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The incidence of cerebral stroke in children has significantly increased in last years. Heart diseases are one of the best known risk factors in pediatric stroke. AIM. To describe the characteristics of the cerebral stroke in children with heart diseases, emphasizing in predisposing conditions, time to diagnosis, management and follow-up of patients.We performed a retrospective study in children suffering from heart diseases with cerebral stroke admitted to our hospital that comprised 10 years. Type of cardiopathy, associated factors like surgery or catheterization, study of thrombophilia, clinical findings, methods and time to diagnosis, primary and secondary prophylaxis, treatment and long-term neurologic outcome were analyzed.Twenty patients were included in our study (age: from neonatal period to 15 years), eighteen of them were children with congenital cardiac disease. Eleven patients had antecedent of surgery and/or catheterism in the previous month. The most common clinical presentation was focal seizures. Time from clinical onset to diagnosis of stroke was longer than 24 hours in 60% of our patients. Fifty per cent of our patients did not receive any primary prophylaxis. Treatment was initiated in 70% of patients, but no one received thrombolysis.It is crucial to consider stroke when children with heart diseases show any neurologic symptom. Optimal diagnostic strategies must be established to low the delay of diagnosis in these patients, as well as randomised clinical trials in order to establish uniform guidelines in pediatric stroke.

To report one patient with slowly progressive encephalopathy, ataxia, central hypomyelination, hypodontia and hypogonadotropic hypogonadism, the 4H syndrome. This clinical picture has been described recently and there are only four patients reported previously.A girl with a previously normal early psychomotor development, presented a slowly progressive deterioration since 15 months of age. Now, she is 14 years old, and has a severe cerebellar ataxia, with tremor and dysmetria. She can't neither walk nor remain standing alone. She has lost the sphincter control and has an immature expressive language. She has no puberal development and definitive hypodontia of upper central incisors. The brain magnetic resonance imaging shows a diffuse hypomyelination, that is confirmed with diffusion and spectroscopy studies.The hypomyelinating leukoencephalopathies are disorders with abnormally low amount of myelin. The diagnosis is difficult in most of the patients. The hypomyelinating leukoencephalopathies include classic disorders and new leukoencephalopathies, described in the past few years.