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MRI findings of adult-onset sporadic neuronal intranuclear inclusion disease: report of 3 cases and review of literature
FENG Yanyun  XU Zhifeng  ZHAO Hai  LU Ruiliang  GAO Mingyong 

Cite this article as: Feng YY, Xu ZF, Zhao H, et al. MRI findings of adult-onset sporadic neuronal intranuclear inclusion disease: report of 3 cases and review of literature. Chin J Magn Reson Imaging, 2020, 11(7): 581-584. DOI:10.12015/issn.1674-8034.2020.07.021.


[Abstract] Objective: To investigate the MRI findings of adult-onset sporadic neuronal intranuclear inclusion disease (NIID).Materials and Methods: The clinical symptoms, MRI findings and dermatopathological features of adult-onset sporadic NIID were analyzed.Results: Three patients with clinical manifestations such as cognitive dysfunction, extrapyramidal symptoms, cerebellar ataxia, etc. Head MRI showed abnormal white matter signal in bilateral cerebral hemispheres. DWI showed high signals at the cerebral cortico-medullary junction, which persisted and progressive increase. Skin biopsy revealed eosinophilic intranuclear inclusion bodies in adipocytes, fibroblasts, and sweat gland cells which were diagnosed as NIID.Conclusions: NIID is a chronically progressive neurodegenerative disease. MRI-DWI of the head shows high signals at the cerebral cortico-medullary junction, and dermatopathological findings of eosinophilic intranuclear inclusion bodies contribute to the diagnosis.
[Keywords] intranuclear inclusion bodies；neurons；magnetic resonance imaging

FENG Yanyun Department of Radiology, the First People's Hospital of Foshan (the Affiliated Foshan Hospital of Sun Yat-sen University), Foshan 528000, China

XU Zhifeng Department of Radiology, the First People's Hospital of Foshan (the Affiliated Foshan Hospital of Sun Yat-sen University), Foshan 528000, China

ZHAO Hai* Department of Radiology, the First People's Hospital of Foshan (the Affiliated Foshan Hospital of Sun Yat-sen University), Foshan 528000, China

LU Ruiliang Department of Radiology, the First People's Hospital of Foshan (the Affiliated Foshan Hospital of Sun Yat-sen University), Foshan 528000, China

GAO Mingyong Department of Radiology, the First People's Hospital of Foshan (the Affiliated Foshan Hospital of Sun Yat-sen University), Foshan 528000, China

*Corresponding to: Zhao H, E-mail: 13760988108@163.com

Conflicts of interest   None.

ACKNOWLEDGMENTS  This study was supported by Special Fund for Scientific and Technological Innovation of Foshan NO. 2015AG10004
Received  2019-12-09
Accepted  2020-03-28
DOI: 10.12015/issn.1674-8034.2020.07.021
Cite this article as: Feng YY, Xu ZF, Zhao H, et al. MRI findings of adult-onset sporadic neuronal intranuclear inclusion disease: report of 3 cases and review of literature. Chin J Magn Reson Imaging, 2020, 11(7): 581-584. DOI:10.12015/issn.1674-8034.2020.07.021.

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