The adult form, called adrenomyeloneuropathy, presents at a mean age of 28 years as progressive spinal cord dysfunction with spastic paraparesis, sensory loss, and bowel and bladder symptoms. Cerebral involvement may be minimal. Only half of patients with adult-onset disease have brain abnormalities on MRI, most often in the corticospinal tracts. Most patients have diffuse atrophy of the spinal cord. Bone marrow transplantation may be effective if performed before severe symptoms develop.
Prognosis is poor for the childhood form of disease.
Patients with adult-onset disease usually require assistance for ambulation within 10 to 15 years, and a large fraction of patients develop rapidly progressive cerebral lesions five to 10 years after the onset of spinal cord symptoms. Metachromatic leukodystrophy is an autosomal recessive disorder that results in demyelination of axons in the central and peripheral nervous systems. A that lead to accumulation of metachromatically staining sulfatides. Onset is usually in infancy or childhood, and adult onset of disease is rare. The symptoms of adult-onset disease are progressive behavioral abnormalities, dementia, ataxia, and neuropathy.