Un caso de la enfermedad de Hallervorden-Spatz.

Autor: Chavarna, Gonzalo Quezada, Cuevas, Scarlett Elizabeth Quezada, Perez, Gerardo Mercado, Barajas, Marisol Duran
Předmět:
Zdroj: Revista Mexicana de Pediatria; nov/dic2008, Vol. 75 Issue 6, p274-276, 3p, 1 Diagram
Abstrakt: Hallervorden-Spatz disease is a metabolic disorder rare autosomal recessive. Characteristic of this disease are pigmentation degeneration ofglobus pallid, substantia nigra and red nucleus. In 1992 went described for first time in 5 students that presented clinical manifestations of bradypsychia, dysarthria, athaxic walk, spasticity, tremors, dystonic (digestive and respiratory), mental deterioration and visual alteration. Diagnostic realize in base to image study, magnetic resonance is the best. In this study we can to watch hypotenses images to basal nucleus level with central hyperintensity and "eye of tiger" denominated, that indicate iron deposit and neuro-axonal degeneration. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index