Mas J L, Louarn F, Degos J D
Rev Neurol (Paris). 1983;139(8-9):467-84.
Central nervous system manifestations of systemic lupus erythematosus are reported in 25 to 60 p. cent of cases and include mental disturbances, epilepsy, focal deficits, and headache. Cerebrospinal fluid (CSF) changes are inconstantly observed. Cerebral scintigraphy may be useful. CT Scan imaging shows infarcts, hemorrhages, or cortical atrophy. Cerebral angiography is usually normal. Pathological examination shows frequent arteriolar lesions in the CNS but their appearance is rarely that of an angiitis. The mechanism of the CNS lesion involves both angiitis and antineuronal antibodies. Diagnosis is difficult when presenting signs are those of CNS involvement, particularly as the ESR can be normal. Serum complement and anti-DNA antibody levels are frequently normal when there is an isolated CNS involvement. Overall prognosis is poor in cases with CNS lesions. The use of corticoids is discussed. Cerebral angiitis is an exceptional finding during the course of rheumatoid arthritis and scleroderma. Central neurological manifestations of Sharp's and Gougerot-Sjögren's syndromes have recently been reported. CNS lesions are reported in 10 to 20 p. cent of patients with panarteritis nodosa and include mental disorders and disturbance of vigilance, epilepsy, focal deficits, meningeal signs and headache. The CSF is often normal. The CT scan provides images of infarcts, hemorrhages or cortical atrophy. Cerebral angiography may show segmental stenoses and distal occlusions. Pathological examination of the CNS shows mainly lesions in the intracerebral and leptomeningeal arterioles and small caliber arteries. Treatment is by corticoids and immunosuppressors. Angiitis of the CNS is rare and of late onset in Wegener's granulomatosis. Hypersensitivity angiitis rarely affects the CNS, those cases where it is involved being of poor prognosis and probably related to panarteritis nodosa. CNS manifestations in giant cell arteritis and Takayasu's arteritis result from neck artery lesions. The CNS is affected in 20 p. cent of cases in Behcet's disease with resulting isolated aseptic meningitis or a meningo-encephalomyelitis, and intracranial hypertension. The CSF is nearly always abnormal. Cerebral angiography should be directed towards the search for cerebral thrombophlebitis. Pathologically lesions predominate in the brain stem. Meningeal lesions are almost constantly present. Neurological involvement is of poor prognosis, and early corticotherapy must be instituted. The nosological autonomy of Buerger's thromboangiitis obliterans is questioned. Lymphomatoid granulomatosis is a particular type of angiitis in which malignant cell infiltration occurs. The CNS is affected in 20 p. cent of cases, and whatever the treatment the prognosis is poor. Granulomatous angiitis of the CNS has the distinctive feature of lesions affecting the CNS vessels either exclusively or predominantly. The antemortem diagnosis is based on biopsy of the leptomeninges, and treatment with corticoids and immunosuppressors may prove effective.
系统性红斑狼疮的中枢神经系统表现见于25%至60%的病例，包括精神障碍、癫痫、局灶性缺损和头痛。脑脊液（CSF）变化并非总能观察到。脑闪烁扫描可能有用。CT扫描成像显示梗死、出血或皮质萎缩。脑血管造影通常正常。病理检查显示中枢神经系统中常出现小动脉病变，但其表现很少像血管炎。中枢神经系统病变的机制涉及血管炎和抗神经元抗体。当中枢神经系统受累的体征出现时诊断困难，尤其是血沉可能正常时。孤立性中枢神经系统受累时血清补体和抗DNA抗体水平常正常。中枢神经系统病变的病例总体预后较差。讨论了皮质类固醇的使用。脑血管炎在类风湿关节炎和硬皮病病程中是罕见发现。最近报道了夏普综合征和古热-斯约格伦综合征的中枢神经表现。结节性多动脉炎患者中有10%至20%出现中枢神经系统病变，包括精神障碍、警觉性障碍、癫痫、局灶性缺损、脑膜刺激征和头痛。脑脊液通常正常。CT扫描可显示梗死、出血或皮质萎缩图像。脑血管造影可能显示节段性狭窄和远端闭塞。中枢神经系统病理检查主要显示脑内和软脑膜小动脉及小口径动脉的病变。治疗采用皮质类固醇和免疫抑制剂。中枢神经系统血管炎在韦格纳肉芽肿中罕见且发病较晚。过敏性血管炎很少累及中枢神经系统，累及中枢神经系统的病例预后差，可能与结节性多动脉炎有关。巨细胞动脉炎和高安动脉炎的中枢神经系统表现源于颈部动脉病变。白塞病患者中有20%出现中枢神经系统受累，导致孤立性无菌性脑膜炎或脑膜脑脊髓炎，以及颅内高压。脑脊液几乎总是异常。脑血管造影应旨在寻找脑静脉血栓形成。病理上病变以脑干为主。脑膜病变几乎总是存在。神经受累预后差，必须尽早开始皮质激素治疗。血栓闭塞性脉管炎的疾病独立性受到质疑。淋巴瘤样肉芽肿病是一种特殊类型的血管炎，其中发生恶性细胞浸润。20%的病例中枢神经系统受累，无论采用何种治疗，预后均差。中枢神经系统肉芽肿性血管炎的独特特征是病变仅或主要累及中枢神经系统血管。生前诊断基于软脑膜活检，皮质类固醇和免疫抑制剂治疗可能有效。
