Summary:Purpose: To evaluate the outcome of children with cryptogenic infantile spasms treated with high-dose synthetic adrenocorticotropic hormone (ACTH) and the relation between early treatment, within 1 month of onset, and outcome.Methods: We assessed the long-term cognitive and seizure outcomes of 37 patients with cryptogenic infantile spasms (onset, age 3 to 9 months) receiving standardized treatment regimen of high-dose tetracosactide depot, 1 mg IM every 48 h for 2 weeks, with a subsequent 8-to 10-week slow taper and followed by oral prednisone, 10 mg/day for a month, with a subsequent slow taper for 5 months or until the infant reached the age of 1 year, whichever came later. Development was assessed before treatment. Seizure outcomes were followed up prospectively. Cognitive outcomes were determined after 6 to 21 years and analyzed in relation to treatment lag and pretreatment regression.

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...

It is not known why the frequency of seizures sometimes increases in the perimenstrual period (catamenial epilepsy). We have examined the possibility that changes in anticonvulsant pharmacokinetics may be responsible. Seventeen women with seizures who were taking phenytoin (DPH) and whose seizures were more frequent perimenstrually were examined twice each, once on the first or second menstrual day and again after 2 weeks. Mean serum DPH levels were lower during the menses. In seven women with seizures unrelated to menses, the fall of DPH levels was much smaller. In catamenial epilepsy, the fall was due to increased DPH clearance. At the time of ovulation, hepatic DPH metabolism may be slowed by competition from steroid hormones.

Six cases of epileptic children are reported, in whom anticonvulsant therapy, aggravated rather than controlled the seizures or induced minor seizures. This was caused by excessive polytherapy in one case and by the use of the inappropriate drug (for that type of epilepsy) in four other cases. In the sixth case, the appropriate anticonvulsant was used, but it induced multiple astatic-myoclonic seizures. This phenomenon of drug-induced seizures is apt to occur particularly in certain types of childhood epilepsy that are refractory to therapy and are accompanied by slow spike-wave discharges in the EEG, such as the Lennox-Gastaut syndrome.

Benign focal epilepsy of childhood is an entity that includes characteristic clinical and electroencephalographic manifestations. Clinically, it consists of typical brief, hemifacial seizures that tend to become generalized when they occur nocturnally. The EEG findings include slow, diphasic, high-voltage, centro-temporal spikes, often followed by slow waves. Retrospective and prospective studies were carried out on 100 such patients, all of whom recovered before reaching adulthood, with the disappearance of both the clinical manifestations and the EEG findings.