Intravenous immunoglobulin G as adjuvant treatment in drug-resistant childhood epilepsy. / Inmunoglobulina G intravenosa como tratamiento adyuvante en epilepsia infantil farmacorresistente.

BACKGROUND: Epilepsy is the most common neurological disease in childhood; depending on the definition of drug-resistant epilepsy, incidence varies from 10% to 23% in the paediatric population. The objective of this study was to account for the decrease in the frequency and/or monthly duration of epileptic seizures in paediatric patients with drug-resistant epilepsy treated with antiepileptic drugs, before and after adding intravenous immunoglobulin G (iIV IgG). METHODS: This is an analytic, observational, retrospective case-control study. We studied paediatric patients with drug-resistant epilepsy who were treated with IV IgG at the Centro Médico Nacional 20 de Noviembre, in Mexico City, from 2003 to 2013. RESULTS: One hundred and sixty seven patients (19.5%) had drug-resistant epilepsy and 44 (5.1%) started adjuvant treatment with IV IgG. The mean age of patients at the beginning of treatment was 6.12 years±5.14); aetiology was structural acquired in 28 patients (73.6%), genetic in 5 (13.1%), immune in 1 (2.6%), and unknown in 4 (10.5%). At 2 months from starting IV IgG, seizure duration had reduced to 66.66%; the frequency of seizures was reduced by 64% at 4 months after starting treatment (P<.001). CONCLUSIONS: According to the results of this study, intravenous immunoglobulin may be an effective therapy for reducing the frequency and duration of seizures in paediatric patients with drug-resistant epilepsy.

[West syndrome as an epileptic presentation in Menkes' disease. Two cases report]. / Síndrome de West como manifestación epiléptica de enfermedad de Menkes. Presentación de dos casos.

INTRODUCTION: Menkes' disease is a neurodegenerative disorder, recessive X chromosome linked (Xp13.3) that normally codify an ATPasa copper transporter. CASE REPORTS: Case 1: patient exhibit failure in the gastrointestinal copper absorption, which is insufficient to cover the needing during the first twelve months of life. The first case was a 5 months male. His developmental skills were normal until he was 5 months old, when he exhibited visual impairment and failure to continue getting normal developmental skills. One month later he had infantile spasms and hypsarrhythmia in the EEG. He had kinky hair, alopecia zones and copper serum level in 0 microg/dL (range 590-1,180 microg/dL) brain CT scan revealed diffuse cortical atrophy. The patient is 5 years old now, he is free of seizures but he has a severe neurological impairment. Case 2: he is a 7 months old male who developed during the two days of life hypotonia and weak suction. He exhibited later hypertonia, delayed neurological development and infantile spasms, microcephaly, kinky hair, blindness and EEG pattern of hypsarrhythmia. The serum copper level was 84 microg/dL (range: 590-1,180 microg/dL). The brain CT scan showed generalized atrophy, including cerebellum, extradural effusion and MRI with multiple infarcts in different stages. Electronic microscopy revealed pili torti. In both cases the diagnosis was suspected because of the hair and eyebrow features. CONCLUSIONS: We suggest a careful hair and eyebrow clinical exam in those patients with delayed milestones and early epilepsy without a documented etiology, and the copper serum level determination in those patients with suspected disease.

[A follow-up during the first year of life of newborn infants with very low weight at birth]. / Seguimiento durante el primer año de vida, de neonatos con muy bajo peso al nacer.

Groups of low-weight neonates, especially those under 1,500 g, discharged from the Intensive Care Unit, have a greater frequency of having psychoneurologic, audiologic, language and ophthalmologic scars in comparison with those neonates who suffered no complications. These frequencies vary considerably depending on different authors, the type of sequela and the duration of the follow-up. It is difficult to identify early on, which of these children will be affected in his later development. The currently used prognostic indexes have shown the importance of combining the child's psychoneurologic and biologic evolution as well as his surrounding environmental factors. Thirty-eight newborns were studied. Each weighted 1,500 g or less and were discharged from the Neonatal Intensive Care Unit. All of the patients were subsequently evaluated by specialists in Neurology, Psychology, Social Communication, Neuromotor Rehabilitation and Ophthalmology at 3, 6, 9 and 12 months of age (37 weeks) and included in a study conducted by the Program for Pediatric Follow-up at the National Institute of Perinatology. In general, the number of abnormalities found in each of the specialties during the first months of life was notorious, which later decreased as the child got older. For example, Neurology found abnormalities at 3 months in a proportion of 0.61 and at 12 months, it decreased to 0.19 (P less than 0.001). During neuromotor stimulation something similar was seen at three months a proportion of 0.39 abnormalities were recorded while at 12 months this decreased to 0.18. The human communication tests showed a proportion of 0.32 at 3 months and 0.10 at 12 months (P less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

[Hearing and language sequelae in survivors of a neonatal intensive care unit]. / Secuelas de audición y lenguaje en sobrevivientes de una Unidad de Cuidados Intensivos Neonatales.

INTRODUCTION: The purpose of this study is to record the hearing and language sequelae in a sample of children from the Intensive Care Unit between the ages of two and three. MATERIALS AND METHODS: Forty-one children were studied after being submitted to neuropsychological, hearing and language tests. Both normal and abnormal parameters for each test allowed the categorization of the children. RESULTS: In the general sample there were 24 full-term children versus 17 preterm children. The neurological testing showed a tendency towards normality. The same was seen in language testing since the proportion of normality corresponded to those children born after a full term. There were practically no differences in those suspected from both groups and a greater percentage of abnormal children were found among the pre-term infants. From an audiological standpoint there was a predominance of normal children; there was only one patient with severe bilateral hypoacusis who needed an electric auxiliary hearing device and two other patients with peripheral ear dysfunction classified as serous middle ear otitis. CONCLUSIONS: The incidence of hypoacusis in this sampling type is similar to that reported in the literature. Those patients with a history of assisted mechanical ventilation have been later found to have, as a sequelae, serous middle ear otitis, which corresponds to that reported by Paradise. The development of language stages were altered more so in preterm children. In some patients, the lack of stimulation associated with a low socioeconomic back group favors the delay in the development of language skills.

[The prone position as an indicator of deviated motor development during the first year of life]. / La posición prona como indicador de un desarrollo motor desviado en el primer año de la vida.

The adequate control and alignment of a baby in the prone position during the first year of life is a basic necessary milestone for a normal, neuromotor development. In this study the prone position was registered in 246 babies with a previous pediatric evaluation, three options could be made: normal retarded or altered. The Milani Comparetti neuromotor evaluation was also realized for each baby at the same time with the same classification options. Both results were compared. A sensitivity and specificity = 1.0 were found for the prone position registered. This test is fast and simple, so it is recommended at a first attention level in high risk population for neuromotor disability.

[The Apgar score as a predictor of neurologic sequellae]. / El puntaje de Apgar como predictor de secuelas neurológicas.

65 newborns with Apgar score less than 7 were studied. Initially 2 groups were formed. Group 1 with severe asphyxia Apgar 0-3 and group 2 with moderate asphyxia Apgar 4-7. Each group was divided according to Apgar score at 5 minutes; with good recuperation Apgar greater than or equal to 6 and bad recuperation Apgar less than 6. So four subgroups were formed. All patients were evaluated with Amiel Tison neurological examination at 12 months old. Of the 65 newborns, 52 (80%) had a normal neurologic examination, and only 12 (20%) were not normal. There were not significant statistic differences neither between the moderate and severely asphyxiated groups nor between the four subgroups at 5 minutes and their neurological examination. We conclude that the Apgar score at 5 minutes is not a good predictive of neurological sequelae, because there is a myriad of factors difficult to investigate in relation to the etiology of neurologic sequelae. We stress the importance of reviewing the original idea about the Apgar score as a useful method (instrument) to evaluate the hemodynamic and homeostatic conditions of the newborns.

[Neurologic follow-up of polycythemic newborn infants]. / Seguimiento neurológico de neonatos policitémicos.

Seventy five newborn infants with neonatal polycythemia diagnosis (venous hematocrit levels greater than 65% were studied). They were born in the Instituto Nacional de Perinatologia and follow through their first year of life searching for neurologic findings. The more frequent signs related to polycythemia in this group were: presence of plethora, respiratory distress, cyanosis and tremors; the associated findings were hypoglycemia, hyperbilirubinemia, necrotizing enterocolitis and meconium aspiratum. Sixty eight of the seventy five neonates developed symptomatology and seven didn't. The eight neonates who developed neurologic transient impairments belonged to the symptomatic group in a 0.11 proportion. We concluded that the number of babies with neurologic findings in our sample was small in comparison with other reference studies; we also found that the exchange transfusion is not a preventive measure for neurologic sequel, but it is useful to diminish the symptomatology caused by polycythemia; besides we don't recommend this procedure in asymptomatic babies because the complication risks as the enterocolitis. We propose a simple treatment scheme.