We report our pediatric experience with lacosamide, a new antiepileptic drug, approved by the US Food and Drug Administration as adjunctive therapy in focal epilepsy in patients more than 17 years ...old. We retrospectively reviewed charts for lacosamide use and seizure frequency outcome in patients with focal epilepsy (Wilcoxon signed rank test). Sixteen patients (7 boys) were identified (median dose 275 mg daily, 4.7 mg/kg daily; mean age 14.9 years, range 8-21 years). Patients were receiving a median of 2 antiepileptic drugs (interquartile range IQR 1.7-3) in addition to having undergone previous epilepsy surgery ( n = 3), vagus nerve stimulation ( n = 9), and ketogenic diet ( n = 3). Causes included structural (encephalomalacia and diffuse encephalitis, 1 each; stroke in 2) and genetic abnormalities (Aarskog and Rett syndromes, 1 each) or cause not known ( n = 10). Median seizure frequency at baseline was 57 per month (IQR 7-75), and after a median follow-up of 4 months (range 1-13 months) of receiving lacosamide, it was 12.5 per month (IQR 3-75), ( P < 0.01). Six patients (37.5%; 3 seizure free) were classified as having disease that responded to therapy (≥50% reduction seizure frequency) and 10 as having disease that did not respond to therapy (<50% in 3; increase in 1; unchanged in 6). Adverse events (tics, behavioral disturbance, seizure worsening, and depression with suicidal ideation in 1 patient each) prompted lacosamide discontinuation in 4/16 (25%). This retrospective study of 16 children with drug-resistant focal epilepsy demonstrated good response to adjunctive lacosamide therapy (median seizure reduction of 39.6%; 37.5% with ≥50% seizure reduction) without severe adverse events.
Abstract Background In children, functional neurological symptom disorders are frequently the basis for presentation for emergency care. Pediatric epidemiological and outcome data remain scarce. ...Objective Assess diagnostic accuracy of trainee's first impression in our pediatric emergency room; describe manner of presentation, demographic data, socioeconomic impact, and clinical outcomes, including parental satisfaction. Methods (1) More than 1 year, psychiatry consultations for neurology patients with a functional neurological symptom disorder were retrospectively reviewed. (2) For 3 months, all children whose emergency room presentation suggested the diagnosis were prospectively collected. (3) Three to six months after prospective collection, families completed a structured telephone interview on outcome measures. Results Twenty-seven patients were retrospectively assessed; 31 patients were prospectively collected. Trainees' accurately predicted the diagnosis in 93% (retrospective) and 94% (prospective) cohorts. Mixed presentations were most common (usually sensory-motor changes, e.g. weakness and/or paresthesias). Associated stressors were mundane and ubiquitous, rarely severe. Families were substantially affected, reporting mean symptom duration 7.4 (standard error of the mean ± 1.33) weeks, missing 22.4 (standard error of the mean ± 5.47) days of school, and 8.3 (standard error of the mean ± 2.88) of parental workdays (prospective cohort). At follow-up, 78% were symptom free. Parental dissatisfaction was rare, attributed to poor rapport and/or insufficient information conveyed. Conclusions Trainees' clinical impression was accurate in predicting a later diagnosis of functional neurological symptom disorder. Extraordinary life stressors are not required to trigger the disorder in children. Although prognosis is favorable, families incur substantial economic burden and negative educational impact. Improving recognition and appropriately communicating the diagnosis may speed access to treatment and potentially reduce the disability and cost of this disorder.
Hand stereotypies (HS) are a primary diagnostic criterion for Rett syndrome (RTT) but are difficult to characterize and quantify systematically.
We collected video on 27 girls (2-12 years of age) ...with classic RTT who participated in a mecasermin trial. The present study focused exclusively on video analyses, by reviewing two five-minute windows per subject to identify the two most common HS. Three raters with expertise in movement disorders independently rated the five-minute windows using standardized terminology to determine the level of agreement. We iteratively refined the protocol in three stages to improve descriptive accuracy, categorizing HS as “central” or “peripheral,” “simple” or “complex,” scoring each hand separately. Inter-rater agreement was analyzed using Kappa statistics.
In the initial protocol evaluating HS by video, inter-rater agreement was 20.7%. In the final protocol, inter-rater agreement for the two most frequent HS was higher than the initial protocol at 50%.
Phenotypic variability makes standardized evaluation of HS in RTT a challenge; we achieved only 50% level of agreement and only for the most frequent HS. Therefore, objective measures are needed to evaluate HS.
Abstract Background Functional neurological symptom disorders are frequently the basis for acute neurological consultation. In children, they are often precipitated by high-frequency everyday ...stressors. The extent to which a severe traumatic experience may also precipitate functional neurological abnormalities is unknown. Methods For the 2-week period after the Boston Marathon bombings, we prospectively collected data on patients whose presentation suggested a functional neurological symptom disorder. We assessed clinical and demographic variables, duration of symptoms, extent of educational impact, and degree of connection to the Marathon bombing. We contacted all patients at 6 months after presentation to determine the outcome and accuracy of the diagnosis. Results In a parallel study, we reported a baseline of 2.6 functional neurological presentations per week in our emergency room. In the week after the Marathon bombings, this frequency tripled. Ninety-one percent of presentations were delayed by 1 week, with onset around the first school day after a city-wide lockdown. Seventy-three percent had a history of a prior psychiatric diagnosis. At the 6 months follow-up, no functional neurological symptom disorder diagnoses were overturned and no new organic diagnosis was made. Conclusions Pediatric functional neurological symptom disorder may be precipitated by both casual and high-intensity stressors. The 3.4-fold increase in incidence after the Boston Marathon bombings and city-wide lockdown demonstrates the marked effect that a community-wide tragedy can have on the mental health of children. Care providers must be aware of functional neurological symptom disorders after stressful community events in vulnerable patient populations, particularly those with prior psychiatric diagnoses.
Abstract Background Quality improvement is a major component of the Accreditation Council for Graduate Medical Education core competencies required of all medical trainees. Currently, neither the ...Neurology Residency Review Committee nor the Accreditation Council for Graduate Medical Education defines the process by which this competency should be taught and assessed. We developed a quality improvement curriculum that provides mentorship for resident quality improvement projects and is clinically relevant to pediatric neurologists. Methods Before and after implementation of the quality improvement curriculum, a 14-item survey assessed resident comfort with quality improvement project skills and attitudes about implementation of quality improvement in clinical practice using a 5-point Likert scale. We used the Kruskal-Wallis and Fisher exact tests to evaluate pre to post changes. Results Residents' gained confidence in their abilities to identify measures ( P = 0.02) and perform root cause analysis ( P = 0.02). Overall, 73% of residents were satisfied or very satisfied with the quality improvement curriculum. Conclusions Our child neurology quality improvement curriculum was well accepted by trainees. We report the details of this curriculum and its impact on residents and discuss its potential to meet the Accreditation Council for Graduate Medical Education's Next Accreditation System requirements.
Subependymal giant cell astrocytomas are one of the three major intracranial lesions found in tuberous sclerosis complex. Subependymal giant cell astrocytomas are typically slow-growing tumors of ...mixed glioneuronal lineage which can become aggressive and cause obstructive hydrocephalus usually in older children and adolescents. Neonatal subependymal giant cell astrocytomas are extremely rare, and their natural history and prognosis are poorly understood. This report investigates an extremely large neonatal subependymal giant cell astrocytoma which was initially identified in utero at 19 weeks of gestation in a high-risk pregnancy with no family history of tuberous sclerosis complex.
The Diagnostic Rating Scale (DRS) was completed by the parents and teachers of 82 children referred for clinical evaluations, 73 referred children seen twice, and 218 non-referred children from the ...community. The DRS, which uses a categorical rather than a dimensional rating approach, was 70% to 90% sensitive to diagnoses of Attention Deficit/Hyperactivity Disorder (ADHD) made by blind clinical teams. In research and clinical applications, the DRS could improve screening efficiency, especially in situations where it would be desirable to exclude all children who might have ADHD or identify all children with Hyperactive-Impulsive symptoms. Because of its objectivity and consistency with the Diagnostic and Statistical Manual (DSM)-IV criteria, the DRS could facilitate comparison of participant samples across studies.
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BFBNIB, DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
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