摘要
中枢神经系统表面含铁血黄素沉积症是一种由蛛网膜下腔慢性出血造成含铁血黄素在脑和脊髓表面沉积而引起的中枢神经系统退行性变。临床主要表现为感音神经性耳聋、小脑性共济失调和锥体束损伤,腰穿可见脑脊液黄变,MR可见脑和脊髓表面沉积的含铁血黄素低信号。常见的造成出血的原因有血管畸形、动脉瘤、肿瘤、颅脑椎管内手术、硬脊膜缺损、外伤等。最根本的治疗方法是通过影像学明确出血灶并行外科手术切除,人工耳蜗植入和口服驱铁剂是目前对未发现明确病灶或病灶不能切除患者的主要治疗方法 。
Superficial sideroisis （ SS） is a clinical syndrome caused by chronic subarachnoid bleeding due to various reasons.It can lead to deposition of hemosiderin and iron in the surface of the central nervous system （CNS） and consequently cause neurodegeneration .This disease is characterized by sensorineural deafness , cerebellar ataxia and pyramidal signs .The diagnostic character presents linear low-intensity signals along the surface of the brain and spinal cord in T 2 imaging or SWI . Here the article review its clinical characteristics , current diagnosis , etiology, pathophysiology and treatment .
出处
《疑难病杂志》
CAS
2016年第9期979-981,985,共4页
Chinese Journal of Difficult and Complicated Cases
