■炎症与入院时严重脑静脉血栓形成(CVT)的严重程度和出院时预后不良有关。遗传性蛋白C/S缺乏(遗传性PCD/PSD)不仅促进血栓形成,而且激活炎症反应,进一步诱导静脉血栓形成。然而,常规治疗如标准抗凝/血管内治疗(EVT)似乎并不能改善预后.抗炎治疗可能是治疗该疾病的新方法。
■我们从2020年1月至2022年7月招募了5名患有遗传性PCD/PSD的急性/亚急性重度CVT患者。除了标准的抗凝治疗,所有患者均接受短期甲基强的松龙冲击治疗.神经功能缺损,颅内压升高,静脉再通,对治疗前后以及出院后6个月时的血清和脑脊液(CSF)炎症标志物及不良事件进行回顾性描述.
■所有患者入院时炎症指标均显著升高。甲基强的松龙脉冲治疗后,血清炎性指标包括中性粒细胞与淋巴细胞比值(P=0.043);血小板与淋巴细胞比值(P=0.043);全身免疫炎性指标(P=0.043);白细胞介素-6(P=0.043)和超敏C反应蛋白(P=0.022)较基线水平显著降低。脑脊液炎性指标较基线有下降趋势(P>0.05)。在静脉再通方面,一名患者实现了完全再通,4例患者获得部分再通.与入院时的基线相比,NIH卒中量表(NIHSS),出院时改良Rankin量表(mRS)和颅内压均明显降低(P=0.029,P=0.041和P=0.017)。在6个月的随访中,NIHSS和mRS进一步下降。在住院和6个月随访期间,5例患者均未出现严重的类固醇相关不良反应,如静脉血栓复发,自发性骨折或骨坏死,胃十二指肠溃疡.
■患有遗传性PCD/PSD的急性/亚急性重度CVT具有高水平的炎症。除了常规的抗凝治疗,使用类固醇的早期抗炎治疗可能是必要的.然而,大量的随机对照试验需要更大的样本量进行进一步的研究.
UNASSIGNED: Inflammation was associated with the severity of severe cerebral venous thrombosis (CVT) on admission and poor prognosis at discharge. Hereditary protein C/S deficiency (hereditary PCD/PSD) not only promotes thrombosis but also activates the inflammatory response, further inducing venous thrombosis. However, conventional treatments such as standard anticoagulant/endovascular therapy (EVT) do not seem to improve prognosis. Anti-inflammatory therapy may be a new way to treat the disease.
UNASSIGNED: We enrolled five patients with acute/subacute severe CVT with hereditary PCD/PSD from January 2020 to July 2022. In addition to standard anticoagulant therapy, all of them were given short-term methylprednisolone pulse therapy. Neurological deficit, increased intracranial pressure, venous recanalization, serum and cerebrospinal fluid (CSF) inflammatory markers and adverse events were retrospectively described before and after treatment and at 6 months after discharge.
UNASSIGNED: Inflammatory indexes of all patients were significantly elevated on admission. After methylprednisolone pulse therapy, serum inflammatory indexes including neutrophil-to-lymphocyte ratio (P=0.043); platelet-to-lymphocyte ratio (P=0.043); systemic immune inflammatory index (P=0.043); interleukin-6 (P=0.043) and hypersensitive C-reactive protein (P=0.022) reduced dramatically compared with baseline. CSF inflammatory indexes had a decreasing trend compared with baseline (P>0.05). In terms of venous recanalization, one patient achieved complete recanalization, four patients obtained partial recanalization. Compared with baseline on admission, the NIH Stroke Scale (NIHSS), modified Rankin Scale (mRS) and intracranial pressure were all considerably lower at discharge (P=0.029, P=0.041 and P=0.017). At 6-month follow-up, NIHSS and mRS further declined. During hospitalization and 6-month follow-up, none of the five patients experienced severe steroid-related adverse effects such as recurrence of venous thrombosis, spontaneous fracture or osteonecrosis, and gastroduodenal ulcer.
UNASSIGNED: Acute/subacute severe CVT with hereditary PCD/PSD has high levels of inflammation. In addition to conventional anticoagulant therapy, early anti-inflammatory therapy using steroids may be necessary. Nevertheless, substantial randomized controlled trials with larger sample sizes are required for further investigation.