����Ӥ�����49���ٴ����Ե�ͼ�ص�

??Abstract??Objective To summarize the electroclinical features and outcome of benign infantile epilepsy??BIE??. Methods BIE patients were collected in Pediatric Department of Peking University First Hospital.The clinical and EEG data of patients were analyzed.The treatment effects and outcome of patients were followed up.Results In 49 BIE patients??21 were male and 28 were female. The seizure onset age ranged from 3 months to 13 months.Partial seizures were observed in 26 patients??53.1%????secondarily generalized seizures in 23 patients??46.9%????39 patients ??76.9%?? had a history of cluster seizures.No patients had history of status epilepticus??24 patients had a family history of seizures??15 patients had a family history of benign familial infantile epilepsy.The interictal EEG was normal in 33 ??67.3%?? cases.The interictal discharges were recorded in 16 cases.Ten of sixteen cases had interictal discharges in lateral or bilateral Rolandic area. Ictal video EEG was recorded in 4 patients.Ictal discharges originated from temporal region in three patients and from occipital region in one patient.Five patients were not treated with antiepileptic drugs??and 44 patients accepted antiepileptic monotherapy. Treatment time ranged from 2 months to 24 months ???12.5??9.9?? months??.All patients were followedup over two years ??and no one had seizure relapse. Conclusion The features of BIE include the onset age before one year old??manifesting partial seizures or secondarily generalized seizures??and usually a cluster of seizures??normal interictal EEG or small spikes in Rolandic area??good response to antiepileptic drugs and benign outcome.     

眼球阵挛-肌阵挛综合征的临床诊治分析

目的 总结眼球阵挛.肌阵挛综合征(OMS)的临床特征及治疗经验,以提高诊疗水平.方法 对北京大学第一医院儿科2006至2007年收治的6例OMS患儿的临床、实验室资料及治疗情况进行分析.结果 6例中男1例,女5例,发病年龄12～26个月(平均21个月),其中4例病前有前驱感染史.就诊时主要症状为眼球阵挛、肌阵挛、共济失调、睡眠障碍、行为改变(6例).1例尿检3-甲氧基-4-羟基-苦杏仁酸(VMA)阳性,4例患儿腹部B超提示肝脏轻度增大,3例脑电图背景活动减慢,所有患儿均未见癎样放电.头颅MRI检查1例示内侧纵束、顶盖脊髓束T2高信号,1例腹部CT示L3-4蛛网膜囊肿,余患儿胸腹部CT未见异常.6例均给予促肾上腺皮质激素(ACTH)治疗,急性期有效.2例在随访过程中复发.结论 OMS是一种罕见的神经系统疾病,临床表现为眼球阵挛,并伴肌阵挛、共济失调、睡眠障碍和行为改变,容易复发且神经系统后遗症明显.ACTH治疗有效.     

Lennox-Gastaut 综合征的临床表现和脑电图特点

目的 探讨Lennox-Gastaut综合征（LGS）的临床表现和脑电图特点。方法 62例LGS患儿首诊后,定期随诊临床发作和包括睡眠描记在内的脑电变化,根据临床和脑电图变化调整治疗。对首诊和随诊资料进行分析总结。结果 男37例,女25例,1-4岁为发病高峰（61%）,8岁以前发病为94%,43例（69%）为症状性癫痫,围产期损伤为突出的病因,19例（31%）为隐原性癫痫,患儿在病程中均有2种或2种以上发作形式,强直,不典型失神,点头和猝倒发作为特征性发作。都有不同程度的精神和智能发育迟缓,脑电图背景活动异常者为100%,均有特征性的双侧1-2.5次/s棘慢波综合,29例睡眠中出现双侧中短程爆发性10-14次/s快节律,主要根据发作类型。进行联合和个体化治疗,大部分病例能减少发作频率和减轻发作的严重程度。结论 LGS表现复杂,应重视其临床表现和脑电图特点,作出正确的诊断,及早进行积极而有效的治疗,最大程度上减少和减轻发作,以改善患儿的智能发育。     

局灶运动抑制性发作5例患儿临床特征及脑电图分析

目的　探讨局灶运动抑制性癫痫儿童的临床及电生理特点,提高对此类癫痫的认识。方法　回顾性分析2015年1月至2019年4月首都医科大学宣武医院儿科住院收治的5例有局灶运动抑制性癫痫患儿脑电图特点、临床特征和治疗情况。结果　5例患儿中男4例、女1例,起病年龄从2岁3月龄至9岁不等,5例均表现为一侧肢体无力,发作期间意识清楚,2例伴感觉异常,1例出现言语不能,持续时间数秒至十分钟不等。4例脑电图可见对侧中央、顶、颞为主导联异常放电,1例脑电图未监测到痫样放电,进一步行脑磁图可见对侧中央区少量棘波。1例头颅核磁共振发现右侧顶叶皮质胶质神经元肿瘤。2例给予奥拉西平治疗后无发作,1例给予左乙拉西坦治疗后发作减少（随访5年无发作）,2例出现加用奥卡西平过程中出现药物过敏,1例手术评估后行癫痫手术治疗,1例行肿瘤切除术,术后发作明显减轻。结论　局灶运动抑制性癫痫较少见,易被误诊或漏诊,需提高临床对该病的认识,早期诊断并给予有效治疗。     

ƫ�ྪ�ʪ�ƫ̱��ƫ�ྪ�ʪ�ƫ̱����ڼ��ۺ���11���ٴ�����

目的总结偏侧惊厥-偏瘫(HH)综合征和偏侧惊厥-偏瘫-癫癎(HHE)综合征的临床特征.方法对北京大学第一医院儿科1995～2003年收治的11例HH或HHE患儿的临床资料进行分析.11例均做了头颅MRI和(或)CT检查、脑电图(EEG)检查及血生化测定,6例做了尿代谢病筛查.结果8例诊断为HHE,3例诊断为HH.发病年龄1个月至8岁5个月,平均为2岁9个月.4例首次惊厥前伴有发热.5例偏瘫前有惊厥持续状态.5例为右侧偏瘫,6例为左侧偏瘫.头颅影像学显示2例正常,7例大脑半球病变与临床症状相对应,1例为弥漫性病变,1例查出无关病变.11例EEG均为异常,其中8例EEG示双侧半球明显不对称,11例中4例有异常放电.6例代谢癎筛查均为阴性,1例血乳酸、丙酮酸增高,1例血氨增高.7例采用了以卡马西平为主的抗癫癎药治疗.结论HHE为一种公认的惊厥持续状态导致的后遗症,临床上提高对该病的认识并积极进行治疗干预,有助于改善其预后.     

病灶多变的家族性部分性癫(癎)家系的临床研究

目的 探讨病灶多变的家族性部分性癫(FPEVF)家系的临床特征.方法 收集2008-2010年就诊于本院小儿神经专科门诊的2个FPEVF家系资料,建立家系系谱图,对先证者家系成员的临床特征、脑电图(EEG)、头颅MRI等进行总结分析.结果 1.FPEVF 2个家系共48名成员,存活44名,受累患者达21例(1例死亡).男12例,女9例;平均发病年龄5岁,起病年龄:家系A:1～7岁,家系B:2～10岁.2.发作前无明显诱发因素,白天夜间均有发作,临床表现为单纯部分性发作10例,复杂部分性发作6例,局灶继发全面性发作3例,亚临床发作2例,其中1例伴热性惊厥史.3.EEG检查19例,均有样放电,表现为频发尖波、棘波、尖慢波或棘慢波,其中起源于颞区、额颞区各5例,额区、额顶区各4例,颞枕区1例.4.存活患者20例的神经系统检查均正常,1例双侧海马异常,余MRI均正常.5.自行缓解6例,2例先证者经丙戊酸钠、托吡酯治疗,发作和EEG均有明显改善.结论 1.FPEVF是一种少见的家族性部分性癫综合征,呈常染色体不完全显性遗传,昼夜均可发作,临床多见部分性发作.2.具有明显的表型异质性和遗传异质性,临床易误诊为其他家族性部分性癫,家族史调查是诊断FPEVF的关键.3.不同家系成员脑电图部分性样放电起源于不同脑区,多见于额颞区,且大脑结构无异常.     

甲基丙二酸血症患儿治疗前后的脑电图监测

目的观察甲基丙二酸血症(MMA)患儿治疗前后脑电图的变化,探讨其对MMA疗效评估的意义。方法对2000—2005年在首都儿科研究所确诊为MMA的26例患儿采用16导视频脑电图仪检查,并将治疗前后的脑电图结果进行对比分析。结果(1)脑电图结果:异常16例(16/26,62%),主要为高峰节律紊乱、慢波背景、癎样放电等;正常10例(10/26,38%)。(2)惊厥发作:16例脑电图异常患儿中12例(12/16,75%)有惊厥发作,主要为痉挛样、强直-阵挛发作,病程1个月至4年。(3)转归:脑电图异常伴惊厥发作的12例患儿中,11例发作停止、脑电图改善(正常7例,异常慢波活动4例);1例死于感染后多脏器功能衰竭,其脑电图由高峰节律紊乱转为放电持续状态;11例患儿惊厥控制2~6个月后停用抗癫癎药,随诊1~5年无发作。4例脑电图异常无惊厥发作患儿,3例随诊中无发作,脑电图无改善,1例脑电图持续低电压死亡。10例脑电图正常患儿中1例随诊中脑电图有棘波发放,无临床发作。结论(1)对婴幼儿难治性癫癎伴有智力低下者应进行病因学诊断;(2)脑电图是了解MMA患儿脑功能的客观指标之一,无惊厥发作的MMA患儿亦应进行脑电图的检测;(3)脑电图是评估病因治疗疗效的客观指标之一;(4)合并癫癎的MMA患儿在病因治疗的同时应辅以抗癫癎药物治疗,疗程个体化。     

儿童抗谷氨酸脱羧酶65抗体相关自身免疫性脑炎二例并文献复习

目的探讨儿童抗谷氨酸脱羧酶65(GAD65)抗体相关自身免疫性脑炎的临床特点及预后。方法对2019年于首都医科大学附属北京儿童医院神经内科住院的2例抗GAD65抗体相关自身免疫性脑炎患儿的临床资料进行回顾性分析。并分别以“抗GAD65抗体”“脑炎”“癫痫”“共济失调”和“anti-GAD65 antibody”“encephalitis”“epilepsy”“cerebellar ataxia”为关键词,对中文期刊全文数据库、万方数据知识服务平台、生物医学文献数据库(Pubmed)建库至2020年1月的文献进行检索,选取临床资料完整的儿童病例,总结临床特点及预后。结果2例抗GAD65抗体相关自身免疫性脑炎的患儿均为女性,例1于4岁9月龄起病,以发热伴意识障碍为主要临床表现,头颅磁共振成像提示颅内弥漫性T2加权序列(T2WI)稍高信号,脑电图提示慢波。例2于6岁8月龄起病,以反复局灶性癫痫发作、记忆力减退、头痛为主要临床表现,头颅磁共振成像提示双侧海马T2WI高信号,脑电图提示累及颞区的异常放电。2例患儿均应用了甲泼尼龙及丙种球蛋白免疫治疗,短期症状均有所改善。分别随访6个月及1年,例1完全恢复,例2仍有局灶性癫痫。文献检索共收集6篇英文文献,报道了6例患儿,结合本组共8例。8例患儿中癫痫发作6例,记忆力下降4例,意识障碍、行为异常各3例,认知障碍、头痛各2例,自主神经功能障碍、共济失调、吞咽困难、失语各1例。急性期或亚急性期5例头颅磁共振成像异常,其中3例累及边缘系统,2例边缘系统外受累为主。慢性期3例出现海马萎缩或硬化。8例均加用免疫治疗,免疫治疗后所有患者均短期获得不同程度的改善。随访6个月至6年,3例边缘叶外脑炎预后良好,5例边缘叶脑炎预后欠佳,其中死亡1例,4例遗留局灶性癫痫。结论儿童抗GAD65抗体相关免疫性脑炎是一种罕见可治疗的疾病,包括边缘叶脑炎及边缘叶以外脑炎,以癫痫发作及记忆力下降为常见临床表现。早期诊断并积极免疫治疗可短期内改善症状。边缘叶脑炎慢性期易出现难治性癫痫,长期预后不佳。     

Neurological findings at follow-up in neonatal hypoglycaemia.

Follow-up examination was carried out in 37 children who had been hypoglycaemic during the neonatal period. Mean age was 3 1/2 years. Five out of 7 children with asymptomatic hypoglycaemia neonatally were completely normal, while minimal brain dysfunction was evident in one, and another child showed pathological EEG. Symptomatic, transient hypoglycaemia seemed to carry a poor prognosis as only one out of 9 individuals was normal. Four patients in this group had convulsions after the neonatal period; two of these had recurrence of hypoglycaemia. One had infantile spasms and was severely mentally retarded with spastic diplegia and epilepsy. One girl was blind due to optic nerve atrophy. Four cases of cerebral palsy were detected in this group. Among 21 cases of secondary hypoglycaemia there were no cases of serious neurological sequelae. It is reasonable to assume that neonatal hypoglycaemia is an important prognostic factor. The deleterious effect on the CNS seems to be related to the duration and severity of the hypoglycaemia.     

Benign infantile familial convulsions

Five infants, three girls and two boys, first had convulsions between the ages of 4 and 6 months. Although the aetiology of the attacks was unknown, all the infants had a family history of similar convulsions occurring at the same age and having a benign outcome. The attacks, which always occurred in a cluster, were promptly controlled, in four cases with phenobarbital and in one case with valproate. Seizures were partial with secondary generalization and were characterized by head and eye deviation (not always the same side in each attack) diffuse hypertonia and then bilateral limb jerks. The interictal EEG was normal. The ictal EEG showed diffuse discharge with onset in the central-occipital region. Laboratory, radiological and neurological findings were normal. A history in at least one paternal relative (the father in four cases) of similar seizures, occurring at the same age suggested a genetic predisposition. No seizures or EEG anomalies were observed during the follow up.     

EEG abnormalities preceding the epilepsy onset in tuberous sclerosis complex patients – A prospective study of 5 patients

Tuberous sclerosis complex (TSC) is a multisystem, autosomal dominant disorder characterized by multiple hamartomas development. Epilepsy is the most common symptom appearing in 80–90% of the patients mainly in the first year of life. A prompt and early seizure control is crucial and can prevent development of an epileptic encephalopathy and secondary mental retardation. Therefore the very early identification of seizures seems to be of a great importance. We present the cases of 5 patients diagnosed with TSC prenatally or perinatally and regularly monitored (at 4–6 weeks intervals) with EEG before the epilepsy onset. The patients' age at baseline varied from 9 days to 9 weeks. In all of the patients epileptiform discharges preceded the epilepsy onset. The time interval between abnormality detection on EEG and the epilepsy onset varied from 1 to 8 days. The patient's age at the epilepsy onset ranged from the 17th day to the 5th month of life. In one patient the EEG was abnormal from the beginning and in this patient the epileptic seizures started from the neonatal period. In the rest of the patients (4/5) the EEG remained normal throughout the first months of life. In all of the children epilepsy started with focal motor seizures. Our study is the first prospective one showing the results of the EEG monitoring in TSC patients and the natural evolution of the EEG patterns in patients with the seizures types other than infantile spasms.     

过度惊吓反应症临床及遗传学特征（附６例报告）

目的　分析6例过度惊吓反应症患儿的临床及遗传学特征。 方法　对2011年6月至2018年5月于北京大学第一医院诊断的6例过度惊吓反应症患儿的临床表现、诊治过程、脑电图（EEG）及神经影像学、遗传学结果等进行分析。 结果　6例中男5例、女1例。6例均为新生儿早期起病,均可因不经意的听觉或触觉刺激诱发过度惊跳反应和全身僵硬症状,3例曾出现短暂窒息发作,1例曾惊吓后摔倒致外伤。6例点鼻反射均阳性,4例合并疝。EEG示1例正常,5例为不典型放电或一过性异常。4例曾行头颅磁共振检查未见明显异常。经遗传学分析,6例中3例携带GLRA1基因杂合突变,2例携带GLRB基因复合杂合突变,1例经全外显子测序未发现基因突变。6例给予氯硝西泮治疗, 1例全身僵硬及过度惊吓反应均基本消失, 5例全身僵硬症状消失、偶有惊跳反应。6例智力发育均正常,2例存在宽基底步态状行走姿势。1例有相同症状而窒息死亡的明确家族史。 结论　过度惊吓反应症具有典型临床表现,点鼻反射阳性,可行基因诊断确诊。该病对氯硝西泮有良好的治疗反应。早期经临床诊断及基因确诊,及时、恰当治疗,对于改善预后至关重要。     

过度惊吓反应症临床及遗传学特征（附６例报告）

目的　分析6例过度惊吓反应症患儿的临床及遗传学特征。 方法　对2011年6月至2018年5月于北京大学第一医院诊断的6例过度惊吓反应症患儿的临床表现、诊治过程、脑电图（EEG）及神经影像学、遗传学结果等进行分析。 结果　6例中男5例、女1例。6例均为新生儿早期起病，均可因不经意的听觉或触觉刺激诱发过度惊跳反应和全身僵硬症状，3例曾出现短暂窒息发作，1例曾惊吓后摔倒致外伤。6例点鼻反射均阳性，4例合并疝。EEG示1例正常，5例为不典型放电或一过性异常。4例曾行头颅磁共振检查未见明显异常。经遗传学分析，6例中3例携带GLRA1基因杂合突变，2例携带GLRB基因复合杂合突变，1例经全外显子测序未发现基因突变。6例给予氯硝西泮治疗， 1例全身僵硬及过度惊吓反应均基本消失， 5例全身僵硬症状消失、偶有惊跳反应。6例智力发育均正常，2例存在宽基底步态状行走姿势。1例有相同症状而窒息死亡的明确家族史。 结论　过度惊吓反应症具有典型临床表现，点鼻反射阳性，可行基因诊断确诊。该病对氯硝西泮有良好的治疗反应。早期经临床诊断及基因确诊，及时、恰当治疗，对于改善预后至关重要。     

A Follow-up Study of Children with Neonatal Herpes Simplex Virus Infections with Particular Regard to Late Nervous Disturbances

Forty-five children with neonatal herpes simplex virus (HSV) infection, representing all known cases in the diagnostic records of four virological laboratories within a 15-year period, were followed up. Twelve children had died. Sixteen of the 33 survivors were healthy. Thirteen children had severe disabilities: all of them showed severe mental retardation; moreover, 11 were tetraplegic, one was hemiplegic with hydrocephalus and one had a pronounced behavioural abnormality. Four children had slight to moderate disabilities: one child was mildly mentally retarded and three, although mentally normal, had hemiplegia and delayed speech development, one of them having a learning disorder as well. Of these 17 neurologically impaired children 16 had ophthalmological abnormalities. EEG recordings were made in 29 patients in the neonatal period. They were markedly abnormal in 24 patients, 14 of whom had localized periodic complexes. An abnormal EEG was a bad prognostic sign. The neurological outcome was better in the HSV-l-infected children (10 cases) than in the HSV-2-infected ones (35 cases). Progressive or recurrent encephalitis was strongly suspected in two preterm children.     

A follow-up study of children with neonatal herpes simplex virus infections with particular regard to late nervous disturbances.

Forty-five children with neonatal herpes simplex virus (HSV) infection, representing all known cases in the diagnostic records of four virological laboratories within a 15-year period, were followed up. Twelve children had died. Sixteen of the 33 survivors were healthy. Thirteen children had severe disabilities: all of them showed severe mental retardation; moreover, 11 were tetraplegic, one was hemiplegic with hydrocephalus and one had a pronounced behavioural abnormality. Four children had slight to moderate disabilities: one child was mildly mentally retarded and three, although mentally normal, had hemiplegia and delayed speech development, one of them having a learning disorder as well. Of these 17 neurologically impaired children 16 had ophthalmological abnormalities. EEG recordings were made in 29 patients in the neonatal period. They were markedly abnormal in 24 patients, 14 of whom had localized periodic complexes. An abnormal EEG was a bad prognostic sign. The neurological outcome was better in the HSV-1-infected children (10 cases) than in the HSV-2-infected ones (35 cases). Progressive or recurrent encephalitis was strongly suspected in two preterm children.     

婴儿良性癫癎的临床观察和远期随访研究

目的 研究婴儿良性癫痫的发作特征,脑电图及治疗反应,探讨早期诊断方法。方法 对出生后3-24个月内起病,排除热性惊厥,症状性癫痫及发育异常的婴儿惊厥进行临床观察及寻像脑电图（VEEG）监测,并随访治疗效果和远期预后,结果 42例经2年以上随访确诊为婴儿良性癫痫,其中3例有良性婴儿惊厥家族史,19%惊厥伴有轻微腹泻,67%为短期内频繁发作,无癫痫持续状态,3例VEEG监测证实分别为起源于颞区,枕区及多灶性的部分性发作,发作间期脑电图背景正常,24%睡眠中有Rolandic区小棘波,39例接受抗癫痫单药治疗,平均用药时间9个月,3例未用药物治疗,起病1年内发作均消失,结论 起病早期具有以下特征应考虑有婴儿良性癫痫的可能;（1）起病年龄在3-12个月,不超过24个月,可有婴儿良性惊厥家族史;（2）发病前后精神运动发育正常;（3）发作无诱因,或仅有轻度腹泻等非特异性感染;（4）以部分性发作为主,可继发全身性发作,起病时发作可以很频繁,但无癫痫持续状态;（5）发作间期脑电图背景正常,无典型癫痫样放电,可有睡眠期Rolandic区小棘波;（6）神经影像学正常。     

婴儿严重肌阵挛癫痫的临床特征和基因突变分析

目的 探讨婴儿严重肌阵挛癫癎(SMEI)的临床特点和基因诊断.方法分析13例SMEI患儿的临床和脑电图(EEG)特点及钠离子通道SCN1A基因突变筛查结果.结果男10例,女3例.8例有热性惊厥和癫痫家族史.惊厥起病年龄2～9个月,平均5.6个月.首次发作为热性惊厥9例.13例在病程早期均以反复发热诱发的全面性或一侧性阵挛或强直阵挛发作为主,其中8例有热性惊厥持续状态.出现无热惊厥的年龄为2～21个月.病程中均出现多种发作类型.发作均有热敏感的特点,诱发因素包括发热、洗热水澡和疫苗接种.起病后出现智力发育落后11例.共济失调5例,锥体束征阳性2例.EEG在1岁前多数正常,1岁后出现全导或局灶放电.头颅MBI检查异常2例.13例均应用多种抗癫痫药治疗,发作均未完全控制.卡马西平和拉莫三嗪使部分患儿发作加重.10例发现有SCN1A基因突变.结论 SMEI的临床特点是:1岁以内起病,首次发作常为热性惊厥;1岁以后出现多种发作形式和智力发育落后;发作具有热敏感的特点;EEG早期正常,以后出现全导或局灶放电.筛查SCN1A基因突变有助于早期明确诊断,指导选择抗癫癎药物.     

Cerebellar atrophy: an important feature of carbohydrate deficient glycoprotein syndrome type 1

We report three children, all younger than 2 years of age, presenting with cerebellar atrophy related to carbohydrate-deficient glycoprotein syndrome type 1, an autosomal recessive metabolic disease. One patient had multisystem disease; two others had mental retardation with ataxia. In all cases the cerebellar atrophy was diagnosed on magnetic resonance imaging and, in one case, confirmed by autopsy. The cerebellar atrophy predominantly affected the anterior lobe. Vertical orientation of the tentorium cerebelli from the neonatal period in two cases suggests antenatal onset of the disease. Biological tests confirmed the diagnosis in all cases. Received: 23 February 1998 Accepted: 6 July 1998     

全面性癫癎伴热性惊厥附加症一家系随访分析

目的探讨全面性癫伴热性惊厥附加症(GEFS )的临床意义。方法回顾性分析GEFS 一家系的临床发作情况,作详细体格检查。进行脑电图、24 h动态脑电监测,部分患者作头颅CT检查。结果先证者Ⅳ12,以抽搐频发3 d入院,生后8个月开始高热惊厥(FS)。此次发作为无热性频发全面性强直-阵挛发作。该家系5代共36人。其中有14例患者;男8例,女6例;年龄4岁5个月~82岁,除Ⅰ2发作类型不详外,Ⅱ2、Ⅲ1、Ⅲ4、Ⅲ6、Ⅳ1、Ⅳ11、Ⅳ17、Ⅴ2为FS,Ⅳ2、Ⅳ12、Ⅳ13、Ⅳ14为FS ,Ⅴ1为FS 和失神发作。除Ⅳ13、Ⅳ14目前予丙戊酸镁治疗外,其他患者已减量停药或未用药,均无发作。全家系成员智能发育、全身及神经系统检查均正常。3例行头颅CT检查,均正常。结论GEFS 为常染色体显性遗传性疾病,具有显著遗传异质性和表型异质性。认识该综合征对诊断和鉴别诊断儿童时期癫具有重要的临床意义。     

儿童狼疮性脑病临床特征

目的探讨儿童狼疮性脑病(NPSLE)的临床特点、辅助检查及治疗。方法对11例NPSLE患儿的临床资料进行分析。结果11例NPSLE患儿均有不同程度的头痛症状,意识障碍及失语各1例,抽搐、记忆力减退和共济失调各3例,出现病理反射及脑膜刺激征各4例。血清学均呈狼疮活动表现。3例脑脊液常规无异常,寡克隆抗体明显升高。其中10例行头颅CT检查,异常9例;10例患儿行脑电图检查,正常1例,且与CT无交叉。经个体化综合治疗,11例患儿神经精神症状均有不同程度的改善。结论系统性红斑狼疮累及神经系统症状最常表现为头痛。目前NPSLE主要根据临床表现作出诊断,血清学、影像学及脑电图检查有助于狼疮性脑病的诊断。对NPSLE的治疗强调个体化的综合治疗。