Ihl Thomas, Arlt Friederike A, Machule Marie-Luise, Prüss Harald, Audebert Heinrich J
Klinik und Hochschulambulanz für Neurologie, Charité - Universitätsmedizin Berlin, Berlin, Germany.
German Center for Neurodegenerative Diseases (DZNE) Berlin, Berlin, Germany.
Case Rep Neurol. 2021 Sep 30;13(3):656-663. doi: 10.1159/000518642. eCollection 2021 Sep-Dec.
Anti-NMDA receptor (NMDAR) encephalitis (NMDARE) is an important treatable cause of autoimmune psychosis in all age-groups, which is sometimes associated with tumors, especially ovarian teratomas. Tuberous sclerosis complex (TSC) is an autosomal dominant inherited neurocutaneous disease predisposing for development of benign tumors. We present a case of a 35-year-old woman with recurrent episodes of schizophrenia-like symptoms. Accidentally, MRI revealed TSC-related brain tumors. NMDAR antibody titers were strongly positive in serum and cerebrospinal fluid. This is the first case describing an overlap of NMDARE and TSC-related brain tumors. A review of brain tumors and NMDARE is given in the supplementary material. Although a causal link seems interesting from a pathophysiological point of view, we are in favor of a coincidence.
抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎(NMDARE)是各年龄组自身免疫性精神病的一个重要可治疗病因,有时与肿瘤相关,尤其是卵巢畸胎瘤。结节性硬化症(TSC)是一种常染色体显性遗传性神经皮肤疾病,易发生良性肿瘤。我们报告一例35岁女性,反复出现精神分裂症样症状。偶然间,磁共振成像(MRI)显示与TSC相关的脑肿瘤。血清和脑脊液中的NMDAR抗体滴度呈强阳性。这是首例描述NMDARE与TSC相关脑肿瘤重叠的病例。补充材料中给出了脑肿瘤和NMDARE的综述。尽管从病理生理学角度来看,因果关系似乎很有意思,但我们认为这是一种巧合。