Huprikar Nikhil, Gossweiler Marisa, Callaghan Maureen, Bunge Paul
Internal Medicine Department, Madigan Army Medical Center, Tacoma, Washington, USA.
BMJ Case Rep. 2013 Aug 7;2013:bcr2013010099. doi: 10.1136/bcr-2013-010099.
Superficial siderosis of the central nervous system is a rare neurological disorder caused by deposits of haemosiderin on subplial brain matter. Characterised by a thin dark layer surrounding the brain stem, cerebellum and cortical fissures on the T2-weighted MRI, symptoms include sensorineural hearing loss and progressive gait ataxia. A specific aetiology for the blood in the subarachnoid space is identified in less than 50% of cases. While identification of a specific vascular defect allows for vascular repair, treatment options are limited for idiopathic superficial siderosis. Recently, a pilot safety study demonstrated promising results using an iron chelator, deferiprone. While this approach is promising, we present a potential serious complication of this therapy-the first report of agranulocytosis in the treatment of superficial siderosis following deferiprone therapy.
中枢神经系统表面铁沉积症是一种罕见的神经系统疾病，由含铁血黄素沉积于脑皮质下物质引起。在T2加权磁共振成像上，其特征为脑干、小脑和皮质沟周围有一层薄薄的深色层，症状包括感音神经性听力损失和进行性步态共济失调。不到50%的病例能确定蛛网膜下腔出血的具体病因。虽然确定特定的血管缺陷可进行血管修复，但特发性表面铁沉积症的治疗选择有限。最近，一项初步安全性研究表明，使用铁螯合剂去铁酮有令人鼓舞的结果。虽然这种方法很有前景，但我们报告了这种治疗的一种潜在严重并发症——去铁酮治疗表面铁沉积症后出现粒细胞缺乏症的首例报告。
