):710-9
12901777
J Neurolinguistics. 2009 Jul 1;22(4):370-383
22180700
J Neurol Neurosurg Psychiatry. 2009 Jul;80(7):808-9
19531690
Neuropsychology. 2007 Jan;21(1):9-19
17201526
Ann Neurol. 2001 Apr;49(4):425-32
11310619
Arch Neurol. 1990 Dec;47(12):1329-35
2252450
J Neurolinguistics. 2008 Sep;21(5):418-432
19727332
Neurology. 2010 Aug 17;75(7):588-94
20713947
Neuropsychology. 2009 Jul;23(4):492-9
19586212
Neuroimage. 2010 Jan 1;49(1):984-93
19679189
Nat Rev Neurosci. 2007 Dec;8(12):976-87
18026167
Brain. 2000 Mar;123 Pt 3:484-98
10686172
Lancet Neurol. 2007 Nov;6(11):1004-14
17945154
Neurology. 2007 Jan 16;68(3):238-9
17224582
Ann Neurol. 2006 Jan;59(1):156-65
16374817
Neurology. 2008 Oct 28;71(18):1445-51
18955688
Brain. 2004 Jul;127(Pt 7):1479-87
15090478
Neurology. 2008 Oct 14;71(16):1227-34
18633132
Brain. 2006 Nov;129(Pt 11):3066-80
17071925
J Neurolinguistics. 2007 Nov;20(6):482-494
18978927
Neurocase. 2004 Feb;10(1):3-18
15849155
Nature. 1996 Nov 14;384(6605):159-61
8906789
Med Image Comput Comput Assist Interv. 2007;10(Pt 2):303-10
18044582
Alzheimer Dis Assoc Disord. 2007 Oct-Dec;21(4):S23-30
18090419
Ann Neurol. 2004 Mar;55(3):335-46
14991811
Neurology. 2009 May 5;72(18):1562-9
19414722
Brain Lang. 2009 Sep;110(3):121-34
19477502
Arch Neurol. 2007 Nov;64(11):1601-9
17998442
Neurology. 2004 Apr 13;62(7):1163-9
15079017