Not only a matter of epilepsy: early problems of cognition and behavior in children with "epilepsy only"--a prospective, longitudinal, controlled study starting at diagnosis.

OBJECTIVE: To understand early educational and behavioral predicament in childhood "epilepsy only." METHODS: A multicenter, prospective, longitudinal study was conducted of 51 outpatient schoolchildren with newly diagnosed idiopathic or cryptogenic epilepsy and 48 sex-matched classmate control subjects. All children underwent neuropsychological assessment 3 times within the first year after diagnosis; parents and teachers completed behavior questionnaires, and patients' parents were interviewed to inventory contextual adversity. Principal components analysis of cognition and behavior disclosed 6 major components that were related with the interview data (repeated measures analysis of variance). RESULTS: Despite similar intelligence and educational background, significantly more patients (51%) than control subjects (27%) required special educational assistance. Patients obtained worse scores across components of cognition and behavior. Parents and teachers perceived patients to have more behavioral problems. Differences between groups existed at pretreatment baseline. Over time, notwithstanding stable percentages of poor scores in both groups, nonpersistence of poor scores was impressive (each time other children scored poorly in other domains). Rather than epilepsy characteristics, contextual adversities were significant risk factors. CONCLUSION: Already in the earliest stage of the illness, children with epilepsy are liable to vicissitudes in cognitive and behavioral functioning. Contextual variables are all-important.

Case report: severe central nervous system involvement in juvenile dermatomyositis.

We present 3 patients with juvenile dermatomyositis (JDM) and severe central nervous system (CNS) complications. All patients had at least 4 positive criteria of Bohan and Peter, which confirmed a definite diagnosis of JDM. They were all male, and had a relatively high creatinine kinase value at admission (1532-4260 U/l). Besides, progressive proximal muscle weakness and rash, one patient presented with rapid irreversible decline of vision. Ophthalmologic examination showed active vasculitis of the retina. After 2 weeks of treatment with immunosuppressive drugs and being in improved, relatively stable clinical condition, all 3 patients developed generalized tonic-clonic convulsions. Other causes of the neurological symptoms could be excluded. In all 3 patients, the course of JDM was fatal. The clinical symptoms and further investigations in our patients show CNS involvement in JDM. Although rarely reported, CNS vasculopathy can be a serious and life-threatening complication of JDM.

Prognosis of haemorrhagic stroke in childhood: a long-term follow-up study.

Little is known about long-term physical sequelae, cognitive functioning, and quality of life of children who have had a haemorrhagic stroke. Fifty-six patients (29 females, 27 males) under 16 years of age at time of the bleeding were studied. Mean age at time of bleeding was 7.7 years (range 1 month to 15.9 years). The primary site and cause of the bleeding at baseline were determined. Occurrences of death, re-bleedings, and seizures during follow-up were recorded. Patients who survived were invited for a follow-up examination including physical check-up, general screening of cognition, and an inventory of subjective health perception. Thirteen children died directly as a result of the haemorrhage; nine experienced a recurrent bleeding, which was fatal in three; six children developed epileptic seizures. At follow-up 36 of 56 patients were still alive. Mean follow-up time was 10.3 years (range 1.3 to 19.9 years) and mean age was 18.6 years (range 1.8 to 34.1 years). There was no patient lost to follow-up. Five patients declined to visit the hospital. In 15 out of 31 patients who could be examined, no physical impairment was observed, 11 had a hemiparesis of varying severity, and three had symptoms of cerebellar ataxia. One child had persisting tetraparesis and one persisting paraparesis. Signs of cognitive deficits were found in 15 patients. Of the children who survive haemorrhagic stroke, the physical and functional prognosis is relatively good, as almost all children were independent at follow-up. However, only a quarter of the surviving children had no physical or cognitive deficit after a mean follow-up period of 10 years. The majority had low self-esteem as well as emotional, behavioural, and health problems.

Long-term prognosis of cerebral venous sinus thrombosis in childhood.

Cerebral venous sinus thrombosis (CVST) is a rare but potentially serious disorder in children. There is no literature on the long-term neuropsychological and emotional sequelae and implications for quality of life. We studied 17 children who had CVST after the neonatal period, aged between 1 month and 16 years at the time of CVST (mean age at CVST was 6 years, median 4 years 8 months). Five children died during follow-up. The cause of death was related to CVST in one child. Twelve children participated in a clinical follow-up assessment. Mean follow-up was 2 years 8 months. One child had physical sequelae with impairment of skilled movement. All children had average or high intelligence scores. Two children with CVST due to an uncomplicated mastoiditis had mild cognitive deficits: one child had difficulty with written language; the other had diminished cognitive efficiency with concentration and attention problems associated with decreased psychosocial functioning. Decreased physical well-being was reported in three of 12 children. We conclude that children who had survived CVST had a fair prognosis. Most had normal cognitive and physical development, although mild cognitive deficits or decreased physical and psychosocial well-being can occur.