[Dysgenesis of the anterior horns and nuclei of the brain stem in multiple congenital arthrogryposis. Presentation of a case].
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Biomedical subjects
Publications and source records attributed to J Torres Mohedas.
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Captopril, an orally active angiotensin I-converting inhibitor, is an effective drug in the treatment of hypertension in adults and children. The use in newborn infants has been, nevertheless, short. We report five cases of hypertension in newborn infants poorly responsive to large doses of other potent antihypertensive agents: furosemide, propanolol and hydralazine. We have got a reduction in mean blood pressure in three cases, with doses of 0.1-0.2 mg/kg/day for a time lesser than a month. Another patient had a reduction with high doses (5 mg/kg), showing increase in mean serum urea, creatinin, potassium and phosphorus levels. This effect disappeared after captopril was discontinued. We suggest the use of captopril in hypertension non-responsive to other antihypertensive agents, mainly in those of renovascular cause.
Clinical features and outcome of 15 children with Guillain-Barré syndrome were retrospectively analyzed. In 53% of the patients there was an infectious illness previous to the neurologic symptoms and in five children the etiologic agent was demonstrated. Motor deficit affecting limbs was the most common clinical presentation and facilal paralysis was the most frequent accompanying sign. One of these children developed the Fisher syndrome variant. The outcome was excellent except in one case. We want to remark from this serie high percentage of agents identified in the infectious process previous to Guillain-Barré syndrome and the good outcome of these patients, due to the supportive treatment in the pediatric intensive care unit where they were admitted.
Two cases of benign intracraneal hypertension or pseudotumor cerebri, associated with non specific infective illness, in infants are reported. Presenting symptoms were vomiting, irritability and bulging anterior fontanelle. Both infants had rapid resolution of their symptoms after lumbar puncture, although the second case had recurrences with further febrile diseases. We suggest that a non specific infective illness can interfere transiently with cerebrospinal fluid dynamics.
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In this publish we analyze etiologic, clinic, electroencephalografic, radiologic, therapeutic and evolutionary facts of a serie of 31 children with diagnostic of West's syndrome. Advances in diagnostic methodes, overall of neuroimagen have allowed us to identify the etiology of 87% of cases, with a great variability of causes. The treatment with ACTH and/or anticonvulsive drugs was effective; although for children's prognostic, etiology and previous neurologic situation before initial symptomatology was more important. So, the clinical evolution was favorable for all criptogenetic cases and those secondary one with previous normal neurologic state. The rest, 70%, remained with mental delay, associated or no with crisis. Likewise the EEG persisted to be pathologic in 67.7% of cases. Five cases progressed towards Lennox-Gastaut's syndrome, that is to say, 16% of whole.