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Neuronal intranuclear inclusion disease with mental abnormality: a case report.
BMC Neurol. 2020 Sep 23; 20(1):356.BN

Abstract

BACKGROUND

Neuronal intranuclear inclusion disease (NIID) is a chronic progressive neurodegenerative disease that is characterized by the discovery of eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous systems and visceral organs. In this paper, we report a case of an adult-onset neuronal intranuclear inclusion disease presenting with mental abnormality in China.

CASE PRESENTATION

A 62-year-old woman presented with mental abnormality and forgetfulness for 3 months before she was admitted to our hospital. There were prodromal symptoms of fever before she had the mental disorder. Encephalitis was first suspected, and the patient underwent lumbar puncture and brain magnetic resonance imaging (MRI). A cerebrospinal fluid (CSF) examination indicated normal pressure, a normal white blood cell count, and slightly elevated protein and glucose levels. Coxsackie B virus, enterovirus, and cytomegalovirus tests showed normal results. Bacterial culture and Cryptococcus neoformans test results were negative. The contrast-enhanced MRI of the brain was normal. The brain diffusion-weighted imaging (DWI) showed a symmetrically distributed strip-shaped hyperintensity signal of the corticomedullary junction in the bilateral frontal, parietal, and temporal lobes. We considered the diagnosis of the NIID, and therefore, skin biopsy was performed. The electron microscopy revealed that intranuclear inclusions in the nucleus of fibrocytes existed and were composed of filaments.

CONCLUSIONS

NIID is a rare neurodegenerative disease with diverse clinical manifestations. In clinical work, when a patient presents with abnormal mental behavior and exhibits hyperintensity signals on DWI images of the corticomedullary junction, it is crucial to consider the diagnosis of NIID.

Authors+Show Affiliations

Department of Neurology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.Department of Neurology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.Department of Neurology, The First People's Hospital of Zaoyang, Zaoyang, 441299, China.Department of Neurology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.Department of Neurology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China. 459511971@qq.com.Department of Neurology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China. 1983xh0564@hust.edu.cn.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

32967628

Citation

Chi, Xiaosa, et al. "Neuronal Intranuclear Inclusion Disease With Mental Abnormality: a Case Report." BMC Neurology, vol. 20, no. 1, 2020, p. 356.
Chi X, Li M, Huang T, et al. Neuronal intranuclear inclusion disease with mental abnormality: a case report. BMC Neurol. 2020;20(1):356.
Chi, X., Li, M., Huang, T., Tong, K., Xing, H., & Chen, J. (2020). Neuronal intranuclear inclusion disease with mental abnormality: a case report. BMC Neurology, 20(1), 356. https://doi.org/10.1186/s12883-020-01933-8
Chi X, et al. Neuronal Intranuclear Inclusion Disease With Mental Abnormality: a Case Report. BMC Neurol. 2020 Sep 23;20(1):356. PubMed PMID: 32967628.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Neuronal intranuclear inclusion disease with mental abnormality: a case report. AU - Chi,Xiaosa, AU - Li,Man, AU - Huang,Ting, AU - Tong,Kangyong, AU - Xing,Hongyi, AU - Chen,Jixiang, Y1 - 2020/09/23/ PY - 2020/05/05/received PY - 2020/09/14/accepted PY - 2020/9/24/entrez PY - 2020/9/25/pubmed PY - 2020/11/24/medline KW - Intranuclear inclusion KW - Mental abnormality KW - Neuronal intranuclear inclusion disease KW - Skin biopsy SP - 356 EP - 356 JF - BMC neurology JO - BMC Neurol VL - 20 IS - 1 N2 - BACKGROUND: Neuronal intranuclear inclusion disease (NIID) is a chronic progressive neurodegenerative disease that is characterized by the discovery of eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous systems and visceral organs. In this paper, we report a case of an adult-onset neuronal intranuclear inclusion disease presenting with mental abnormality in China. CASE PRESENTATION: A 62-year-old woman presented with mental abnormality and forgetfulness for 3 months before she was admitted to our hospital. There were prodromal symptoms of fever before she had the mental disorder. Encephalitis was first suspected, and the patient underwent lumbar puncture and brain magnetic resonance imaging (MRI). A cerebrospinal fluid (CSF) examination indicated normal pressure, a normal white blood cell count, and slightly elevated protein and glucose levels. Coxsackie B virus, enterovirus, and cytomegalovirus tests showed normal results. Bacterial culture and Cryptococcus neoformans test results were negative. The contrast-enhanced MRI of the brain was normal. The brain diffusion-weighted imaging (DWI) showed a symmetrically distributed strip-shaped hyperintensity signal of the corticomedullary junction in the bilateral frontal, parietal, and temporal lobes. We considered the diagnosis of the NIID, and therefore, skin biopsy was performed. The electron microscopy revealed that intranuclear inclusions in the nucleus of fibrocytes existed and were composed of filaments. CONCLUSIONS: NIID is a rare neurodegenerative disease with diverse clinical manifestations. In clinical work, when a patient presents with abnormal mental behavior and exhibits hyperintensity signals on DWI images of the corticomedullary junction, it is crucial to consider the diagnosis of NIID. SN - 1471-2377 UR - https://www.unboundmedicine.com/medline/citation/32967628/Neuronal_intranuclear_inclusion_disease_with_mental_abnormality:_a_case_report_ L2 - https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-020-01933-8 DB - PRIME DP - Unbound Medicine ER -