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- W2595277798 abstract "OBJECTIVE: To expand the spectrum of neuropathology in semantic variant primary progressive aphasia (svPPA), and to introduce a novel coexisting glioneuronal tauopathy which, based on its localization, may have influenced the patient9s clinical manifestation. BACKGROUND: svPPA is clinically defined as impaired confrontation naming, single word comprehension and object knowledge with relative preservation of repetition and speech production. Unlike other types of Frontotemporal Dementia with pathological heterogeneity, svPPA has relatively consistent neuropathology of FTLD-TDP43 Harmonized type C. Co-existing neurodegenerative phenomena are commonly encountered in FTLD autopsies, but their impact on clinical phenotype is poorly understood. DESIGN/METHODS: We present and correlate the clinical, neuroimaging and pathologic features of a case of svPPA with unique autopsy findings. RESULTS: A 72 year old man developed progressive language dysfunction with impaired object knowledge, confrontation naming and comprehension with fluent speech. There was worsening left anterior temporal atrophy on sequential MRI with coincident hypoperfusion on SPECT. Extensive neuropsychological and cognitive testing was consistent with a diagnosis of svPPA. At autopsy there were widespread TDP-43 immunopositive neuronal cytoplasmic inclusions and short neurites consistent with FTLD-TDP Harmonized type A. In addition there was an unusual co-existing glioneuronal tauopathy with a predilection for white matter comprised of granular astrocytic inclusions, tufted and thorny-like astrocytes and coiled bodies, most pronounced in the left anterior temporal lobe. The patient9s twin is becoming symptomatic and genetic testing for a progranulin mutation is underway. CONCLUSIONS: We describe novel pathological findings in a clinically well-delineated case of potentially familial svPPA. The remarkable features of this case include the Harmonized type A FTLD-TDP43 designation and the peculiar glioneuronal tau inclusions in the neuroanatomical region implicated clinically and on structural and functional neuroimaging. We speculate that the co-existing tauopathy may have contributed to the patient9s svPPA phenotype. Disclosure: Dr. Mitchell has nothing to disclose. Dr. Black has received personal compensation for activities with Novartis Pharmaceuticals, Pfizer, GlaxoSmithKline, Roche Pharmaceuticals, and Bristol-Myers Squibb, and Elan. Dr. Black has received research support from Novartis Pharmaceuticals, Pfizer, Roche, and GlaxoSmithKline. Dr. Rogaeva has nothing to disclose. Dr. Bilbao has nothing to disclose. Dr. Keith has nothing to disclose." @default.
- W2595277798 created "2017-03-23" @default.
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- W2595277798 date "2013-02-12" @default.
- W2595277798 modified "2023-09-29" @default.
- W2595277798 title "Co-Existing Glioneuronal Tauopathy as a Potential Explanation for Semantic Variant Primary Progressive Aphasia Phenotype in FTLD-TDP43 Harmonized Type A (P05.124)" @default.
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