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 conjuction with other anticonvulsants. 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. Results indicated that sulthiame is the drug of choice, often as the sole anti-convulsive agent, in cases of "juvenile myoclonic epilepsy". In the myoclonic encephalopathies of childhood (the so-called "minor motor epilepsy" or Lennox-Gastaut syndrome), which are notoriously refractory to therapy, sulthiame appears to be an efficacious adjunct to currently-used agents, including benzodiazepines, succinimides, dipropyl acetate, steriods and a ketogenic diet.

Benign focal epilepsy of childhood. A follow-up study of 100 recovered patients.

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.

Phenytoin pharmacokinetics in catamenial epilepsy.

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.

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.

Metastatic disease to kidney from lung.

Two cases are reported of primary carcinoma of the lung, presenting as tumors of the kidney. It was found that metastases to the kidney from the lung is frequent. It is recommended that in all cases of primary lung carcinoma the kidneys be examined for metastatic disease. If cancer is found and is treatable, the cure rate may be improved with early and aggressive treatment.

Ruptured renal cyst in polycystic disease.

A patient with polycystic disease was admitted with acute abdominal pain. This was caused by a rupture of one of his right renal cysts into the collecting system, with extravasation into the subcapsular space. With conservative therapy, the patient returned to his status prior to this episode. The extravasation was absorbed, and the cyst became smaller. He will continue under supportive care for his polycystic disease.

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.

Effect of valproic acid therapy on zinc metabolism in children with primary epilepsy.

The effect of long-term treatment with valproic acid (VPA) on zinc (Zn) metabolism was studied in 15 children with absence seizures. During treatment with VPA the erythrocyte Zn content was significantly lower than that found in controls matched for sex and age. Plasma and urine values of Zn and of copper were within normal limits. It is suggested that the anticonvulsive action of VPA may be mediated through its effect on the metabolism of Zn in the brain and the concomitant changes in the activity of the enzymes glutamic acid decarboxylase and carbonic anhydrase.

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.

Low erythrocyte zinc content in acquired aphasia with convulsive disorder (Landau-Kleffner syndrome).

Abnormal zinc metabolism in a 6-year-old male patient with Landau-Kleffner syndrome (acquired aphasia and convulsive disorder) is the subject of our report. We describe a significant decrease of erythrocyte and plasma zinc levels in the patient as compared with normal. Red blood cell zinc content is normally 38.5 +/- 2.7 micrograms/g hemoglobin v 16.5 micrograms/g hemoglobin found in the patient. Plasma zinc level is 94.6 +/- 10.4 micrograms/dL in normal in comparison with 50.4 micrograms/dL in the patient. Normal oral zinc tolerance test performed on the patient precludes altered zinc absorption or increased urinary loss. The possible connection between abnormal zinc metabolism and the pathophysiology of Landau-Kleffner syndrome is discussed.

Meningioma associated with parathyroid adenoma.

A woman hospitalized because of attempted suicide with diazepam tablets was found to have hypercalcemia and other signs of hyperparathyroidism. Electroencephalogram indicated a brain lesion which was confirmed by a brain scan and angiogram. The hypercalcemia persisted after removal of the meningioma and serum levels of calcium returned to normal only after a parathyroid adenoma was removed during an additional intervention. This association of meningioma with hyperparathyroidism is unique in the literature. The difficulties of diagnosis resulting from the neuropsychiatric symptoms common to the two disorders are discussed.

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.

Deinstitutionalization and welfare policies.

Before America began creating a federally based welfare state in the 1930s, most publicly funded responses to social problems had an institutional bias. The ways in which the welfare programs initiated 50 years ago have helped to influence institutional trends, and are likely to continue doing so in the future, constitute the major focus of this analysis. Four special problem groups are assessed from a historical perspective: (1) the dependent aged and the movement from local almshouses and state insane asylums to nursing homes; (2) the mentally ill and the movement from state hospitals to a variety of local medical and nonmedical residences; (3) the developmentally disabled--formerly the mentally retarded--and the movement from state schools to private community residential facilities; and (4) the dependent/neglected and delinquent youth and the movement away from orphan asylums and training schools to group homes, treatment centers, adolescent psychiatric units, halfway houses, and outdoor camps. Recent trends and projections, as well as present and future policy issues, are assessed.

[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.

[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.