BACKGROUND: Despite recent work, the nosology of nonfluent primary progressive aphasia (PPA) remains unresolved. METHODS: We describe a clinical and neurolinguistic cross-sectional analysis of a cohort of 24 patients with nonfluent PPA. Patients were initially classified based on analysis of spontaneous speech into 4 groups: apraxia of speech (AOS)/agrammatism (10 patients); AOS/no agrammatism (4 patients); no AOS/agrammatism (3 patients); no AOS/no agrammatism (7 patients). These groups were further characterized using a detailed neurolinguistic and neuropsychological battery. Parkinsonism was present in 3/10 patients in the AOS/agrammatism group. All patients in the no AOS/agrammatism group had mutations in the progranulin (GRN) gene, while 5/7 cases in the no AOS/no agrammatism group had CSF findings compatible with Alzheimer disease. RESULTS: The groups without AOS showed more severe neurolinguistic impairments for a given disease stage, and sentence comprehension, speech repetition, and reading were impaired in all groups. Prolonged word-finding pauses and impaired single word comprehension were salient features in the no AOS/agrammatism group. Additional impairments of executive function and praxis were present in both groups with agrammatism, and impaired episodic memory was a feature of the no AOS/no agrammatism group. CONCLUSION: PPA with AOS is aligned with the syndrome previously designated progressive nonfluent aphasia; agrammatism may emerge as the syndrome evolves, or alternatively, the pure AOS group may be pathophysiologically distinct. PPA without AOS resembles the syndrome designated logopenic/phonologic aphasia; however, there is evidence for a distinct subsyndrome of GRN-associated aphasia. The findings provide a rationale for further longitudinal studies with pathologic correlation.
BACKGROUND: Despite recent work, the nosology of nonfluent primary progressive aphasia (PPA) remains unresolved. METHODS: We describe a clinical and neurolinguistic cross-sectional analysis of a cohort of 24 patients with nonfluent PPA. Patients were initially classified based on analysis of spontaneous speech into 4 groups: apraxia of speech (AOS)/agrammatism (10 patients); AOS/no agrammatism (4 patients); no AOS/agrammatism (3 patients); no AOS/no agrammatism (7 patients). These groups were further characterized using a detailed neurolinguistic and neuropsychological battery. Parkinsonism was present in 3/10 patients in the AOS/agrammatism group. All patients in the no AOS/agrammatism group had mutations in the progranulin (GRN) gene, while 5/7 cases in the no AOS/no agrammatism group had CSF findings compatible with Alzheimer disease. RESULTS: The groups without AOS showed more severe neurolinguistic impairments for a given disease stage, and sentence comprehension, speech repetition, and reading were impaired in all groups. Prolonged word-finding pauses and impaired single word comprehension were salient features in the no AOS/agrammatism group. Additional impairments of executive function and praxis were present in both groups with agrammatism, and impaired episodic memory was a feature of the no AOS/no agrammatism group. CONCLUSION: PPA with AOS is aligned with the syndrome previously designated progressive nonfluent aphasia; agrammatism may emerge as the syndrome evolves, or alternatively, the pure AOS group may be pathophysiologically distinct. PPA without AOS resembles the syndrome designated logopenic/phonologic aphasia; however, there is evidence for a distinct subsyndrome of GRN-associated aphasia. The findings provide a rationale for further longitudinal studies with pathologic correlation.
Authors: F Hulstaert; K Blennow; A Ivanoiu; H C Schoonderwaldt; M Riemenschneider; P P De Deyn; C Bancher; P Cras; J Wiltfang; P D Mehta; K Iqbal; H Pottel; E Vanmechelen; H Vanderstichele Journal: Neurology Date: 1999-05-12 Impact factor: 9.910
Authors: V Deramecourt; F Lebert; B Debachy; M A Mackowiak-Cordoliani; S Bombois; O Kerdraon; L Buée; C-A Maurage; F Pasquier Journal: Neurology Date: 2009-11-25 Impact factor: 9.910
Authors: Maria Luisa Gorno-Tempini; Nina F Dronkers; Katherine P Rankin; Jennifer M Ogar; La Phengrasamy; Howard J Rosen; Julene K Johnson; Michael W Weiner; Bruce L Miller Journal: Ann Neurol Date: 2004-03 Impact factor: 10.422
Authors: Jonathan D Rohrer; William D Knight; Jane E Warren; Nick C Fox; Martin N Rossor; Jason D Warren Journal: Brain Date: 2007-10-18 Impact factor: 13.501
Authors: Maya L Henry; H Isabel Hubbard; Stephanie M Grasso; Heather R Dial; Pélagie M Beeson; Bruce L Miller; Maria Luisa Gorno-Tempini Journal: J Speech Lang Hear Res Date: 2019-08-07 Impact factor: 2.297
Authors: Edmond Teng; Tritia R Yamasaki; Michelle Tran; Julia J Hsiao; David L Sultzer; Mario F Mendez Journal: Dement Geriatr Cogn Disord Date: 2013-12-31 Impact factor: 2.959
Authors: Murray Grossman; John Powers; Sherry Ash; Corey McMillan; Lisa Burkholder; David Irwin; John Q Trojanowski Journal: Brain Lang Date: 2012-12-04 Impact factor: 2.381