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歲,既往因腦干海綿狀血管畸形行多次手術(shù)切除
診斷:表面鐵沉積
表面鐵沉積(SS)通常因長時間、反復(fù)蛛網(wǎng)膜下腔出血或腦室內(nèi)出血而引起。
含鐵血黃素具有神經(jīng)元毒性,因此患者可以表現(xiàn)為緩慢進行性雙側(cè)耳聾和共濟失調(diào)。
含鐵血黃素沉積在MRI磁敏感成像序列顯示最佳,表現(xiàn)為室管膜或軟腦膜線樣低信號(箭頭)。
一旦診斷該病,就應(yīng)開始認真尋找引起反復(fù)蛛網(wǎng)膜下腔出血的病因。
From AJNR Classic Case May 28, 2012
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