Rosenbaum T, Gärtner J, Körholz D, Janssen G, Schneider D, Engelbrecht V, Göbel U, Lenard H G
Department of Neuropediatrics, Heinrich-Heine-Universität Düsseldorf, Germany.
Neuropediatrics. 1998 Jun;29(3):159-62. doi: 10.1055/s-2007-973554.
Paraneoplastic neurological disorders represent remote effects of cancer without invasion of tumor cells into the nervous system. Limbic encephalitis is a distinct entity mostly associated with small-cell carcinoma of the lung. We present the cases of two teenage girls who were admitted with clinical symptoms typical for limbic encephalitis. In the course of the disease, they exhibited characteristic evolutionary changes of brain MRI abnormalities. Onset of neurological symptoms and type of underlying neoplasia were different in both patients. In one girl the initial workup led to the diagnosis of nodular sclerosing Hodgkin disease which so far had not caused any symptoms besides the described neurological abnormalities. A diagnostic brain biopsy showed inflammatory changes and excluded invasion of malignant cells into the central nervous system. The other patient had been diagnosed with a small cell carcinoma of the ovary several months before neurological and brain MRI abnormalities were observed. This is the first report in which clinical picture, evolution of MRI abnormalities, and--in one case--characteristic neuropathological changes are suggestive of paraneoplastic limbic encephalitis in two adolescent girls.
副肿瘤性神经系统疾病是指癌症的远隔效应,肿瘤细胞未侵入神经系统。边缘叶脑炎是一种独特的疾病,主要与小细胞肺癌相关。我们报告了两名青少年女性患者的病例,她们因边缘叶脑炎的典型临床症状入院。在疾病过程中,她们表现出脑MRI异常的特征性演变。两名患者的神经症状发作和潜在肿瘤类型均不同。其中一名女孩的初步检查诊断为结节硬化型霍奇金病,除了所述神经异常外,迄今为止未引起任何症状。诊断性脑活检显示有炎症改变,排除了恶性细胞侵入中枢神经系统。另一名患者在观察到神经和脑MRI异常前几个月被诊断为卵巢小细胞癌。这是首次报告两名青春期女孩的临床症状、MRI异常演变以及(在一例中)特征性神经病理改变提示副肿瘤性边缘叶脑炎。