Uyama E, Ueno N, Uchino M, Narahara T, Owada M, Taketomi T, Ando M
First Department of Internal Medicine, Kumamoto (Japan) University School of Medicine, Japan.
Headache. 1995 Sep;35(8):498-501. doi: 10.1111/j.1526-4610.1995.hed3508498.x.
This report concerns an 18-year-old boy who is hemizygote for Fabry's disease. Varying degrees of nonpulsating headache crises, lasting from a few hours to several days, began when he was 16 years of age. Painful crises in the extremities, characteristic of Fabry's disease, were not present. Although only occasional, he had several episodes of throbbing headache with vomiting without aura. The meningeal signs were equivocal, although the patient had noninfectious pleocytosis, intracranial hypertension, delayed radioisotope clearance on cisternography, and multiple old cerebral infarcts. Nonsteroidal anti-inflammatory drugs, antidepressants, carbamazepine, and glycerol were of no benefit for his headache. Although its mode of action remains obscure, prednisolone was effective for treating the headache and the aseptic meningeal reaction.
本报告涉及一名患有法布里病的半合子18岁男孩。16岁时开始出现不同程度的非搏动性头痛发作，持续数小时至数天。法布里病典型的四肢疼痛发作并不存在。虽然只是偶尔发作，但他有几次搏动性头痛伴呕吐且无先兆。脑膜刺激征不明确，尽管患者有非感染性淋巴细胞增多、颅内高压、脑池造影放射性同位素清除延迟以及多处陈旧性脑梗死。非甾体抗炎药、抗抑郁药、卡马西平和甘油对他的头痛均无效。尽管其作用方式仍不清楚，但泼尼松龙对治疗头痛和无菌性脑膜反应有效。
