Jacquet G, Plouvier E, Billerey C, Godard J, Steimle R
Service de Neurochirurgie, Centre hospitalier universitaire Jean Minjoz, Besançon.
Presse Med. 1988 May 7;17(17):855-7.
A 19-year-old girl had been suffering since the age of 4 from Hand-Schüller-Christian disease. The condition was diagnosed by biopsy of a cranial bone lacuna. Later on, she developed diabetes insipidus and bilateral exophthalmos, both being typical signs of Hand-Schüller-Christian disease. At the age of 14, an intracranial tumour in the posterior cerebral fossa was discovered by computerized tomography, but in view of her parent's refusal of surgery, she was operated upon only 4 years later for obstructive hydrocephalus with intracranial hypertension. Two years after this operation, she presented with neurological signs of compression of the medulla oblongata, including tetraparesis and respiratory disorders, and the tumour was resected. Following a brief improvement she died of major hydroelectrolytic disorders due to her irreducible unstable diabetes insipidus. A review of the literature on Hand-Schüller-Christian disease and its rare association with intracranial tumours is analysed. The occurrence of such tumours raise important therapeutic problems and make the prognosis considerably worse.
一名19岁女孩自4岁起就患有汉-许-克病。通过颅骨腔隙活检确诊了该病。后来，她出现了尿崩症和双侧眼球突出，这两者都是汉-许-克病的典型症状。14岁时，通过计算机断层扫描发现后颅窝有一个颅内肿瘤，但鉴于其父母拒绝手术，4年后她才因梗阻性脑积水伴颅内高压接受手术。这次手术后两年，她出现了延髓受压的神经症状，包括四肢轻瘫和呼吸障碍，于是肿瘤被切除。经过短暂好转后，她因无法控制的不稳定尿崩症导致严重水电解质紊乱而死亡。本文分析了关于汉-许-克病及其与颅内肿瘤罕见关联的文献综述。此类肿瘤的发生引发了重要的治疗问题，并使预后明显变差。
