Rare Spontaneous Attenuation of Childhood Inflammatory Cerebral Adrenoleukodystrophy.

J Pediatr Neurol

Division of Pediatric Blood and Marrow Transplantation, Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota, United States.

Published: April 2020

X-linked adrenoleukodystrophy (ALD) is a neurodegenerative peroxisomal disorder with variable clinical phenotypes. Childhood cerebral ALD (CCALD) is at the most severe end of the disease spectrum. In CCALD, the clinical manifestations include increasing deficits in behavior, vision, hearing, coordination, and motor function, as well as seizures. Without treatment, CCALD often results in apparent vegetative state within 1 to 2 years of appearance of initial signs and symptoms. We present the case of a boy with classic inflammatory CCALD who exhibited spontaneous attenuation in disease progression. While extremely rare, spontaneous arrest of disease progression may occur in boys with inflammatory CCALD.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7410095PMC
http://dx.doi.org/10.1055/s-0039-1677805DOI Listing

Publication Analysis

Top Keywords

rare spontaneous
8
spontaneous attenuation
8
inflammatory ccald
8
disease progression
8
ccald
5
attenuation childhood
4
childhood inflammatory
4
inflammatory cerebral
4
cerebral adrenoleukodystrophy
4
adrenoleukodystrophy x-linked
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!