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[浅表性铁沉积症:病例报告与文献综述]

[Superficial siderosis: case report and literature review].

作者信息

Kresojević Nikola D, Petrović Igor N, Dragaević-Misković Natasa T, Kostić Vladimir S

出版信息

Srp Arh Celok Lek. 2013 Mar-Apr;141(3-4):219-22. doi: 10.2298/sarh1304219k.

Abstract

INTRODUCTION

Superficial siderosis (SS) is caused by chronic subarachnoid bleeding and is characterized by free iron and hemosiderin deposition along the pial and subpial structures of central nervous system. SS leads to progressive and irreversible CNS damage. The most common causes of chronic subarachnoidal bleeding are tumors, head and spinal cord trauma, arteriovenous malformations and aneurysms. SS is characterized by clinical triad: sensorineural hearing loss, cerebellar ataxia and piramydal signs. Brain MR imaging is the investigation of choice for the diagnosis of SS.Typical findings include hypointensities seen on T2-weighted MR imaging around the brain, cerebellum, brain stem, spinal cord, VIII cranial nerve and atrophy of cerebellum and medulla.

CASE OUTLINE

A 71-year-old female patient noticed hand tremor in the middle of the third decade of life, and later slowly progressive bilateral hearing loss. At the age of 64 she developed unsteady gate, hand clumsiness and dysarthria, to became incapable of independent walking and standing five years later. Clinical course and brain MRI findings were typical for SS, but additional investigation did not reveal the couse of subarahnoidal bleeding.

CONCLUSION

SS represents a rare and under-recognized condition that must be considered in all patients with cerebellar syndrome of unknown cause. Early diagnosis of SS in some cases with identified cause of chronic bleeding allowes therapeutic interventions that may prevent further progression of the disease.

摘要

引言

浅表性铁沉积症(SS)由慢性蛛网膜下腔出血引起,其特征是游离铁和含铁血黄素沿中枢神经系统软脑膜和软膜下结构沉积。SS会导致中枢神经系统进行性和不可逆的损害。慢性蛛网膜下腔出血最常见的原因是肿瘤、头部和脊髓创伤、动静脉畸形和动脉瘤。SS的临床特征为三联征:感音神经性听力丧失、小脑共济失调和锥体束征。脑部磁共振成像(MR成像)是诊断SS的首选检查方法。典型表现包括在T2加权MR成像上可见围绕脑、小脑、脑干、脊髓、第八对脑神经的低信号以及小脑和延髓萎缩。

病例概述

一名71岁女性患者在30岁左右时注意到手部震颤,随后出现缓慢进展的双侧听力丧失。64岁时,她出现步态不稳、手部笨拙和构音障碍,5年后无法独立行走和站立。临床病程和脑部MRI表现符合SS,但进一步检查未发现蛛网膜下腔出血的原因。

结论

SS是一种罕见且未得到充分认识的疾病,所有不明原因的小脑综合征患者都必须考虑到这种疾病。在某些已确定慢性出血原因的病例中,早期诊断SS可进行治疗干预,这可能会阻止疾病的进一步发展。

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