Objective To explore influential factors and features of relapse of presenting with an initial episode of acute central nervous system inflammatory demyelinating disease(IDDCNS) in children.Methods By group-control study,20 relapsing patients with IDDCNS compared with 50 single episoding patients with IDDCNS whose case history longer than 0.5 year,whose age at onset,gender,preceding infection or immunization,neurologic syndromes at presentation,therapeutic response,and final neurologic outcome after initial episode were analyzed by mono-factor analysis and multi-factor nonconditional Logistic regression model.Results 1.The risk of relapse increased in the near future,75.0% relapsed before 6 months.There were 18 cases diagnosed multiple sclerosis(MS),1 case neurooptic myelitis and 1 case immaturity myelitis in relapsing patients.There were 39 cases diagnosed acute disseminated encephalomyelitis in single episoding patients.2.Mono-factor analysis:8 factors were not statistically significant,including age at onset,gender,seizure,palsy,conscious disturbance,ataxia,natural relief,sequelae after the first attack;6 factors were statistically significant,including infectious or vaccination agents before episode,fever,visual disorder,multiple neurological signs,positive pathologic reflex,with marked improvement to cortical hormone.3.In the multi-factor stepwise regression analysis,visual disorder(OR=42.50)and positive pathologic reflex(OR=8.666)were the independent influencing factors,multiple neurological signs(OR=0.068)was independent protecting factor.Conclusions The rate of relapse in IDDCNS is lower than abroad and most relapse before 0.5 year,the main diagnosis of relapsing case is MS.When visual disorder and positive pathologic reflex presenting in the fist episode of IDDCNS,the opportunity of relapse will increase,and will decrease when multiple neurological signs presenting.

Anticonvulsants ; therapeutic use ; Brain ; pathology ; physiopathology ; Brain Waves ; physiology ; Child ; Electroencephalography ; Humans ; Landau-Kleffner Syndrome ; physiopathology ; Sleep ; physiology ; Sleep Stages ; physiology ; Status Epilepticus ; epidemiology ; etiology ; physiopathology ; therapy

Objective To study the etiology,clinical characteristics,electroencenphalography(EEG),mental degree of Lennox-Gastaut syndrome(LGS). Method Retrospectively analyzed etiology,sex,age,seizure types,EEG,mental degree of 54 children diagnosed as LGS. Results The number of male was 36,female was 18,seizure onset from 1 month to 8 years,diagnosing age from 3 months to 11years. The EEG reveals 1.5-2.5 Hz spike-wave discharges and a slow baseline activity. Conclusions LGS is one of the most difficult epilepsys to treat and need frequently more than 2 antiepilepsy drugs. It is characterized by variable etiology,multiple types of intractable seizures, and has enormous detrimental effects on patient′s developmental health.

Objective To comparison of the quality of life in children with migraine and quality of life in children with primary epilepsy(EP)or Tourette's syndrome(TS).Methods There were 239 children with moderate migraine,the time of which lasted from 6 to 36(12.14?4.67)months,headache index 4-20(9.98?3.74).There were 250 cases and 424 cases with EP or TS,respectively,both team members were under good control with single drug therapy,the diagnosed according to the international classification of headache disorders-Ⅱ.The pediatric quality of life inventory,version 4.0,age 8-12 years,and child report forms were used to evaluate the quality of life in children with migraine and the other two kinds of samples by Bonferroni and Mann-Whitney tests.Results The scores of quality of life in children with moderate migraine were lower than those in children with EP(total score 69.06?10.48 vs 81.26?13.80;physical function scores 67.43?14.37 vs 83.14?14.70;psychological function scores 69.92?10.56 vs 80.26?14.32;emotional function scores 66.76?14.09 vs 80.90?18.93;social function scores 76.81?14.67 vs 83.36?17.40;school function scores 66.20?13.62 vs 76.52?13.80).The scores of quality of life in children with moderate migraine were lower than those in children with TS(total scores 69.06?10.48 vs 79.18?11.45;physical function scores 67.43?14.37 vs 81.52?12.61;psychological function scores 69.92?10.56 vs 77.90?12.28;emotional function scores 66.76?14.69 vs 74.07?16.34;social function scores 76.81?14.07 vs 89.06?16.23;school function scores 66.20?13.62 vs 70.35?16.96).Two sets of data between children with moderate migraine and those with EP,TS showed statistical significance(Pa

-2,and T)was detected on exon 11 in the mutational analysis of GABRG2.Our results indicate that genomic variations of GABRG2 are not likely to be substantially involved in the etiology of GEFS+in this family. Conclusion Our study fails to provide evidence supporting a causal relation between the SCN1A,SCN1B, GABRG2 mutation and the etiologic genes in this family,which indicates that GEFS+has with phenotypic and genotypic heterogeneity.

Objective To explore the clinical characteristics and diagnostic criteria of migraine in children.Methods The migraine cli-nical characteristics of patients that consistent with the chronic headache diagnostic criteria, excluding other diseases,were analyzed.ICHD-Ⅱ childhood migraine diagnostic criteria was used as gold standard to explore the migraine diagnostic criteria in children.Results 1.In 346 patients, 157 fitted ICHD-Ⅱchildhood migraine diagnostic criteria.2.Type of migraine: probable migraine was the most common(68.8%), the most commonly unfulfilled criterion was associated gastroenteritis symptoms; migraine without aura was the second (19.7%).3.The migraine diagnostic criteria: the most important single-variable was headache associated symptoms,with sensitivity of 70.7%,specificity of 83.6%,the positive predictive value(PPV) was 78.2%,positive likelihood ratio (PLR) was 4.31 and area under curve(AUC) was 0.771.The most important three-variables was headache duration of 1-72 hours, moderate-severe headache and headache associated symptoms, with the sensitivity of 52.2%,specificity of 96.2%,the PPV was 90.1%, the PLR was 13.7 and the AUC was 0. 657.Conclusions The most important single-variable is headache associated symptoms,the most important three-variables are headache duration 1-72 hours, moderate-severe headache and headache associated symptoms.

OBJECTIVETo study the clinical characteristics of myasthenia gravis (MG) in children and the changes in AchR-Ab-seronegative (SNMG) MG and AchR-Ab-seropositive MG (SPMG) patients.

METHODSThe study was done on 77 MG patients who were diagnosed at The Pediatric Hospital, Fudan University from 1992 to 2002. This clinical trial was a non-randomized, controlled open study.

RESULTS(1) The age of onset ranged from 3 months to 16 years, and the most common ages of onset were before 3 years; 32 cases were males and 45 females. The extraocular muscles were more frequently involved. According to the modified Osserman's criteria, 54 patients (70%) were classified as type I, 21 cases (27%) as type II and 2 cases (3%) as type III. (2) Eighteen of 55 cases (35%) were positive for anti-acetylcholine receptor antibodies (AchRab) and 16 of 55 cases (31%) were positive for acetylcholine premembrane receptor antibody (PremRab) on the initial examination. The clinical state of the patient during the examination did not show any clear correlation with the level of these antibodies. There was no significant difference between clinical type and AchRab positive rate among the three groups. Two of 18 patients (11%) were positive for thymoma associated antibody (Tintinab). The serological test on follow-up showed that 6 of 10 SNMG cases (60%) turned to be SPMG. In 85% of the cases the results of CD cells examination was abnormal, most of them showed reduced levels of CD4(+) or CD3(+) and CD8(+). (3) The thymus proliferation was found in 22 patients (42%) by CT and changes of thymoma were found in 2 cases (4%) and were confirmed by operation. (4) In 50% of the cases the electromyography (EMG) was abnormal. (5) After anticholinesterase drugs and steroids treatment the prognosis of patients with MG was usually good.

CONCLUSIONSMG in our children's hospital has increased, the age of onset became younger, and type II MG cases increased. Seronegative patients could turn positive, so monitoring the patient's serology is helpful for finding more SPMG cases. Steroids have been proven effective and safe in treatment of MG in children. Patients in methylprednisolone group experienced less side effects of steroid therapy than group treated with oral prednisone.

Adolescent ; Antibodies ; blood ; Child ; Child, Preschool ; Cholinesterase Inhibitors ; therapeutic use ; Female ; Follow-Up Studies ; Hospitals, University ; Humans ; Infant ; Male ; Myasthenia Gravis ; blood ; classification ; drug therapy ; Prognosis ; Receptors, Cholinergic ; immunology ; Steroids ; therapeutic use ; Treatment Outcome

OBJECTIVETo study the clinical and genetic characteristics of generalized epilepsy with febrile seizures plus (GEFS).

METHODSData of two probands of the disease were collected through outpatient clinic. DNA was extracted from peripheral blood leukocytes using RelaxGene Blood DNA System. Twenty-six exons of SCN1A were amplified by polymerase chain reaction (PCR), the PCR products were screened by denaturing high performance liquid chromatography (DHPLC), then the abnormal fragments were sequenced by Sanger method in order to find the mutations of SCNIA gene.

RESULTS(1) There were 28 affected individuals in the two families of GEFS+ (14 males and 14 females). Febrile seizures (FS) were present in 7 cases, febrile seizures plus (FS+) in 6 cases, FS+ and absence seizures in 1 case, FS+ and myoclonic seizures in 1 case, uncertain type in 13 cases. No severe phenotype was seen. Bilineal inheritance occured in one GEFS+ family. (2) A samesense mutation (c. 1212A > G) of SCN1A gene was found in the proband and an unaffected individual of pedigree B of GEFS.

CONCLUSIONS(1) GEFS+ is a syndrome with the characteristics of heterogeneous clinical phenotypes; bilineal inheritance suggests the possibility of complex inheritance with additive gene effects. (2) Our study failed to provide evidence supporting a causal relation between the SCN1A mutation and the etiologic gene in the GEFS+ family B, which indicates that GEFS+ has the phenotypic and genotypic heterogeneity.

Adolescent ; Child ; Child, Preschool ; DNA Mutational Analysis ; Epilepsy, Generalized ; complications ; genetics ; Female ; Genetic Testing ; Genotype ; Humans ; Infant ; Male ; NAV1.1 Voltage-Gated Sodium Channel ; Nerve Tissue Proteins ; genetics ; Pedigree ; Phenotype ; Seizures, Febrile ; complications ; genetics ; Sodium Channels ; genetics

OBJECTIVETo explore the prevalence and characteristics of chronic headache in children and adolescents in Shanghai and to collect messages concerning the impact and compliance of medication for migraine.

METHODSA population-based questionnaire study was conducted among subjects 6 - 15 years of age sampled from primary and junior high schools in Shanghai and the subjects were followed up.

RESULTS(1) The prevalence: 8701 (88.6%) out of 9857 pupils responded to the questionnaire; 17% of the respondents had headache and in 86.4% of them the reason of headache was unknown. The prevalence of chronic headache in Shanghai children and adolescents was 7.8%, there was no significant difference between both genders (chi(2) = 0.010, P > 0.05). (2) The prevalence of chronic headache increased with age, the incidence was higher in boys before 12 years of age, while higher in girls after 12 years of age. (3) Characteristics of chronic headache: the proportion of unilateral, bilateral and headache of unknown site was similar; in most of the cases headache was localized in the temple (35.2%) and forehead region (25.6%), the duration of headache was short, always accompanied by gastrointestinal symptoms. Half of the patients reported that the headache had affected their study and daily life. (4) The status of using health care facilities: 24% of the students sought medical assistance during their headache episodes and among them only 30.9% took medicine. (5) Over-fatigue (51.4%), followed by insufficient sleep (40.4%), emotional changes (38.5%) were the main aggravating factors. The headache was also associated with positive family history and stress in studying.

CONCLUSIONSHeadache is a common complaints of children, affecting the patients' study and daily life. But many patients with headache were not treated properly, therefore, the medical and educational sectors and the society should pay more attention to this problem.

Adolescent ; Child ; China ; epidemiology ; Female ; Headache Disorders ; epidemiology ; Humans ; Male ; Prevalence ; Surveys and Questionnaires

Objective To explore the clinical features and video-electroencephalogram (VEEG) monitoring of nonconvulsive status epilepticus (NCSE) in children.Methods 1.Object of study:Seventeen patients of NCSE diagnosed with Kaplan's criteria were analyzed in Children's Hospital of Fudan University between Oct.2009 and Sep.2012.2.Data analysis:Data on demographics,etiology,clinical manifestation and response to clonazepam therapy were analyzed.3.Therapies:Clonazepam 0.05 mg/kg was intravenously injected twice a day.Treatment of poor efficacy patients was combined with other antiepileptic drugs.4.Therapeutic effect:Clinical assessment of cognitive improvement and VEEG monitoring of background activity or paroxysmal abnormalities were analyzed.Results Nine male and 8 female of 17 patients with NCSE were involved,from 11 months to ll.4-year old.The clinical attacks lasted ranging variously time from 4 hours to 3 months.Each patient had a prolonged change of consciousness,accompanied by psychological or behavioral changes.Definite medical causes were identified in 65％ (11/17 cases) of the patients.Secondary epilepsy was the dominating cause.The characteristics of ictal VEEG in NCSE generally included slow activity and focal or generalized δ or θ activity.After clonazepam treatment,the conditions of 13 patients were under complete control,in which 4 had improvement.Six cases of unknown cause were fully controlled within 72 hours after intravenous injection of clonazepam.The prognosis of CNS infection sequelae patients,metabolism disorders and brain structural damage was poor.Conclusions NCSE may present with confusion,behavioral disturbances and psychiatric conditions.The diagnosis can be made by the ictal and interictal VEEG monitoring.It is necessary to make the diagnosis and control the seizures as quickly as possible.Clonazepam is useful in NCSE.