Characteristics of seizures among boys with standard risk childhood cerebral adrenoleukodystrophy treated with hematopoietic cell transplantation
Abstract number :
3.453
Submission category :
11. Behavior/Neuropsychology/Language / 11B. Pediatrics
Year :
2022
Submission ID :
2232951
Source :
www.aesnet.org
Presentation date :
12/5/2022 12:00:00 PM
Published date :
Nov 22, 2022, 05:28 AM
Authors :
Allison Foy, PhD – University of Minnesota Medical School; Evelyn Elizondo, N/A – University of Minnesota Medical School; Sam Kroening, BA – University of Minnesota Medical School; Dante Rogers, BS – University of Minnesota Medical School; Daniel Kenney-Jung, MD – Duke University; David Nascene, MD – University of Minnesota Medical School; Igor Nestrasil, MD, PhD – University of Minnesota Medical School; Paul Orchard, MD – University of Minnesota Medical School; Troy Lund, MD, PhD – University of Minnesota Medical School; Ashish Gupta, MD – University of Minnesota Medical School; Elizabeth Pierpont, PhD – University of Minnesota Medical School
This is a Late-Breaking abstract.
Rationale: Childhood cerebral adrenoleukodystrophy (cALD) is a rare X-linked genetic disorder affecting males characterized by progressive cerebral demyelination and neurologic decline. Standard intervention for cALD is hematopoietic cell transplantation (HCT). Although seizures are a potentially serious complication in boys with cALD who undergo HCT, this topic has not been comprehensively studied. We aimed to characterize the timing and presentation of seizures in patients with cALD, identify potential risk factors for the development of seizures, and characterize associations between seizures and long-term neurocognitive functioning.
Methods: Neurological and neuropsychological records were retrospectively reviewed for all patients with standard risk cALD (Loes MRI severity score < 10) who underwent allogeneic HCT at our center between 1991-2022 (N = 77). Data regarding seizure presence, type, and timing were extracted for all patients. Neurocognitive test data were extracted for patients with a history of seizures.
Behavior