Patients typically present in the 2nd or 3rd year of life with hypotonia, seizures, and hepatomegaly. This is also known as Pseudo neonatal adrenoleukodystrophy.
On imaging patients have increased T2/FLAIR signal in the corticospinal tracts, periventricular white matter, and corpus callosum. They appear similar to Infantile Refsum disease and X-linked adrenoleukodystrophy
Reference
Barkovich AJ, Pediatric Neuroimaging