Risk-Taking Behaviors in Children with ADHD Compared to Children with Primary Headaches.

The study examined the engagement in risk-taking behaviors and their onset in children with attention-deficit/hyperactivity disorder (ADHD) compared with children with primary headaches. Whether ADHD is associated with higher engagement in risk-taking behavior compared with other neurodevelopmental disorders (and not only typical development) has yet to be demonstrated. A sample of 189 children, 10 to 18 years old, undergoing neurological surveillance for ADHD (N = 144) or primary headaches (N = 45) participated in the study. The children and their parents reported the children's engagement in various risk-taking behaviors. The ADHD group reported a higher level of general risk-taking behavior relative to the headache group. The differences remained significant even after controlling for demographic and clinical variables. No differences in the age of onset of risk-taking behaviors were found. It is concluded that risk-taking behavior is more common in children with ADHD under active neurological surveillance than in children followed for primary headaches.

Intrathecal Baclofen Therapy-Practical Approach: Clinical Benefits and Complication Management.

Intrathecal baclofen is an expanding accepted treatment for children with cerebral palsy and other causes of spasticity and dystonia. The aims of this review are therefore to (1) delineate the clinical benefits of intrathecal baclofen therapy in pediatric spasticity and dystonia and (2) increase awareness of the potential complications and emergency management measures of intrathecal baclofen therapy. A current literature review demonstrates the benefits and complications of this minimally invasive device. Practical guides for recognizing acute conditions and management recommendations are included. Intrathecal baclofen is increasingly being used to help individuals attain realistic functional goals. Therefore, families and health care professionals should be aware of potential complications, symptoms, and emergency management.

Clown-care reduces pain in children with cerebral palsy undergoing recurrent botulinum toxin injections- A quasi-randomized controlled crossover study.

OBJECTIVE: We investigated the impact of clown-care on pain in 45 children with cerebral palsy who underwent recurrent Botulinum-toxin injections (age 7.04± 4.68 years). Participants were randomized to receive either clown (n = 20) or standard (n = 25) -care. METHODS: Pain Visual-Analogue-Scale (range 1-5) was reported before and after procedures. Pain assessment was lower for children undergoing Botulinum-toxin injections with clown-care (2.89± 1.36) compared to standard-care (3.85± 1.39; p = 0.036) even though pain anticipated prior to procedures was similar (~3). FINDINGS: Children who underwent the first procedure with clown-care reported lower pain even after they crossed-over to the following procedure which was standard (p = 0.048). Carryover effect was more prominent in injection-naïve children (p = 0.019) and during multiple procedures (p = 0.009). Prior pain experience correlated with pain in subsequent procedures only when first experience was standard-care (p = 0.001). CONCLUSIONS: Clown-care alleviated pain sensation during Botulinum-toxin injections and initial clown-care experience reduced pain during subsequent injections even though clowns were not present. TRIAL REGISTRATION: clinicaltrials.gov ID # NCT01377883.

Mutations in the histone methyltransferase gene KMT2B cause complex early-onset dystonia.

Histone lysine methylation, mediated by mixed-lineage leukemia (MLL) proteins, is now known to be critical in the regulation of gene expression, genomic stability, cell cycle and nuclear architecture. Despite MLL proteins being postulated as essential for normal development, little is known about the specific functions of the different MLL lysine methyltransferases. Here we report heterozygous variants in the gene KMT2B (also known as MLL4) in 27 unrelated individuals with a complex progressive childhood-onset dystonia, often associated with a typical facial appearance and characteristic brain magnetic resonance imaging findings. Over time, the majority of affected individuals developed prominent cervical, cranial and laryngeal dystonia. Marked clinical benefit, including the restoration of independent ambulation in some cases, was observed following deep brain stimulation (DBS). These findings highlight a clinically recognizable and potentially treatable form of genetic dystonia, demonstrating the crucial role of KMT2B in the physiological control of voluntary movement.

Neurologic sequela in a patient with galactosemia potentially mediated by interleukin-11 dysfunction.

A 16-year-old galactosemic patient, homozygous for the 5.5-kb gene deletion, suffered severe neurologic regression following streptococcal infection. Since the gene deletion includes the promoter of interleukin-11a receptor involved in neuronal apoptosis, we questioned whether this patient had no interleukin-11a receptor activity-resulting in neuronal toxicity during septicemia. We hypothesized that interleukin-11 levels would be elevated because of a loss of feedback induced by the absent interleukin-11Ra receptor complex. To assess this, we compared interleukin-11 levels in the proband and 2 of his siblings with the same genetic deletion, to age-matched controls. No differences were found in interleukin-11 levels between groups. Our study was not carried out during acute infective states, when the disrupted immunoregulation triggered by sepsis is relevant, and is thus limited. In conclusion, although interleukin-11 was not chronically elevated in individuals with galactosemia and 5.5-kb gene deletion, data do not rule out potential interleukin-11 dysfunction during acute infection.