Biomedical subjects

J Rignani

Publications and source records attributed to J Rignani.

3 recordsLinked to original sources

PubMed1997

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). Most women (10/16) had abnormal median SSEPs all involving central pathways, whereas most men (59/67) had abnormal median SSEPs involving both peripheral and central pathways. Tibial SSEPs were abnormal in both sexes (14/15 women, 67/67 men), with either peripheral or central pathway involvement. This study demonstrates the frequent widespread involvement of both peripheral nerve and central somatosensory pathways in men with AMN and the predominantly central involvement in women.

Adolescent↗

PubMed1995

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). The association between neurobehavioral test performance and duration of exposure to lead or solvents was also examined and compared. The neurobehavioral test battery consisted of examiner and computer-administered neurobehavioral tests, a test of olfactory function, and questionnaires that assessed neuropsychiatric symptoms. Adjusted mean differences on the neurobehavioral test scores were estimated by comparing the exposed group to the referent group using linear regression and adjusting for premorbid intellectual ability, age, and race. Both lead and solvents were associated with diminished neurobehavioral performance in all neurobehavioral areas tested. Specifically, while lead and solvent exposure had the same magnitude of adverse effects on tests of manual dexterity, lead exposure was associated with greater adverse effects on memory and learning tests but with less adverse effects on executive/motor tests and on a test of olfaction than solvent exposure. An elevated number of neuropsychiatric symptoms was reported by 7% of the referent group, 43% of the lead group, and 15% of the solvent group. For exposure duration of < or = 10 years, more neurobehavioral decrements were found in the solvent group relative to the lead group. However, for exposure duration of > or = 18 years, the lead group showed more decrements than the solvent group. Overall, these data suggest differences in neurobehavioral functioning between the lead (organic and inorganic) and solvent exposed workers examined in this study.

Adult↗

PubMed1995

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.

Adrenoleukodystrophy↗