原发性中枢神经系统血管炎影像1例

2019-05-22 不详 影像园

患者,女,46岁,头痛5月、加重2月,视物模糊进行性加重1月

【所属科室】

神经内科

【基本资料】

患者,女,46岁

【主诉】

头痛5月、加重2月,视物模糊进行性加重1月

【现病史】

患者5月前无明显诱因出现头痛,表现为胀痛,右侧明显,半月前就诊当地医院神经科,给予营养神经、改善循环治疗后头痛无明显缓解,1月前开始出现左眼视物模糊,眼内窥镜发现眼底出血。

【实验室检查】

脑脊液蛋白136mg/ml。AQP-4、寡克隆区蛋白及自身免疫抗体均阴性。

【影像图片】







































【影像表现】

右侧放射冠、双侧额叶顶叶皮层及白质见多发斑片状稍长T1稍长T2信号,FLAIR呈稍高信号,DWI呈稍高信号,ADC map呈高信号,增强扫描病灶未见明显强化,但相应区域增强扫描可见多发迂曲小血管影,SWI呈低信号。

临床诊断诊断依据】

原发性中枢神经系统血管
 
【病例小结】

原发性中枢神经系统血管炎(PACNS)是一种仅局限于中枢神经系统,主要侵犯脑实质的中小血管和软脑膜微血管管壁的炎性疾病,也称原发性脑血管炎或孤立性中枢神经系统血管炎,病变主要在脑,也可侵犯脊髓,动脉和静脉均可受累。发病率仅占所有中枢神经系统血管炎的1%。在每年发生的脑血管病中,可能占3%~5%。病因及发病机制不明,可能由微生物原或非微生物原所诱发的自身免疫异常。主要是脑实质和软脑膜小动脉的非特异性肉芽肿性炎症,任何大小动脉均可累及,但主要影响小血管和微血管。炎症可造成血管壁增厚、管腔闭塞、血栓栓塞或坏死的管壁破裂,最终导致缺血性或出血性损害。儿童及成人均可发病,但以40~50岁为发病高峰,平均年龄42岁。男女发病率无明显差异。多呈急性或亚急性起病,部分患者隐袭起病。病程较长,以亚急性或慢性波动性进展或缓解与复发交替病程为特征,一般从出现症状到确诊平均5个月。无明显或有轻度全身非特异性症状,表现为发热、肌痛、关节痛、乏力、体质量减轻、红细胞沉降率升高等。辅助检查多无异性改变,ANCA检查胞浆型抗中性粒细胞胞浆抗体(c-ANCA)及环核型抗中性粒细胞胞浆抗体(p-ANCA)可呈阴性或弱阳性,即使阳性但目前多认为应归属ANCA相关性血管炎,不属于PACNS。脑脊液检查有或无异常,有报道80%~90%的患者可呈无菌性脑膜炎样表现,主要为白细胞计数稍高和蛋白增高,糖正常。有时可见寡克隆带(OB)阳性和鞘内IgG合成增加,但多认为即使阳性也缺乏特异性。脑组织活检是诊断金标准,敏感性达53%~80%。主要病理为皮层和软脑膜血管透壁性单核炎性细胞浸润。

影像学异常是诊断的重要依据之一。1/3~2/3患者头CT显示不同程度的异常低密度信号。几乎全部患者均可见MRI异常改变:经典改变为皮层、皮层下白质、深部灰质受累,多表现为单侧或双侧脑内数目多发、形态多样、新老不一、形态不典型且不符合脑血管分布的点片状或脑回样梗死病变;其他少见者尚可呈类脱髓鞘样脑白质病变,脑炎样、线粒体脑病样病变,甚至可呈伴明显水肿的占位性病变,但均无特异性。头MRI呈长T1、长T2信号及FLAIR高信号,个别患者出现短T1信号,可能与合并渗血有关。有报道称DWI高信号、ADC低信号、SWI见微出血者多支持血管炎。增强扫描可见不同类型的强化,表现为皮质下不规则条纹状强化、局灶性皮质带状强化或弥漫性脑实质血管强化,或片状、不规则、脑回样强化;但也可不强化;硬脑膜、软脑脊膜可强化也可不强化,但其强化是诊断的重要支持条件。MRA是常用检查手段,但敏感性和特异性不高。DSA典型改变包括单发或多发性局限性狭窄、节段性狭窄、闭塞、串珠样改变、动脉瘤形成或瘤样扩张等,伴或不伴侧支血管形成,这些改变一是缺乏特异性,二是异常率低,其改变仅见于25%患者,40%经病理证实的患者DSA也可显示正常。

PACNS的诊断目前无统一标准。目前原发性中枢神经系统血管炎临床症状体征、实验室检查结果、影像学表现、脑组织活检均无特异性或不具有通用性,诊断困难、检出率低。为适宜临床应用实际需求,我们提出以下诊断方案供参考。PCNSA诊断依据:(1)有仅限于中枢神经系统的脑损害症状、体征;(2)脑脊液检查无异常或仅有轻度非特异性异常;(3)符合上述血管炎性改变的MRI病灶特点及血管成像学特点;(4)对免疫抑制剂治疗较敏感;(5)经持续3~6个月的病程随访,除可有复发病变外,未发现其他相关性血管炎改变的疾病,可排除系统性、感染性血管炎;(6)经病理检查有符合PCNSA的证据。符合上述(1)~(4)+(6)条者为确诊;符合(1)~(5)条为临床确诊;符合(1)~(4)条为可能。这个标准的优点在于不受难于操作的病理检查的限制,而以临床及影像为主,可提高PACNS的临床检出率。

需要与以下疾病鉴别:1、脑梗死,多发生于高龄患者,常有高血压、动脉硬化及糖尿病病史,病变几乎均累及灰白质,典型者呈楔形,较大病灶MRA可见支配血管狭窄或闭塞。2、多发性硬化,时间积空间多发性, 病灶多位于两侧脑室旁, 病灶形状多为类圆形斑片状且与脑室垂直排列,增强扫描可见环状、结节样及半环状。中毒性脑病的鉴别, 将是否存在中毒史作为其区分鉴别的关键。

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