[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.

[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.

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