How do parents perceive adverse drug events of their children's anticonvulsant medication?

BACKGROUND: The main source of knowledge on adverse drug events (ADE) are physicians' reports in controlled clinical trials. In contrast, little is known about the parents' perception of ADE of anticonvulsants their children receive. METHODS: After approval by the local ethics committee, we performed a survey in a neuropediatric outpatient clinic of a university hospital. Based on a structured questionnaire, we interviewed parents of children with current anticonvulsant treatment regarding (i) their fears about potential ADE, (ii) experienced ADE according to parents, and (iii) implications of ADE on the child's life. RESULTS: Parents of 150 patients took part in the interview. (i) 95 (63.3%) parents expressed fears concerning ADE, mostly liver injury/liver failure (33 [22%]). (ii) 129 (86%) parents reported experienced ADE, mostly sedation (65 [43.3%]) and abnormal behavior (54 [36%]). (iii) Parents reported substantial implications of ADE on the child's daily life for 84 (56%) children, and 63 (42%) parents expressed a negative impact on the child's development. CONCLUSION: We recognized a great discrepancy between those ADE that were feared and those that were experienced. Parents feared life-threatening ADE and experienced less severe ADE that nevertheless have a negative impact on the child's daily life.

Is there a predictive value of EEG and MRI after a first afebrile seizure in children?

BACKGROUND: After a first afebrile seizure, EEG in addition to cMRI is recommended for pediatric patients. Once indications requiring immediate treatment are excluded, it is of interest to determine if the results provide a prognostic tool for seizure relapses. METHODS: Patients aged between 1 month and 18 years who had a first afebrile seizure between 2006 and 2008 were retrospectively studied and monitored for another 48 months. RESULTS: Out of 248 patients, 62.5% had generalized and 36.3% focal seizures. 34.7% of the EEG results were pathological. 176 patients had a cMRI that showed in 23.3% probable epileptogenic lesions. 3 patients with benign cerebral tumours needed surgical therapy. In the following 48 months 29.4% of the children showed seizure relapses. There was a correlation between epileptic patterns in the EEG and further seizures (p=0.0001). However, the sensitivity of the EEG based diagnoses was 0.6, the specificity 0.78 and the positive predictive value 0.52. There was no correlation between epileptogenic lesions and the probability of seizure relapses. The sensitivity of the cMRI to this effect was 0.36, the specificity 0.74 and the positive predictive value 0.34. DISCUSSION: The EEG is superior to cMRI for predicting seizure relapses. The percentage of noticeable cMRI findings is high but this has low therapeutic relevance and is assumed to largely represent "incidental findings". It is important to question the value of MRI investigations for sedated small children except in the case of emergencies. The key question is whether the cMRI should be deployed to diagnose epilepsy, the probability of seizure recurrences or to classify the entity of a most likely epilepsy.

[Botulinum toxin injections for chronic migraine in adolescents - an early therapeutic option in the transition from neuropaediatrics to neurology]. / Botulinumtoxin-Injektionen bei chronischer Migräne im Jugendalter - eine frühzeitige Therapieoption in der Transition von der Neuropädiatrie zur Neurologie.

BACKGROUND: The prevalence of chronic headaches in children and adolescents is up to 2 % resulting in the beginning of the later typical headache careers of adults. The therapy for chronic migraine with botulinum toxin is now established in adults. However, there is only limited experience in the use of botulinum toxin in paediatric patients. METHODS: 10 patients aged 13 - 17 years who suffered from chronic migraine according to the IHS criteria were injected at 31 specific injection points of the head and neck muscles with a total amount of 150 IE of botulinum toxin A (Botox®) according to the approved scheme. The number of headache days per month over the following 9 months was recorded and side effects were retrospectively determined. RESULTS: The responder rate (that is reduction of headache days per month more than 50 %) was 7/10 at three months after the injection. On average the number of headache days per month was reduced from 19.2 days to a minimum of 10.1 days. After three to six months the number of headache days increased again in all responders. Slight local side effects such as redness or temporary pain were observed in all patients, but severe side effects such as infections, fever, ptosis or allergic reactions did not occur. DISCUSSION: This small case series shows that the therapy for chronic migraine with botulinum toxin A can also be effective and safe in adolescents. As many adolescents still suffer from headaches later as adults a link between neuropaediatricians and neurologists is justifiable. An early botulinum toxin therapy followed by the transition of the adolescents would be helpful.

[How safe are dimenhydrinate suppositories?]. / Wie sicher sind Dimenhydrinat-Suppositorien?

HISTORY: A 13-month-old girl suffered from 3 generalized tonic-clonic seizures for several minutes within a total period of 9 hours. History revealed that the child received a total of 5 dimenhydrinate containing suppositories à 40 mg during the previous 2 days (i. e. 23 mg dimenhydrinate per kg body weight) due to enteritis with vomiting. The first seizure occurred 10 hours after the last administration. INVESTIGATIONS: The plasma level of diphenhydramin was 230 µg/l approximately one hour after the first seizure. Electroencephalography showed no pathological signs, an MRI scan of the brain was normal except of several small gliotic spots and body temperature was regularly. TREATMENT AND COURSE: Two stationary occurring seizures were stopped with 5 mg diazepam rectally. Continued surveillance and an EEG two days later showed age-appropriate normal findings. There were no further seizures in the next 4 years. CONCLUSION: Infants have the risk to develop dimenhydrinate intoxication, especially in cases where suppositories were given repeatedly because of intermittent defecation.

Ambulatory care of children treated with anticonvulsants - pitfalls after discharge from hospital.

BACKGROUND: Anticonvulsants require special consideration particularly at the interface from hospital to ambulatory care. PATIENTS AND METHOD: Observational study for 6 months with prospectively enrolled consecutive patients in a neuropediatric ward of a university hospital (age 0-<18 years) with long-term therapy of at least one anticonvulsant. Assessment of outpatient prescriptions after discharge. Parent interviews for emergency treatment for acute seizures and safety precautions. RESULTS: We identified changes of the brand in 19/82 (23%) patients caused by hospital's discharge letters (4/82; 5%) or in ambulatory care (15/82; 18%). In 37/76 (49%) of patients who were deemed to require rescue medication, no recommendation for such a medication was included in the discharge letters. 17/76 (22%) of the respective parents stated that they had no immediate access to rescue medication. Safety precautions were applicable in 44 epilepsy patients. We identified knowledge deficits in 27/44 (61%) of parents. CONCLUSION: Switching of brands after discharge was frequent. In the discharge letters, rescue medications were insufficiently recommended. Additionally, parents frequently displayed knowledge deficits in risk management.

[Neurofibromatosis type 1 and associated clinical abnormalities in 27 children]. / Neurofibromatose Typ 1 und assoziierte Krankheiten bei 27 Kindern und Jugendlichen.

Neurofibromatosis type 1 is the most common of the phakomatoses and the clinical follow-up is an interdisciplinary challenge. The data of 27 patients with NF1 were systematically reviewed and compared to data from the literature. All of our patients had clinical signs of NF1. Besides the classic criteria café-au-lait spots (100%), freckling (48,1%), positive family history (44,1%), neurofibromas (40,7%), Lisch nodules (22,2%) and optic pathway tumors (22,2%) there were developmental delay (40,7%), macrocephaly (33,3%), strabism (29,6%), scoliosis (18,5%), epilepsy (14,8%), pubertal anomalies (14,8%), short stature (11,1%) and tics. Morphologically, CNS hamartomas (55,5%), astrocytomas (22,2%) and one pheochromocytoma became apparent. Special findings consist of one aneurysm of internal carotic arteria, juvenile xanthogranulomas, a case of pulmonary stenosis and an intracardial tumor. Four new mutations in the NF1 gene were found. Regular screening of optic glioma with MRI had no clinical significance. In contrast to other authors, one of our patients with optic glioma showed clinical progress after twelve years of age. The detection of astrocytomas led only to therapeutic consequences, when clinical signs or symptoms occurred. As with other authors, we found no potential for CNS hamartoma to proliferate. In three cases with pubertal anomalies we found CNS gliomas, which indicates the need for MRI. The expense of screening, apart from clinical surveillance, seems inadequate in relation to clinical relevance and costs. We describe four new mutations in the NF1 gene; there have been no specific genotype-phenotype correlations. Neurofibromatosis type 1 and associated clinical abnormalities in 27 children.