Long-term cognitive outcomes after cerebral sinovenous thrombosis in childhood.

AIM: To assess long-term cognitive function in children after cerebral sinovenous thrombosis (CSVT). METHOD: Children with CSVT, who had neuropsychological testing for intellectual ability, executive function, attention, language, or behavior, were included in a prospective observational study. Outcomes were compared with normative means using one-sample t-tests. Predictors of abnormal function were examined using logistic regression. RESULTS: Fifty children with CSVT were included (median age at diagnosis 2y 10mo, interquartile range 7d-6y 10mo; 35 males, 15 females). The median follow-up time was 4 years 2 months (interquartile range 2y 8mo-6y 4mo). Compared with normative means, children with CSVT had lower mean (± standard deviation) full-scale IQ, working memory, and processing speed scores (93.3±16, p=0.01; 93.6±16, p=0.04; 93.7±15.3, p=0.02 respectively). They also had lower scores in executive function, attention, and language domains. Refractory seizure at presentation was associated with a trend in behavioral problems (odds ratio [OR] 6.3, 95% confidence interval [CI] 0.9-46, p=0.07). Females were less likely to experience processing speed difficulties (OR 0.22, 95% CI 0.04-1.3, p=0.09). Incomplete recanalization was associated with a greater risk of abnormal verbal comprehension (OR 5.3, 95% CI 0.93-30.5, p=0.059). INTERPRETATION: Children with CSVT as a group performed below age expectations on standardized neuropsychological tests, although there was variability across individuals and cognitive domains. Larger studies are needed to evaluate predictors of cognitive deficits in children with CSVT.

Successful pregnancy, epidural anaesthesia, labour, and delivery in a woman with Sturge-Weber syndrome and previous hemispherectomy.

BACKGROUND: The outcomes of pregnancy and subsequent delivery of healthy neonates in women who have undergone previous near total hemispherectomy for Sturge-Weber syndrome (SWS) have rarely been reported. CASE: A woman with SWS had two successful and uncomplicated pregnancies and deliveries under epidural anaesthesia after undergoing a near total hemispherectomy with subsequent unilateral cerebral palsy. Non-contrast MRI of the brain revealed post hemispherectomy changes with no residual meningeal angiomatosis. CONCLUSION: As more women who undergo surgical resection for underlying SWS grow into the child-bearing years, additional reports of pregnancy and delivery outcomes in this patient population can be expected and will be valuable.

Contexte : Les issues de grossesse et l'accouchement subséquent d'enfants en santé chez des femmes ayant au préalable subi une hémisphérectomie presque totale en raison du syndrome de Sturge-Weber (SSW) n'ont que rarement fait l'objet de signalements. Cas : Une femme présentant le SSW a connu deux grossesses et accouchements réussis et sans complications sous anesthésie péridurale, après avoir subi une hémisphérectomie presque totale ayant subséquemment donné lieu à une infirmité motrice cérébrale unilatérale. Une IRM du cerveau menée sans produits de contraste a révélé la présence de modifications post-hémisphérectomie, sans angiomatose méningée résiduelle. Conclusion : Au fur et à mesure que nous verrons un nombre de plus en plus important de femmes, ayant subi une résection chirurgicale en raison d'un SSW sous-jacent, atteindre l'âge de procréation, nous pouvons nous attendre à d'autres signalements d'issues de grossesse et d'accouchement portant sur cette population de patientes; ces signalements prendront alors toute leur valeur.

Persons With Spinal Cord Injury Report Peripherally Dominant Serotonin-Like Syndrome After Use of Serotonergic Psychedelics.

Psychedelic-assisted therapy (PAT) may treat various mental health conditions. Despite its promising therapeutic signal across mental health outcomes, less attention is paid on its potential to provide therapeutic benefits across complex medical situations within rehabilitation medicine. Persons with spinal cord injury (SCI) have a high prevalence of treatment-resistant mental health comorbidities that compound the extent of their physical disability. Reports from online discussion forums suggest that those living with SCI are using psychedelics, though the motivation for their use is unknown. These anecdotal reports describe a consistent phenomenon of neuromuscular and autonomic hypersensitivity to classical serotonergic psychedelics, such as psilocybin and lysergic acid diethylamide (LSD). Persons describe intense muscle spasms, sweating, and tremors, with an eventual return to baseline and no reports of worsening of their baseline neurological deficits. The discomfort experienced interferes with the subjective beneficial effects self-reported. This phenomenon has not been described previously in the academic literature. We aim to provide a descriptive review and explanatory theoretical framework hypothesizing this phenomenon as a peripherally dominant serotonin syndrome-like clinical picture-that should be considered as such when persons with SCI are exposed to classical psychedelics. Raising awareness of this syndrome may help our mechanistic understanding of serotonergic psychedelics and stimulate development of treatment protocols permitting persons with SCI to safely tolerate their adverse effects. As PAT transitions from research trials into accepted clinical and decriminalized use, efforts must be made from a harm reduction perspective to understand these adverse events, while also serving as an informed consent process aid if such therapeutic approaches are to be considered for use in persons living with SCI.

The spectrum of epilepsy in children with 15q13.3 microdeletion syndrome.

PURPOSE: To further define the epilepsy phenotype in a cohort of children with 15q13.3 microdeletion syndrome. METHODS: We retrospectively reviewed the phenotypic spectrum of all children aged < 18 years with epilepsy and 15q13.3 microdeletion syndrome. RESULTS: Thirteen children were included, 69% were female. The median age of children in the cohort was 12 years (age range: 3 years-15 years). Median age at seizure onset was 4 years. Eleven children (85%) had intellectual disability. Nine of 13 children (69%) had a history of typical absence seizures with median age of onset at 5 years (2 had absence status epilepticus). Thirty-one percent (4/13) had focal with impaired awareness non-motor onset seizures. ILAE recognized absence epilepsy syndromes were diagnosed in 6/13 (46%). The remainder were classified as having genetic generalized epilepsies with overlap clinical features, combined or focal epilepsies. Electroencephalogram in the cohort showed generalized (85%) and focal epileptiform discharges (62%) and posterior dominant rhythm slowing (33%). One child had electrical status epilepticus of sleep. Neuroimaging was performed in 5 children (38%) and revealed abnormal findings in 3. Seizures were drug resistant in a third of the cohort. Valproate resulted in seizure freedom in 5 (42%). Oxcarbazepine caused clinical worsening in one child with combined seizure types. Two children tried cannabidiol and one tried the ketogenic diet; neither was effective. CONCLUSIONS: The epilepsy phenotype in children with 15q13.3 microdeletion syndrome is defined by childhood onset absence seizures, and may have atypical features such as, early onset absences, persistence into adolescence, status epilepticus, intellectual disability and treatment resistance. Focal seizures and focal EEG findings may be observed and should be treated cautiously, given the possibility of combined seizure types. Valproate appeared effective, although other treatments must be explored further.

Measuring tissue oxygen saturation via spectrometer in children.

BACKGROUND: : Near-infrared spectroscopy is a new noninvasive method of monitoring oxygen saturation at a tissue level. The purpose of this study was to evaluate new near-infrared tissue spectrometer InSpectra (Hutchinson Technology Inc.) in children and to determine preferable areas of the body to measure tissue oxygen saturation (StO2). METHODS: : Prospective study at a pediatric emergency department. Children aged 0 years to 17 years with no respiratory distress participated in this study. StO2 on deltoid muscle, thenar eminence, forearm, calf, bicep, and tricep was measured at triage with a 25-mm probe. RESULTS: : A total of 310 patients were recruited. The mean age of participants was 6.8 years +/- 4.4 years and 53% were males. Average StO2 was 84% (95% confidence interval, 81-87%) on the bicep muscle, 83.5% (95% confidence interval, 82-85%) on the deltoid muscle and significantly (p < 0.05) lower on other areas. Variation of StO2 was lower on the bicep, deltoid, and thenar muscles. Regression analysis showed significant linear relationship between patients' age and StO2 measured on the thenar eminence (beta = 0.3, R = 0.08, p < 0.001) and between patients' weight and StO2 on the thenar eminence (beta = 0.3, R = 0.07, p < 0.001). StO2 in febrile patients was similar to afebrile children, except thenar eminence where StO2 was significantly lower (p = 0.002). Less than 5% reported any type of pain or cried during StO2 measurement, which did not differ from pulse oximetry. CONCLUSION: : Bicep and deltoid muscles are the most appropriate areas to measure StO2 using the 25-mm transducer in children of different ages. The use of near-infrared spectroscopy on the thenar eminence, which is usually used for measurement in adults, has varied results in children depending on the age, weight, and presence of fever.