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一位患有表浅性铁质沉着症、脊髓内囊肿、低压性头痛和脊髓低位的患者。

A patient with superficial siderosis, intraspinal cyst, low-pressure headache and low-lying cord.

机构信息

Neuroscience Laboratory, Department of Neurology, China Medical University Hospital, Taichung.

出版信息

Acta Neurol Belg. 2011 Dec;111(4):357-9.

Abstract

Superficial siderosis is a rare condition caused by deposition of hemosiderin in the central nervous system. In recent years, it has been used to identify dural defects connecting the intrathecal space with the intraspinal fluid-filled collection seen in patients with superficial siderosis. However rare reports describe the association of low-lying cord in superficial siderosis patients. We present a patient with progressive cerebellar ataxia, sensorineural hearing loss, low-pressure headache, pyramidal signs and additional cervical and lumbar radiculopathy with neurogenic bladder which might be related to cervical disc herniation and tethered cord. CSF and Magnetic resonance imaging (MRI) studies confirmed the diagnosis of superficial siderosis. Dural defect is indirectly suspected by existence of fluid-filled collection ventral to the spinal cord and low-pressure headache in our patient. Underlying causes of low-pressure headache and possible dural defect should be searched for as an associated and possibly treatable condition in superficial siderosis and provide further insights into the mechanism of bleeding in our patient who have an intraspinal fluid-filled collection.

摘要

表浅性铁沉着症是一种罕见的疾病,由铁卟啉在中枢神经系统中的沉积引起。近年来,它被用于识别硬脑膜缺陷,这些缺陷将蛛网膜下腔与脊髓内充满液体的集合连接起来,见于表浅性铁沉着症患者。然而,很少有报道描述低位脊髓在表浅性铁沉着症患者中的关联。我们介绍了一位患有进行性小脑共济失调、感觉神经性听力损失、低压性头痛、锥体征以及额外的颈腰椎神经根病和神经性膀胱的患者,这可能与颈椎间盘突出和脊髓栓系有关。CSF 和磁共振成像 (MRI) 研究证实了表浅性铁沉着症的诊断。在我们的患者中,存在脊髓腹侧充满液体的集合和低压性头痛,间接怀疑存在硬脑膜缺陷。应寻找低压性头痛和可能的硬脑膜缺陷的潜在原因,因为它们是表浅性铁沉着症的一种相关且可能可治疗的病症,并进一步了解我们的患者脊髓内充满液体的出血机制。

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