Summary:Purpose: Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified.Methods: This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting.Results: One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3); 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixationoff sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year.Conclusions: Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group. Key Words: Benign epilepsy-Partial seizures-Occipital seizures-Aversive seizures-Status epilepticus.The syndromic approach to the epilepsies has been a major nosologic advance (1,2). In children with and without seizures, occipital spikes that disappear with age were noted by Gibbs et al. more common form of benign CEOP occurring in younger children with infrequent, predominantly nocturnal seizures manifested by tonic deviation of the eyes and vomiting and often first seen as partial motor status epilepticus. Despite similar accounts by others (16-23), recognition of this early-onset variant of CEOP has been delayed. Furthermore, reservations have been expressed about the benign character of CEOP after reports that EEG occipital abnormalities related to the eyes-closed state may also occur in lesional epilepsies (24-26), in the visually impaired (27), and in normal children (28).The purpose of this report is to document fully the clinical features of occipital childhood epilepsy characterised by ictal tonic deviation of the eyes and vomiting, rather than with ictal visual hallucinations, to determine whether these are sufficiently distinctive to warrant classification separate from CEOP. METHODThe study was a collaborative project: Participating investigators collected details of patients with idiopathicoccipital seizures seen over the last 10-to 20-year period. Inclusion criteria were the occurrence of occipital seizures characterise...

Absence status (AS) is a heterogenous epileptic syndrome that can occur at any age, usually in a context of prior epilepsy. Eleven cases of AS occurring in middle-aged patients who had no history of epilepsy were retrospectively collected over a 10-year period (10 women and one man; mean age, 58.6 years). Eight patients were receiving high doses of psychotropic drugs. Clinical and EEG presentation was similar to AS occurring in patients with prior epilepsy. Evaluation of precipitating factors revealed that AS coincided with benzodiazepine withdrawal in eight cases. Cofactors included excessive use of other psychotropic drugs, nonpsychotropic treatment, hypocalcemia, hyponatremia, and chronic alcoholism. CT demonstrated mild cerebral atrophy in six cases. There was no recurrence, even without chronic antiepileptic treatment. These data indicate that (1) most cases of "de novo" AS of middle age or late onset result from the addition of various epileptogenic factors; (2) AS can be considered a new and uncommon complication of benzodiazepine withdrawal, and (3) long-term administration of anticonvulsant medication may not be required.

Sodium valproate enteric-coated tablets were administered as monotherapy to 118 patients (median age, 19 years) with primary generalized epilepsies. More than half (56%) of these patients were transferred from prior drug therapy, most of them because of inadequate seizure control, and some because of adverse effects. Seventy-one percent of the patients experienced tonic-clonic seizures, either alone or in combination with other types of seizures, principally absences. Mean duration of follow-up was 18 months (median, 17 months; range, 1-68 months). At a mean daily dosage of less than 20 mg/kg, 83% of the patients became seizure-free. Therapy was equally effective against tonic-clonic seizures, absences, and myoclonic seizures. Tonic-clonic seizures were suppressed in 85% of cases (89% when patient had only one seizure type), absences in 82% (95% when patient had only one seizure type), and myoclonic seizures in 82%. Paroxysmal activity was present in 88% of the electroencephalogram (EEG) records before valproate monotherapy, and in 32.4% at the study's end. These results were achieved with generally mild and mostly transient side effects; side effects were reported by 16% of patients during the first month, and 2% at the last follow-up. No hematologic or hepatic toxicity was observed. The lag time between attaining steady-state serum concentrations and achieving maximal clinical improvement suggests that sodium valproate monotherapy should be given an adequate trial to ensure that patients derive the greatest possible benefit before adding or switching to another drug.

Summary Twenty‐six cases of “benign epilepsy of childhood” with Rolandic spikes, thoroughly studied clinically and electroencephalographically, were compared with 26 patients with petit mal or petit mal and grand mal seizures. Absences could be detected in a fair number of children with “benign epilepsy.” Rolandic spikes and MU rhythms were found almost exclusively in patients having convulsions, whether focal or generalized. Rolandic spikes associated with MU rhythms and bilaterally synchronous or asynchronous discharges of spikes or of spike‐and‐slow‐waves seem, therefore, to be the features of an epilepsy whose clinical manifestations (absences or convulsions) may depend on the psycho‐biophysiological background. RÉsumé 26 cas d' “Epilepsie bénigne de L'enfance avec pointes Rolandiques” ont étéétudiés du point de vue clinique et électroencéphalographique, ils sont compareés à 26 observations de patients présentant des crises petit mal, ou petit mal et grand mal associées. Les absences petit mal électrocliniques sont fréquentes chez les sujets porteurs d'une épilepsie bénigne de L'enfance. Les pointes Rolandiques et les rythmes MÛ sont exclusivement enregistrés chez les patients présentant des crises convulsives focales ou et généralisées. L'association pointes Rolandiques, rythme MÛ, décharges bilatérales de pointes ou de pointes ondes, synchrones ou asynchrones, semble caractéristique des épilepsies bénignes dont la forme convulsive ou nonconvulsive dépend des conditions psycho‐biophysiologiques actuelles. ZUSAMMEnfassung 26 vollständig klinisch und elektroencephalographisch untersuchte Fälle von kindlicher benigner Epilepsie mit Rolandischen spikes, wurden mit 26 Kindern mit Petit‐Mai oder Petit‐Mal und Grand‐Mal verglichen. In einer beträchtlichen Anzahl von Kindern mit benigner Epilepsie konnten Absencen beobachtet werden. Rolandische spikes und MU‐Rhythmen wurden fast ausschliesslich bei Patienten mit fokalen oder generalisierten Anfällen gefunden. Das gemeinsame Auftreten von Rolandischen spikes, MU‐Rhythmen und bilateral synchronen oder asynchronen Entladungen von spikes oder spike‐slow‐waves scheinen somit Zeichen einer Epilepsie zu sein, deren klinische Manifestation (Absencen oder Krampfanfälle) vom psycho‐biophysiologischem Hintergrund abhängen. RESUMEN Se han comparado 26 casos de “epilepsyía benigna del niño con puntas Rolándicas,” concienzudamente estudiados clínica y electroencefalográficamente con 26 enfermos que padecían episodios de petit‐mal o ataques de gran mal asociados a petit‐mal. Se recogieron ausencias en un considerable número de niños con “epilepsyía benigna.” Puntas Rolándicas y ritmos MU se encontraron exclusivamente con convulsiones, ya fueran focales o generalizadas. La asociación de puntas Rolándicas, ritmos MU y descargas bilaterales síncronas o asíncronas, de puntas o punta‐onda lenta parece ser un hallazgo de las epilepsyías cuyas manifestaciones clínicas (ausencias o convulsiones) pueden depender de un fondo psico‐biofisiológico.

The authors studied 18 cases of infantile epilepsy with occipital focus and good prognosis. The patients were selected according to clinical and EEG criteria. Those criteria are: neurological and neuroradiological examinations must not show any abnormality; the patients must be free from seizures for at least 3 years and 6 months; the fundamental EEG activities must be normal, and a spike and/or spike and wave occipital focus has to be present. The medical story, the clinical and EEG follow-up, the seizures semiology (clinical and EEG) during the awake and sleep stages are described. The data show a new primary benign epileptic entity which is probably associated to maturation disturbances.