[Clinical and prognostic heterogeneity in Aicardi's syndrome: a description of two cases].

Introduction: Aicardi's syndrome is characterized by infantile spasms, agenesis of the corpus callosum and ocular lesions. Clinically it presents as severe mental retardation, severe limitation of motor development and of language, with a prognosis of survival for only a few months or years. We present two new cases of this uncommon syndrome and describe the heterogeneity of its clinical and prognostic severity.

Variable clinical and biochemical presentation of seven Spanish cases with glutaryl-CoA-dehydrogenase deficiency.

In this report, we describe seven new patients with a severe deficiency of glutaryl-CoA dehydrogenase in cultured skin fibroblasts. Three of the patients studied excreted high levels of glutaric acid. The remaining four patients presented a lack of significant glutaric aciduria.

[Wallenberg syndrome in childhood secondary to the striking by lightening].

Wallenberg's Syndrome (WS) is exceptional in childhood. We present a case of a 10 year-old girl with a WS caused by an embolism in the right lateral portion of the medulla secondary to an electrofulguration, but with an unusual progressive outcome, developing clinical signs of medial medullar injury, leading even into a respiratory failure 30 days after admittance. She only became stable after surgical lesion decompression.