SUMMARYPurpose: Benign epilepsy with centrotemporal spikes (BECTS) is associated with mild cognitive deficits, especially language impairment. This study aimed to clarify whether children with BECTS with left-or righthemispheric, or bilateral focus have specific neuropsychological language deficits when compared to healthy controls, whether these deficits correlate functionally with language network organization (typical vs. atypical), and whether cofactors such as duration, handedness, and medication have a relevant impact on language reorganization processes. Methods: Twenty-seven patients and 19 healthy controls were examined with several neuropsychological tests (German version of the Wechsler Intelligence Scale for Children [WISC-IV], Regensburger verbal fluency test [RWT], Corsiblock forward and backward and HandDominanz-Test [HDT]) and with two language paradigms on functional magnetic resonance imaging (fMRI): silent reading of word-pairs and silent generation of simple sentences.Key Findings: Although neuropsychological test results only differed by trend between BECTS patients and controls, language laterality indices (LIs) in fMRI were significantly lower in patients than in controls. In particular, the anterior language network with Broca's area and the supplementary motor area (SMA) revealed the lowest LIs and showed the most bilateral or right hemispheric activations in the sentence generation task. Medication and duration of epilepsy did not have any significant effect on language reorganization and patients' performances. Significance: Language reorganization in BECTS patients takes place in bilateral or right hemispheric language networks, with a strong focus in anterior language regions. These functional changes can be interpreted as important compensatory strategies of the central nervous system (CNS) to stabilize cognitive, especially language performance.
When compared with published national or international data, our pro-active treatment approach to ELGANs was associated with higher or equal survival rates without increasing rates of severe neonatal morbidity or neuro-developmental impairment at the age of 18 to 24 months.
IntroductionThe aim of this study was to determine the feasibility and clinical utility of point-of-care electroencephalogram (pocEEG) in the paediatric emergency department (ED) for children presenting with acute non-traumatic central nervous system (CNS) disorders.MethodsRetrospective observational study of prospectively collected data in paediatric patients (0–16 years) with acute non-traumatic CNS-disorders presenting between April 2014 and February 2017 to a single paediatric ED in Switzerland.The 2-channel EEG was applied to all patients presenting with acute seizures or impaired consciousness to the ED. For a pocEEG, scalp surface electrodes are applied in five locations, thus allowing registration of fronto-temporal bilateral cortical activity. Neurology consultants assisted with interpretation of readings. EEG findings and clinical characteristics were collected. Feasibility and usefulness were rated via Likert scale.Results36 patients with acute seizures or altered mental status were analysed. Age range was 9 months to 15 years, median age of 34 months. 21 of 36 (58%) patients arrived out of hours. Application of electrodes was rated as ‘easy’ in 28 (77.8%) patients and rated as ‘difficult’ in 8 (22.2%). The utility of the EEG was rated by physicians as ‘very useful/diagnostic’ in 13 cases (36%), ‘useful’ in 21 cases (58%), ‘not useful’ in two cases (8%). None were rated ‘negative.’ConclusionUptake of pocEEG introduction has been very encouraging. Provider ratings were overwhelmingly positive. Recognition of non-convulsive status epilepticus was improved and pocEEG facilitated more targeted interventions.
Prenatal disruptive injuries within one or both cerebellar hemispheres, which are mostly caused by hemorrhages, are well known. Primary disruptive lesions of the brainstem, however, are exceptional. Here we report on clinical and neuroimaging findings, as well as outcome of four male infants with prenatal brainstem disruptions that have been seen between 2005 and 2015. Two infants with extensive brainstem defects (from the midbrain to the medulla) had respiratory insufficiency and died at the age of 12 weeks and 22 months, respectively. Two patients had smaller, unilateral/asymmetrical lesions in the pontomesencephalic and medullary regions, respectively, and presented with permanent multiple cranial nerve deficits and long-tract signs. Recognition of prenatal brainstem disruptions and distinction from malformations are essential for the management and the estimation of a recurrence risk.
The incidence of PVL in Switzerland is 1.2% for preterm infants with a birth weight less than 1500 g. Cranial ultrasonography on infants at risk for PVL is important because 44% of the infants with PVL didn't show neurologic abnormalities at discharge.
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