Timing the clinical onset of epileptic spasms in infantile epileptic spasms syndrome: A tertiary health center's experience.

OBJECTIVE: Lead time to treatment (clinical onset of epileptic spasms [ES] to initiation of appropriate treatment) is known to predict outcomes in infantile epileptic spasms syndrome (IESS). Timing the clinical onset of ES is crucial to establish lead time. We investigated how often ES onset could be established to the nearest week. We aimed to (1) ascertain the exact date or estimate the nearest week of ES onset and (2) compare clinical/demographic factors between patients where date of ES onset was determined or estimated to the nearest week and patients whose date of ES onset could not be estimated to the nearest week. Reasons for difficulties in estimating date of ES onset were explored. METHODS: Retrospective chart review of new onset IESS patients (January 2019-May 2022) extracted the date or week of the clinical onset of ES. Predictors of difficulty in date of ES onset estimation to the nearest week were examined by regression analysis. Sources contributing to difficulties determining date of ES onset were assessed after grouping into categories (provider-, caregiver-, disease-related). RESULTS: Among 100 patients, date of ES onset was estimated to the nearest week in 47%. On univariable analysis, age at diagnosis (p = .021), development delay (p = .007), developmental regression/stagnation (p = .021), ES intermixed with other seizures (p = .011), and nonclustered ES at onset (p = .005) were associated with difficulties estimating date of ES onset. On multivariable analysis, failure to establish date of ES onset was related to ES intermixed with other seizures (p = .004) and nonclustered ES at onset (p = .003). Sources contributing to difficulties determining date of ES onset included disease-related factors (ES characteristics, challenges interpreting electroencephalograms) and provider/caregiver-related factors (delayed diagnosis). SIGNIFICANCE: Difficulties with estimation of lead time (due to difficulties timing ES onset) can impact clinical care (prognostication), as even small increments in lead time duration can have adverse developmental consequences.

Inequities in Therapy for Infantile Spasms: A Call to Action.

OBJECTIVE: The aim of this study was to determine whether selection of treatment for children with infantile spasms (IS) varies by race/ethnicity. METHODS: The prospective US National Infantile Spasms Consortium database includes children with IS treated from 2012 to 2018. We examined the relationship between race/ethnicity and receipt of standard IS therapy (prednisolone, adrenocorticotropic hormone, vigabatrin), adjusting for demographic and clinical variables using logistic regression. Our primary outcome was treatment course, which considered therapy prescribed for the first and, when needed, the second IS treatment together. RESULTS: Of 555 children, 324 (58%) were non-Hispanic white, 55 (10%) non-Hispanic Black, 24 (4%) non-Hispanic Asian, 80 (14%) Hispanic, and 72 (13%) other/unknown. Most (398, 72%) received a standard treatment course. Insurance type, geographic location, history of prematurity, prior seizures, developmental delay or regression, abnormal head circumference, hypsarrhythmia, and IS etiologies were associated with standard therapy. In adjusted models, non-Hispanic Black children had lower odds of receiving a standard treatment course compared with non-Hispanic white children (odds ratio [OR], 0.42; 95% confidence interval [CI], 0.20-0.89; p = 0.02). Adjusted models also showed that children with public (vs. private) insurance had lower odds of receiving standard therapy for treatment 1 (OR, 0.42; CI, 0.21-0.84; p = 0.01). INTERPRETATION: Non-Hispanic Black children were more often treated with non-standard IS therapies than non-Hispanic white children. Likewise, children with public (vs. private) insurance were less likely to receive standard therapies. Investigating drivers of inequities, and understanding the impact of racism on treatment decisions, are critical next steps to improve care for patients with IS. ANN NEUROL 2022;92:32-44.

Body mass index trajectories in pediatric multiple sclerosis.

AIM: To characterize growth trajectories of children who develop multiple sclerosis compared to typically developing, regional peers and Centers for Disease Control (CDC) normative values. METHOD: This case-control study collected weight, height, and body mass index (BMI) in 40 consecutive pediatric patients with multiple sclerosis (28 females, 12 males), in addition to 120 typically developing peers (84 females, 36 males), identified and matched for year of birth, sex, ethnicity, and socio-economic status. BMI values were converted to z-scores based on CDC reference values and were compared with respect to age between our two cohorts and by years relative to multiple sclerosis onset for cases. RESULTS: Median age for the clinical onset of multiple sclerosis was 15 years. BMI z-scores are significantly higher for patients with multiple sclerosis compared to typically developing, demographically-matched peers and CDC standards. These significant differences in BMI are noted from 4 years of age and onward. Height trajectories were similar among case and control individuals and CDC normative values. INTERPRETATION: BMI in pediatric multiple sclerosis is markedly higher, beginning in early childhood, years before the clinical-onset of the disease. WHAT THIS PAPER ADDS: Children with multiple sclerosis are significantly more overweight than typically developing peers at the time of diagnosis. Body mass index trajectories are significantly higher years before the clinical manifestation(s) of multiple sclerosis.

TRAYECTORIAS DEL ÍNDICE DE MASA CORPORAL EN ESCLEROSIS MÚLTIPLE PEDIÁTRICA: OBJETIVO: Caracterizar las trayectorias de crecimiento de los niños que desarrollan esclerosis múltiple en comparación con los valores normativos de desarrollo típico, los pares regionales y los Centros para el Control de Enfermedades (CDC). MÉTODO: Este estudio de casos y controles recopiló el peso, la estatura y el índice de masa corporal (IMC) en 40 pacientes pediátricos consecutivos con esclerosis múltiple (28 mujeres, 12 varones), además de 120 compañeros con desarrollo típico (84 mujeres, 36 varones), identificados y emparejados por año de nacimiento, sexo, etnia y estatus socioeconómico. Los valores de IMC se convirtieron en puntuaciones z basándose en los valores de referencia de los CDC y se compararon con respecto a la edad entre nuestras dos cohortes y por años en relación con el inicio de esclerosis múltiple para los casos. RESULTADOS: La edad media para el inicio clínico de la esclerosis múltiple fue de 15 años. Las puntuaciones z de IMC son significativamente más altas para los pacientes con esclerosis múltiple en comparación con los pares con desarrollo demográfico y estándares CDC. Estas diferencias significativas en el IMC se observan a partir de los 4 años de edad. Las trayectorias de altura fueron similares entre los individuos de casos y controles y los valores normativos de los CDC. INTERPRETACIÓN: El IMC en la esclerosis múltiple pediátrica es notablemente más alto, comenzando en la primera infancia, años antes del inicio clínico de la enfermedad.

TRAJETÓRIAS DO ÍNDICE DE MASSA CORPORAL EM ESCLEROSE MÚLTIPLA PEDIÁTRICA: OBJETIVO: Caracterizar as trajetórias de crescimento de crianças que desenvolvem esclerose múltipla comparadas com pares regionais que se desenvolvem tipicamente e valores normativos do Centro para Controle de Doenças (CDC). MÉTODO: Este estudo de caso-controle coletou peso, altura e índice de massa corporal (IMC) em 40 pacientes pediátricos consecutivos com esclerose múltipla (28 do sexo feminino, 12 do sexo masculino), além de 120 pares com desenvolvimento típico (84 do sexo feminino, 36 do sexo masculino), pareados para ano de nascimento, sexo, etnia, e nível sócio-econômico. Os valores de IMC foram convertidos para escores z com base nos valores de referência do CDC e comparados com relação à idade entre as duas coortes, e por anos com relação ao início da esclerose múltipla para os casos. RESULTADOS: A idade mediana para o início clínico da esclerose múltipla foi 15 anos. Os escores z do IMC foram significativamente mais altos para os pacientes com esclerose múltipla comparados aos pares com desenvolvimento típico e demograficamente pareados, e aos padrões do CDC. Estas diferenças significativas no IMC são notadas a partir dos 4 anos de idade. As trajetórias de altura foram similares entre casos, controles e valores normativos do CDC. INTERPRETAÇÃO: O IMC em esclerose múltipla pediátrica é marcadamente mais alto, iniciando cedo na infância, anos antes do início clínico da doença.