| Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia. | |
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MedLine Citation:
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PMID: 16093229 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Progressive supranuclear palsy (PSP) is a clinicopathological entity typically presenting as an akinetic rigid syndrome with early falls, axial rigidity, vertical supranuclear gaze palsy and levodopa resistance. Pathological features consist of tau deposition in neuronal and glial cells located mainly in subcortical and brainstem structures. Rare cases with the pathological diagnosis of atypical PSP have been described in which neocortical tau deposition is more widespread than what is usually seen in typical PSP. Progressive nonfluent aphasia (PNFA) is a syndrome characterized by spontaneous nonfluent speech and early preserved comprehension of language. Apraxia of speech (AOS) is a motor speech disorder that may be a feature of PNFA. We report the clinical and pathological findings of four cases that presented with features most consistent with PNFA predominated by AOS. Pathological features in these four cases included the typical features of PSP subcortically and in brainstem structures, but combined with tau-positive neuronal and glial pathology in the neocortex. Comprehensive semiquantitative analyses of tau burden including neurofibrillary tangles and pretangles, coiled bodies, tufted astrocytes and threads were undertaken in the four cases of atypical PSP and compared to 10 cases of typical PSP. Semiquantitative analysis demonstrated that in atypical PSP, the pathology shifts from subcortical grey and brainstem regions, commonly affected in typical PSP, towards neocortical regions. This shift in pathology accounts for the presentation of PNFA and AOS observed in our patients, as well as the lack of classic features of PSP. These cases demonstrate that atypical PSP can present as AOS and PNFA without the classic features of PSP. |
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Authors:
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K A Josephs; B F Boeve; J R Duffy; G E Smith; D S Knopman; J E Parisi; R C Petersen; D W Dickson |
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Publication Detail:
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Type: Case Reports; Comparative Study; Journal Article |
Journal Detail:
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Title: Neurocase Volume: 11 ISSN: 1355-4794 ISO Abbreviation: Neurocase Publication Date: 2005 Aug |
Date Detail:
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Created Date: 2005-08-11 Completed Date: 2005-10-19 Revised Date: 2011-03-01 |
Medline Journal Info:
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Nlm Unique ID: 9511374 Medline TA: Neurocase Country: England |
Other Details:
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Languages: eng Pagination: 283-96 Citation Subset: IM |
Affiliation:
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Department of Neurology, Division of Behavioural Neurology, Mayo Clinic, Mayo Foundation, Rochester, MN 55905, USA. josephs.keith@mayo.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aphasia, Broca / complications*, pathology* Apraxias / complications*, pathology* Cerebral Cortex / pathology, physiopathology Female Humans Magnetic Resonance Imaging / methods Male Middle Aged Neurofibrillary Tangles / pathology Neurologic Examination / methods Neuropsychological Tests / statistics & numerical data Speech / physiology Statistics, Nonparametric Supranuclear Palsy, Progressive / complications*, pathology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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