Pinchas Lerman

Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone

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.

Seizures induced or aggravated by anticonvulsants.

Summary: 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 astaticmyoclonic 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.

Focal Epileptic EEG Discharges in Children Not Suffering from Clinical Epilepsy: Etiology, Clinical Significance, and Management

Summary: Epileptic discharges in the EEG record may be found in children not suffering from convulsive seizures. Such discharges often consist of spiking in the rolandic and midtemporal areas (labelled centrotemporal spikes) and less often in the occipital region. They may be multifocal. These focal discharges seem to be age‐dependent, tending to disappear during the teenage years. Children displaying such discharges are discovered either through large‐scale EEG studies of asymptomatic children or among those who are referred for EEG because of various nonrelated complaints such as headaches, bed‐wetting, syncope, behaviour problems, and learning difficulties. Many of them are labelled as suffering from “masked epilepsy” and are considered as epileptics. One hundred such cases are reviewed, and the clinical and EEG data are analysed. Three illustrative case reports are presented, and the literature is critically reviewed. It is maintained that these children are not to be considered as epileptic and should not be treated with anticonvulsant drugs.

Absence Seizures Aggravated by Valproic Acid

Summary:  Purpose: To report on pediatric patients with absence epilepsy who experienced absence seizure aggravation while receiving valproic acid (VPA).

The benign partial nonrolandic epilepsies.

Although rolandic epilepsy is the only epileptic syndrome that, at present, fully meets all the criteria of benignity and is widely recognized as such, there are also other forms of partial epilepsy that are benign. These are also genetically determined and age-dependent, occurring in neurologically intact children older than 18 months of age. As a rule, the seizures are brief and infrequent, usually responding well to antiepileptic drugs and remitting spontaneously before adulthood. The EEG background is normal, the morphology of the spikes is typical, and they are activated by sleep; generalized spike-wave discharges may occur. During the past decade, there have been reports of a number of additional syndromes of benign partial epilepsy. These include benign occipital epilepsy (the most common in this group), benign frontal epilepsy, benign epilepsy with affective symptomatology (or benign psychomotor epilepsy), benign epilepsy with extreme somatosensory evoked potentials, benign partial epilepsy of adolescence, and benign epilepsy associated with multiple spike foci. The Landau-Kleffner syndrome is often included among the benign epilepsies. These nonrolandic benign epilepsies are described in detail, and the relevant literature is reviewed.

A Prospective Evaluation of Pediatric Patients With Syncope

Fifty-eight children with syncope were evaluated prospectively to determine the characteristics of syncope in the pediatric age group and the yield of various diagnostic tests. The age at first syncope ranged from 0.5 to 15 years. Twenty-five children presented after a single episode and 33 after multiple episodes. Ten had a history of breath-holding spells. Nineteen had a family history of syncope. A diagnosis was established in 53 patients (91%): vasodepressor (31), cardioinhibitory (13), tussive (3), hyperventilation (2), and mixed syncope (4). In five patients (9%), the cause remained unknown. The diagnosis was established from the history in 45 cases, by a positive oculocardiac reflex in 11, and by the head-up tilt test in four. We conclude that the cause of most cases of pediatric syncope is vasodepressor or cardioinhibitory and can be diagnosed by good history-taking. Costly evaluations are rarely necessary.

Sulthiame in childhood epilepsy

Abstract Background : Sulthiame is a central carbonic anhydrase inhibitor found to be effective for both partial and generalized seizures. It has been in use in some European countries and in Israel for over 30 years. The aim of the present study was to evaluate the efficacy and tolerability of sulthiame in childhood epilepsy by conducting a multicenter, retrospective study of patients who received this drug.

Methotrexate-Induced Leukoencephalopathy is Treatable with High-Dose Folinic Acid: A Case Report and Analysis of the Literature

An episode of leukoencephalopathy is reported in a 13-year-old girl who, after standard radiotherapy for a posterior fossa medulloblastoma, received 8 treatments with a protocol containing a 4-hour infusion of 500 mg/m2 methotrexate and 12 mg intrathecal methotrexate. The leukoencephalopathy, documented clinically and by CT and EEG, cleared after 2350 mg of leucovorin (citrovorum factor, folinic acid) was given in addition to the 135 mg given as part of the therapy. A review of the literature suggests that leukoencephalopathy may be prevented by high doses of leucovorin and can be treated by high doses, if lower doses were used initially. When high dose leucovorin was not used, residual neurological damage is not unusual.

THE USE OF SULTHIAME IN MYOCLONIC EPILEPSY OF CHILDHOOD AND ADOLESCENCE

Sulthiame has been used by most investigators in psychomotor seizures, other focal seizures and grand‐mal, usually in conjunction with other anti‐convulsants. Reports on its use in myoclonic epilepsy and as a sole anti‐convulsant are few and inconclusive. The present report presents the results of a study carried out on the use of sulthiame in 54 cases of myoclonic epilepsies originating in infancy, childhood and adolescence. The different types of myoclonic epilepsy are defined. An illustrative case report is included.

Dramatic Effect of Lamotrigine in Young Adults Suffering from Intractable Absences and Generalized Tonic–Clonic Seizures Since Childhood

Abstract The purpose of this study was to evaluate the efficacy of adding lamotrigine to valproic acid in patients with refractory absence seizures starting in childhood and persisting into adulthood. Lamotrigine was added to previous anticonvulsants (always including valproic acid) in 10 patients aged 23–44 years, with intractable absence and generalized tonic–clonic seizures. Seven patients became seizure free, three patients had > 75% reduction in seizure frequency. The patients have remained on comedication with valproic acid and lamotrigine, after gradually discontinuing most other anticonvulsants, for a follow-up period ranging from 1–4 years. There has been no increase in seizure frequency during this period. None of the patients reported any side effects. This study supports the growing evidence of therapeutic synergy between lamotrigine and valproic acid. This combination may be the therapy of choice for refractory absence and generalized tonic–clonic seizures both in children and adults.