A 32-year-old woman with history of multiple operations for resection of a brainstem cavernous malformation.
A
B
What is your diagnosis?
The answer is Superficial Siderosis
Superficial siderosis (SS) is typically cause by recurrent episodes of subarachnoid or intraventricular hemorrhage that occur over extended time periods.
Hemosiderin is cytotoxic to neurons; therefore, patients may present with slowly progressive bilateral deafness and ataxia. Hemosiderin deposition is best seen on susceptibility-weighted MR sequences as dark lines on ependyma or pial matter (arrows).
Once diagnosis of SS is made, a dedicated search for the cause of recurrent subarachnoid hemorrhage must commence.
中文版:
女性,32岁,既往因脑干海绵状血管畸形行多次手术切除
诊断:表面铁沉积
表面铁沉积(SS)通常因长时间、反复蛛网膜下腔出血或脑室内出血而引起。
含铁血黄素具有神经元毒性,因此患者可以表现为缓慢进行性双侧耳聋和共济失调。
含铁血黄素沉积在MRI磁敏感成像序列显示最佳,表现为室管膜或软脑膜线样低信号(箭头)。
一旦诊断该病,就应开始认真寻找引起反复蛛网膜下腔出血的病因。
From AJNR Classic Case May 28, 2012
联系客服