Prognostic value of continuous electroencephalography monitoring in children with severe brain damage.

PURPOSE: The purpose of this study is to determine whether there is a relationship between continuous electroencephalography (EEG) monitoring patterns and prognosis for children with severe brain damage. Patients and METHODS: The different patterns of EEG were analyzed for 103 children (Glasgow Coma Scale [GCS] score < 8) who were monitored with continuous video-EEG (CVEEG) within 72 hours after the onset of coma. The clinical outcomes were scored and evaluated at hospital discharge by the modified Pediatric Cerebral and Overall Performance Category Scale (PCOPCS). EEG parameters of the different prognosis groups were compared and risk factors for prognosis were identified. RESULTS: Of the 103 children, 36 were in the good prognosis group (PCOPCS scores 1 and 2) and 67 were in the poor prognosis group (PCOPCS scores 3-6). The poor prognosis group had the lower proportion of events in reactive EEG patterns and sleep architecture, and a higher proportion of low-voltage events. Multivariate analyses showed that the lower GCS score and no sleep architecture were significantly associated with poor prognosis. CONCLUSIONS: Comatose children with higher GCS score and sleep architecture have better clinical outcomes in terms of morbidity and mortality.

[Clinical and EEG features in children with febrile seizures after antiepileptic drug therapy].

OBJECTIVE: To investigate the changes of clinical and EEG features in children with febrile seizures which are prone to epilepsy four years after antiepileptic drugs valproate and/or topiramate treatment. METHODS: One hundred and thirty-two children with febrile seizures between 2004 and 2005 and who had the indications of antiepileptic drugs treatment were administered with oral valproate and/or topiramate treatment. The children were followed up for four years. Routine blood tests, liver and renal function tests were performed twice a year. Sleeping activation EEG examination was performed once a year. RESULTS: During the follow-up of 1 to 10 years, 108 (98.2%) out of 110 children with valproate monotherapy were seizure-free. In the 110 cases, 95 were in the drug withdrawl and 10 were in the drug reduction. All of 13 cases receiving topiramate monotherapy were seizure-free and were in the drug withdrawl. None of the patients showed abnormalities in routine blood tests, liver and renal functions tests. Sleeping activation EEG showed normal in 102 cases, focal discharges in 8 cases, bilateral synchronized spikes in 4 cases and 3Hz spikes and polyspikes in 2 cases. CONCLUSIONS: Early use of antiepileptic drugs valproate or topiramate is effective and safe in children with febrile seizures which are prone to epilepsy. The majority of the children have a normal sleeping activation EEG after antiepileptic drug therapy.

[Clinical analysis and screening for SCN1A gene mutation in two pedigrees of generalized epilepsies with febrile seizures plus].

OBJECTIVE: To study the clinical and genetic characteristics of generalized epilepsy with febrile seizures plus (GEFS). METHODS: Data 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.

[Myasthenia gravis in children: clinical study of 77 patients].

OBJECTIVE: To 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. METHODS: The 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. CONCLUSIONS: MG 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.