INS NYC 2024 Program

Custom Content	Poster Session 09 Program Schedule 02/16/2024 03:30 pm - 04:45 pm Room: Shubert Complex (Posters 1-60) Poster Session 09: Epilepsy \| Oncology \| MS \| Infectious Disease Final Abstract #10 Poster Symposium: Neuropsychological Care in Early Life Epilepsies: From Referrals to Outcomes — Abstract 3 Performance of the Developmental Profile, Fourth Edition in Children with Profound Impairment Associated with Developmental and Epileptic Encephalopathy Mary Wojnaroski, Nationwide Children's, Columbus, United States Natasha Ludwig, KennedyKrieger Institute, Baltimore, United States Jenny Downs, Telethon Kids Institute, Perth, Australia Sarah Te, Decoding Developmental Epilepsies, Washington DC, United States Rebecca Hommer, University of Maryland, College Park, United States Gabrielle Conecker, Decoding Developmental Epilepsies, Washington DC, United States JayEtta Hecker, Decoding Developmental Epilepsies, Washington DC, United States Anne Berg, Northwestern Univeristy, Chicago, United States Category: Epilepsy/Seizures Keyword 1: pediatric neuropsychology Objective: Children with Developmental and Epileptic Encephalopathy (DEE) experience epilepsy and often profound impairment very early in development. The impact to the developing brain is pervasive and these children typically exhibit substantial cognitive and functional impairments throughout life. While disease modifying therapies are on the horizon for DEEs, many neuropsychological assessment tools have limited ability to capture the range and variability of skills and detect change over time, which is imperative for both clinical care and clinical trial readiness in this group. The Developmental Profile-Fourth edition (DP-4) Parent/Caregiver Checklist measures development in individuals birth-21 years; however, performance of this measure has yet to be examined children with profound impairment associated with DEE. Here, we evaluated the initial performance of the DP-4 in a sample of individuals with profound impairment associated with DEE to inform potential utility of the measure in this context. Participants and Methods: Forty-five caregivers completed the DP-4 as part of an online survey of child functioning and caregiver priorities in individuals with profound impairment and DEE. The DP-4 measures five areas of development and functioning: Physical, Adaptive, Social-Emotional, Cognitive, and Communication. Mean age of individuals with DEE was 9.19 years (SD=5.31, range 2-18). Most had an identified pathogenic genetic etiology (N=41). Results: Given the severity of impairment in this sample, raw scores and age-equivalencies (AEs) are reported as standard scores offer limited information. On the Physical subscale, mean raw score was 6 (SD=5.10, range 0-19, maximum points possible=37) and mean AE was 8.5 months (range 0-22.5); two individuals scored at the floor (i.e., raw=0; 4%). On the Adaptive Behavior subscale, mean raw score was 5 (SD=4.42, range 0-20, maximum points possible=41) and mean AE was 8.5 months (range 0–33.5); two individuals scored at the floor (4%). On the Social-Emotional subscale, mean raw score was 6 (SD=3.78, range 0-15, maximum points possible=36) and mean AE was 4.5 months (range 0 – 25.5); two individuals scored at the floor (4%). On the Cognitive subscale, mean raw score was 5 (SD=4.48, range 0-23, maximum points possible=42) and mean AE was 8.5 months (range 0–50.5); one individual scored at the floor (2%). On the Communication subscale, mean raw score was 4 (SD=4.47, range 0 to 20, maximum points possible=34) and mean AE was 6.5 months (range 0–29.5); three individuals received a score at the floor (7%). Conclusions: Results show relatively minimal floor effects on the DP-4 in this profoundly impaired sample of children with DEE. Specifically, floor effects only occur 2-7% of the time across domains. These findings suggest that the DP-4 may be a particularly useful tool in measuring skills at the lowest end of the developmental spectrum. Further evaluation of the DP-4 and its ability to measure meaningful change in individuals with DEE is warranted, given the need to sensitively measure development and functioning in this population.

Custom Content

02/16/2024
03:30 pm - 04:45 pm
Room: Shubert Complex (Posters 1-60)

Poster Session 09: Epilepsy | Oncology | MS | Infectious Disease

Final Abstract #10

Poster Symposium: Neuropsychological Care in Early Life Epilepsies: From Referrals to Outcomes — Abstract 3

Performance of the Developmental Profile, Fourth Edition in Children with Profound Impairment Associated with Developmental and Epileptic Encephalopathy

Mary Wojnaroski, Nationwide Children's, Columbus, United States
Natasha Ludwig, KennedyKrieger Institute, Baltimore, United States
Jenny Downs, Telethon Kids Institute, Perth, Australia
Sarah Te, Decoding Developmental Epilepsies, Washington DC, United States
Rebecca Hommer, University of Maryland, College Park, United States
Gabrielle Conecker, Decoding Developmental Epilepsies, Washington DC, United States
JayEtta Hecker, Decoding Developmental Epilepsies, Washington DC, United States
Anne Berg, Northwestern Univeristy, Chicago, United States

Category: Epilepsy/Seizures

Keyword 1: pediatric neuropsychology

Objective:

Children with Developmental and Epileptic Encephalopathy (DEE) experience epilepsy and often profound impairment very early in development. The impact to the developing brain is pervasive and these children typically exhibit substantial cognitive and functional impairments throughout life. While disease modifying therapies are on the horizon for DEEs, many neuropsychological assessment tools have limited ability to capture the range and variability of skills and detect change over time, which is imperative for both clinical care and clinical trial readiness in this group. The Developmental Profile-Fourth edition (DP-4) Parent/Caregiver Checklist measures development in individuals birth-21 years; however, performance of this measure has yet to be examined children with profound impairment associated with DEE. Here, we evaluated the initial performance of the DP-4 in a sample of individuals with profound impairment associated with DEE to inform potential utility of the measure in this context.

Participants and Methods:

Forty-five caregivers completed the DP-4 as part of an online survey of child functioning and caregiver priorities in individuals with profound impairment and DEE. The DP-4 measures five areas of development and functioning: Physical, Adaptive, Social-Emotional, Cognitive, and Communication. Mean age of individuals with DEE was 9.19 years (SD=5.31, range 2-18). Most had an identified pathogenic genetic etiology (N=41).

Results:

Given the severity of impairment in this sample, raw scores and age-equivalencies (AEs) are reported as standard scores offer limited information. On the Physical subscale, mean raw score was 6 (SD=5.10, range 0-19, maximum points possible=37) and mean AE was 8.5 months (range 0-22.5); two individuals scored at the floor (i.e., raw=0; 4%). On the Adaptive Behavior subscale, mean raw score was 5 (SD=4.42, range 0-20, maximum points possible=41) and mean AE was 8.5 months (range 0–33.5); two individuals scored at the floor (4%). On the Social-Emotional subscale, mean raw score was 6 (SD=3.78, range 0-15, maximum points possible=36) and mean AE was 4.5 months (range 0 – 25.5); two individuals scored at the floor (4%). On the Cognitive subscale, mean raw score was 5 (SD=4.48, range 0-23, maximum points possible=42) and mean AE was 8.5 months (range 0–50.5); one individual scored at the floor (2%). On the Communication subscale, mean raw score was 4 (SD=4.47, range 0 to 20, maximum points possible=34) and mean AE was 6.5 months (range 0–29.5); three individuals received a score at the floor (7%).

Conclusions:

Results show relatively minimal floor effects on the DP-4 in this profoundly impaired sample of children with DEE. Specifically, floor effects only occur 2-7% of the time across domains. These findings suggest that the DP-4 may be a particularly useful tool in measuring skills at the lowest end of the developmental spectrum. Further evaluation of the DP-4 and its ability to measure meaningful change in individuals with DEE is warranted, given the need to sensitively measure development and functioning in this population.