ナカムラ　マサタカ   NAKAMURA M.   中村　正孝    所属   関西医科大学  神経内科学講座    職種   講師
言語種別	英語
発表タイトル	Two cases of neuronal intranuclear inclusion disease preceded by urinary disturbance for many years
会議名	第60回日本神経学会学術大会
学会区分	全国規模の学会
発表形式	ポスター掲示
講演区分	一般
発表者・共同発表者	◎Nakamura M, Ueki S, Kubo M, Yagi H, Sasaki R, Okada Y, Akiguchi I, Kusaka H, Kondo T
発表年月日	2019/05/25
開催地 （都市, 国名）	大阪
概要	Objective: Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease defined by the presence of eosinophilic hyaline intranuclear inclusions. The initial and main clinical feature of adult-onset NIID is dementia. We present herein 2 cases of adult-onset NIID with longstanding urinary disturbance prior to development of other neurological symptoms. Case presentation: Case 1: A 71-year-old woman was admitted after she lost consciousness while bathing. She presented slowly progressive bladder dysfunction starting at the age of 40. Recently, she complained of recurrent light-headedness on standing. Her neurological findings showed miosis, muscle weakness, rigidity, hyporeflexia, sensory disturbance, cerebellar ataxia, and orthostatic hypotension. Case 2: A 68-year-old man was admitted because of episodes of transient loss of consciousness. Ten years earlier, he had developed urinary dysfunction. His neurological findings revealed cognitive dysfunction, cerebellar ataxia, and hyporeflexia. Both patients had leukoencephalopathy and motor-sensory neuropathy. In both cases, DWI showed high-intensity signals in the corticomedurally junction; and skin biopsy samples revealed ubiquitin-positive intranuclear inclusions. Conclusion: Although numerous cases of this disorder have been reported in the past, there were only a few cases showing the development of other neurological symptoms after longstanding urinary disturbance. Our cases suggest that it is worthwhile considering the possibility of NIID in cases with a long-term history of neurogenic bladder dysfunction.