[Show abstract][Hide abstract] ABSTRACT: We report on seven patients with a novel neuroimaging finding that involves exclusively the cerebellar gray matter at the bottom of several fissures of both hemispheres but spares the vermis. The abnormal fissures were predominantly located in the lower and lateral parts of the cerebellar hemispheres. The affected cerebellar cortex was hypointense on T1-weighted and hyperintense on T2-weighted and fluid attenuation inversion recovery sequences. In some patients, the involved cerebellar gray matter was mildly thickened and the affected fissures slightly widened. In three of seven patients, the neuroimaging findings were unchanged on follow-up studies up to 6 years. The seven patients had various indications for the brain magnetic resonance imaging studies, and none of them had cerebellar dysfunction. Based on the similarity of the neuroimaging pattern with the cerebral "bottom-of-sulcus dysplasia," we coined the term "cerebellar bottom-of-fissure dysplasia" to refer to this novel neuroimaging finding. The neuroimaging characteristic as well as the unchanged findings on follow-up favors a stable "developmental" (malformative) nature. The lack of cerebellar dysfunction in the affected patients suggests that cerebellar bottom-of-fissure dysplasia represents most likely an incidental finding that does not require specific diagnostic investigation but allows a reassuring attitude.
The Cerebellum 11/2015; DOI:10.1007/s12311-015-0736-y · 2.72 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to elucidate the feasibility, efficacy, and sustainability of a home-based, two-week, forced-use therapy (FUT) program for children with hemiplegic cerebral palsy (CP).
A single-blinded, randomized controlled design was chosen. The Melbourne Assessment of Unilateral Upper Limb Function (MA) was carried out at baseline, pretest, post-test, and follow-up at two weeks, three months, and 12 months. Additionally, a questionnaire was used to evaluate the clinical relevance and integration of FUT in the home setting. 23 children, ages six to 16 years, took part in the study and were randomized into either an intervention group (n=12, mean age 9.8 ± 3.5 years) or a control group ($n=$ 11, mean age 11.7 ± 3.7 years). The intervention consisted of constraint of the unaffected hand for six hours per day and promotion of different activities of daily living according to an age-related manual for the use of the non-constraint hand.
Unpaired t-tests for the change in MA scores relative to the pre-test values showed no difference between the groups at any time point: post-test (p=0.304), two weeks (p=0.193), or three months (p=0.957). Improvements in Activities of Daily Living (ADLs) assessed by questionnaires were observed by 64% of parents of the intervention group. Fifty-five percent of parents stated that the FUT program was exhausting and only 45% indicated that they achieved constraint for 6 hours per day.
Our results evaluating a home-based FUT program of 14 days show no statistically significant improvement of upper extremity function in children with CP. The lack of compliance and absence of structured exercises proved to be considerable pitfalls of the home-based FUT program. Therefore, future home based FUT concepts should put special emphasis on the close monitoring and support of children and their families, as well as the integration of structured exercise sessions.
Journal of pediatric rehabilitation medicine 06/2012; 5(2):65-74. DOI:10.3233/PRM-2012-0198
[Show abstract][Hide abstract] ABSTRACT: Objective: To describe the characteristics and epidemiology of neonatal arterial ischemic stroke (AIS) in Switzerland. Methods: Data on clinical manifestation, neuroimaging, risk factors (RF’s), and treatment procedure were gathered prospectively for all neonates diagnosed with AIS and born in
Switzerland between 2000 and 2007. A re-evaluation of available neuroimaging studies was performed by two experienced investigators. Clinical follow-up assessments were performed. Predictors of poor outcome (including symptoms, infarct characteristics, RF’s and treatment) were determined. Results: Seventy-nine neonates (53 boys, 26 girls) have been reported. The incidence of neonatal AIS in Switzerland
was 14 per 100 000. Seizures were the most common symptoms (91%). RF’s including maternal conditions,
birth complications, neonatal comorbidities and prothrombotic states were found in 77%. Eighty percent had unilateral lesions (80% left-sided) and 20% had bilateral lesions. The anterior circulation (mainly the medial cerebral artery) was mostly involved (85%). At follow-up (mean 6mo) 41%
showed hemiplegia and 26% received anticonvulsant medication. Respiratory symptoms at presentation (OR 5.580, 95% CI 1.104–28.203, p=0.038), muscle tone abnormalities at presentation (OR 2.032, 95% CI 1.001–8.588, p=0.050) and the presence of neonatal comorbidities (OR 2.932, 95% CI 1.001–8.588, p=0.050) were significantpredictors of poor outcome in an univariate regression
Annual Meeting of the European Academy of Childhood Disability, Istanbul, Turkey; 05/2012
[Show abstract][Hide abstract] ABSTRACT: Children with epilepsy have a significant risk for attention-deficit/hyperactivity disorder (ADHD), which is often accompanied by deficits in working memory performance. However, it is not yet clear whether there are specific differences in the underlying mechanisms of working memory capability between children with epilepsy-related ADHD and those with developmental ADHD. There is evidence that methylphenidate can improve the behavioral difficulties in children with developmental ADHD. Whether this medication has the same effect on ADHD symptoms in patients with epilepsy is not yet well understood. The aim of the present study is, therefore, to evaluate whether boys with epilepsy-related ADHD and developmental ADHD share a common behavioral, pharmacoresponsive, and neurofunctional pathophysiology.
Seventeen boys with diagnosed combined epilepsy/ADHD, 15 boys with developmental ADHD, and 15 healthy controls (aged 8-14 years) performed on working memory tasks (N-back) while brain activation was recorded using functional magnetic resonance imaging. Each patient was tested twice: once after the intake of methylphenidate and once without in a counterbalanced order.
On a behavioral level, we show that boys with epilepsy-related ADHD as well as those with developmental ADHD performed similarly poorly on tasks with high cognitive load when compared to healthy controls, and that intake of methylphenidate improved performance almost to normal levels in both ADHD groups. On the functional level, both patient groups showed similar reductions of activation in all relevant parts of the functional network of working memory when compared to controls. Of interest, intake of methylphenidate did not significantly alter this activity pattern.
Our data show strong similarities between epilepsy-related and developmental ADHD on the behavioral, pharmacoresponsive, and neural level, favoring the view that ADHD with and without epilepsy shares a common underlying neurobehavioral pathophysiology.
[Show abstract][Hide abstract] ABSTRACT: AIMo describe the characteristics of paediatric cerebral sinus venous thrombosis (CSVT) in Switzerland.
data on clinical features, neuroimaging, risk factors, and treatment were collected for all children in Switzerland younger than 16 years of age who had CSVT between January 2000 and December 2008. A follow-up examination and a cognitive assessment were performed (mean follow-up period 26mo). Differences between neonates and children (patients older than 28d) were assessed and predictors of outcome were determined.
twenty-one neonates (14 males, seven females; mean age 9d, SD 8d) and 44 children (30 males, 14 females; mean age 8y 7mo, SD 4y 5mo) were reported. The incidence of paediatric CSVT in Switzerland was 0.558 per 100000 per year. In neonates, the deep venous system was more often involved and parenchymal injuries were more common. The strongest predictor of poor outcome was neonatal age (odds ratio 17.8, 95% confidence interval 0.847-372.353). Most children showed global cognitive abilities within the normal range, but impairments in single cognitive subdomains were frequent.
paediatric CSVT is rare. Its outcome is poor in neonates. Most children have good neurological outcomes, but some patients have individual neuropsychological impairments.