朗格汉斯细胞组织细胞增多症中的脱髓鞘和胶质增生性小脑病变。
Demyelinating and gliotic cerebellar lesions in Langerhans cell histiocytosis.
作者信息
Poe L B, Dubowy R L, Hochhauser L, Collins G H, Crosley C J, Kanzer M D, Oliphant M, Hodge C J
机构信息
Department of Radiology, State University of New York Health Science Center at Syracuse.
出版信息
AJNR Am J Neuroradiol. 1994 Nov;15(10):1921-8.
PURPOSE
To describe the involvement of the cerebellum by a gliotic and demyelinating process in Langerhans cell histiocytosis.
METHODS
A retrospective analysis of all (N = 30) cases of Langerhans cell histiocytosis followed at our institution since 1975 yielded four patients with CT and/or MR evidence of cerebellar abnormalities.
RESULTS
Four patients manifested strikingly similar findings of symmetric nonenhancing hypodensities in the dentate nuclei region of the cerebellum, which were hypointense on short-repetition-time/short-echo-time MR and hyperintense on long-repetition-time/long-echo-time MR. Biopsy in one patient yielded areas of demyelination, cell loss, and gliosis without histiocytic infiltration.
CONCLUSION
Langerhans cell histiocytosis involves the cerebellum in a specific and poorly understood manner. Lesions on imaging may precede clinical findings by years. Lesions may occur in patients who have never experienced radiation therapy and may act as a marker for eventual central nervous system deterioration.
目的
描述朗格汉斯细胞组织细胞增多症中胶质增生和脱髓鞘过程对小脑的累及情况。
方法
对自1975年以来在我们机构随访的所有（N = 30）例朗格汉斯细胞组织细胞增多症病例进行回顾性分析，发现4例患者有小脑异常的CT和/或MR证据。
结果
4例患者在小脑齿状核区域表现出惊人相似的对称非强化低密度影，在短重复时间/短回波时间MR上呈低信号，在长重复时间/长回波时间MR上呈高信号。1例患者的活检显示有脱髓鞘、细胞丢失和胶质增生区域，无组织细胞浸润。
结论
朗格汉斯细胞组织细胞增多症以一种特定且尚不清楚的方式累及小脑。影像学上的病变可能比临床发现早数年出现。病变可能发生在从未接受过放射治疗的患者中，并且可能作为最终中枢神经系统恶化的一个标志。
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