中枢神经系统表面铁沉积症一例

Superficial siderosis in the central nervous system:report of a case with asymptomatic hypoglycemia

目的讨论中枢神经系统表面铁沉积症的病例特点及诊断。方法对1例患者进行临床相关资料分析。结果中枢神经系统表面铁沉积症是由于铁沉积在中枢神经系统表面而产生。临床典型特征为：进行性感音性听力下降，小脑性共济失调和锥体束征，磁共振的梯度回波序列T2·加权像上可见颅脑及脊髓表面显著低信号带，脑脊液中铁蛋白含量增高。结论中枢神经系统表面铁沉积症容易误诊，通过病史及体格检查并结合相关检查可以在疾病的早期确诊。磁共振的梯度回波序列T2·加权像及脑脊液的相关检查有助于诊断。

Objective To investigate the clinic characters and diagnosis of superficial siderosis in the central nervous system （SSCN）. Methods One patient was systematically studied by the authors. Results SSCN was a rare entity, resulting in the deposition of ferric pigments and ions on the surface of the central nervous system. The clinical features included progressive sensorineural hearing loss, cerebellar ataxia and pyramidal sign, widespread hypointensity band at surfaces of the cerebral or cerebellar hemispheres, the brain stem and the spinal cord on Gradient Echo T2^＊ -weighted images （ GRE-T2^＊ WI） of MR, elevation of the levels of ferritin in the cerebrospinal fluid. Conclusions This disease can be identified at early stage with history and physical examination. GRE-T2^＊ WI and some related cerebrospinal fluid tests will contribute to diagnosis.