Mathis S, Levillain P, Vandermarcq P, Gil R, Auche V, Ciron J, Neau J-P
Clinique neurologique, université de Poitiers, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers cedex 05, France.
Rev Med Interne. 2007 Sep;28(9):645-50. doi: 10.1016/j.revmed.2007.04.018. Epub 2007 May 25.
Langerhans cell histiocytosis is a multivisceral pathology. Neurological manifestations are rare.
We report the case of a 31 year old man hospitalized for left partial motor seizure revealing a right frontal tumor with criteria for histiocytosis X. The histological and biological examination found criteria for Langerhans cell histiocytosis (CD1a and S100 reactivity). The check-up for extracerebral localisations of the disease was negative. The outcome was favourable after a total surgical resection. The review of the literature and a discussion on neurological manifestations of this disease were carried out.
A neurological manifestation can be the first and only symptom of a Langerhans cell histiocytosis.
朗格汉斯细胞组织细胞增多症是一种多脏器病变。神经系统表现较为罕见。
我们报告一例31岁男性,因左侧部分性运动性癫痫入院,检查发现右侧额叶肿瘤,具有组织细胞增多症X的标准。组织学和生物学检查发现符合朗格汉斯细胞组织细胞增多症的标准(CD1a和S100反应性)。对该疾病脑外定位的检查为阴性。全手术切除后预后良好。我们进行了文献回顾并讨论了该疾病的神经系统表现。
神经系统表现可能是朗格汉斯细胞组织细胞增多症的首发及唯一症状。