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Biomedical subjects

P Lerman

Publications and source records attributed to P Lerman.

At least 19 recordsLinked to original sources

Absence seizures aggravated by valproic acid.

PURPOSE: To report on pediatric patients with absence epilepsy who experienced absence seizure aggravation while receiving valproic acid (VPA). METHODS: The charts of all children from four pediatric epilepsy clinics receiving VPA for absence epilepsy were reviewed. Patients were evaluated and followed up between 1994 and 2000. RESULTS: Eight cases (six boys) of absence seizure aggravation were detected. Mean age at seizure onset was 5.8 years (range, 3-12 years). Six patients had simple absence seizures, one had myoclonic absences, and one had absences with automatisms. The electroencephalogram in all cases depicted generalized 3-Hz spike-and-wave activities. All eight patients experienced an increase in the frequency of absence seizures within days of VPA introduction. Dose increments resulted in further seizure aggravation. Serum levels of VPA were within therapeutic range in all patients. No case was attributed to VPA-induced encephalopathy. All patients improved on VPA discontinuation. In five children, VPA was reintroduced, resulting in further seizure aggravation. CONCLUSIONS: VPA can occasionally provoke absence seizure aggravation in patients with absence epilepsy.

Acute Disease↗

[Benign childhood epilepsy with centro-temporal spikes].

Benign childhood epilepsy with centro-temporal spikes is a specific pediatric syndrome, characterized by typical clinical symptoms including hemifacial convulsions which tend to generalize in sleep. There is no loss of consciousness. The typical EEG shows blunt diphasic spikes, localized in the rolandic or mid-temporal area. The spikes are high voltage and increase in drowsiness and sleep. In all these patients there is a normalization of the EEG and cessation of seizures in a few years, even without treatment. All these pediatric patients recover before adulthood.

Anticonvulsants↗

Phenobarbital still has a role in epilepsy treatment.

Phenobarbital, the oldest of the currently available antiepileptic drugs, has lost its popularity in this era of multiple new drugs and is seldom used after infancy in developed countries. We report a group of patients with generalized epilepsy in whom phenobarbital proved to be the drug of choice. Phenobarbital monotherapy was given to 20 patients with idiopathic generalized epilepsy beginning in childhood but persisting into adulthood. All the patients have normal intelligence and neurologic status. The electroencephalogram has either been normal or showed generalized spike-wave discharges. All patients' seizures are fully controlled on once-a-day low-dose phenobarbital with no side effects. Attempts to discontinue the medication have led to recurrence of seizures. We believe that in patients with mild generalized tonic-clonic seizures phenobarbital could still be the drug of choice since it is cheap, safe, convenient, and effective.

Adolescent↗

Early-onset benign occipital seizure susceptibility syndrome.

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 fixation-off 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.

Adolescent↗

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.

Adolescent↗

Unverricht-Lundborg disease: absence of nonallelic genetic heterogeneity.

Unverricht-Lundborg disease is a clinically recognizable form of progressive myoclonus epilepsy. Recently, in several families of both Finnish and Mediterranean extraction segregating Unverricht-Lundborg disease, the gene for this disease was linked to the same region of the long arm of chromosome 21. We performed linkage analysis in eight families, including four of neither Baltic nor Mediterranean origin, using a polymorphic (CA)n repeat marker for the human liver-type 6 phosphofructokinase (PFKL) gene, previously mapped to 21q22.3. No recombinations were observed between the disease phenotype and the PFKL marker and a maximum lod score of 5.63 was obtained. These findings confirm tight linkage between PFKL and the gene for Unverricht-Lundborg disease and strongly suggest a lack of nonallelic genetic heterogeneity of the disease.

Adolescent↗

Stormy onset with prolonged loss of consciousness in benign childhood epilepsy with occipital paroxysms.

In nine of 62 children with benign occipital epilepsy (BOE) the onset was stormy and alarming. The first and often only seizure was characterised by prolonged loss of consciousness lasting up to 12 hours, suggesting an acute cerebral insult. In all but one case there was a tonic aversion either of eyes alone or of both head and eyes which was interpreted as conjugate deviation. The other accompanying ictal motor phenomena were either partial or generalised convulsions. In five patients the seizure was heralded by a headache, and in five cases was accompanied by vomiting. The seizure began with visual symptoms in only one patient. The seizure occurred while awake in seven and during sleep in two. The age at onset was from 3 1/4 to 10 years. Interictal EEGs showed occipital discharges typical of BOE, and the clinical course was benign. In four cases a few partial or complex partial seizures recurred during subsequent anticonvulsant therapy, but in five cases seizures never recurred. Anticonvulsants were discontinued in five patients who remained free from seizures for one to 11 1/2 years after withdrawal of treatment. Sudden coma in a child associated with focal features such as tonic deviation of the head or eyes or both may represent a benign seizure disorder.

Child↗

Behavioral profile and social competence in temporal lobe epilepsy of adolescence.

A group of 26 adolescents with temporal lobe epilepsy was compared with a matched control group of 26 adolescents with chronic bronchial asthma as well as with a group of 90 healthy adolescents, using the social competence measure and the behavior profile of the Child Behavior Checklist. Both chronically ill groups exhibited more social and behavioral problems than the healthy adolescents but were similar to each other on the social and behavioral profiles. The one area in which the temporal lobe epilepsy and asthmatic groups differed from one another was that of "schizoid" psychopathology in male subjects.

Adolescent↗

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.

Adolescent↗

Effect of early corticosteroid therapy for Landau-Kleffner syndrome.

Four children treated for seizures between 1980 and 1986 were diagnosed as having Landau-Kleffner syndrome (acquired aphasia with convulsive disorder), following the onset of aphasia. They received early and prolonged ACTH or corticosteroid therapy, with high initial doses. In all four cases the EEG promptly became normal, with subsequent long-lasting remission of the aphasia and improvement of seizure control. Three to six years after discontinuation of hormone therapy the children are off medication and free from seizures and language disability.

Adrenal Cortex Hormones↗

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.

Adolescent↗

[Corpus callosotomy in intractable epilepsy].

6 males and 3 females, 2-17 years old, underwent partial or complete corpus callosotomy as treatment for medically intractable epileptic seizures. All benefited from the procedure. Evidence for unilateral cerebral damage on computerized tomography or a nonlocalized focus with secondary contralateral spread were the criteria for patient selection. The best results were obtained in atonic seizures. Long term side effects were minor and were minimized by staging the callosal resection. If partial callosotomy is effective, complete resection is unnecessary.

Adolescent↗

Corpus callosotomy in the treatment of secondary generalizing intractable epilepsy.

Over the past 5 years 9 patients underwent partial or complete corpus callosotomy for treatment of medically intractable epileptic seizures. A beneficial effect of the procedure was observed in 8 patients. Criteria for performance of the procedure included CT evidence of unilateral cerebral damage and non-localized epileptic foci in that hemisphere with secondary spread to the uninvolved side. The best results were obtained in atonic seizures. Long-term side effects were minor. The procedure is recommended in those patients with medically uncontrolled atonic or secondary generalized motor seizures in whom the corpus callosum is thought to mediate the spread of epileptic activity from a damaged cerebral hemisphere. Side effects are minimized by staging of the callosal section. If a partial callosotomy was beneficial, complete section can then be avoided.

Adolescent↗

Psychopathology and temporal lobe epilepsy in adolescents.

Although high rates of psychopathology in children and adolescents suffering from temporal lobe epilepsy (TLE) have been reported in the literature, the subject remains controversial. In this investigation we made a psychiatric study of 26 adolescents with TLE using a detailed structure interview and rigorous diagnostic criteria. These were compared to 26 matched controls suffering from chronic bronchial asthma (BA). In neither group was there a high rate of specific psychiatric illness, although many adolescents in both samples showed moderate to severe depression. We conclude that TLE may not be a specific cause of psychopathology in adolescence, and that the depression might result from the suffering involved in chronic illness.

Adjustment Disorders↗