Somatosensory evoked potentials in adrenomyeloneuropathy.

Adrenomyeloneuropathy (AMN) is an X-linked metabolic disorder causing accumulation of very-long-chain fatty acids with multifocal nervous system demyelination of the peripheral nerves, spinal cord, and cerebrum. The extent to which the disorder affects upper versus lower limbs or peripheral versus CNS has not been electrophysiologically defined in a large population nor differentiated in men and women. To determine patterns of nervous system demyelination and define gender differences, we studied 83 AMN patients with short latency median and posterior tibial nerve somatosensory evoked potentials (SSEPs).

Clinical course of neuropsychological functioning after chronic exposure to organic and inorganic lead.

This case series describes the clinical course (12- to 28-month follow-up) of neuropsychological functioning in 23 workers who had chronic occupational exposure to a mixture of organic and inorganic lead. Significant improvement in performance was seen in 123 tests and deterioration in 323 tests. However, there was no significant change in the majority of tests (1923).

Visual system abnormalities in adrenomyeloneuropathy.

We studied the visual system in 59 men with adrenomyeloneuropathy. Pattern-reversal visual evoked potentials, magnetic resonance imaging, and clinical examination revealed that visual pathways are affected in 63% of patients, involving the optic discs, optic nerves, lateral geniculate bodies, optic radiations, and parietooccipital cortex. This indicates both primary demyelination of the optic nerves and discs, and more diffuse involvement of postchiasmal structures.

Comparison of neurobehavioral function in workers exposed to a mixture of organic and inorganic lead and in workers exposed to solvents.

The neurobehavioral effects of lead (organic and inorganic) and organic solvents were compared in 386 U.S. workers (52 reference, 190 lead, and 144 solvent workers).