孤独症合并癫患儿的临床及脑电图特征

目的 探讨孤独症合并癫患儿的临床、脑电图(EEG)特征及治疗效果.方法 对56例孤独症合并癫患儿应用抗癫药物治疗,治疗前后行EEG检查,观察其预后,并对临床特点及EEG进行分析.结果 孤独症合并癫患儿中,癫发作类型以局限性发作最为常见(75%),其中63%是复杂局限性发作.其他较常见的发作形式是强直性发作(36%)和热性惊厥(27%),强直-阵挛性发作、失张力发作、不典型失神发作等发作形式也可出现.EEG主要表现为阵发性异常,包括背景活动减慢、阵发性快活动、阵发性δ活动、阵发性θ活动等非特异改变10例,以及局灶性、多灶性、弥散性尖(棘)波、尖(棘)慢波活动等性放电46例,其中位于颞叶26例(46%),额叶10例(18%),中央区9例(16%),枕叶6例(10%).病变部位双侧局灶性29例(52%),单侧局灶性19例(34%),弥散性异常改变8例(14%).经正规抗癫治疗,77%的病例获得1 a以上无发作控制效果,其中63%单药治疗有效,37%的患儿联合用药有效,23%的患儿未能获得控制.结论 孤独症合并癫患儿存在不同类型的癫发作形式,局限性发作为其最常见的发作形式,大部分为复杂局限性发作.EEG主要表现为阵发性异常,部分出现癫样放电,EEG异常多起源于颞叶,多双侧出现.抗癫药物对于这类患者的癫发作有很好的控制效果,与其他原因所致癫治疗效果类似.     

On the origin of EEG-slowing and encephalopathy during induction treatment of acute lymphoblastic leukemia

BACKGROUND: Neurological complications and EEG slowing frequently occur in children undergoing induction treatment for acute lymphoblastic leukemia (ALL). Disease-related factors and treatment-related toxicity are believed to play causative roles. We wanted to elucidate the etiology further by serial EEG examinations and parallel CSF amino acid analyses. PROCEDURE: Twenty-nine children participated in the study. EEG examinations with quantitative computerized analysis were scheduled on day 1, 10, 29, and 59 of protocol I of BFM-ALL 90 and 95 Study Protocols. CSF analysis for amino acids was carried out on day 1, 15, 29, 45, and 59. RESULTS: A total of 21 of 25 available EEGs showed slight-to-moderate slowing already at diagnosis. The abundance of slow waves was significantly correlated to the white blood count and the CSF glutamine concentration. The EEGs significantly worsened during the first 10 days of treatment with prednisone, VCR, daunorubicin, and intrathecal methotrexate. The following treatment including asparaginase (ASP) gave rise to depletion of CSF from asparagine and a rise of aspartate; glutamine, and glutamate did not follow this pattern. The EEGs remained abnormal, but did not worsen further; the CSF amino acid changes were not related to the EEG. During the subsequent consolidation treatment, the EEGs normalized despite administration of cyclophosphamide, cytara bine, intrathecal methotrexate, and mercaptopurin. CONCLUSIONS: The greater part of EEG changes observed in the early treatment of ALL is due to disease-related factors. Treatment with prednisone, vincristine, and to a much lesser degree asparaginase aggravates the pre-existing encephalopathy. Depletion of CSF from asparagine does not give rise to additional changes. In the second month, the EEG normalizes despite ongoing treatment with different cytotoxic drugs.     

Electroencephalographic changes after pediatric cardiac surgery with cardiopulmonary bypass: is slow wave activity unfavorable?

Pediatric cardiac surgery with cardiopulmonary bypass (CPB) is frequently associated with neurologic deficits. We describe the postoperative EEG changes, assess their possible causes, and evaluate their relevance to neurologic outcome. Thirty-one children and five neonates with congenital heart disease were included. EEG recording started after intubation and continued until 22-96 h after CPB. In addition to conventional analysis, spectral analysis was performed for occipital and frontal electrodes, and differences between pre- and postoperative delta power (delta-deltaP) were calculated. Maximum values of occipital delta-deltaP that occurred within 48 h after CPB were correlated with clinical variables and with perioperative markers of oxidative stress and inflammation. Occipital delta-deltaP correlated with frontal delta-deltaP, and maximum delta-deltaP correlated with conventional rating. Distinct rise of deltaP was detected in 18 of 21 children without any acute or long-term neurologic deficits but only in five of 10 children with temporary or permanent neurologic deficits. Furthermore, maximally registered delta-deltaP was inversely associated with duration of CPB and postoperative ventilation. Maximal delta-deltaP was also inversely associated with the loss of plasma ascorbate (as an index of oxidative stress) and plasma levels of IL-6 and IL-8. Slow wave activity frequently occurs within 48 h after CPB. However, our data do not support the notion that EEG slowing is associated with adverse neurologic outcome. This is supported by the fact that EEG slowing was associated with less oxido-inflammatory stress.     

Antibody response to Escherichia coli L-asparaginase. Prognostic significance and clinical utility of antibody measurement

By using a modification of the microtiter solid-phase radioimmunoassay, we have measured Escherichia coli L-asparaginase (L-ASP) specific IgG, IgG4, and IgE antibodies in children who received L-ASP as part of their chemotherapy for leukemia and lymphoma. In 13 children with acute lymphoblastic leukemia induced with vincristine, prednisone, and L-ASP (10,000 IU/M2 i.v. each week for 3 weeks), seven developed high titer specific IgG antibodies. Four of the seven relapsed at the time of their peaking IgG response (6-10 months). None of the six with low or absent L-ASP antibody response have relapsed (followed for 20-35 months). In six children with allergic reactions to L-ASP reinduction, all had high titers of L-ASP specific IgG4 (greater than or equal to 20 U/ml) at the time of their reaction. In 16 other children with low L-ASP IgG4 (less than 13 U/ml), none demonstrated allergic reactions to rechallenge. Specific IgE was not consistently detectable in either group. In 21 patients with leukemia or lymphoma on L-ASP with cyclophosphamide-containing regimens, none developed significant IgG antibody response, compared with seven of 13 not receiving cyclophosphamide (p less than 0.001). We conclude: (a) development of L-ASP antibodies may have prognostic significance; (b) the detection of specific IgG4 can predict L-ASP allergy; and (c) cyclophosphamide-containing regimens reduce antibody formation to L-ASP and may allow repetitive (without anaphylaxis) and more effective (avoiding neutralizing antibodies) use of L-ASP.     

EEG development in early treated PKU patients from birth to 6 years of age

In 126 early treated PKU patients (type I and type II) a close EEG follow up was performed from birth up to 6 years of age. A total of 1465 EEGs were performed before and after onset of dietary treatment and on 11 more subsequent occasions. The composition of the background activity was normal up to 6 years when only a small number of the children (19) showed no dominant alpha activity. The frequency of epileptiform activity of generalised as well as focal type was low in the first 2 years of life, but afterwards slightly enhanced in comparison to normal control groups. Other findings like generalised theta paroxysms or focal slow waves were rarely observed. Under a standardised protein load at 6 months (52 patients) and at 5 years of age (42 patients) a moderate generalised slowing of the background activity but no other abnormalities were noted.     

CNS late effects after ALL therapy in childhood. Part II: Conventional EEG recordings in asymptomatic long-term survivors of childhood ALL- An evaluation of the interferences between neurophysiology,neurology, psychology,and CNS morphology

Monitoring of therapy-related late effects after acute lymphoblastic leukemia (ALL) therapy in childhood has become an increasingly important area in posttherapeutic patient surveillance. The usefulness of conventional electroencephalographic (EEG) investigations as part of these attempts is controversially discussed. However, EEG recordings have become a popular approach for judgement on the functional integrity of the central nervous system in this subject group. The present report focuses on this problem and discusses the question whether and to what extent conventional EEG recordings were correlated with further measures of central nervous system (CNS) integrity and therapeutic differences. EEGs were recorded in 110 subjects, asymptomatic long-term survivors of ALL in childhood, during a large retrospective multicenter study evaluating CNS late sequelae following antileukemic therapy in Germany and Austria. EEG findings were correlated with demographic data, illness- and treatment-related parameters, as well as with data on the morphological, neurological and psychological status of the participating subjects. At the time of follow-up the EEG was abnormal in 47 cases (42.7%). The most frequent EEG abnormalities observed were disturbances of the background activity (n=45, 95.8%), followed by hypersynchrone activities (n=10, 21.3%) and interhemispheric differences/focal slowings (n=6, 12.8%). With exception of age at diagnosis, none of the observed EEG abnormalities showed a correlation with any of the aforementioned illness- or treatment-related parameters. Eighty percent of the observed EEG abnormalities were found in children younger than 5 years at diagnosis. Children less than 2 years of age as well as those above 5 years at onset of disease showed a significantly reduced prevalence of EEG disturbances compared to subjects between 2 and 5 years at diagnosis. Neither the degree of illness nor therapy-specific differences showed any relationship to EEG outcome. There was no specific EEG finding for a specific morphological substrate, neurological or psychological deficiency and vice versa. Overall, there was no beneficial effect of routine EEG testing in children following therapy for ALL. According to our data, the evaluation of conventional EEG recordings of otherwise asymptomatic ALL long-term survivors is not a very helpful measure for predicting the degree of behavioral deficiencies, neurological disturbances, or morphological CNS abnormalities, which may be present or will develop in this special subject group. Med. Pediatr. Oncol. 29:121–131, 1997. © 1997 Wiley-Liss, Inc.     

Prospective neurophysiological study in children treated for acute lymphoblastic leukaemia: Serial EEG during treatment and long-term follow up with evoked potentials

In 79 children treated for acute lymphoblastic leukaemia according to protocol ALL-BFM 81, serial EEG examinations were performed before, during and after therapy. Diffuse changes of the background activity were observed in 64% of the children at the time of diagnosis. During induction and reinduction treatment with vincristine andl-asparaginase, and with some delay after CNS irradiation, a marked slowing developed in up to 65% of patients. Children who had not been irradiated showed transient disturbances during treatment with medium-dose-methotrexate. Reinduction induced more abnormal EEGs in the children who had been irradiated. At the end of maintenance therapy, only slight EEG changes were found. No differences between the irradiated and non-irradiated group were then seen. Children with CNS leukaemia or seizures differed from those with an uncomplicated treatment in that they more often showed focal and persistent disturbances. In 39 patients who stayed in first remission for at least 18 months after the termination of treatment, a follow up investigation was performed. From the EEG examination, including power spectral analysis, no differences were found between irradiated and non-irradiated patients. Slowing of the dominant frequency was seen in the patients with more severe leukaemia and in those whose EEG had been markedly abnormal at diagnosis. The visually evoked potentials were normal in all groups of patients. In the brainstem auditory evoked potential, a prolongation of the latency of wave I and a decrease of the I–V interval was found in irradiated patients. We conclude that the diffuse EEG changes frequently emerging during treatment are reversible. Persistent or lateralized changes can indicate a neurological complication. Neurophysiological abnormalities found at long-term follow up, for the most part cannot be attributed to side-effects of the treatment but rather to disease related factors.     

NATURAL HISTORY OF JUVENILE RHEUMATOID ARTHRITIS A Follow-up Study of a Case with Special Reference to Clinical, Electroencephalographic and Neuropathological Findings

ABSTRACT. A detailed comparison between the clinical and EEG findings is made in a case of a boy with juvenile rheumatoid arthritis (JRA) who died at 15 years, 6.5 years after the beginning of the follow-up period. In the course of the disease, seven EEG recordings were made, showing a progressive diffuse slowing and disorganization with some improvement during short remissions. In relapses, diffuse slowing was associated with grave asymmetries in the EEG which, however, fluctuated and later disappeared without accompanying clinical or neuroradiological abnormalities. An abundancy of different residual findings, however, remained in the EEG after relapses. There were spike-and-wave paroxysms in every record except at the terminal stage. A stepwise slowing and disorganization was also seen in these paroxysms as background activity. The final cause of death was an intraventricular haemorrhage. No cerebral amyloidosis was found at autopsy. In conclusion, it is suggested that JRA is also a brain disease manifested as a cerebral vasculitis.     

Factors predicting the risk of relapse after antiepileptic drug discontinuation in children with partial seizures

The purpose of this study is to identify possible factors which could influence the seizure recurrence after anti-epileptic drug (AED) withdrawal in children with partial epilepsy. AED was discontinued in 82 children who had been free of partial epileptic seizures for 2.0–11.0 years (mean 4.7 years). Twenty-four patients (29.3%) had a relapse from a few days to 6.1 years (mean 1.2 years) after AED discontinuation. Significantly more common in children who relapsed were: younger age at beginning of AED withdrawal, occurrence of complicated febrile convulsions (5/24 vs 1/58,P<0.01), abnormal neurological examination (8/24 vs 8/58,P<0.05), delayed psychomotor development (7/24 vs 7/58,P<0.05), focal slowing (6/24 vs 3/58,P<0.01) and focal epileptiform discharges (7/24 vs 6/58,P<0.05) in the last EEG before AED discontinuation. Between the two groups no statistical significant differences no statistical significant differences concerning the age at onset of seizures, the duration at AED therapy after the last seizure, the familial occurrence of epilepsy and background EEG abnormalities in the last EEG before AED discontinuation were found. On the basis of EEG, occurrence of febrile convulsions, and neurological and developmental examination it may be possible to predict which children have the best chance to remain free of recurrence after AED discontinuation.Presented in part at the European Congress of Epileptology, Oporto, Portugal, September 6–10, 1994     

Low response rate to 5 aza-cytidine, vincristine, and prednisone therapy in previously treated childhood acute nonlymphocytic leukemia: a Southwest Oncology Group Study

A combination therapy with 5 aza-cytidine (5 AZA-C), vincristine (VCR), and prednisone (PRED) was given to 53 children with acute nonlymphocytic leukemia resistant to conventional therapy. Of these, eight children obtained a complete remission and seven a partial remission. Maintenance therapy was initiated in 12 children. The remission duration varied between 19 and 176 days (median 69 days). The main toxicity was vomiting and myelosuppression manifested as severe neutropenia and thrombocytopenia. It is concluded that VCR and PRED do not increase the response rate to that of 5 AZA-C as a single agent.     

Initial Electroencephalographic Findings in Children with Acute Lymphoblastic Leukaemia

ABSTRACT. Electroencephalography (EEG) was performed on 66 children with acute lymphoblastic leukaemia, 45 before treatment and 21 during the first 5 days of chemotherapy. The patients, aged 7 months to 16 years, 33 boys and 33 girls, had been admitted to the Department of Paediatrics, University of Oulu, between March 1976 and January 1987. The EEG findings were compared with those in 66 age and sex-matched control children chosen at random from the local population. The patients had significantly more frequent and more severe disturbances in background activity ( p < 0.001) than the controls and increased slow waves in the occipital ( p < 0.001) and temporal regions ( p < 0.01). The patients who had received chemotherapy before the EEG recording had EEG disturbances significantly more frequently than the other patients ( p < 0.01), but the latter still had EEG abnormalities significantly more frequently than their matched controls, although they did not have severe changes (grade 3). The results suggest that chemotherapy increases EEG changes during the early days of induction therapy and possibly induces long-term disturbances in brain function. The associations between EEG changes and clinical findings were also analysed and the results show that a long duration of leukaemic symptoms or an aggressive disease may lead to EEG abnormalities.     

Differential diagnosis of visual hallucinations

OBJECTIVE: Visual hallucinations in children need a differential diagnostic effort. METHODS AND PATIENTS: In a retrospective cohort study we identified all children, admitted to the Department of Neuropediatrics of a University Hospital between 1.1.2001 and 31.12.2003 suffering from visual hallucinations. All children underwent neurologic examination and electroencephalography (EEG). RESULTS: 14 children with visual hallucinations were identified. Disturbed perception of the size (9 of 14 cases), of the form (5 of 14 cases), and irregular perceptions of movements (5 of 14 cases) were most frequently reported. One child showed a transient hemihypesthesia, the only pathologic finding in the neurologic examination. Three children had features of hypersynchronic activity in EEG: one child undergoing immunosuppressive drug therapy and with a visual hallucination in context of a reversible posterior leucoencephalopathy showed a focal slow background activity, whereas three children had a sharp wave activity. Two of these children fulfilled the criteria for a focal epilepsy, one of them of the frontal lobe, one of the temporal lobe. CONCLUSION: Recurrent visual hallucinations are frequently transient and show clinical and pathophysiologic features reminiscent of infantile migraine. Psychic etiology, focal epilepsy and, under special circumstances, a reversible posterior leucoencephalopathy have to be considered when making a differential diagnosis.     

Stage III abdominal non-Hodgkin's lymphoma in costa rican children: Comparison of two consecutive trials of treatment

Seventy-three patients with Stage III abdominal non-Hodgkin's lymphoma were prospectively treated following two sequential protocols (P): L278 P (group A, 33 patients) (1978-1983) and L384 P (group B, 40 patients), (1984-1991). No patient received radiotherapy. The L278 P included 7 drugs: cyclophosphamide, vincristine (VCR), adriamycin (ADR), prednisone, methotrexate (MTX), dexamethasone, and 6-mercaptopurine, given for remission induction, maintenance, and CNS prophylaxis. In the L384 P we introduced a consolidation phase consisting of intravenous MTX and citrovorum factor rescue, and IV cytosine arabinoside. VCR was also added to the monthly doses and the maintenance phase was reduced from 18 to 15 months. From January 1988 we changed ADR for epirubicin in the same doses. Prophylactic treatment of the CNS, in the L384 P, was intensified by increasing the number of doses of MTX IT in the remission, induction, and consolidation phases, and with the use of ara-C IT. Laparotomy in 50 patients allowed partial resection in 16, and second-look laparotomy was performed in 27 patients. Viable tumor was found in four patients. Three patients (G-A) died from metabolic complications and another 4 (2 G-A and 2 G-B) failed to attain CR and died. A total of 28 (85%) of 33 children of G-A and 38 (95%) of 40 children in G-B achieved CR. Five children died in remission (2 G-A, 3 G-B). Three patients (G-A) relapsed in the CNS and one (G-B) relapsed in the abdomen and died. Disease-free survival at 120 months was 70% in G-A and 84% in G-B.     

EEG-results in early-treated children with phenylketonuria 4 through 10 years under treatment (author's transl)]

EEG-results in 21 children with Phenylketonuria (PKU), put on a diet at least since their 3rd month of life, now aged 4 through 10 years, with normal psychomotor development, lacking abnormal neurological signs, are compared with the results in 796 healthy children of the same age. Visual evaluation and frequency analysis of the background activity of the EEGs reveal no differences between children with PKU and controls. Generally, there is no close correlation between mean plasma levels of phenylalanine (phe-means) during treatment, and the composition of the EEG when combined age groups are compared. But a trend can be demonstrated: In the small group of 8 years old children phe-means up to 6 mg-% can be associated with a faster (alpha), and phe-means above 6 mg-% with a slower (theta) background activity. The frequency of metabolic derailment (single phe-values above 10 mg-%) does not correlate with the EEG. Focal and generalized hypersynchronous activity (HSA) is observed significantly more often even in early treated, normally developing children with PKU. In children aged 4--8 years, HSA is associated with a high proportion of slow waves compared with those who do not display HSA. In children 9 and 10 years old, however, no such differences can be seen. It is hypothesised that even early treated children with PKU, in their first years show a retardation in the development of their background activity but catch up by 9--10 years. It still remains uncertain whether further age-appropiate development of the EEG can be observed after liberating or discontinuing the dietary regimen at this age.     

In vitro effect of antitumor drugs on lymphocytic blastogenesis in childhood acute lymphoblastic leukemia (ALL) and non-Hodgkin's lymphoma (NHL).

"In vitro" sensitivity of lectin (PHA, Con A)-stimulated lymphocytes to antitumor drugs (ARA-C, ADR, VM26, MTX, CP, VCR, Vepesid, ACLA) and the clinical efficiency of the complex therapy was compared in 7 patients with ALL and 2 patients with NHL. H3-thymidin incorporation of lymphocytes labelled prior to the drug exposure was used as "in vitro" method. A fairly good correlation was found between the "in vitro" test and the clinical response to the drug administered. These results suggest that this "in vitro" test is useful in choosing the drugs to be administered in case of malignancies of children.     

肌阵挛患儿神经电生理的临床研究

目的 借助多项神经电生理技术观测不同起源和不同性质肌阵挛的临床-电生理特征.方法应用视频脑电-肌电多导记录(VEEG-EMG)、抽搐逆向锁定的脑电平均技术(jerk-lockedback averaging,JLA)以及短潜伏期躯体感觉诱发电位(SSEP),对32例肌阵挛发作患儿进行临床和多项电生理的实时联合分析及分类.结果 32例患儿的年龄为1个月～16岁,平均2.8岁,其中皮层性和皮层下性起源各14例,其他肌阵挛4例.(1)皮层性起源组:14例中11例主要表现局灶性或多灶性肌阵挛,3例还同时有全身性肌阵挛发作.11例呈非节律性,3例伴节律性肌阵挛.10例肌阵挛对声响、闪光或叩击肌腱等刺激异常敏感.肌阵挛同步EMG时程为10～52 ms.发作间期脑电图(EEG)有局灶、多灶、或全部性棘-慢波,或高度失律等多种异常.发作期EEG 8例可见全部性1～4 Hz(多)棘-慢波暴发,1例为多灶性1～2.5 Hz棘-慢波,1例无明显改变,其余4例部分肌阵挛发作伴有全部性2～3 Hz棘-慢波.JLA分析13例存在与肌阵挛相关的棘波,1例正常.10例SSEP检测中3例存在巨大皮层反应电位.(2)皮层下起源组:14例中8例为全身性,另6例伴多灶性肌阵挛发作,均为非节律性.对各种感觉刺激皆不敏感.同步EMG时程60～400 ms.间期EEG无恒定异常,包括背景活动正常6例,伴(癎)性放电者8例.9例发作期EEG无明显改变,5例部分肌阵挛发作期伴(癎)性放电.JIA分析12例无异常,2例虽有叠加后棘波,但与肌阵挛发作无锁时关系.14例SSEP皆正常.(3)未能确认起源组:同步EMG时程60～400 ms,EEG及SSEP均正常.根据多项电生理检测结果 ,确认本组32例患儿中,14例为癫(癎)性肌阵挛.结论 (1)对肌阵挛发作及其性质的判定不能仅靠发作间期和发作期EEG;(2)借助多项神经电生理技术,尤其JLA分析,能较好区分不同起源肌阵挛并确认肌阵挛的癫(癎)性质.     

促肾上腺皮质激素治疗婴儿痉挛55例临床研究

目的 总结首次应用促肾上腺皮质激素(ACTH)治疗婴儿痉挛的临床疗效、不良反应、用药前后的脑电图变化,为ACTH的普及应用提供临床借鉴.方法 对我中心既往3年间收治的首次应用ACTH治疗的婴儿痉挛患儿的临床资料进行回顾性分析.结果 (1)控制痉挛发作显效率为54.7%,好转率为15.1%,无效率为30.2%,且疗效与发病年龄、病程及病因有关;(2)不良反应有睡眠周期改变(85.5%)、皮肤颜色变深(81.8%)、心律不齐(65.5%)、血压升高(38.2%)、低钾血症(21.8%)、感染(20.0%)等;(3)用药前脑电图多以高峰节律紊乱为主要背景,用药两周后脑电图高峰节律消失41例(74.5%),好转14例(25.5%).结论 首次应用ACTH治疗婴儿痉挛疗效肯定,尤其可明显改善患儿高峰节律紊乱脑电背景,用药期间也存在一定的不良反应,应密切观察.     

Virilizing hepatoblastoma—Significance of alpha-1-fetoprotein and human chorionic gonadotropin as tumor markers in diagnosis and follow-up

Hepatoblastoma was diagnosed in a 12 month old boy presenting with hepatomegaly and isosexual precocious puberty. Preoperative levels of both alpha-l-fetoprotein (AFP) and human chorionic gonadotropin (HCG) were highly elevated. The tumor was removed by hepatic trisegmentectomy. Tumor tissue contained high concentrations of AFP and HCG. On combination chemotherapy with cyclophosphamide (CYC), vincristine (VCR), adriamycin (ADR) and 5-fluoruracil (5-FU) HCG dropped over a period of 9 months to normal values. In contrast, AFP was undetectable following surgery. One year after initiation of therapy there is no clinical or radiological evidence of recurrence of the malignancy but the observation period is too short to draw any conclusions on the effect of therapy and the final outcome.     

偏侧惊厥-偏瘫-癫(间)综合征的临床特点及诊断

目的探讨偏侧惊厥-偏瘫-癫癎(HHE)综合征的临床特点及诊断。方法对5例HHE综合征患儿的临床资料进行回顾性分析,总结临床特征及其发病的危险因素,进行必要的辅助检查,包括头颅CT和(或)MRI、脑电图及诊断性智力测定或精神运动发育评价。结果4例4岁内起病,3例伴热性惊厥,最长惊厥时间均在2 h以上,并惊厥侧肢体偏瘫。5例均在偏瘫后2年内出现癫癎反复发作。4例为局限性运动发作,1例为精神运动性发作。5例均存在智力障碍或精神发育迟滞。头颅MRI检查4例发现左侧海马硬化,1例CT示右半球萎缩。脑电图均异常,4例见异常放电,1例示明显不对称。5例均予卡马西平为主的药物治疗,癫癎发作得到控制或部分控制。结论HHE综合征是持续偏侧惊厥导致的偏瘫-癫癎综合征,海马硬化可能是反复癫癎所致海马的继发性损伤,而非癫癎的起源灶。应提高对该病的认识,早期正确处理惊厥持续状态将减少HHE综合征发生。     

POSTIRRADIATION SYNDROME AND EEG FINDINGS IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKAEMIA

ABSTRACT: Garwicz, S., Aronson, A. S., Elmqvist, D. and Landberg, T. (Departments of Paediatrics, Clinical Neurophysiology, Radiotherapy, University Hospital, Lund, Sweden). Postirradiation syndrome and EEG findings in children with acute lymphoblastic leukaemia. Acta Paediatr Scand, 64:399, 1975.–Out of 12 children with acute lymphoblastic leukaemia treated with craniospinal irradiation during primary haematologic remission, 8 developed a postirradiation syndrome characterized by fever and tiredness. The symptoms lasted 1–2 weeks and subsided spontaneously. Longitudinal EEG studies revealed no acute disturbances during the irradiation therapy but in all cases studied, moderate to severe diffuse general slowing developed during the postirradiation syndrome. Complete normalization of the EEG occurred in all children at follow-up. It is concluded that the described EEG abnormalities constitute an integral part of the postirradiation syndrome.