原发性中枢神经系统脉管炎
James Jinxing Wang, MD,PhD
原发性中枢神经系统脉管炎是脑内的一种肉芽肿性脉管炎。这一疾病 并不罕见，临床上初诊时经常被误诊。根据文献及个人经验，目前脑卒中发病人群中，原发性中枢神经系统脉管炎的比例高达0.5%。其临床症状的非特异性，使得该病的诊断有一定的难度。临床上最重要的三个症状为：意识障碍，头痛和定位体征。诊断检查也很不敏感。CT对于脉管炎的诊断意义不大，MRI仅供参考，而MRA则毫无用处。动脉血管造影对于中等或大血管来说有一定的敏感性，但特异性差。最可靠的诊断方法是脑组织活检。但需要神经外科大夫取的活检组织必须包括：灰质，白质和脑膜，才可能得到阳性结果。脑脊液检测最为敏感，90%以上的情况下可见异常，但也缺少特异性。治疗最主要的方法还是激素疗法。它需要长程使用激素，至少要连续使用6个月时间，最好长达两年。若激素无效，也可用免疫抑制剂来取代激素疗法。如果过早停用激素，可导致疾病复发。
Primary Central Nervous System Vasculitis
James Jinxing Wang, MD,PhD President Wesley Neurology Clinic 1211 Union Ave, #400 Memphis, TN38104 USA
The primary central nervous system vasculitis is a granulomatous angiitis in the brain. It is not a rare disorder. However, it is usually missed the first time. Based on the literature and personal experience, the central nervous system vasculitis account for up to 0.5% of total strokes. Because the symptoms are nonspecific, that makes the diagnosis difficult. The three most common clinical symptoms are altered level of consciousness, headaches, and focal neurological deficit. The diagnostic testing is also difficult to interpret sometimes. CT is basically useless for vasculitis. MRI may give some suggestions, but MRA is not useful. Arterial angiogram is useful if it is medium sized and large vessels. It is sensitive, but nonspecific. Brain biopsy is the most reliable testing. However, the neurosurgeon must be notified to get all three matters; gray matter, white matter, and meninges to get positive test. Actually the most sensitive testing is the spinal fluid. More than 90% of the time it is abnormal, but it is nonspecific. In terms of the treatment, steroids are still the main stay. However, it is a long-term treatment, at least six months ideally two years. If the steroids do not work, immunosuppressant may be added on top of steroids. If the steroids are stopped early, the disease may relapse., http://www.100md.com
原发性中枢神经系统脉管炎是脑内的一种肉芽肿性脉管炎。这一疾病 并不罕见，临床上初诊时经常被误诊。根据文献及个人经验，目前脑卒中发病人群中，原发性中枢神经系统脉管炎的比例高达0.5%。其临床症状的非特异性，使得该病的诊断有一定的难度。临床上最重要的三个症状为：意识障碍，头痛和定位体征。诊断检查也很不敏感。CT对于脉管炎的诊断意义不大，MRI仅供参考，而MRA则毫无用处。动脉血管造影对于中等或大血管来说有一定的敏感性，但特异性差。最可靠的诊断方法是脑组织活检。但需要神经外科大夫取的活检组织必须包括：灰质，白质和脑膜，才可能得到阳性结果。脑脊液检测最为敏感，90%以上的情况下可见异常，但也缺少特异性。治疗最主要的方法还是激素疗法。它需要长程使用激素，至少要连续使用6个月时间，最好长达两年。若激素无效，也可用免疫抑制剂来取代激素疗法。如果过早停用激素，可导致疾病复发。
Primary Central Nervous System Vasculitis
James Jinxing Wang, MD,PhD President Wesley Neurology Clinic 1211 Union Ave, #400 Memphis, TN38104 USA
The primary central nervous system vasculitis is a granulomatous angiitis in the brain. It is not a rare disorder. However, it is usually missed the first time. Based on the literature and personal experience, the central nervous system vasculitis account for up to 0.5% of total strokes. Because the symptoms are nonspecific, that makes the diagnosis difficult. The three most common clinical symptoms are altered level of consciousness, headaches, and focal neurological deficit. The diagnostic testing is also difficult to interpret sometimes. CT is basically useless for vasculitis. MRI may give some suggestions, but MRA is not useful. Arterial angiogram is useful if it is medium sized and large vessels. It is sensitive, but nonspecific. Brain biopsy is the most reliable testing. However, the neurosurgeon must be notified to get all three matters; gray matter, white matter, and meninges to get positive test. Actually the most sensitive testing is the spinal fluid. More than 90% of the time it is abnormal, but it is nonspecific. In terms of the treatment, steroids are still the main stay. However, it is a long-term treatment, at least six months ideally two years. If the steroids do not work, immunosuppressant may be added on top of steroids. If the steroids are stopped early, the disease may relapse., http://www.100md.com