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当血液留下痕迹时:诊断老年患者的浅表性铁沉积症

When Blood Leaves a Trace: Diagnosing Superficial Siderosis in a Geriatric Patient.

作者信息

Viju Jerin G, Krishnan Parameswaran, M Anand

机构信息

Neurology, Indo American Brain and Spine Center, Vaikom, IND.

Neuroradiology, Indo American Brain and Spine Center, Vaikom, IND.

出版信息

Cureus. 2025 Jul 29;17(7):e89025. doi: 10.7759/cureus.89025. eCollection 2025 Jul.

Abstract

Superficial siderosis (SS) is a rare neurodegenerative condition caused by chronic or recurrent bleeding into the subarachnoid space, leading to the deposition of hemosiderin in the subpial layers of the brain, brainstem, spinal cord, or cranial nerves. SS is categorized into two main forms based on its anatomical distribution: cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS). Among these, iSS is further subdivided into Type 1 (classical iSS) and Type 2 (secondary iSS). Classical iSS refers to cases in which there is no radiologically confirmed single spontaneous or traumatic intracranial hemorrhage and is typically associated with chronic bleeding sources, such as cranial or spinal dural defects. In contrast, secondary iSS is characterized by the presence of a radiologically identifiable single intracranial hemorrhagic event, either spontaneous or traumatic, which is considered the likely cause of the siderosis. Patients typically present with SS in the fourth to sixth decades of life. The prevalence of SS has been increasing in recent years due to the more widespread use of magnetic resonance imaging (MRI). Early imaging and diagnosis are crucial for identifying the bleeding source and enabling timely intervention, which may prevent further neurological deterioration. We report the case of a 69-year-old woman with a history of subdural hematoma and surgical intervention who presented with progressive bilateral hearing loss, imbalance, headache, and hypogeusia. MRI of the brain revealed classical imaging features of SS. Although further evaluation was recommended, the patient declined extensive diagnostic work-up. Empirical treatment with the iron-chelating agent deferiprone was initiated. This case highlights the diagnostic challenges of SS and emphasizes the importance of comprehensive imaging and early intervention.

摘要

浅表性铁沉积症(SS)是一种罕见的神经退行性疾病,由蛛网膜下腔慢性或反复出血引起,导致含铁血黄素在脑、脑干、脊髓或颅神经的软膜层沉积。根据其解剖分布,SS主要分为两种形式:皮质浅表性铁沉积症(cSS)和幕下浅表性铁沉积症(iSS)。其中,iSS又进一步细分为1型(经典iSS)和2型(继发性iSS)。经典iSS是指没有放射学证实的单一自发性或创伤性颅内出血的病例,通常与慢性出血源有关,如颅骨或脊髓硬脑膜缺损。相比之下,继发性iSS的特征是存在放射学可识别的单一颅内出血事件,无论是自发性还是创伤性,这被认为是铁沉积症的可能原因。患者通常在40至60岁出现SS症状。近年来,由于磁共振成像(MRI)的更广泛应用,SS的患病率一直在上升。早期成像和诊断对于确定出血源和及时进行干预至关重要,这可能会防止进一步的神经功能恶化。我们报告了一例69岁女性病例,该患者有硬膜下血肿病史并接受过手术干预,出现进行性双侧听力丧失、平衡失调、头痛和味觉减退。脑部MRI显示出SS的典型影像学特征。尽管建议进行进一步评估,但患者拒绝了广泛的诊断检查。开始使用铁螯合剂去铁酮进行经验性治疗。该病例突出了SS的诊断挑战,并强调了全面成像和早期干预的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e68/12395121/c3876c49d66a/cureus-0017-00000089025-i01.jpg

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