Evidence map›Paper›PMID 39138714›Full record

ArticleNeurocritical care2025

Seizure Characteristics and EEG Features in Intoxication Type and Energy Deficiency Neurometabolic Disorders in the Pediatric Intensive Care Unit: Single-Center Experience Over 10 Years.

Kuntal Sen, Dana Harrar, Nicole Pariseau, Karis Tucker, Julia Keenan, Anqing Zhang, Andrea Gropman

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Article in Neurocritical care, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

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2citing papers in PubMed
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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Kuntal SenDivision of Neurogenetics and Neurodevelopmental Pediatrics, Center for Neuroscience and Behavioral Medicine, GWU School of Medicine and Health Sciences, Children's National Hospital, 111 Michigan Ave, NW, Washington, DC, 20010, USA. ksen2@childrensnational.org.
Dana HarrarDivision of Child Neurology, Children's National Hospital, Washington, DC, USA.
Nicole PariseauDivision of Child Neurology, Children's National Hospital, Washington, DC, USA.
Karis TuckerDivision of Neurogenetics and Neurodevelopmental Pediatrics, Center for Neuroscience and Behavioral Medicine, GWU School of Medicine and Health Sciences, Children's National Hospital, 111 Michigan Ave, NW, Washington, DC, 20010, USA.
Julia KeenanDivision of Child Neurology, Children's National Hospital, Washington, DC, USA.
Anqing ZhangDepartment of Biostatistics, Children's National Hospital, Washington, DC, USA.
Andrea GropmanDivision of Neurogenetics and Neurodevelopmental Pediatrics, Center for Neuroscience and Behavioral Medicine, GWU School of Medicine and Health Sciences, Children's National Hospital, 111 Michigan Ave, NW, Washington, DC, 20010, USA.

Funding

Children's National Board of Visitors/ Founders Auxiliary Board Grant FY 2021 CHB Award 9 6005920
6 · The paper itself

Abstract

backgroundAcute metabolic crises in inborn errors of metabolism (such as urea cycle disorders, organic acidemia, maple syrup urine disease, and mitochondrial disorders) are neurological emergencies requiring management in the pediatric intensive care unit (PICU). There is a paucity of data pertaining to electroencephalograms (EEG) characteristics in this cohort. We hypothesized that the incidence of background abnormalities and seizures in this cohort would be high. Neuromonitoring data from our center's PICU over 10 years are presented in this article.

methodsData were collected by retrospective chart review for patients with the aforementioned disorders who were admitted to the PICU at our institution because of metabolic/neurologic symptoms from 2008 to 2018. Descriptive statistics (χ

resultsOur cohort included 40 unique patients (8 with urea cycle disorder, 7 with organic acidemia, 3 with maple syrup urine disease, and 22 with mitochondrial disease) with 153 admissions. Presenting symptoms included altered mentation (36%), seizures (41%), focal weakness (5%), and emesis (28%). Continuous EEG was ordered in 34% (n = 52) of admissions. Twenty-three admissions were complicated by seizures, including eight manifesting as status epilepticus (seven nonconvulsive and one convulsive). Asymmetry and focal slowing on EEG were associated with seizures. Moderate background slowing or worse was noted in 75% of EEGs. Among those patients monitored on EEG, 4 (8%) died, 3 (6%) experienced a worsening of their Pediatric Cerebral Performance Category (PCPC) score as compared to admission, and 44 (86%) had no change (or improvement) in their PCPC score during admission.

conclusionsThis study shows a high incidence of clinical and subclinical seizures during metabolic crisis in patients with inborn errors of metabolism. EEG background features were associated with risk of seizures as well as discharge outcomes. This is the largest study to date to investigate EEG features and risk of seizures in patients with neurometabolic disorders admitted to the PICU. These data may be used to inform neuromonitoring protocols to improve mortality and morbidity in inborn errors of metabolism.

Indexed as

ElectroencephalographyIntensive Care Units, PediatricMaple Syrup Urine DiseaseMetabolism, Inborn ErrorsMitochondrial DiseasesSeizuresUrea Cycle Disorders, InbornAdolescentChildChild, PreschoolFemaleHumansInfantMaleRetrospective StudiesAcute metabolic crisisHyperammonemiaInborn errors of metabolismMaple syrup urine diseaseMitochondrial disordersOrganic acidemiasUrea cycle disorders

Identifiers

PMID39138714

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.