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Biomedical subjects

I Alfonso

Publications and source records attributed to I Alfonso.

10 recordsLinked to original sources

Reversible dementia and apparent brain atrophy during valproate therapy.

Two children developed severe cognitive and behavioral deterioration suggestive of a degenerative disease while being treated with sodium valproate for idiopathic, localization-related epilepsy with centrotemporal spikes. Magnetic resonance imaging revealed marked central and generalized cortical and cerebellar atrophy. In both patients, clinical symptoms and signs cleared in a few weeks following valproate withdrawal. The magnetic resonance imaging appearance improved within 3 months in 1 of the patients and normalized in both after 6 and 12 months. No metabolic changes were associated with the clinical or imaging abnormalities. Although the mechanism of this rare idiosyncratic complication of valproate therapy is unknown, we advocate discontinuing valproate therapy in all epileptic patients with neuromental deterioration or brain atrophy of unknown etiology.

Atrophy

Cuban embargo.

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Commerce

Linear nevus sebaceous syndrome. A review.

We report a case of linear nevus sebaceous syndrome with large areas of calcification involving multiple layers of the posterior aspects of both eyes. This patient had a normal neurological examination despite dysplastic brain changes. We discuss and review the multiple clinical presentation and embryopathogenesis of this disorder.

Calcinosis

Spinal cord involvement in encephalocraniocutaneous lipomatosis.

Encephalocraniocutaneous lipomatosis is a rare hamartomatosis involving the craniofacial region and the central nervous system. The most prominent clinical features are large areas of scalp alopecia, soft subcutaneous craniofacial masses, lipomas, connective tissue nevi of the eyelids and surrounding areas, pterygium-like choriostoma of the ocular conjunctiva, mental retardation, motor deficit, and seizures. Of the eight patients reported previously, three had spinal cord evaluations and two had evidence of lipomatosis. We report the third patient with this association, review the literature of encephalocraniocutaneous lipomatosis, and stress the importance of spinal cord evaluation during the newborn period.

Adolescent