[Neonatal sciatic palsy: etiology and outcome of 21 cases]. / Parálisis ciática neonatal: etiología y seguimiento a propósito de 21 casos.

INTRODUCTION: Sciatic nerve paralysis is a rare entity in the newborn. Few reference in specialized tests indicate that in the majority of cases the sciatic palsy has been observed after misplaced injections into the buttocks. The prognosis is variable and appears to be better after umbilical vessel catheterization for injection of medications than after misplaced muscular injections. In case of recovery it takes place within 3 to 12 months. OBJECTIVE: The objective of the present study is to know the evolution of neonatal sciatic palsy and to determine their injury noxe in regard to perinatal factors, and their relationship with long-time outcome, and to look for prognostic clues of clinical utility. MATERIAL AND METHODS: We evaluated perinatal factors of newborn children with sciatic nerve paralysis, followed for more than 18 months of clinical evolution, in a neuropediatric centre. RESULTS: Twenty one newborn with such criteria were evaluated. Gestational age was within 32 and 42 weeks (median 38.2). The birth weight was between 2,100 and 4,100 g (median 2,973). The majority of cases obtained total recovery (16 of 21). The time of recovery was 4 to 14 months (median 8.8). Free ambulation was obtained by all cases (at 10 to 24 months). No apparent cause was observed in the majority of cases. Cesarean delivery was more frequent specially in cases with permanent consequences. CONCLUSION: Long-time prognostic of neonatal sciatic palsy is generally good. In our series all the cases with consequences were associated to cesarean delivery. The duration of cesarean intervention and the anesthesic hypotony of the newborn could be implicated in the sciatic nerve injury of poor outcome.

[Rett's syndrome: study of 15 cases]. / Síndrome de Rett: estudio de 15 casos.

A multicentric study of 15 cases of Rett syndrome selected with the diagnostic criteria according HAG-BERG et al: female sex, normal pre and perinatal period, normal psychomotor development through the first months of life, early dementia between 1-3 years of age with autistic behaviour, loss of acquired purposeful hand skill, "washing hands" stereotypies, normal head circumference at birth with later deceleration of head growth and truncal ataxia with gait apraxia. Waking EEG showed unspecific abnormalities while sleep recording demonstrated extremely frequent multifocal spike and sharp waves mainly over the rolandic region and generalized, and also pseudo-periodic suppression of background activity. In 3 cases the EMG showed a peripheral axonal neuropathy. Only in one case we found hyperammonemia. Karyotypic studies performed in 12 cases demonstrated non specific fragile sites. CT scan was normal in almost all cases. The QD was extremely low.

[Continuous oral phenobarbital or intermittent rectal diazepam for the prevention of febrile convulsions]. / Fenobarbital oral continuado o diazepam rectal intermitente para la prevención de las crisis febriles.

During 18 months 100 children were submitted to a longitudinal study, in order to compare the efficacy of treatment with administration of intermittent rectal diazepam versus oral BID phenobarbital therapy, for prevention of febrile seizures. The group was divide in a randomized trial. Rectal diazepam was administered at dose os 0.5 mg/kg dose and the dose was repeated every eight hours during the period of fever. Diazepam was found to be as effective as oral phenobarbital in the prevention of new febrile seizures.

[Hyperinsulinism probably caused by diffuse pancreatic hyperplasia]. / Hiperinsulinismo por probable hiperplasia difusa pancreática.

A case history of a two years seven months old boy with hyperinsulinism and marked psychomotor retardation is presented. In this patient, biochemical analysis revealed persistent hypoglycemia. Basal insulin levels (RIA) were consistently superior to 10 mU/ml. and were higher than would be expected from the child's glycemias. Angiographic study of the celiac trunk revealed diffuse pancreatic enlargement. Therapy was initiated with oral diazoxide normalizing and stabilizing the patient's glycemia. The importance of an early diagnosis of this condition and the necessary biochemical analyses involved are discussed as well as the use of diazoxide in the medical treatment of hypoglycemia.