Ikeda Tokuhei, Noto Daisuke, Noguchi-Shinohara Moeko, Ono Kenjiro, Takahashi Kazuya, Ishida Chiho, Yoshita Mitsuhiro, Kawaguchi Masahito, Kawahara Norio, Iwasa Kazuo, Tomita Katsurou, Yamada Masahito
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1 Takara-machi, Kanazawa 920-8640, Japan.
Clin Neurol Neurosurg. 2010 Jan;112(1):62-4. doi: 10.1016/j.clineuro.2009.08.020. Epub 2009 Sep 11.
We report two cases of superficial siderosis (SS) of the central nervous system (CNS), which is caused by chronic haemorrhaging into the subarachnoid space with haemosiderin deposition in the superficial portion of the CNS. Patient 1 had fluid collection in the spinal canal, which was reported as the source of the chronic bleeding. Patient 2 was bleeding from thickened dura at the level of the sacral vertebrae. Both of the patients had xanthochromic cerebrospinal fluid. We surgically repaired the sources of bleeding. Subsequently the cerebrospinal fluid (CSF) cleared and their symptoms were not aggravated for about 1 year. We measured several CSF markers of SS before and after surgery. Total tau protein (CSF-t-tau), phosphorylated tau protein (CSF-p-tau), iron (CSF-iron) and ferritin (CSF-ferritin) in the CSF were highly elevated at diagnosis. After surgery, the levels of CSF-t-tau and CSF-p-tau were markedly reduced while CSF-iron and CSF-ferritin had not decreased. It is suggested that CSF-t-tau and CSF-p-tau reflected the neural damage in SS and were useful to evaluate the effectiveness of SS therapies.
我们报告了两例中枢神经系统(CNS)浅表性铁沉积症(SS),其由蛛网膜下腔慢性出血伴含铁血黄素沉积于CNS浅表部位所致。病例1椎管内有积液,据报告这是慢性出血的来源。病例2在骶椎水平增厚的硬脑膜处出血。两名患者的脑脊液均呈黄变。我们对出血源进行了手术修复。随后脑脊液(CSF)变清,且他们的症状在约1年时间内未加重。我们在手术前后测量了SS的几种脑脊液标志物。诊断时,脑脊液中的总tau蛋白(CSF-t-tau)、磷酸化tau蛋白(CSF-p-tau)、铁(CSF-iron)和铁蛋白(CSF-ferritin)水平显著升高。术后,CSF-t-tau和CSF-p-tau水平明显降低,而CSF-iron和CSF-ferritin水平未下降。提示CSF-t-tau和CSF-p-tau反映了SS中的神经损伤,有助于评估SS治疗的效果。