학술논문

Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report.
Document Type
Journal Article
Source
BMC Neurology. 4/15/2020, Vol. 20 Issue 1, p1-3. 3p. 1 Color Photograph, 1 Diagram.
Subject
*DEMENTIA
*ASPIRATION pneumonia
*GRAIN
*DISEASES
*GENETIC testing
*CEREBELLUM degeneration
*SPINOCEREBELLAR ataxia
Language
ISSN
1471-2377
Abstract
Background: Spinocerebellar ataxia type 31 (SCA31) is not usually associated with dementia, and autopsy in a patient with both conditions is very rare.Case Presentation: An 87-year-old male patient presented with ataxia and progressive dementia. Genetic testing led to a diagnosis of SCA31. Fifteen years after his initial symptoms of hearing loss and difficulty walking, he died of aspiration pneumonia. A pathological analysis showed cerebellar degeneration consistent with SCA31 and abundant argyrophilic grains in the hippocampal formation and amygdala that could explain his dementia.Conclusions: This is the first autopsy report on comorbid argyrophilic grain disease with SCA31. [ABSTRACT FROM AUTHOR]