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 #11 Poster Symposium: Neuropsychological Care in Early Life Epilepsies: From Referrals to Outcomes — Abstract 4 Initial Use of a Parent-Report Measure of Responsivity in Children with Profound Impairment Associated with Developmental and Epileptic Encephalopathy Natasha Ludwig, Kennedy Krieger Institute, Baltimore, United States Mary Wojnaroski, Nationwide Children's Hospital, Columbus, United States Jenny Downs, Telethon Kids, 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 University, Chicago, United States Category: Epilepsy/Seizures Keyword 1: pediatric neuropsychology Objective: Standardized neurodevelopmental measures provide limited information about children with profound impairments associated with developmental and epileptic encephalopathy (DEE). The Coma Recovery Scale, Pediatric (CRS-P) is a clinician-administered assessment of responsivity, alertness, and attention for children in a disorder of consciousness due to severe acquired brain injury. Considering that the CRS-P was designed to accommodate the measurement needs of a profoundly impacted group, it may have utility for capturing meaningful aspects of cognition in children with DEEs. In a sample of 10 children with SCN2A-DEE, the CRS-P demonstrated greater range and variability compared to standardized measures. Furthermore, a parent-reported version of the CRS-P, the Caregiver Responsiveness Inventory (CRI), was highly correlated with CRS-P. The current study extends these preliminary findings by exploring the characteristics of the CRI in a larger sample of children with DEE of mixed genetic etiologies. Participants and Methods: Thirty-five caregivers of children with profound impairment associated with DEE were recruited to complete an online survey about their child’s functioning through a DEE-focused patient advocacy group. Children were 2-18 years old (Mean=8.5, SD=4.8; N=14 male) and most had an identified pathogenic genetic etiology (97%), were prescribed >1 anti-seizure medication (80%), and did not use spoken words (97%). Approximately half were unable to pick up objects (46%) and had a gastronomy tube (49%). Cognitive abilities approximated an 8.5 month-level per the Developmental Profile, Fourth Edition Parent/Caregiver Checklist (DP-4) Cognition scale (Mean raw score=5.7, SD=5.2). The CRI asks caregivers to rate their child’s responsivity over the past two weeks across six domains of including auditory, visual, motor, oromotor, communication and arousal. The maximum possible total score is 23 (higher scores mean higher responsivity). Results: Mean CRI total score was 14.1 (SD=5.4). No child scored the minimum or maximum possible CRI total score, but this occurred on domain scores. The communication domain yielded the most with the minimum possible score (77%) and the oromotor domain yielded the most with the maximum possible score (86%). The relationship between the CRI total and DP-4 Cognition raw score was moderate, r=.39, p=.04. When Percent of Maximum Possible (POMP) scores were considered, the CRI total yielded a higher mean and greater range/variability (M=61.7%, SD=23.6, range=13.0-95.8%) than the DP-4 Cognition raw (M=13.5%, SD=12.9, range=0-54.8%). Conclusions: In this sample of children with profound impairment associated with DEE, caregiver ratings of responsivity on the CRI demonstrated minimal floor and ceiling effects when the total score was considered, but findings indicate some modifications may improve the utility of domain scores. The CRI was moderately related to the DP-4 Cognition, but POMP metrics indicate more range and variability was captured on the CRI than on the DP-4 in this sample, which is important when considering the utility of an outcome measure. Findings support further investigation of the utility of CRI as a measure for clinical care and possibly in clinical trials for this patient 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 #11

Poster Symposium: Neuropsychological Care in Early Life Epilepsies: From Referrals to Outcomes — Abstract 4

Initial Use of a Parent-Report Measure of Responsivity in Children with Profound Impairment Associated with Developmental and Epileptic Encephalopathy

Natasha Ludwig, Kennedy Krieger Institute, Baltimore, United States
Mary Wojnaroski, Nationwide Children's Hospital, Columbus, United States
Jenny Downs, Telethon Kids, 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 University, Chicago, United States

Category: Epilepsy/Seizures

Keyword 1: pediatric neuropsychology

Objective:

Standardized neurodevelopmental measures provide limited information about children with profound impairments associated with developmental and epileptic encephalopathy (DEE). The Coma Recovery Scale, Pediatric (CRS-P) is a clinician-administered assessment of responsivity, alertness, and attention for children in a disorder of consciousness due to severe acquired brain injury. Considering that the CRS-P was designed to accommodate the measurement needs of a profoundly impacted group, it may have utility for capturing meaningful aspects of cognition in children with DEEs. In a sample of 10 children with SCN2A-DEE, the CRS-P demonstrated greater range and variability compared to standardized measures. Furthermore, a parent-reported version of the CRS-P, the Caregiver Responsiveness Inventory (CRI), was highly correlated with CRS-P. The current study extends these preliminary findings by exploring the characteristics of the CRI in a larger sample of children with DEE of mixed genetic etiologies.

Participants and Methods:

Thirty-five caregivers of children with profound impairment associated with DEE were recruited to complete an online survey about their child’s functioning through a DEE-focused patient advocacy group. Children were 2-18 years old (Mean=8.5, SD=4.8; N=14 male) and most had an identified pathogenic genetic etiology (97%), were prescribed >1 anti-seizure medication (80%), and did not use spoken words (97%). Approximately half were unable to pick up objects (46%) and had a gastronomy tube (49%). Cognitive abilities approximated an 8.5 month-level per the Developmental Profile, Fourth Edition Parent/Caregiver Checklist (DP-4) Cognition scale (Mean raw score=5.7, SD=5.2). The CRI asks caregivers to rate their child’s responsivity over the past two weeks across six domains of including auditory, visual, motor, oromotor, communication and arousal. The maximum possible total score is 23 (higher scores mean higher responsivity).

Results:

Mean CRI total score was 14.1 (SD=5.4). No child scored the minimum or maximum possible CRI total score, but this occurred on domain scores. The communication domain yielded the most with the minimum possible score (77%) and the oromotor domain yielded the most with the maximum possible score (86%). The relationship between the CRI total and DP-4 Cognition raw score was moderate, r=.39, p=.04. When Percent of Maximum Possible (POMP) scores were considered, the CRI total yielded a higher mean and greater range/variability (M=61.7%, SD=23.6, range=13.0-95.8%) than the DP-4 Cognition raw (M=13.5%, SD=12.9, range=0-54.8%).

Conclusions:

In this sample of children with profound impairment associated with DEE, caregiver ratings of responsivity on the CRI demonstrated minimal floor and ceiling effects when the total score was considered, but findings indicate some modifications may improve the utility of domain scores. The CRI was moderately related to the DP-4 Cognition, but POMP metrics indicate more range and variability was captured on the CRI than on the DP-4 in this sample, which is important when considering the utility of an outcome measure. Findings support further investigation of the utility of CRI as a measure for clinical care and possibly in clinical trials for this patient population