title: Neuromyelitis optica accompanied by nephrotic syndrome and autoimmune-related pancytopenia.
abstract: Neuromyelitis optica (NMO) associated with nephrotic syndrome and autoimmune-related pancytopenia has not been reported previously. We report herein a young woman who initially presented with bilateral blurring of vision and numbness in her hands. MRI disclosed multiple white matter lesions and a long cervical spinal cord lesion extending to the medulla oblongata. Serum aquaporin-4 antibody was positive and the patient was diagnosed with NMO. While in the hospital, she presented with hypoproteinemia and heavy proteinuria, meeting the diagnostic criteria of nephrotic syndrome. After high-dose methylprednisolone treatment, her vision improved significantly and urine protein quantity decreased. However, the patient subsequently developed severe pancytopenia with a positive Coombs' test. Thrombocytopenia finally led to uncontrollable gastrointestinal bleeding as the direct cause of the patient's death. This case illustrates the extremely rare condition of concurrence of NMO, nephrotic syndrome, and autoimmune pancytopenia in one patient, which suggests the involvement of organs beyond the central nervous system in NMO spectrum disorders.
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Jingzi
authors.initials: J
authors.lastname: ZhangBao
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Zhou
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Jiahong
authors.initials: J
authors.lastname: Lu
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Jianying
authors.initials: J
authors.lastname: Xi
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China; Department of Neurology, Jing'an District Centre Hospital of Shanghai, China. Electronic address: zhao_chongbo@fudan.edu.cn.
authors.forename: Chongbo
authors.initials: C
authors.lastname: Zhao
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China. Electronic address: chao_quan@fudan.edu.cn.
authors.forename: Chao
authors.initials: C
authors.lastname: Quan
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000009:adverse effects / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008775:Methylprednisolone / Q000009:adverse effects / Q000627:therapeutic use; D009404:Nephrotic Syndrome / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging / Q000628:therapy; D009471:Neuromyelitis Optica / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D010198:Pancytopenia / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging / Q000628:therapy; D012867:Skin / Q000473:pathology
chemical_list: D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone
keywords: Nephrotic syndrome; Neuromyelitis optica (NMO); Pancytopenia