Department of Neurology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy.
J Neurol Sci. 2012 Jan 15;312(1-2):170-2. doi: 10.1016/j.jns.2011.07.034. Epub 2011 Aug 24.
Superficial siderosis (SS) of the central nervous system is a rare disorder caused by chronic or recurrent hemorrhages into the subarachnoid space with hemosiderin and ferritin deposition, which leads to neuronal damage. The source of bleeding remains unknown in 50% of cases. Recently, attention has been focused on fluid-filled collection in the spinal canal, suggesting the presence of a dural defect which may be the bleeding point. We present a patient with SS and spinal extradural fluid collection due to midthoracic dural defect with spinal cord herniation. The reduction of the spinal cord herniation and the repair of the dural defect resulted in the disappearance of the fluid collection and cerebrospinal fluid abnormalities. The case here reported is, to our knowledge, the first case of spinal cord herniation presenting with SS and confirms the key role played by dural lacerations in the pathogenesis of both SS and spinal cord herniation. The search for dural lacerations should be one of the primary aims in patients with SS.
中枢神经系统表面铁沉积症（SS）是一种罕见的疾病，由蛛网膜下腔反复或慢性出血引起，导致含铁血黄素和铁蛋白沉积，从而导致神经元损伤。在 50%的病例中，出血源仍不清楚。最近，人们的注意力集中在椎管内充满液体的集合上，这表明存在硬脑膜缺陷，而该缺陷可能是出血点。我们报告了 1 例因中胸段硬脑膜缺损伴脊髓疝导致 SS 和硬脊膜外腔积液的患者。脊髓疝的复位和硬脑膜缺损的修复导致了积液和脑脊液异常的消失。据我们所知，本例是首例以 SS 和脊髓疝为表现的脊髓疝病例，证实了硬脑膜撕裂在 SS 和脊髓疝发病机制中的关键作用。在 SS 患者中，应首先寻找硬脑膜撕裂。
