Evolución atípica del síndrome de Panayiotopoulos a síndrome de punta-ondas continuas durante el sueño lento / Atypical evolution of Panayiotopoulos syndrome to continuous spikes and wave's syndrome during slow sleep

Introducción: Las epilepsias focales idiopáticas constituyen uno de los grupos de epilepsias más frecuentes en la infancia. Excepcionalmente los pacientes con este tipo de epilepsias tienen evoluciones atípicas que constituyen un reto diagnóstico y terapéutico. Objetivo: Ilustrar la evolución atípica de la epilepsia focal idiopática tipo Panayiotopoulos. Presentación del caso: Adolescente de 13 años que presentó su primera crisis epiléptica a los 5 años de edad, de breve duración, mientras dormía tuvo apertura ocular, desviación de los ojos a la izquierda, abundante salivación y presentó un vómito. En tres años tuvo solo tres crisis. No recibió tratamiento con fármacos antiepilépticos hasta después de la tercera crisis, que fue más prolongada. Tras iniciar tratamiento con carbamazepina comenzó a presentar dificultades en el aprendizaje y marcada hiperactividad. Un electroencefalograma interictal de sueño demostró descargas de punta-ondas continuas en el sueño lento. Después de dos años de tratamiento se alcanzó la normalidad en el estudio electroencefalográfico de sueño, con retirada inicial de la carbamazepina, e introducción progresiva de clobazam y valproato de magnesio. Evolutivamente el paciente mantuvo las dificultades en el aprendizaje, con mejoría notable de su hiperactividad, sin recurrencia de crisis epilépticas. Conclusiones: El caso presentado constituye un ejemplo infrecuente de un paciente con una epilepsia focal idiopática con evolución atípica, probablemente inducida por la carbamazepina, con cuadro clínico-electroencefalográfico de más de dos años de duración, con mejoría favorecida por el tratamiento finalmente empleado, la evolución natural del síndrome o el efecto de ambos (AU)

Introduction: Idiopathic focal epilepsies are one of the most frequent epilepsy groups in childhood. Exceptionally, patients with this type of epilepsy have atypical evolutions that constitute a diagnostic and therapeutic challenge. Objective: To illustrate the atypical evolution of idiopathic focal epilepsy, type Panayiotopoulos. Case presentation: A 13-year-old adolescent who presented his first epileptic seizure at 5 years old, of short duration, while he slept had an eye opening, deviation of the eyes to the left, abundant salivation and vomiting. In three years he had only three seizures. He did not receive treatment with antiepileptic drugs until after the third seizure, which was longer. After starting treatment with carbamazepine, he began to have learning difficulties and marked hyperactivity. A sleep's interictal electroencephalogram showed continuous spikes and wave's discharges during the slow sleep. After two years of treatment, the normalization of the sleep electroencephalogram was achieved, with withdrawal of carbamazepine, and progressive introduction of clobazam and magnesium valproate. The patient remained evolutionarily with learning difficulties, with significant improvement in hyperactivity, without recurrence of seizures. Conclusions: The case presented is an infrequent example of a patient with idiopathic focal epilepsy with atypical evolution, probably induced by carbamazepine, with clinical-electroencephalographic symptoms during more than two years, with improvement favored by the final treatment used, the natural evolution of the syndrome or the effect of both(AU)

Panayiotopoulos syndrome: a clinico-electroencephalographic presentation of three cases from Nigeria

Panayiotopoulos syndrome (PS) is a common benign, childhood, focal, seizure susceptibility syndrome presenting with mainly focal aware or focal impaired awareness autonomic seizures and autonomic status epilepticus that is under diagnosed in our locality. Increased awareness through reporting will aid diagnosis, reduce misdiagnosis and prevent aggressive and deleterious interventions. The classic clinical and electroencephalographic (EEG) features of three cases are described and the literature reviewed. The aim is to underscore the presence in our locality of this remarkably benign epileptic syndrome that must be differentiated from febrile seizures, encephalopathy, migraine or cardiogenic syncope.

Childhood Idiopathic Occipital Lobe Epilepsy: Clinical Characteristics and Prognostic Factors / 대한소아신경학회지

PURPOSE: Among syndromes of idiopathic occipital lobe epilepsy there are Panayiotopoulos syndrome (PS) and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G). However, the classification of epilepsy syndrome is difficult to diagnose at the first seizure because of varying characteristics. We analyzed the different clinical characteristics and prognostic factors of idiopathic occipital lobe epilepsy. METHODS: Forty-three patients who had at least two unprovoked seizures, no abnormalities on magnetic resonance imaging, no known etiology of seizure, and mainly occipital spikes on electroencephalography were retrospectively enrolled at the Korea University Medical Center. A good prognosis was defined as being seizure-free and taking two or less drugs for 1 year. RESULTS: Among all patients, the proportion of PS was 44.2%, ICOE-G was 7.0% and unclassified group was 48.8%. The age at seizure onset was 4.5±2.6 years (mean±SD) in the PS group, and 8.3±2.1 years in the ICOE-G group. The follow-up duration was 10.8±6.0 years. The percentage that the initial diagnosis had not been changed was 66.7% in the PS group and 100% in the ICOE-G group. Among other related symptoms only emesis (P<0.001) and visual symptoms (P=0.007) had varying characteristics between the PS and ICOE-G groups. Patients with PS had a better prognosis than those with ICOE-G or unclassified group (odds ratio [OR]=58.8). Patients with 1 more autonomic symptoms had a worse prognosis (OR=3.8). CONCLUSION: This study showed that only symptoms of emesis and visual symptoms can differentiate between PS and ICOE-G. More autonomic symptoms were associated with worse prognosis in childhood occipital lobe epilepsy.

Cyclical Vomiting Syndrome with Bilateral Epileptiform Discharges.

Cyclical vomiting syndrome is a paroxysmal, condition characterized by recurrent severe episodes of vomiting lasting for hours to days, with variable intervals of normal health in between with no apparent cause of the vomiting. We hereby report a 10yr old girl with cyclical vomiting syndrome with multiple, bisynchronous occipitally predominant, bilateral epileptiform discharges representing a diagnostic confusion.

Clinical Considerations of Panayiotopoulos Syndrome / 대한소아신경학회지

PURPOSE: Panayiotopoulos syndrome(PS) is a benign childhood focal seizure disorder characterized by seizures with emetic symptoms and other autonomic phenomena, and its EEG shows shifting and/or multiple foci, often with occipital predominance. More than 800 cases have been reported worldwide, but no case has been reported in Korea. This paper describes the characteristics of the patients that satisfy the diagnostic criteria of PS and gives its clinical considerations. METHODS: A total of 140 children with non-febrile seizures visited Bundang Cha General Hospital from June 2004 to June 2005. Among them seven patients satisfied the diagnostic criteria of PS. We analyzed their clinical findings and EEG results retrospectively. RESULTS: Boys predominated. The mean age at the first seizures was 6.21 years old. Nearly 57% of the cases had at least one seizure lasting more than 30 minutes. Seizures during sleep were more common than those in wakefulness. Seizures started with emesis while the children were usually fully conscious. Other conventional seizure manifestations such as loss of consciousness, eyeball deviation, and convulsions often ensued. The EEG records showed occipital spikes in four patients. Of the other three cases, two had extraoccipital spikes and one had a normal EEG. CONCLUSION: PS seems to be commonly observed in Korea, although it remains largely unknown in Korea. Since PS, in its early phases, is often indistinguishable from several serious diseases, a good knowledge of the condition can prevent unnecessary examinations and excessive treatment. Further studies are required to establish systematic practice parameter guidelines.