Striatal Involvement on MRI in Adrenomyeloneuropathy

The document reported a case of adrenomyeloneuropathy (AMN), a variant of adrenoleukodystrophy, in a patient who began losing hair at age 12 and developed progressive neurological symptoms by age 25. By age 33, the patient exhibited diffuse baldness, spastic paraparesis, and peripheral neuropathy, with MRI revealing unusual high signal areas in the right striatum. Despite normal routine laboratory results, the patient had increased very long chain fatty acids and low cortisol response to ACTH. Nerve conduction studies indicated peripheral polyneuropathy, and a sural nerve biopsy showed mild myelin loss. These findings confirmed the diagnosis of AMN.