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Clinicopathologic analysis of progressive non-fluent aphasia and corticobasal degeneration: Case report and review

OBJECTIVE: To investigate progressive non-fluent aphasia and histopathologically-proven corticobasal degeneration. METHODS: We evaluated symptoms, signs, neuropsychological deficits, and radiology data longitudinally, in a patient with autopsy-proven corticobasal degeneration and correlated these ob...

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Detalles Bibliográficos
Autores principales: de Brito-Marques, Paulo Roberto, Vieira-Mello, Roberto José, Montenegro, Luciano, Aragão, Maria de Fátima Vasco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Associação de Neurologia Cognitiva e do Comportamento 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5619310/
https://www.ncbi.nlm.nih.gov/pubmed/29213736
http://dx.doi.org/10.1590/S1980-57642011DN05020013
Descripción
Sumario:OBJECTIVE: To investigate progressive non-fluent aphasia and histopathologically-proven corticobasal degeneration. METHODS: We evaluated symptoms, signs, neuropsychological deficits, and radiology data longitudinally, in a patient with autopsy-proven corticobasal degeneration and correlated these observations directly to the neuroanatomic distribution of the disease. RESULTS: At presentation, a specific pattern of cognitive impairment was evident with an extreme extrapyramidal motor abnormality. Follow-up examination revealed persistent impairment of praxis and executive functioning, progressive worsening of language performance, and moderately preserved memory. The motor disorder manifested and worsened as the condition progressed. Many of the residual nerve cells were ballooned and achromatic with eccentric nuclei. Tau-immunoreactive pathology was significantly more prominent in neurons in the frontal and parietal cortices and dentate nuclei than in temporal neocortex, hippocampi and brainstem. CONCLUSION: The clinical diagnosis of progressive non-fluent aphasia secondary to corticobasal degeneration hinged on a specific pattern of impaired cognition as well as an extrapyramidal motor disorder, reflecting the neuroanatomic distribution of the disease in frontal and anterior temporal cortices and the dentate nuclei.