McLean B N, Miller D, Thompson E J
Department of Neurochemistry, Institute of Neurology, London, UK.
J Neurol Neurosurg Psychiatry. 1995 May;58(5):548-54. doi: 10.1136/jnnp.58.5.548.
A retrospective study of CSF and serum analysis from a total of 43 patients with sarcoidosis, 20 with systemic lupus erythematosus, and 12 with Behçet's disease with neurological involvement found local synthesis of oligoclonal IgG using isoelectric focusing and immunoblotting in 51%, 25%, and 8% respectively at some stage in their disease. Blood-brain barrier breakdown, when assessed with an albumin ratio found 47% of patients with sarcoidosis, 30% of those with systemic lupus erythematosus, and 42% of patients with Behçet's disease exhibiting abnormal barrier function at some time. Serial CSF analysis showed that clinical relapses were associated with worsening barrier function and in some patients the development of local oligoclonal IgG synthesis; conversely steroid treatment led to a statistically significant improvement in barrier function, and in two patients a loss of oligoclonal IgG bands. A higher proportion of patients had MRI abnormalities than oligoclonal IgG or blood-brain barrier breakdown, MRI being abnormal in 16 of 19 patients with sarcoidosis, three of four patients with systemic lupus erythematosus, and seven of nine patients with Behçet's disease, although this may have been due to temporal factors. In the differential diagnosis of chronic neurological disorders, locally synthesised oligoclonal IgG cannot distinguish between diseases, but the loss of bands seen in two patients contrasts with what is seen in multiple sclerosis, and thus may be a useful diagnostic clue.
一项回顾性研究对43例结节病患者、20例系统性红斑狼疮患者和12例有神经受累的白塞病患者的脑脊液和血清进行分析,发现采用等电聚焦和免疫印迹法检测,分别有51%的结节病患者、25%的系统性红斑狼疮患者和8%的白塞病患者在疾病的某个阶段出现寡克隆IgG的局部合成。用白蛋白比率评估血脑屏障破坏情况时发现,47%的结节病患者、30%的系统性红斑狼疮患者和42%的白塞病患者在某些时候表现出异常的屏障功能。连续的脑脊液分析显示,临床复发与屏障功能恶化相关,在一些患者中还与局部寡克隆IgG合成的出现有关;相反,类固醇治疗使屏障功能有统计学意义的改善,在两名患者中寡克隆IgG条带消失。MRI异常的患者比例高于寡克隆IgG或血脑屏障破坏的患者比例,19例结节病患者中有16例、4例系统性红斑狼疮患者中有3例、9例白塞病患者中有7例MRI异常,不过这可能是由于时间因素所致。在慢性神经疾病的鉴别诊断中,局部合成的寡克隆IgG无法区分不同疾病,但两名患者中出现的条带消失与多发性硬化症中的情况不同,因此可能是一个有用的诊断线索。