Histiocytic disorders, such as Langerhans cell histiocytosis and Erdheim-Chester disease, are characterised by inflammation and accumulation of cells derived from the monocyte and macrophage lineages, resulting in tissue damage.¹ Neurodegenerative lesions in Langerhans cell histiocytosis are a devastating type of CNS involvement—different from tumour infiltration—in which patients present with progressive symmetric cerebellar syndrome, tetrapyramidal syndrome with or without motor deficits, pseudobulbar palsy, or cognitive impairment.

中文翻译：

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组织细胞增生的神经变性可能始于子宫

组织细胞性疾病，例如朗格汉斯细胞组织细胞增生症和埃德海姆-切斯特病，其特征在于炎症和源自单核细胞和巨噬细胞谱系的细胞蓄积，从而导致组织损伤。¹郎格罕斯细胞组织细胞增生症的神经退行性病变是毁灭性的中枢神经系统受累类型（与肿瘤浸润不同），其中患者表现为进行性对称性小脑综合征，四棱锥体综合征，伴或不伴运动障碍，假性球麻痹或认知障碍。