Grois N G, Favara B E, Mostbeck G H, Prayer D
St. Anna Children's Hospital, Vienna, Austria.
Hematol Oncol Clin North Am. 1998 Apr;12(2):287-305. doi: 10.1016/s0889-8588(05)70511-6.
Central nervous system (CNS) disease in Langerhans cell histiocytosis (LCH) is a poorly understood complication of yet unknown frequency. By far the most common manifestation is in the hypothalamic-pituitary system with diabetes insipidus as the leading sign, followed by other endocrinopathies and hypothalamic dysfunction. However, essentially all other parts of the CNS may be involved. On the one hand, space-occupying histiocytic infiltrates may lead to size- and site-depending symptoms, extending from adjacent bone lesions or arising from the meninges or choroid plexus. On the other hand, a progressive neurological deterioration can occur with mainly cerebellar-pontine symptoms. In this article, these clinical patterns are described in correlation with the morphology on MR imaging and histopathology. Further, the therapeutic strategies are reviewed critically, and guidelines for the management of patients with LCH-related CNS disease are presented.
朗格汉斯细胞组织细胞增多症（LCH）中的中枢神经系统（CNS）疾病是一种频率未知且了解甚少的并发症。迄今为止，最常见的表现是下丘脑 - 垂体系统受累，尿崩症是主要症状，其次是其他内分泌疾病和下丘脑功能障碍。然而，CNS的几乎所有其他部位都可能受累。一方面，占位性组织细胞浸润可能导致取决于大小和部位的症状，这些症状可由相邻骨病变扩展而来，或起源于脑膜或脉络丛。另一方面，主要出现小脑脑桥症状时可发生进行性神经功能恶化。在本文中，将这些临床模式与磁共振成像（MR）和组织病理学的形态学相关联进行描述。此外，对治疗策略进行了批判性回顾，并提出了LCH相关CNS疾病患者的管理指南。
