Boselie Toon Fm, van Aalst Jasper, Staals Julie
Department of Neurosurgery, Maastricht University Medical Centre+, Maastricht, The Netherlands
Department of Neurosurgery, Maastricht University Medical Centre+, Maastricht, The Netherlands.
BMJ Case Rep. 2021 Mar 17;14(3):e239194. doi: 10.1136/bcr-2020-239194.
Superficial siderosis is a rare disorder characterised by the deposition of haemosiderin on the surface of the central nervous system. Cognitive dysfunction has sporadically been reported in relation with superficial siderosis. We present a 61-year-old man with cognitive dysfunction in the presence of the typical radiological image of temporal and cerebellar superficial siderosis, most likely due to pseudomeningocoele 14 years after resection of a meningioma at the cervicothoracic junction. Xantochromia was present on cerebrospinal fluid investigation and a source of bleeding was seen during surgical exploration. Despite surgical treatment of the suspected bleeding source, the patient deteriorated and neuropsychological examination 1 year after surgery showed progression of cognitive dysfunction to dementia. It is likely that in the absence of other typical symptoms such as cerebellar ataxia and hearing loss, the cognitive dysfunction was not related to the superficial siderosis.
浅表性含铁血黄素沉着症是一种罕见的疾病,其特征是含铁血黄素沉积于中枢神经系统表面。认知功能障碍偶尔会与浅表性含铁血黄素沉着症相关联。我们报告一名61岁男性,其存在颞叶和小脑浅表性含铁血黄素沉着症的典型影像学表现,伴有认知功能障碍,最可能是由于在颈胸交界处脑膜瘤切除术后14年出现假性脑膜膨出所致。脑脊液检查发现有黄变现象,手术探查时可见出血源。尽管对疑似出血源进行了手术治疗,但患者病情仍恶化,术后1年的神经心理学检查显示认知功能障碍进展为痴呆。在没有小脑共济失调和听力损失等其他典型症状的情况下,认知功能障碍很可能与浅表性含铁血黄素沉着症无关。