PDF(Pubmed)
Abstract：
Superficial siderosis is a disease in which hemosiderin is deposited under the leptomeninges and subpial layers of hindbrain structures, e.g., the cerebellum, brainstem, and eighth cranial nerve. The main symptoms of superficial siderosis are cerebellar ataxia, hearing loss, cognitive decline, and myelopathy. The activities of daily living of patients with superficial siderosis are severely impaired due to the progressive symptoms. Here, we report a patient with superficial siderosis whose symptoms deteriorated after lumbar subarachnoid-peritoneal (L-P) shunt surgery. She received L-P shunt surgery based on the diagnosis of idiopathic normal pressure hydrocephalus at another hospital. The patient had a history of cervical surgery, and a dural defect was identified at the C4-5 level by a detailed magnetic resonance imaging study. We hypothesized that the L-P shunt reduced cerebrospinal pressure and increased bleeding from the fragile vessels in the dural defect, which might have increased hemosiderin deposition.
摘要：
浅表性铁质沉着症是一种铁血黄素沉积在后脑结构的软脑膜和软脑膜下的疾病，例如，小脑,脑干,和第八脑神经。浅表铁质沉着症的主要症状是小脑共济失调，听力损失,认知能力下降,和脊髓病。由于进行性症状，浅表铁质沉着症患者的日常生活活动严重受损。这里,我们报告了一名患有浅表铁质沉着症的患者，其症状在腰椎蛛网膜下腔-腹膜（L-P）分流手术后恶化。她在另一家医院根据特发性正常压力脑积水的诊断接受了L-P分流手术。患者有颈椎手术史,并且通过详细的磁共振成像研究在C4-5水平确定了硬脑膜缺损。我们假设L-P分流术降低了脑脊髓压，并增加了硬膜缺损中脆弱血管的出血，这可能增加了铁血黄素的沉积.
