ABSTRACT Objective: Ictal semiology interpretation for differentiating psychogenic nonepileptic seizures (PNESs) and epileptic seizures (ESs) is important for the institution of appropriate treatment. Our objective was to assess the ability of different health care professionals (HCPs) or students to distinguish PNES from ES based on video-recorded seizure semiology. Methods: This study was designed following the Standards for Reporting of Diagnostic Accuracy Studies (STARD) guidelines. We showed in a random mix 36 videos of PNES or ES (18 each) and asked 558 participants to classify each seizure. The diagnostic accuracy of various groups of HCPs or students for PNES versus ES was assessed, as well as the effect of patient age and sex. Measures of diagnostic accuracy included sensitivity, specificity, and area under the curve (AUC). Results: The descending order of diagnostic accuracy (AUC) was the following (p ≤ 0.001): (1) neurologists and epileptologists; (2) neurology residents; (3) other specialists and nurses with experience in epilepsy; and (4) undergraduate medical students. Although there was a strong trend toward statistical difference, with AUC 95% confidence intervals (CIs) that were not overlapping, between epileptologists (95% CI 93, 97) compared to neurologists (95% CI 88, 91), and neurologists compared to electroencephalography technicians (95% CI 82, 87), multiple pairwise comparisons with the conservative Tukey–Kramer honest significant difference test revealed no statistical difference (p = 0.25 and 0.1, respectively). Patient age and sex did not have an effect on diagnostic accuracy in neurology specialists. Conclusion: Visual recognition of PNES by HCPs or students varies overall proportionately with the level of expertise in the field of neurology/epilepsy.
Abstract BACKGROUND Infants with childhood-onset epilepsies, including epileptic encephalopathies, are at high risk of developmental challenges such as motor and language delays. There has been a growing focus on developmental screening instruments due to the association between delayed development and long-term adverse outcomes, including negative impacts on health-related quality of life, school achievement and social participation. Therefore, children with developmental delays should be referred as early as possible to rehabilitation services such as occupational, physical, or speech therapy. Developmental screening using standardized tools is recommended in order to identify the high-risk children that require additional developmental assessments. The Ages and Stages Questionnaires (ASQ), a parent-completed developmental screening tool, is one of the more cited and recommended developmental screening tools. However, its utility in children with early-onset epilepsy has yet to be validated. OBJECTIVES This study aimed to determine the validity of the ASQ for identifying children with new-onset epilepsy who require further professional developmental rehabilitation. DESIGN/METHODS Fifty infants (25 males) with seizure onset under 3 years of age were prospectively recruited. Parents completed the ASQ-3 before the first clinic appointment. The ASQ-3 evaluates 5 domains of development. An ASQ score below 2 standard deviations (SD) on any one of the domains was considered abnormal. Once completed, all infants were evaluated by a multidisciplinary team including neurologists, occupational therapists, and physical therapists to determine if they should be referred for further rehabilitation interventions. Accuracy of the ASQ was calculated in terms of sensibility, specificity, positive predictive value (PPV) and negative predictive value (NPV). RESULTS Mean age at the initial evaluation was 13.1 months (SD=8.22). Among the 50 infants, 36 (72%) received a score <-2 SD in at least one domain of the ASQ and 43 (86%) were referred for further rehabilitation services after the multidisciplinary assessment. The ASQ had a sensitivity of 81%, a specificity of 71%, a PPV of 93% and a NPV of 36% in identifying children with new-onset epilepsy who would require further rehabilitation services. CONCLUSION The ASQ, a well-known parent-completed developmental screening tool, when abnormal shows adequate validity for the identification of children with early-onset epilepsy who require developmental assessments and interventions by rehabilitation professionals. Our results also provide further evidence that development delay is the rule rather than the exception in infants with new onset epilepsy.
To validate a case definition of multiple sclerosis (MS) in the pediatric population using administrative (health claims) data, and to estimate the incidence and prevalence of MS in the pediatric population for Ontario, Canada.
Methods
We used population-based administrative data to identify persons aged ≤18 years with MS. We assessed the performance of multiple administrative case definitions using a clinical reference cohort including children with MS, children with monophasic demyelinating syndromes, and healthy children; we report sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). We applied 2 preferred case definitions to estimate the incidence and prevalence of MS from 2003 to 2014.
Results
The Canadian Chronic Disease Surveillance System definition of ≥1 hospitalization or ≥5 physician claims for MS within 2 years had a sensitivity of 81.1%, specificity of 100%, PPV of 100%, and NPV of 86%. The Marrie definition of ≥3 hospital or physician claims for MS ever had a sensitivity of 89.2%, specificity of 100%, PPV of 100%, and NPV of 91.5%. Depending on the administrative case definition used, in 2014, the annual age-standardized annual incidence of MS in the pediatric population ranged from 0.99 to 1.24 per 100,000 population, and the age-standardized prevalence ranged from 4.03 to 6.8 per 100,000 population. The prevalence of MS rose over time.
Conclusion
Administrative data provide a feasible, valid means of estimating the incidence and prevalence of MS in the pediatric population. MS prevalence in the Ontario pediatric population is among the highest reported in pediatric populations worldwide.
Few studies have looked at long-term epileptic and cognitive outcome of frontal lobe epilepsy (FLE) in children. Most are limited by inclusion of lesional and nonlesional patients.To define the epileptic and functional outcome of children with nonlesional FLE.We reviewed medical records and neuropsychological evaluations of patients with nonlesional FLE diagnosed between 1994 and 2004. We included children with either focal or regional frontal EEG and/or functional imaging abnormalities. We reviewed their charts for seizure and neuropsychological outcome.We included 21 children. Twelve (57.1%) presented with daily seizures. Seizures were nocturnal in 8 of 21, secondarily generalized in 6 of 21, adversive in 5 of 21, and focal motor in 6 of 21. Although, initial seizure control was poor in 14 of 21, long-term control was achieved in 10 of 21 after 14.6+/-22.3 months. Early development was normal in 12 of 21 but at later formal neuropsychological evaluation only 3 of 12 still had a normal profile. The majority of children had learning difficulties requiring special education prior to seizure onset (6 of 10). A clearly defined regression after seizure onset was observed in three children. The majority exhibited attention deficit and hyperactivity or impulsivity (14 of 21), behavioral problems (8 of 21), and cognitive impairments (10 of 21). Early seizure control was associated with a better cognitive outcome.Nonlesional FLE is associated with poor seizure and behavioral outcomes. Whether this is secondary to MRI-silent developmental lesions or to the progressive repercussion of seizures on frontal lobe functions remains uncertain. A prospective study with early neuropsychological assessment could help confirm the latter.
Stroke-like episodes, defined as periods of acute localized neurological dysfunction during which brain imagery suggests cerebral ischemia but vascular anatomy is normal, occurred in 3 patients with autosomal recessive Saguenay-Lac St-Jean (SLSJ) cytochrome oxidase (COX) deficiency. The patients developed focal neurological deterioration and frontal hypodensities on cerebral computerized tomography (CT). Arteriography, performed in 1 patient during an acute episode, showed normal vascular anatomy. Nevertheless, capillary shunting was evident both in regions that appeared abnormal on the initial cerebral CT study and in regions that appeared normal but subsequently developed Leigh disease. Stroke-like episodes did not exacerbate systemic acidosis, and acidotic decompensations occurred independently of stroke-like episodes. In conclusion, stroke-like episodes occur in autosomal recessively inherited congenital lactic acidoses as well as in those caused by mitochondrial DNA mutations. In some cases, acute localized neurovascular changes occur in regions that subsequently develop Leigh disease. Ann Neurol 1999;45:389–392
