Shuta Toru1, Shoko Ishida2, Toshiki Uchihara3, Katsuiku Hirokawa4, Masanobu Kitagawa2, Kinya Ishikawa5. 1. Department of Neurology, Nitobe Memorial Nakano General Hospital, 4-59-16 Chuo, Nakano-ku, Tokyo, 164-8607, Japan. shuta-toru@nakanosogo.or.jp. 2. Department of Pathology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan. 3. Department of Neurology, Nitobe Memorial Nakano General Hospital, 4-59-16 Chuo, Nakano-ku, Tokyo, 164-8607, Japan. 4. Department of Pathology, Nitobe Memorial Nakano General Hospital, 4-59-16 Chuo, Nakano-ku, Tokyo, 164-8607, Japan. 5. Department of Neurology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
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.
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.
Entities:
Keywords:
Argyrophilic grain disease; Autopsy; Case report; Spinocerebellar ataxia type 31