Levetiracetam in juvenile myoclonic epilepsy: long-term efficacy in newly diagnosed adolescents

The aim of this study was to evaluate the efficacy and tolerability of levetiracetam (LEV) monotherapy in juvenile myoclonic epilepsy (JME). The study group consisted of 32 patients with epilepsy (20 males, 12 females) with a mean age of 13 years 3 months (SD 7y 11mo) at seizure onset. LEV was administered as the first drug; all patients were followed up at 6 and 12 months. The dose that achieved seizure control ranged from 1000 to 2500mg/daily. At 6-month evaluation: 15 patients were seizure free; 14 patients were responders (>50% reduction in seizures); and three patients had marginal effects (<50% reduction of seizures). At 12-month evaluation: 29 patients were seizure free; three patients were responders. No patients reported adverse events. These data provide preliminary evidence that LEV may be effective for treating patients with newly diagnosed JME.     

Disentangling the effects of Tourette syndrome and attention deficit hyperactivity disorder on cognitive and behavioral phenotypes

Eighty participants (62 males; 18 females; age range: 6-16 years) took part in the study, comprising four groups of 20 subjects each: TS-only, ADHD-only, TS+ADHD, controls. The age distributions, did not differ significantly among the four groups. The severity of symptoms, assessed by the TSGS, did not differ significantly between the two TS groups. Standardised measures were used throughout. The "cases" (i.e. TS-only, TS+ADHD, ADHD-only) were significantly different from controls on most measures of behavior. There were also differences amongst the various clinical subgroups, with, in general, TS-only participants being similar to controls with regards to both "total behavior" ratings and cognitive testing results. A diagnosis of ADHD, either or its own or in association with TS, was associated with greater maladaptive behavior and worse cognitive functioning. With regards to affective symptoms and anxiety, the three clinical groups did not differ from each other, but each of them was more affected than the control group. One finding in our study which differed from previous literature was that TS-only patients were rated as more "delinquent" than controls by their parents: possible reasons for this are discussed. Oppositional defiant disorder (ODD) was seen in a few (2,3,3 ODD patients in each clinical group), but as numbers were small no statistics were undertaken. Family histories were in accord with both TS and ADHD being genetic disorders, but sharing an overlap in only some cases. The "additive effect" hypothesis is discussed in detail in the light of our results and recent literature.     

Preterm birth and neurodevelopmental outcome: a review

Background  

The incidence of preterm delivery and the survival rate of preterm newborns are rising, due to the increased use of assisted reproductive technology associated with multiple gestations and improved technology in obstetrics and neonatology, which allow saving preterm infants at earlier gestational ages. As a consequence, the risk of developmental disabilities in preterm children is high, and clinical pictures need to be fully defined.     

Conceptual and methodological challenges for neuroimaging studies of autistic spectrum disorders

Autistic Spectrum Disorders (ASDs) are a set of complex developmental disabilities defined by impairment in social interaction and communication, as well as by restricted interests or repetitive behaviors. Neuroimaging studies have substantially advanced our understanding of the neural mechanisms that underlie the core symptoms of ASDs. Nevertheless, a number of challenges still remain in the application of neuroimaging techniques to the study of ASDs. We review three major conceptual and methodological challenges that complicate the interpretation of findings from neuroimaging studies in ASDs, and that future imaging studies should address through improved designs. These include: (1) identification and implementation of tasks that more specifically target the neural processes of interest, while avoiding the confusion that the symptoms of ASD may impose on both the performance of the task and the detection of brain activations; (2) the inconsistency that disease heterogeneity in persons with ASD can generate on research findings, particularly heterogeneity of symptoms, symptom severity, differences in IQ, total brain volume, and psychiatric comorbidity; and (3) the problems with interpretation of findings from cross-sectional studies of persons with ASD across differing age groups. Failure to address these challenges will continue to hinder our ability to distinguish findings that outline the causes of ASDs from brain processes that represent downstream or compensatory responses to the presence of the disease. Here we propose strategies to address these issues: 1) the use of simple and elementary tasks, that are easier to understand for autistic subjects; 2) the scanning of a more homogenous group of persons with ASDs, preferably at younger age; 3) the performance of longitudinal studies, that may provide more straight forward and reliable results. We believe that this would allow for a better understanding of both the central pathogenic processes and the compensatory responses in the brain of persons suffering from ASDs.     

Lumbar epidural fentanyl: segmental spread and effect on temporal summation and muscle pain

Background. Despite extensive use, different aspects of thepharmacological action of epidural fentanyl have not been clarified.We applied a multi-modal sensory test procedure to investigatethe effect of epidural fentanyl on segmental spread, temporalsummation (as a measure for short-lasting central hyperexcitability)and muscle pain. Methods. Thirty patients received either placebo, 50 or 100µg single dose of fentanyl epidurally (L2–3), ina randomized, double-blind fashion. Heat pain tolerance thresholdsat eight dermatomes from S1 to fifth cranial nerve (assessmentof segmental spread), pain threshold to transcutaneous repeatedelectrical stimulation of the sural nerve (assessment of temporalsummation) and pain intensity after injection of hypertonicsaline into the tibialis anterior muscle (assessment of musclepain) were recorded. Results. Fentanyl 100 µg, but not 50 µg, producedanalgesia to heat stimulation only at L2. Surprisingly, no effectat S1 was detected. Both fentanyl doses significantly increasedtemporal summation threshold and decreased muscle pain intensity. Conclusions. The findings suggest that a single lumbar epiduraldose of fentanyl should be injected at the spinal interspacecorresponding to the dermatomal site of pain. Increased effecton L2 compared with S1 suggests that drug effect on spinal nerveroots and binding to opioid receptors on the dorsal root gangliamay be more important than traditionally believed for the segmentaleffect of epidurally injected fentanyl. Epidural fentanyl increasestemporal summation threshold and could therefore contributeto prevention and treatment of central hypersensitivity states.I.M. injection of hypertonic saline is a sensitive techniquefor detecting the analgesic action of epidural opioids. Br J Anaesth 2003; 90: 467–73