Febrile convulsions increase risk of Tourette syndrome

PurposeFebrile convulsion (FC) and Tourette syndrome (TS) are both common neurological disorders in infants and children. Both disorders share clinical similarities, such as paroxysmal symptoms with normal neurodevelopment and expected remission over time. This population-based study investigated the association between FC with TS during childhood neurodevelopment.MethodWe used the Taiwan National Health Insurance Research Database to conduct a retrospective cohort analysis on 1586 FC patients. A reference cohort of 6344 non-FC patients, matched for age, sex, urbanization level, parental occupation, and index year, was used for comparison. The risk of the occurrence of TS in FC patients was assessed using a Cox proportional hazard regression model.ResultsThe overall incidence of TS was higher in the FC cohort than in the non-FC cohort (28.5 vs 13.9 per 10,000 person-years; adjusted hazard ratio = 1.91, 95% confidence interval = 1.32–2.75). The associated risk factors for FC patients to develop TS were boys, children living in rural areas, and children whose parents held blue-collar positions. Moreover, the risk of TS in FC patients rose from 0.89 to 16.0 (trend test P < 0.0001) when the frequency of FC-related medical visits increased from 1 to 2 times to more than 4 times. The adjusted hazard ratio for TS in related to FC-related medical visits was 1.02 (95% CI = 1.02–1.03) per one frequency increment.ConclusionFC may increase the risk of subsequent TS occurrence in children. Children who had frequent medical visits for FC were particularly vulnerable.     

PRRT2 mutations in a cohort of Chinese families with paroxysmal kinesigenic dyskinesia and genotype–phenotype correlation reanalysis in literatures

Purpose of the study: Though rare, children are susceptible to paroxysmal dyskinesias such as paroxysmal kinesigenic dyskinesia, and infantile convulsions and choreoathetosis. Recent studies showed that the cause of paroxysmal kinesigenic dyskinesia or infantile convulsions and choreoathetosis could be proline-rich transmembrane protein 2 (PRRT2) gene mutations.

Material and methods: This study analysed PRRT2 gene mutations in 51 families with paroxysmal kinesigenic dyskinesia or infantile convulsions and choreoathetosis by direct sequencing. In particular, we characterize the genotype–phenotype correlation between age at onset and the types of PRRT2 mutations in all published cases.

Results: Direct sequencing showed that 12 out of the 51 families had three different pathogenic mutations (c.649dupC, c.776dupG, c.649C>T) in the PRRT2 gene. No significant difference of age at onset between the patients with and without PRRT2 mutations was found in this cohort of patients. A total of 97 different PRRT2 mutations have been reported in 87 studies till now. The PRRT2 mutation classes are wide, and most mutations are frameshift mutations but the most common mutation remains c.649dupC. Comparisons of the age at onset in paroxysmal kinesigenic dyskinesia or infantile convulsions patients with different types of mutations showed no significant difference.

Conclusions: This study expands the clinical and genetic spectrums of Chinese patients with paroxysmal kinesigenic dyskinesia and infantile convulsions and choreoathetosis. No clear genotype–phenotype correlation between the age at onset and the types of mutations has been determined.     

Pre- and Perinatal Factors in Febrile Convulsions

Forsgren, L., Sidenvall, R., Blomquist, H. K:son, Heijbel, J. and Nyström, L. (Departments of Neurology, Paediatrics and Epidemiology and Health Care Research, University Hospital, Umeå, Sweden). Acta Paediatr Scand 80: 218, 1991.
In a community based study, 110 children with febrile convulsions (FC) were identified prospectively. Pre- and perinatal risk factors were compared with 213 age and sex matched controls sampled from the community. During pregnancy, proteinuria and preeclampsia/eclampsia Occurred more often in mothers of cases. Premature birth and bilirubinemia ≥ 200 μmol/l were also more common in cases. There were no differences between cases and controls in Occurrence of chronic illnesses in mothers, parents age at birth, birth order, and factors occurring during delivery such as type of anesthesia, Occurrence of acute or elective cesarean section, use of vacuum extraction, mode of presentation, signs of fetal distress in amnion fluid, umbilical problems, abnormalities of fetal heart rate or duration of delivery. Perinatal asphyxia was uncommon and there was no difference between cases and referents. Occurrence of complications during the first neonatal week did not differ between groups.     

Hypoparathyroidism simulating brain tumor—an unusual presentation

A 13 yr old boy presented with generalised tonic-clonic convulsions and papilledema simulating an intracranial space occupying lesion. CT scan showed bilateral basal ganglia calcification. Biochemical investigations established the diagnosis of hypoparathyroidism. On treatment with oral calcium and vitamin D the patients became seizure free and papilledema regressed. The possibility of hypoparathyroidism should be considered when basal ganglia calcification is seen and CT scan.     

VIRAL INFECTIONS OF THE RESPIRATORY TRACT IN HOSPITALIZED CHILDREN A Study from Oslo during a 90 Months'Period

ABSTRACT. A diagnosis of 979 respiratory viral infections was made in hospitalized children. Respiratory syncytial virus greatly outnumbered the other viruses: it caused 58% of the total virus infections and occurred in winter epidemics. Influenza A and B virus occurred during late winter and spring, rhinovirus had a seasonal distribution towards spring and autumn, whereas adenovirus types 1, 2 and 5 had no distinct seasonal distribution. Whereas respiratory syncytial virus were mainly associated with bronchiolitis and adenovirus type 7 with pneumonia, rhinovirus infections were most often found in children with episodes of acute bronchial asthma. The influenza A and B and adenovirus types 1, 2 and 5 infections often occurred with extrarespiratory symptoms, especially febrile convulsions.     

Convulsions following axillary brachial plexus blockade with levobupivacaine

Neurotoxicity manifesting as convulsions is a recognised complication of the administration of local anaesthetic drugs as part of a regional anaesthetic technique. We describe a case of self-limiting convulsions following the institution of an axillary brachial plexus block with levobupivacaine. Although the occurrence of convulsions following the administration of racemic bupivacaine is a well-recognised complication, there have been no clinical case reports published describing convulsions following the use of levobupivacaine in regional anaesthesia.     

Parental responses to first and recurrent febrile convulsions

OBJECTIVE: To compare parental knowledge, attitudes, concerns, and first-aid practices for children experiencing febrile convulsions (FC). SUBJECTS AND METHODS: A questionnaire was mailed to 326 FC parents from 11 emergency departments in southern Taiwan. RESULTS: A total of 109 first- and 107 recurrent-FC parents responded 1-3 months after the FC. Most incorrectly believed an electroencephalogram (EEG) or computed tomography (CT) was necessary; immunization should be postponed, and overestimated the risk of subsequent epilepsy. Parents were concerned about further attacks in the night, fever episodes, and frequently measured the child's body temperature. During the first episode, objects were inserted into the child's mouth and they were rushed to a hospital. One third lowered the child's body temperature, and 15% positioned the children on their side. For subsequent seizures, 80% anticipated rushing the child to a hospital, and 44% would put objects into the child's mouth. In comparison, although the recurrent-FC parents had higher scores in knowledge and attitudes than the first-FC parents, low knowledge scores (40% correct) were seen in both groups. No significant differences were found on parental concerns, performed/anticipated first aid for FC. CONCLUSION: Most FC parents had inadequate knowledge, high concerns, and improper first-aid practices. This suggests that parents with either first- or recurrent-FC children need information, emotional support, and first-aid demonstrations.     

Long-lasting antiepileptic effects of levetiracetam against epileptic seizures in the spontaneously epileptic rat (SER): differentiation of levetiracetam from conventional antiepileptic drugs

PURPOSE: Some evidence suggests that levetiracetam (LEV) possesses antiepileptogenic characteristics. The purpose of this study was to investigate the time course of seizure protection by LEV compared with that of phenytoin (PHT), phenobarbital (PB), valproate (VPA), and carbamazepine (CBZ) in the spontaneously epileptic rat (SER). The SER is a double mutant (tm/tm, zi/zi) showing both tonic convulsions and absence-like seizures. METHODS: The effect of single (40, 80, and 160 mg/kg, i.p.) and 5-day (80 mg/kg/day, i.p.) administration of LEV on tonic convulsions and absence-like seizures in SERs were studied. Tonic convulsions induced by blowing air onto the animal's head at 5-min intervals for 30 min and spontaneous absence-like seizures characterized by 5- to 7-Hz spike-wave-like complexes in the cortical and hippocampal EEG were recorded for 30 min. In the single-administration study, observations for seizure activity were performed once before and 3 times (45, 75, and 135 min) after drug administration. In the 5-day administration study, seizure observation was performed 4 times for 30 min (once before and 3 times after drug administration) during the 5-day drug-administration period, and continued once a day until 8 days after the final administration. The antiepileptic effects of 5-day administration of conventional AEDs (PHT, PB, VPA, and CBZ) were examined by using similar methods. RESULTS: Tonic convulsions and absence-like seizures were inhibited by a single administration of LEV at 80 and 160 mg/kg, i.p., but not significantly at 40 mg/kg, i.p. When LEV was repeatedly administered at 80 mg/kg/day, i.p., for 5 days to SERs, the inhibitory effects on seizures increased with administration time. The number of tonic convulsions and absence-like seizures were significantly reduced to 39.1% and 38.4% compared with previous values, respectively, after 5-day LEV administration. Furthermore, significant inhibition of tonic convulsions was detected convulsions more potently compared with LEV in SERs. The maximal antiseizure effects of these drugs were reached after the initial administration, with almost the same antiseizure effects observed through day 5, despite continued drug administration. Moreover, a long-lasting treatment effect was not observed with any of these drugs except for PHT and CBZ, both of which showed moderately prolonged antiseizure effects. CONCLUSIONS: These results show that LEV is effective in the treatment of both convulsive and absence-like seizures in SERs after single- and multiple-dose administration. Interestingly, in the 5-day administration study, it was found that the antiepileptic effects for tonic convulsions and absence-like seizures were observed both during the drug-administration period and 相似文献   

Genotypic association of exonic LGI4 polymorphisms and childhood absence epilepsy

The LGI4 gene is located in a region linked to benign familial infantile convulsions (BFIC) and idiopathic generalized epilepsy. Screening of the LGI4 coding region in BFIC and childhood absence epilepsy (CAE) revealed several frequent exonic polymorphisms. A genotypic association was found for the c.1914GCAT polymorphism in 42 CAE patients compared with 110 population controls (²=6.66, df=1, P=0.01), providing evidence for a so far undetected susceptibility allele for CAE in the LGI4 region.