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Liberato and colleagues retrospectively analyzed brain magnetic resonance imaging (MRI) scans for brain lesion volume, Loes score, and growth over time in a cohort of boys biochemically or genetically diagnosed with adrenoleukodystrophy. Demographic data, genotype information, biochemical data, follow-up intervals, and brain lesion characteristics were recorded in all MRI scans. White matter changes were scored using the Loes MRI severity scoring system, and lesions were subdivided into 5 patterns based on primary anatomic distribution.
Sixty percent of patients (28/47) showed brain lesions (median Loes score of 3.0 points; range 0.5–11). Seventy-nine percent of patients with CCALD (22/28) had contrast enhancement on first lesional or subsequent MRI. Lesion progression (Loes increase of ≥0.5 point) was seen in 50% of patients (14/28). The rate of lesion growth (mL/mo) was faster in younger patients (r = −0.745; p < 0.0001). Older patients (median age 14.4 y/o) tended to undergo spontaneous arrest of disease. Early lesions grew 46× faster when still limited to the splenium, genu of the corpus callosum, or the brainstem (p = 0.001).
The paper provide a description of childhood cerebral adrenoleukodystrophy (CCALD) lesion development in a cohort of asymptomatic boys. Understanding the early stages of CCALD is crucial to optimize treatments for children diagnosed by newborn screening.