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小儿霍奇金病伴发的副肿瘤性小脑变性

Paraneoplastic cerebellar degeneration in pediatric Hodgkin disease.

作者信息

Hahn A, Claviez A, Brinkmann G, Altermatt H J, Schneppenheim R, Stephani U

机构信息

Department of Neuropaediatrics, University of Kiel, Germany.

出版信息

Neuropediatrics. 2000 Feb;31(1):42-4. doi: 10.1055/s-2000-15297.

Abstract

Paraneoplastic cerebellar degeneration (PCD) is a rare neurological complication in adults with extracerebral neoplasms. It is characterized by a diffuse cerebellar dysfunction, usually leading to severe neurological sequelae. In childhood, this complication is extremely rare. We report on PCD as primary manifestation of Hodgkin disease (HD) in a thirteen-year old boy. On magnetic resonance imaging, irreversible atrophy of the cerebellum developed within three months. Antibodies against Purkinje cells were detectable at diagnosis and normalised after successful treatment of the lymphoma. Cerebellar symptoms, however, only partially resolved. The necessity of a search for a malignant tumour is emphasised in the presence of an otherwise unexplained, subacutely developing, diffuse cerebellar dysfunction.

摘要

副肿瘤性小脑变性(PCD)是成人脑外肿瘤罕见的神经系统并发症。其特征为弥漫性小脑功能障碍,通常会导致严重的神经后遗症。在儿童期,这种并发症极为罕见。我们报告了一名13岁男孩以霍奇金病(HD)为首发表现的PCD。磁共振成像显示,小脑在三个月内出现不可逆萎缩。诊断时可检测到抗浦肯野细胞抗体,淋巴瘤成功治疗后抗体水平恢复正常。然而,小脑症状仅部分缓解。当出现无法解释的、亚急性发展的弥漫性小脑功能障碍时,强调了寻找恶性肿瘤的必要性。

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