Document Detail

Syndromes of nonfluent primary progressive aphasia: a clinical and neurolinguistic analysis.
MedLine Citation:
PMID:  20713949     Owner:  NLM     Status:  MEDLINE    
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.
Jonathan D Rohrer; Martin N Rossor; Jason D Warren
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neurology     Volume:  75     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-08-17     Completed Date:  2010-09-13     Revised Date:  2014-02-19    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  603-10     Citation Subset:  AIM; IM    
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MeSH Terms
Aged, 80 and over
Aphasia, Broca / physiopathology
Cognition Disorders / complications
Cohort Studies
Cross-Sectional Studies
Executive Function / physiology*
Middle Aged
Neurologic Examination
Neuropsychological Tests
Primary Progressive Nonfluent Aphasia / classification,  physiopathology*,  psychology*
Severity of Illness Index
Speech / physiology
Verbal Learning / physiology
Grant Support
G0401247//Medical Research Council; //Medical Research Council; //Wellcome Trust

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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