Allard P, Kermarec J, Goasguen J, Ferry M, de Muizon H, Girard P, Herning R
Rev Pneumol Clin. 1984;40(5):305-9.
The authors report a case of histiocytosis X which presented with neurological manifestations in a 20 year old man: episodes of headache with vomiting, followed, three months later, by the development of paralysis of the right 6th and 7th cranial nerves associated with nystagmus. The CT scan revealed a tumour-like lesion on the floor of the IVth ventricle with a long axis of 18 mm. The chest x-ray revealed diffuse nodular opacities in the pleural and apical regions with features suggestive of histiocytosis X. The diagnosis was confirmed by surgical biopsy of the typical pulmonary nodules which were rich in histiocytes with X bodies on electron microscopy. The neurological signs disappeared after one month of treatment with Prednisone (1 mg/kg/day) and Vincaleukoblastine (10 mg/week). By the 3rd month, the pulmonary lesions were reduced and the intra-ventricular formation had regressed by 40%. In the authors' series of 29 cases of confirmed histiocytosis X in adults, the present case is the only one with a clinical neurological presentation, apart from 3 cases of diabetes insipidus. A review of the literature confirms the rarity of this type of presentation. The suggestion of the diagnosis by the chest x-ray appearance enabled a dangerous neuro-surgical operation to be avoided.
作者报告了一例组织细胞增多症X，该病例发生在一名20岁男性身上，表现为神经症状：头痛伴呕吐发作，三个月后，右侧第6和第7颅神经麻痹并伴有眼球震颤。CT扫描显示第四脑室底部有一个瘤样病变，长轴为18毫米。胸部X光显示胸膜和肺尖区域有弥漫性结节状混浊，具有组织细胞增多症X的特征。通过对典型肺结节进行手术活检确诊，电镜检查显示这些结节富含带有X小体的组织细胞。使用泼尼松（1毫克/千克/天）和长春新碱（10毫克/周）治疗一个月后，神经症状消失。到第3个月时，肺部病变减轻，脑室内肿物消退了40%。在作者的29例确诊的成人组织细胞增多症X系列病例中，除了3例尿崩症外，本病例是唯一有临床神经症状表现的病例。文献回顾证实了这种表现类型的罕见性。胸部X光表现提示了诊断，从而避免了危险的神经外科手术。
