原发性中枢神经系统血管炎：45 例患者的临床特征和结局。
Primary angiitis of the central nervous system: Clinical profiles and outcomes of 45 patients.
机构信息
Comprehensive Stroke Care Program, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
Department of Neurology, University of Cincinnati, Cincinnati, OH, USA.
出版信息
Neurol India. 2019 Jan-Feb;67(1):105-112. doi: 10.4103/0028-3886.253578.
OBJECTIVE
To describe the clinical profile, treatment response and predictors of outcome in patients with primary angiitis of the central nervous system (PACNS) from a single tertiary care center.
METHODOLOGY
Retrospective analysis of consecutive patients diagnosed with PACNS from January 2000 to December 2015. Outcome was defined as poor when the 6-month modified Rankin scale (mRS) was ≥3.
RESULTS
The median age of the 45 patients included in this study was 36 (range 19-70) years at disease onset and 31 (68.9%) were males. The initial presentation was ischemic stroke in 15 (33.3%), hemorrhagic stroke in 4 (8.9%), headache in 11 (24.4%), seizures in 8 (17.8%) and cognitive dysfunction in 5 (11.1%) patients. Diagnosis was confirmed by a four vessel cerebral digital subtraction angiogram (DSA), biopsy and by both biopsy and DSA in 26 (57.8%), 15 (33.3%) and 4 (8.9%) patients, respectively. All patients received glucocorticoids and 14 patients received in addition either cyclophosphamide or azathioprine as their first treatment. The median duration of follow-up was 33.1 (0.7-356) months. A poor 6-month outcome was observed in 12 (26.7%) patients. Relapse occurred in 25 (55.6%) patients and 7 (15.6%) died. Predictors of a poor outcome consisted of cognitive dysfunction at diagnosis (80% vs 20%; P = 0.014) and NIHSS ≥5 (62.5% vs 37.5%; P <.0005). None of the patients with a normal EEG had a poor outcome (P = 0.046). Predictors of relapse were a higher NIHSS at admission (P =.032) and a normal DSA (P = 0.002).
CONCLUSION
In this cohort, severe deficits and cognitive symptoms at onset and an abnormal EEG were associated with a poor 6-month outcome.
目的
描述单一三级医疗中心原发性中枢神经系统血管炎（PACNS）患者的临床特征、治疗反应和预后预测因素。
方法
对 2000 年 1 月至 2015 年 12 月期间连续诊断为 PACNS 的患者进行回顾性分析。将 6 个月改良 Rankin 量表（mRS）评分≥3 定义为预后不良。
结果
本研究纳入的 45 例患者的中位年龄为发病时 36 岁（范围 19-70 岁），31 例（68.9%）为男性。初始表现为缺血性卒中 15 例（33.3%）、出血性卒中 4 例（8.9%）、头痛 11 例（24.4%）、癫痫发作 8 例（17.8%）和认知功能障碍 5 例（11.1%）。通过四血管脑数字减影血管造影（DSA）、活检以及活检和 DSA 联合诊断 26 例（57.8%）、15 例（33.3%）和 4 例（8.9%）患者。所有患者均接受糖皮质激素治疗，14 例患者还接受环磷酰胺或硫唑嘌呤作为一线治疗。中位随访时间为 33.1 个月（0.7-356 个月）。12 例（26.7%）患者 6 个月预后不良。25 例（55.6%）患者复发，7 例（15.6%）患者死亡。认知功能障碍（80% vs 20%；P = 0.014）和 NIHSS≥5（62.5% vs 37.5%；P<.0005）是预后不良的预测因素。EEG 正常的患者均无不良预后（P = 0.046）。入院时 NIHSS 较高（P =.032）和 DSA 正常（P = 0.002）是复发的预测因素。
结论
在本队列中，发病时严重的功能缺损和认知症状以及正常的 EEG 与 6 个月预后不良相关。
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