[Electroencephalographic discoveries in children with infantile massive spasms in Mexico]. / Hallazgos electroencefalográficos en niños con espasmo masivo infantil en México.

INTRODUCTION: Infantile massive spasms (IMS) is an age dependent epileptic syndrome that appears before the first year, and is characterised by delayed psychomotor development, massive spasms and hypsarrhythmia. There are classifications that only take the electroencephalogram (EEG) into account, without linking it with the IMS associated crisis pattern which can establish a treatment to improve the recurrence of the crises in this population, according to EEG discoveries and patterns of epileptic fits. PATIENTS AND METHODS: We include 100 EEGs of patients diagnosed with IMS, between 2 and 12 months old, selected by using the classification of Hrachovy to identify classic and modified hypsarrhythmia, and to correlate it with the pattern of epileptic seizures associated with IMS. RESULTS: The hypsarrhythmia found was mainly of the modified variety, and classic hypsarrhythmia only accounted for 9% of cases. The first case presented flexion IMS with generalised tonic seizures, and in the second, generalised tonic seizures and mixed IMS. Start age was between 2 and 4 months old. CONCLUSIONS: In comparison with results in other publications, in the EEG it was found that modified hypsarrhythmia with IMS associated partial and generalised tonic seizures with was the most common, while in the classic variety mixed IMS predominated. It must be taken into account that the absence of this variety does not rule out a diagnosis of IMS, but its presence makes the prognosis worse.

Hallazgos electroencefalográficos en niños con espasmo masivo infantil en México / Electroencephalographic discoveries in children with infantile massive spasms in mexico

Introducción. El espasmo masivo infantil (EMI) es un síndrome epiléptico edad dependiente antes del año, caracterizado por retraso en el desarrollo psicomotor, espasmos masivos e hipsarritmia. Existen clasificaciones que incluyen sólo el electroencefalograma (EEG), sin correlacionar con patrón de crisis asociado a EMI, que pueda establecer un tratamiento que mejore la recurrencia de las crisis en esta población, de acuerdo a los hallazgos de EEG y patrones de crisis epilépticas. Pacientes y métodos. Incluimos 100 EEG de pacientes con diagnóstico de EMI, entre los 2 y 12 meses, seleccionados con la clasificación de Hrachovy, para identificar la hipsarritmia clásica y la modificada, y correlacionarlas con el patrón de crisis epilépticas asociadas a EMI. Resultados. La variante de hipsarritmia encontrada fue la modificada y sólo en el 9 por ciento se encontró la clásica. En el primer caso, presentó EMI en flexión con crisis tónicas generalizadas, y, en el segundo, fueron las tónicas generalizadas y EMI mixtos. La edad de inicio fue de 2-4 meses de edad. Conclusiones. Comparados los resultados obtenidos en otras publicaciones, en el EEG fue más común la hipsarritmia modificada, con crisis parciales y tónicas generalizadas asociadas a EMI, y en la clásica, EMI mixta; se debe considerar que la falta de esta variante no descarta el diagnóstico de EMI, pero la presencia empeora el pronóstico (AU)

[Neurologic disorders in children with chronic terminal renal insufficiency]. / Alteraciones neurológicas en niños con insuficiencia renal crónica terminal.

Neurological alterations are very frequent in children with end stage renal disease (ESRD). In order to assess the type of these complications, a neurological study was undertaken in 30 children with ESRD. Three of the patients had convulsions and hyperreflexia was the only clinical sign found in all the patients. Low serum levels of hemoglobin and calcium and high concentrations of phosphates, aluminum and parathyroid hormone were found in all cases, but correlations with any neurological alteration were not made. Abnormal EEG with slow waves in the occipital region were obtained in 23 patients. Sensorial hypoacusia was registered in 6 children who were previously treated with aminoglucosides. The cerebral tomography showed calcifications in three instances, but only in one patient were of clinical significance. In five, out of the 30 patients, cerebral atrophy was detected. In conclusion, assessment of the neurological condition is mandatory in children with ESRD before renal transplantation is performed, since the complication itself or the therapy may interfere with rehabilitation.

Rett's syndrome with Lennox-Gastaut pattern.

Three Latin American female patients studied in two different centers (Mexico City and Houston, Texas) had the clinical criteria of Rett's Syndrome, including progressive intellectual deterioration, stereotyped movements, loss of purposeful hand use, ataxia, and progressive disability. The three patients had normal laboratory studies including imaging studies (CT and MRI) but consistently abnormal EEGs with Lennox-Gastaut pattern. The usefulness of the EEG in the differential diagnosis and the association of this electrographic phenomenon are stressed.

Valproic acid in the treatment of epilepsy during the pediatric age.

We studied 85 pediatric patients with different types of epilepsy treated with valproic acid. The clinical and EEG follow-up were performed up to 10 months. Our EEG-clinical observations in this study show that this group of epilepsies during the pediatric age are extremely sensitive to valproic acid with minimal and tolerable side effects. Within a brief period of time an effective response was obtained in the case of epilepsy with myoclonicastatic seizures juvenile myoclonic epilepsy (impulsive petit mal), classical absence and photosensitive epilepsy.

Neonatal status epilepticus II: Electroencephalographic aspects.

A prospective study of 15 newborns were identified with NSE. In 11 cases the findings were electrical only without clinical manifestations (ENSE), and the other four presented simultaneously clinical seizures and electrical discharges (ECNSE).

Neonatal status epilepticus I: Clinical aspects.

Fifteen newborns with neonatal status epilepticus were prospectively identified from a protocol of convulsion in the newborn, at the neonatal intensive care unit. The mean postnatal age was 7.1 days, with fourteen full term babies and one premature. Previous complications during childbirth existed in twelve infants. The principal etiology associated was hypoxic-ischemic-encephalopathy, and four patients died. In thirteen cases the antecedent of subtle seizures existed; only in four cases was status epilepticus suspected. Difficulty in clinical recognition makes it necessary to perform an EEG on each newborn with risk factors, abnormal neurological manifestations, or with prolonged and or repeated hypoxic perinatal antecedents.

West syndrome evolving into the Lennox-Gastaut syndrome.

Eight cases of WS developed electroencephalographic characteristics of the LGS in early childhood. The importance of EEG and clinical followups in patients with the WS for early detection of other associated forms of epilepsy, particularly the LGS, is therefore emphasized.

Estado de mal epileptico en el recien nacido. / Status epilepticus in the newborn infant

Se analiza el estudio prospectivo de 15 recien nacidos en quienes se identifico estado de mal epileptico. La etiologia mas frecuente fue la encefalopatia hipoxica; el antecedente de crisis tonicas y la forma sutil fueron las manifestaciones que se presentaron con mayor frecuencia. En cuanto al registro electroencefalografico, en 11 casos las descargas ocurrieron durante su primer estudio sin manifestaciones clinicas (estado de mal epileptico electrico) y en cuatro casos se presentaron simultaneamente descargas electroencefalograficas y crisis clinicas (estado de mal neonatal epileptico electrocilnico). En este trabajo tambien se informa de la respuesta al tratamiento y se enfatiza en las ventajas del control continuo terapeutico y electroencefalografico

Piretoterapia como tratamiento en el sindrome de West. / Pyrethotherapy as treatment of West syndrome

Se presentan cuatro casos de sindrome de West (espasmos mioclonicos generalizados, detencion del desarrollo psicomotor e hipsarritmia), considerados resistentes al tratamiento tradicional (ACTH, nitrazepan, clonazepan, etc.); en los que se empleo fiebre alta (piretoterapia), producida con vacuna TBA, (tifica y paratifica), para el control del mismo. Dentro de los ocho dias subsiguientes al tratamiento con piretoterapia, las condiciones clinicas de estos ninos se reportaron con una disminucion progresiva de los espasmos mioclonicos generalizados y mejoria de su estado mental y desarrollo neurologico. Los electroencefalogramas de control senalaron desaparicion del trazo de hipsarritmia. Despues del analisis de los casos y su correlacion electroclinica, se sugiere que la piretoterapia sea un procedimiento util en esta entidad de epilepsia tan invalidante

Diplejia espastica familiar y arginemia. / Familial spastic diplegia and arginemia

Se comunica la observacion de dos hermanos portadores de una enfermedad neurologica degenerativa con caracter autosomico recesivo; los datos clinicos predominantes en ambos se expresan por diplejia espastica, amiotrofia y retroceso de funciones intelectuales; en los dos casos se encontro ausencia de arginasa en eritrocitos y aumento de arginemia en suero. Los hallazgos clinicos en estos ninos, muestran dos fases de la evolucion de la enfermedad y los de laboratorio, confirman arginemia, senalando que se trata de una variedad de diplejia espastica familiar y arginemia y se suman a los diez casos previamente descritos en la literatura mundial