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Recurrent severe and long‐lasting cerebellar ataxia attacks in adult‐onset neuronal intranuclear inclusion disease
Author(s) -
Osakada Yosuke,
Deguchi Kentaro,
Deguchi Shoko,
Higaki Fumiyo,
Oda Wakako,
Yoshida Mari,
Ohta Yasuyuki,
Yamashita Toru,
Abe Koji
Publication year - 2021
Publication title -
neurology and clinical neuroscience
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.125
0ISSN - 2049-4173
DOI - 10.1111/ncn3.12454
Subject(s) - medicine , ataxia , pathology , hyperintensity , white matter , lesion , eosinophilic , dementia , cerebellar ataxia , cerebellum , disease , magnetic resonance imaging , radiology , psychiatry
Neuronal intranuclear inclusion disease (NIID) is a progressive neurodegenerative disease that is characterized by eosinophilic hyaline intranuclear inclusions. NIID presents highly variable clinical manifestations such as dementia, ataxia, convulsion, neuropathy, and autonomic dysfunction. A 60‐year‐old woman developed recurrent severe cerebellar ataxia (SARA score: 30/40 points). She was diagnosed with adult‐onset NIID by a characteristic brain diffusion‐weighed imaging hyperintensity lesion in subcortical white matter and intranuclear inclusion bodies in a skin biopsy. Her ataxia was more severe and lasted longer (additional 19 days) than a previous case, although she showed remarkable improvement.