经颅多普勒超声诊断儿童狼疮脑病

Significance of transcranial Doppler in diagnosis of neuropsychiatric lupus erythematosus in children

目的 探讨经颅多普勒超声 (TCD)在儿童狼疮脑病 (NPLE)诊断中的意义。方法 5 4例系统性红斑狼疮 (SLE)患儿分为三组 :无NPLE组即SLE组 (15例 ) ;NPLE组 (2 0例 ) ;NPLE亚临床组(19例 )。应用TCD仪 ,常规探查颅内双侧大脑前、中、后动脉和颈内动脉、椎动脉及基底动脉共 11条动脉 ;探测参数有 :脑动脉平均峰流速 (Vm)、左右大脑半球血流速度对称性、频谱形态、血流声频、血管波动指数 (PI) ;对NPLE组中 16例TCD异常者做了动态TCD观察。结果 TCD异常表现为Vm增高或下降、左右半球血流速度不对称、涡流频谱、粗糙血流声频、PI下降等。SLE组正常 12例 ,异常 3例 ;NPLE组正常 2例 ,异常 18例 ;NPLE亚临床组正常 0例 ,异常 19例。NPLE组及NPLE亚临床组TCD异常率高于SLE组 (χ2 =32 13,P <0 0 1) ,而前两者间差异无显著性。χ2 检验显示NPLE组和NPLE亚临床组PI下降、Vm增高或下降、双半球血流速度差、涡流频谱、粗糙血流声频异常率均比SLE组高 ,P均 <0 0 1;NPLE组与NPLE亚临床组各项参数差异均无显著性 (P >0 0 5 )。结论 TCD简便、无创、价优 ,有助于长期随访观察SLE患儿病情 。

Objective Neuropsychiatric lupus erythematosus (NPLE)occurs in about 30%～60% of children with systemic lupus erythematosus (SLE), which is often a critical condition. Some neurological abnormalities in SLE have an ominous prognosis. Childhood NPLE is generally considered to carry a favorable prognosis provided it is recognized and treated by a pediatric specialist as early as possible. Childhood NPLE often cannot be recognized at the early stage of the disease until the symptoms of the central nervous system (CNS) involvement become apparent. The purpose of this study was to evaluate significance of transcranial Doppler (TCD) in diagnosis of NPLE in children.Methods The authors studied 54 cases diagnosed as SLE at the rheumatologic clinic, Beijing Children′s Hospital from December 1995 to March 2000. The ratio of boy to girl was 1∶17. The mean age of onset was 9.6 years. The 54 children with SLE were divided into three groups: SLE without NPLE , 15 patients as group 1; SLE with NPLE in 20 patients as group 2; and SLE with subclinical NPLE in 19 patients as group 3. The following parameters were measured :cerebral arterial blood mean peak velocity (Vm), symmetry of bilateral flow rate; ultra sound spectrum; flow sound wave spectrum; pulsatility index(PI).Sixteen cases with NPLE were checked up in different periods from disease onset. Results TCD abnormal findings included: Vm increased or decreased, asymmetry of bilateral flow rate, vortex flow spectrum, rude flow sound wave spectrum, PI decreased. Three of 15 cases in group 1. 18 of 20 in group 2 and all cases in group 3 showed abnormal results (χ 2=32.13, P <0.01).There was no significant differentce between group 2 and group 3( P >0.05). All parameters between group 1 and group 2, group 1 and group 3 were significantly different( P <0.01).TCD findings were 90 7% consistent with the clinical manfestations. TCD showed 5.1% false negativity, 20% false positivity with sensitivity 94.9%, specificity 80.0%, positive predictive value 92.5%,negative predictive value 85.7%, and Kappa 0.746. Conclusion TCD, an inexpensive and non invasive method, seemed to be sensitive and specific in determining early functional disturbances of NPLE that arise before structural changes. It is significant for detecting and following up of the progression of SLE and its early involvement of the central nervous system.