Resumen

RAMIREZ BENITEZ, Yaser  y  MARCHENA MORERA, Hussimy. Neuropsychological disorders related to congenital adrenal hyperplasia. Rev Cubana Endocrinol [online]. 2008, vol.19, n.3. ISSN 1561-2953.

Congenital adrenal hyperplasia includes a series of genetic diseases of autosomal recessive transmission characterized by a disorder of suprarenal esteroidogenesis. This is a typical case of brain damage by pathologic effect of sexual hormones in developing nervous system, and also, are the secondary sequelae to severe situation of adrenal crises appearing in a classic way in the first days of life. METHODS: a study-case of a child aged 6 presenting with backgrounds of congenital adrenal hyperplasia and salt-losing syndrome. We determine weak and strong points in develop by means of the Luria Inicial neuropsychological battery. As a complement to valuation we applied the Weschler Intelligence Scale and the Infantile Behavior Questionnaire for parents and professors. RESULTS: child presenting with a neuropsychological profile characterized by low scores in: manual motility, visual-spatial, impulsiveness, a poor language, difficulty in operations of simple calculations, in recognition of objects by touch with visual help, and in processing speed. Ability of intelligence is within normal limits with the lower performance in non-verbal scale. In behavior scale, it was possible to identify difficulties to attention and learning. CONCLUSION: neuropsychological profile is characterized by a low level of executions in verbal tasks, non-verbal, and in processing speed. Hormonal treatment during first days of life allows that development follows its "normal" course, but all process has a cost reflected in acquisition of higher psychological abilities

Palabras clave : Congenital adrenal hyperplasia; neuropsychology; learning.