[Is benign childhood paroxysmal eye deviation a non-epileptic disorder?]. / Desviación ocular paroxística benigna infantil: un trastorno no epiléptico?

INTRODUCTION: Benign childhood paroxysmal eye deviation (BCPED) is classified as a 'non-epileptic paroxysmal disorder'. CASE REPORTS: We report the cases of four patients aged between 6 months and 2 years, who suffered brief episodes of upward conjugate gaze deviation, with no clonic movements or associated cognitive deterioration. These episodes, which lasted several seconds, appeared in short repeated bouts that became worse with fatigue. Results of the neurological exploration, laboratory examinations, neuroimaging (CAT, MRI, brain ultrasonography) and a neurophysiological study, which included EEG-video monitoring and EEG performed during the waking state, were all normal. A nocturnal polysomnographic study was later conducted for 7-8 hours and EEG, EMG and EOG readings were recorded. The trace showed focal or generalised paroxysmal discharges during non-REM sleep in the form of polyspike-wave and spike-wave complexes. Sleep analysis (Reschstaffen and Kales) showed only a shortened REM sleep latency, with no clear clinical meaning. Several cases have been reported in the literature with identical symptoms and normal results in the diagnostic tests, including daytime polysomnography. CONCLUSIONS: The appearance of these epileptic anomalies in the nocturnal study makes it necessary to perform a complete nocturnal polysomnography. In spite of these findings, BCPED courses favourably and has a benign prognosis both with and without antiepileptic treatment. We therefore believe that BCPED should be classed within the group of 'benign idiopathic epilepsies of childhood'.

Desviación ocular paroxística benigna infantil: ¿un trastorno no epiléptico? / Is benign childhood paroxysmal eye deviation a non-epileptic disorder?

Introducción. La desviación ocular paroxística benigna infantil (DOPBI) se clasifica como un 'trastorno paroxístico no epiléptico'. Casos clínicos. Presentamos cuatro pacientes de entre 6 meses y 2 años de edad, con breves episodios de desviación conjugada de la mirada hacia arriba, sin movimientos clónicos ni deterioro cognitivo asociado. Estos episodios, con una duración de varios segundos, aparecieron en salvas y se incrementaron con la fatiga. La exploración neurológica, los exámenes de laboratorio, neuroimagen (TAC, RM, ecografía cerebral) y estudio neurofisiológico, incluido registro vídeo-EEG y EEG durante la vigilia, fueron todos ellos normales. Posteriormente, se practicó estudio polisomnográfico nocturno durante 7-8 h, y se registró simultáneamente EEG, EMG y EOG. El trazado mostró descargas paroxísticas, focales o generalizadas, durante el sueño no REM en forma de complejos punta-onda y polipunta-onda. El análisis de sueño (Rechtschaffen y Kales) mostró únicamente una latencia de sueño REM acortada, sin un claro significado clínico. En la bibliografía se han descrito varios casos con síntomas idénticos y normalidad de las pruebas diagnósticas, incluida la polisomnografía diurna. Conclusiones. El hallazgo de estas anomalías epilépticas en el estudio nocturno exige la práctica de polisomnografía nocturna completa. A pesar de estos hallazgos, la DOPBI tiene un curso favorable y un pronóstico benigno con o sin tratamiento antiepiléptico. Por ello, creemos que la DOPBI debería incluirse en el capítulo de 'epilepsias benignas idiopáticas infantiles' (AU)

Introduction. Benign childhood paroxysmal eye deviation (BCPED) is classified as a ‘non-epileptic paroxysmal disorder’. Case reports. We report the cases of four patients aged between 6 months and 2 years, who suffered brief episodes of upward conjugate gaze deviation, with no clonic movements or associated cognitive deterioration. These episodes, which lasted several seconds, appeared in short repeated bouts that became worse with fatigue. Results of the neurological exploration, laboratory examinations, neuroimaging (CAT, MRI, brain ultrasonography) and a neurophysiological study, which included EEG-video monitoring and EEG performed during the waking state, were all normal. A nocturnal polysomnographic study was later conducted for 7-8 hours and EEG, EMG and EOG readings were recorded. The trace showed focal or generalised paroxysmal discharges during non-REM sleep in the form of polyspike-wave and spike-wave complexes. Sleep analysis (Rechtschaffen and Kales) showed only a shortened REM sleep latency, with no clear clinical meaning. Several cases have been reported in the literature with identical symptoms and normal results in the diagnostic tests, including daytime polysomnography. Conclusions. The appearance of these epileptic anomalies in the nocturnal study makes it necessary to perform a complete nocturnal polysomnography. In spite of these findings, BCPED courses favourably and has a benign prognosis both with and without antiepileptic treatment. We therefore believe that BCPED should be classed within the group of ‘benign idiopathic epilepsies of childhood (AU)