Striatal Involvement on MRI in Adrenomyeloneuropathy

The document reported a case of adrenomyeloneuropathy (AMN), a variant of adrenoleukodystrophy, in a patient who began experiencing symptoms at age 12 with hair loss, followed by progressive gait disturbance and erectile impotence by age 25. By age 33, the patient exhibited diffuse baldness, spastic paraparesis, and peripheral nerve involvement. MRI scans revealed unusual high signal areas in the right striatum and bilateral internal capsules. Laboratory tests showed increased very long chain fatty acids and low serum cortisol response to ACTH. Nerve conduction studies indicated peripheral polyneuropathy, and a sural nerve biopsy showed mild loss of myelinated fibers. These findings confirmed the diagnosis of AMN.