Adult-onset neuronal intranuclear inclusion disease: a case report and review of literature
10.3760/cma.j.issn.1006-7876.2018.11.010
- VernacularTitle:成人型神经元核内包涵体病一例并文献复习
- Author:
Weian CHEN
1
;
Xiang LI
;
Weiqian ZHU
;
Yang ZHANG
;
Zaiqiang ZHANG
Author Information
1. 温州医科大学附属第一医院神经内科
- Keywords:
Intranuclear inclusion;
Leukoencephalopathy;
Dementia;
Diffusion-weighted image;
Biopsy
- From:
Chinese Journal of Neurology
2018;51(11):905-908
- CountryChina
- Language:Chinese
-
Abstract:
The clinical, pathological features and diagnostic methods of one case of adult-onset neuronal intranuclear inclusion disease (NIID) were analyzed. The patient was 61-year-old female presented with progressive cognitive impairment, episodic unconsciousness, stroke-like attack and paroxysmal digestive tract symptoms. Diffusion-weighted images showed high signals at the cerebral cortico-medullary junction with lace-type distribution, which persisted. Skin biopsy revealed intranuclear inclusion bodies in adipocytes, fibroblasts, and sweat gland cells. This case suggests that adult neuronal nuclear inclusion disease is a chronically progressive neurodegenerative disease with a highly clinical heterogeneity. The subcortical lace sign and eosinophilic intranuclear inclusion bodies by skin biopsy contribute to the diagnosis.
