神经元核内包涵体病是一种缓慢进展的罕见神经系统退行性疾病，其特征性病理表现为神经系统及内脏器官中广泛分布的核内嗜酸性包涵体。随着皮肤病理活组织检查的出现，该疾病确诊数量增多，但其表现多样，异质性强，常常误诊。报道1例临床表现为单纯自主神经受损的神经元核内包涵体病患者，其病程较长，自主神经功能受损症状突出，认知功能损害不明显。希望能为该病的临床诊断提供新思路。“,”Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous system, and also in the visceral organs. Although in recent years skin biopsy is useful for the antemortem diagnosis of this disease, it is often misdiagnosed due to its highly variable clinical manifestations. A case of NIID is reported here. The patient had a long course of disease, mainly presenting as dysfunction of autonomic nervous system. No significant cognitive impairment was found. Thus, a new idea is provided for the diagnosis of this disease.