Maggioni F, Martinello F, Iavicoli R, Pietrogrande F, Trevisan C P
Department of Neurology, University of Padua, Italy.
Acta Neurol (Napoli). 1994 Jun;16(3):134-41.
A case of idiopathic superficial hemosiderosis (SH) of the central nervous system and a review of the literature are presented. The patient suffered from progressive cerebellar ataxia, hearing loss, anosmia, spastic paraparesis, but no mental deterioration. The diagnosis was made with brain and spinal MRI, that showed in T2 weighted images superficial hypointensity of spinal cord, medulla oblungata, pons, mesencephalon, cerebellum and cerebral hemispheres, images that are considered pathognomonic of SH. Repeated spinal fluid examinations were negative, suggesting that evidence of overt subarachnoidal bleeding is not essential in the diagnosis. In patients with SH of unknown etiology no valid therapy is yet available.
本文报告一例中枢神经系统特发性浅表性 hemosiderosis(SH)病例并对相关文献进行综述。该患者患有进行性小脑共济失调、听力丧失、嗅觉丧失、痉挛性截瘫,但无精神衰退。通过脑部和脊髓 MRI 做出诊断,T2 加权图像显示脊髓、延髓、脑桥、中脑、小脑和大脑半球浅表低信号,这些图像被认为是 SH 的特征性表现。多次脑脊液检查均为阴性,提示蛛网膜下腔明显出血的证据在诊断中并非必需。对于病因不明的 SH 患者,目前尚无有效的治疗方法。