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Two cases of sporadic adult-onset neuronal intranuclear inclusion disease preceded by urinary disturbance for many years.
J Neurol Sci. 2018 09 15; 392:89-93.JN

Abstract

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 predominantly dementia. We present herein 2 cases of sporadic adult-onset NIID with longstanding urinary disturbance prior to development of other neurological symptoms. 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, diffusion-weighted imaging showed high-intensity signals in the corticomedurally junction; and skin biopsy samples revealed ubiquitin-positive intranuclear inclusions. Therefore, we made a diagnosis of adult-onset NIID. 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.

Authors+Show Affiliations

Department of Neurology, Kansai Medical University Medical Center, 10-15, Fumizono-cho, Moriguchi, Osaka 5708507, Japan; Department of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 5731010, Japan. Electronic address: nakamuma@hirakata.kmu.ac.jp.Department of Neurology, Kansai Medical University Medical Center, 10-15, Fumizono-cho, Moriguchi, Osaka 5708507, Japan.Department of Neurology, Koseikai Takeda Hospital, 841-5, Higashi Shiokoji-cho, Shiokoji-dori Nishinotoin-higashiiru, Shimogyo-ku, Kyoto 600-8558, Japan.Department of Neurology, Koseikai Takeda Hospital, 841-5, Higashi Shiokoji-cho, Shiokoji-dori Nishinotoin-higashiiru, Shimogyo-ku, Kyoto 600-8558, Japan.Department of Neurology, Kansai Medical University Medical Center, 10-15, Fumizono-cho, Moriguchi, Osaka 5708507, Japan.Department of Neurology, Kansai Medical University Medical Center, 10-15, Fumizono-cho, Moriguchi, Osaka 5708507, Japan.Department of Neurology, Koseikai Takeda Hospital, 841-5, Higashi Shiokoji-cho, Shiokoji-dori Nishinotoin-higashiiru, Shimogyo-ku, Kyoto 600-8558, Japan.Department of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 5731010, Japan.Department of Neurology, Kansai Medical University Medical Center, 10-15, Fumizono-cho, Moriguchi, Osaka 5708507, Japan.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

30031993

Citation

Nakamura, Masataka, et al. "Two Cases of Sporadic Adult-onset Neuronal Intranuclear Inclusion Disease Preceded By Urinary Disturbance for Many Years." Journal of the Neurological Sciences, vol. 392, 2018, pp. 89-93.
Nakamura M, Ueki S, Kubo M, et al. Two cases of sporadic adult-onset neuronal intranuclear inclusion disease preceded by urinary disturbance for many years. J Neurol Sci. 2018;392:89-93.
Nakamura, M., Ueki, S., Kubo, M., Yagi, H., Sasaki, R., Okada, Y., Akiguchi, I., Kusaka, H., & Kondo, T. (2018). Two cases of sporadic adult-onset neuronal intranuclear inclusion disease preceded by urinary disturbance for many years. Journal of the Neurological Sciences, 392, 89-93. https://doi.org/10.1016/j.jns.2018.07.012
Nakamura M, et al. Two Cases of Sporadic Adult-onset Neuronal Intranuclear Inclusion Disease Preceded By Urinary Disturbance for Many Years. J Neurol Sci. 2018 09 15;392:89-93. PubMed PMID: 30031993.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Two cases of sporadic adult-onset neuronal intranuclear inclusion disease preceded by urinary disturbance for many years. AU - Nakamura,Masataka, AU - Ueki,Syugo, AU - Kubo,Motonori, AU - Yagi,Hideo, AU - Sasaki,Risa, AU - Okada,Yoichiro, AU - Akiguchi,Ichiro, AU - Kusaka,Hirofumi, AU - Kondo,Takayuki, Y1 - 2018/07/17/ PY - 2018/02/26/received PY - 2018/06/05/revised PY - 2018/07/13/accepted PY - 2018/7/23/pubmed PY - 2019/7/19/medline PY - 2018/7/23/entrez KW - Diffusion-weighted image KW - Loss of consciousness KW - Neuronal intranuclear inclusion disease KW - Skin biopsy KW - Urinary disturbance SP - 89 EP - 93 JF - Journal of the neurological sciences JO - J Neurol Sci VL - 392 N2 - 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 predominantly dementia. We present herein 2 cases of sporadic adult-onset NIID with longstanding urinary disturbance prior to development of other neurological symptoms. 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, diffusion-weighted imaging showed high-intensity signals in the corticomedurally junction; and skin biopsy samples revealed ubiquitin-positive intranuclear inclusions. Therefore, we made a diagnosis of adult-onset NIID. 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. SN - 1878-5883 UR - https://www.unboundmedicine.com/medline/citation/30031993/Two_cases_of_sporadic_adult_onset_neuronal_intranuclear_inclusion_disease_preceded_by_urinary_disturbance_for_many_years_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022-510X(18)30299-5 DB - PRIME DP - Unbound Medicine ER -