特发性肥厚性硬脑膜炎12例临床分析

Clinical analysis in twelve patients with idiopathic hypertrophic pachymeningitis

目的总结特发性肥厚性硬脑膜炎的临床表现和实验室特点,并探讨特发性肥厚性硬脑膜炎与自身免疫反应的相关性。方法回顾分析12例特发性肥厚性硬脑膜炎患者的临床表现、实验室检查、MRI检查和治疗经过,与18例颅内静脉窦血栓形成患者的实验室治疗进行比较。结果 12例患者临床主要表现为头痛、脑神经损害症状和小脑症状;血清学指标,2例(2/12)于正常值范围,4例(4/12)血常规白细胞计数升高,7例(7/12)血清C-反应蛋白、红细胞沉降率和血清淀粉样蛋白A (serum amyloid A,SAA)水平升高,2例(2/12)类风湿因子水平升高,1例(1/12)抗DNA-组蛋白复合物抗体,1例(1/12)抗核抗体阳性、1例(1/12)可疑阳性,1例抗心磷脂抗体(anti-cardiolipin antibody,ACA)可疑阳性,1例[1/12髓过氧化物酶(myeloperoxidase,MPO)]阳性;脑脊液指标,6例(6/12)压力升高; 4例(4/12)白细胞数升高,10例蛋白定量增高,2例蛋白定量正常,8例(8/12) Pandy试验阳性,1例(1/12)可疑阳性,11例(11/12) IgA升高、11例(11/12) IgM升高、8例(8/12) IgG升高。特发性肥厚性硬脑膜炎患者仅脑脊液蛋白定量(t=4. 78,P=0. 00)以及IgA(t=5. 12,P=0. 00)、IgM(t=2. 16,P=0. 04)和IgG(t=4. 29,P=0. 00)水平高于颅内静脉窦血栓形成患者; Pearson相关分析显示,特发性肥厚性硬脑膜炎患者脑脊液蛋白定量与脑脊液IgA(r=0. 690,P=0. 01)和IgG(r=0. 702,P=0. 01)水平均呈正相关,而与IgM(r=0. 483,P=0. 11)不相关。激素和免疫抑制剂治疗有效。结论特发性肥厚性硬脑膜炎可能系自身免疫反应所致,免疫球蛋白水平升高考虑以鞘内合成和炎症反应为主要原因。

Objective To summarize the clinical and laboratory characteristics of idiopathic hypertrophic pachymeningitis( IHP) and to explore the correlation between idiopathic hypertrophic cranial hard meningitis and autoimmune reaction. Methods The clinical manifestations,laboratory examinations,MRI examinations and treatment of 12 patients with HIP were retrospectively analyzed and compared with the cerebrospinal fluid results of 18 patients with cerebral venous sinus thrombosis( CVST). Results The main clinical manifestations of 12 patients were headache,cranial nerve damage symptoms and cerebellar symptoms. Serological indices: 2 cases( 2/12) were within normal range,4 cases( 4/12) had elevated routine white blood cell count,7 cases( 7/12) had elevated serum C-reactive protein,erythrocyte sedimentation rate and serum amyloid A( SAA),2 cases( 2/12) had elevated rheumatoid factor levels,one case( 1/12) was positive for anti-DNAhistone complex antibody,one case( 1/12) was positive for anti-nuclear antibody,one case( 1/12) was suspicious for anticardiolipin antibody ACA,one case( 1/12 myeloperoxidase,MPO). In cerebrospinal fluid( CSF),6 cases( 6/12) had elevated pressure,4 cases( 4/12) had elevated white blood cell count,10 cases had elevated protein content,2 cases had normal protein content,8 cases( 8/12) had positive Pandy test,1 case( 1/12) had suspected positive,11 cases( 11/12) had elevated IgA,11 cases( 11/12) had elevated IgM,8 cases( 8/12) had elevated IgG. The levels of cerebrospinal fluid protein( t = 4. 78,P = 0. 00) and IgA( t = 5. 12,P = 0. 00),IgM( t = 2. 16,P = 0. 04) and IgG( t = 4. 29,P = 0. 00) in patients with HIP were higher than those in patients with intracranial venous sinus thrombosis( IVST). Pearson correlation analysis showed that CSF protein content was positively correlated with CSF IgA( r = 0. 690,P = 0. 01) and IgG( r = 0. 702,P = 0. 01),but not with IgM( r = 0. 483,P = 0. 11). Hormones or immunosuppressive agents were effective for treatment.Conclusion Idiopathic hypertrophic duratitis may be cause by autoimmune responses. Increased levels of immunoglobulin may be due to intrathecal synthesis and inflammation.