Jorge J. Asconapé

Some clinical and EEG aspects of benign juvenile myoclonic epilepsy.

Summary: Twelve patients with benign juvenile myoclonic epilepsy (BJME) representing 4% of our population of epileptics (n = 275) are presented. Only two patients (17%) had myoclonic jerks as the only seizure type. Seven (58%) had generalized tonic‐clonic seizures (GTCS) and myoclonus. Three patients (25%) had absence seizures (AS), GTCS, and myoclonic jerks. Electroencephalographic evidence of photosensitivity was found in four (33%). Auditory precipitation of seizures was found in one patient. As is the case with other primary generalized epilepsies, the onset of BJME seems to be age specific. In our series the mean age of onset in years was 4.3 for AS, 14.75 for myoclonic jerks, and 16.4 for GTCS. It took an average of 8.5 years from the onset of BJME (range, 2–20 years) and 6.5 years from the onset of GTCS (range, 2 months‐6 years) until the condition was properly recognized. Five patients experienced at least one episode of myoclonic status epilepticus. Generalized, paroxysmal, symmetric poly spike and slow wave discharges are the typical EEG finding. These complexes, however, showed considerable interpatient variability. Sleep deprivation proved to be the most valuable activating procedure. Valproic acid monotherapy effectively controlled myoclonic jerks as well as associated GTCS in most patients.

Bradycardia and Asystole with the Use of Vagus Nerve Stimulation for the Treatment of Epilepsy: A Rare Complication of Intraoperative Device Testing

Summary: Purposes: A 56‐year‐old man with mild mental retardation, right congenital hemiparesis, and refractory partial seizures was referred for vagus nerve stimulation (VNS).

Clinical and EEG Asymmetries in Juvenile Myoclonic Epilepsy

Summary: We reviewed records of 85 patients with juvenile myoclonic epilepsy (JME) for significant asymmetries in clinical seizures or the EEG. We noted asymmetries in 26 of 85 patients (30.6%). Only 2 patients had both clinical and EEG asymmetries; 12 had clinical asymmetries and 12 had EEG asymmetries exclusively. Analysis of patients with and without asymmetries showed no statistically significant differences in comparisons of sex, age at seizure onset, family history of epilepsy, seizure type, or response to treatment. The delay in diagnosis was greater in JME patients with asymmetries (9.5 years) than in JME patients with no asymmetries (7.5 years), but this difference was not statistically significant. Fourteen of the 26 patients with asymmetries (53.8%) were initially misdiagnosed as having partial seizures. Asymmetries in JME patients are not only common, but are also a frequent cause of misdiagnosis.

Valproate-Associated Pancreatitis

Summary: To assess the clinical characteristics of valproate (VPA)‐associated pancreatitis, information from three sources was gathered: (a) a survey among 507 physicians with a special interest in treatment of epilepsy, (b) a review of the authors’ patient population, and (c) a review of the literature. Of 366 physicians answering the survey, 53 (14.5%) reported a case of pancreatitis. Thirty‐nine cases were available for review (24 from the medical literature, 12 from the survey, and 3 from the authors). Pancreatitis appeared to be more frequent in young persons (mean age 16.4 years) but may occur at any age. The highest risk appears to exist during the first months of treatment: 43.8% of the cases developed during the first 3 months, and 68.8% developed during the first year. Seventy‐six percent of patients were receiving polytherapy, and 41% had some form of associated chronic encephalopathy. In most patients, the reaction was rapidly reversible when VPA was discontinued. It was severe in 6 patients, with 3 deaths reported. Rechallenge with VPA was attempted in 9 patients, with a high incidence of relapses. Asymptomatic elevation of serum amylase in patients receiving VPA was reported by 40 (10.9%) of the physicians surveyed. Awareness of the problem and early discontinuation of VPA may be effective in preventing serious reactions.

Psychogenic seizures in adults: a longitudinal analysis

The clinical characteristics, psychosocial background, neuropsychological testing, clinical and social outcome were analysed in 93 adults with psychogenic seizures (PS). Thirteen (14%) were males and 80 (86%) were females. Mean age was 31.7 years (range 16 to 55 years). Lack of responsiveness associated with motor activity was the most common finding. Neuropsychological testing done in 46 cases revealed hysteroid traits and coping mechanisms and depression to be the most prevalent underlying problems. History of sexual abuse was evident in 10 (10.7%) cases. Social impact analysis revealed that of 62 patients who were working at the onset of PS, 34 were not working at the time of the diagnosis of PS. In 25 cases, PS were the reason for not working. After a mean follow-up of 60.7 months done in 63 patients, 16 (25.4%) patients were seizure-free. There were no obvious significant predictors of poor prognosis.

Psychogenic seizures in children: long-term analysis of 43 cases.

Forty-three patients exhibiting psychogenic seizures with onset before the age of 16 years were studied. All patients underwent intensive electroencephalography and video-electroencephalography monitoring. Thirty-two were female and 11 male. Mean age of the population at seizure onset was 12.4 years (range, 5 to 16 years). Twenty-one patients (48.8%) were taking anticonvulsants. Neurologic past history was abnormal in nine cases. Family history of epilepsy was found in 15 cases (34.9%). Median seizure frequency was one seizure every 5 days. Clinical characteristics of the seizures varied. However, unresponsiveness with generalized violent and uncoordinated movements involving the whole body (n = 19) or with generalized trembling (n = 11) were the most common features. Neuropsychological testing, carried out in 22 cases, failed to show major abnormalities in most of the cases. Significant personal and family distress was found in most of the cases. An important impact on patients quality of life was evident when the seizures were present as compared to the seizure-free periods. There were no statistically significant predictors of clinical outcome. (J Child Neurol 1994;9:404-407).

Clinical management of recurrent postictal psychosis

Abstract Although there are numerous reports of interictal psychosis in epileptic patients, there are few studies describing the longitudinal course and treatment of postictal psychosis. The goal of this study was to define the clinical features, natural history, and possible interventional methods in a group of patients with complex partial seizures and postictal psychosis. We studied seven patients who manifested psychotic behavior following an increase in their seizure frequency. Mean follow-up was 83 months. Patients underwent neuropsychiatric testing, cranial magnetic resonance imaging, and extensive EEG evaluation. All but one patient had severe epilepsy. An increased number of seizures was followed by a brief period of lucidity and then psychosis, which lasted between 2 and 14 days and tended to recur (mean, one episode every 4 months). EEGs done during the psychotic episodes generally demonstrated a mild diffuse background slowing without evidence of ictal epileptiform discharges. Evaluation between episodes revealed no persistent psychiatric features. The patients rarely required hospitalization or neuroleptic medications. Patients usually responded to mild sedation, close observation, and a supportive environment.

Circling seizures in a case of juvenile myoclonic epilepsy.

Summary: Circling seizures (CS) have been described in association with focal lesions as well as with generalized EEG discharges. We report 1 patient with juvenile myoclonic epilepsy (JME) who developed CS. There were no focal findings on clinical examination, EEG, or imaging studies. We propose that CS in this patient may represent a profound asymmetry in expression of an idiopathic generalized epilepsy rather than a partial condition.

Use of Antiepileptic Drugs in the Presence of Liver and Kidney Diseases: A Review

Patients with liver or kidney disease often have adverse reactions to many drugs, even when prescribed at accepted doses. This is not surprising in view of the fact that the liver and kidney are the major organs involved in elimination of drugs. Solid knowledge of basic pharmacological principles as well as familiarity with the pharmacokinetics of the individual drugs and their main metabolites are required in the management of these patients. Although the effects of hepatic and renal failure on the disposition of drugs have been the subject of considerable interest in the last two decades, information on most of the drugs used in clinical practice is at the present time insufficient; antiepileptic drugs are no exception. The increasing accuracy and availability of drug level determinations in plasma or other body fluids doubtlessly represents one of the major developments in this area, providing the physician with precise and objective ancillary information. Renal disease can significantly affect the disposition of a drug. It can impair the urinary excretion of the parent drug as well as of its metabolites, a few of which may be pharmacologically active, thus leading to accumulation and adverse effects. Renal failure can also affect the kinetics of a drug by altering its protein binding, distribution, or metabolism. Dialysis can further modify these parameters and can also result in significant removal of the drug, leading to depletion of therapeutic concentrations. Dialyzability of a drug depends on several factors, including molecular weight, protein binding, and hematocrit, as well as on the intrinsic characteristics of the dialyzer. In general, for a drug that undergoes extensive hepatic metabolism, only a small fraction of that drug is excreted unchanged in the urine, so that renal excretion plays only a minor role in its elimination. The majority of drugs undergo some degree of biotransformation, which results in the formation of more polar substances with increased water and decreased lipid solubility. As a consequence, these polar metabolites show less pharmacological activity and increased renal excretion, compared to the parent compounds. Drug metabolism occurs almost exclusively in the liver, in the microsomal mixed-function oxidase system. Since the rate of drug elimination from plasma is one of the main determinants of the duration of action of a drug, liver damage can lead to significant prolongation of a drug’s half-life. Unless dosage is readjusted, higher steady-state concentrations will occur, resulting in clinical toxicity. Because of the large hepatic reserve capacity, liver failure must be se-

Juvenile myoclonic epilepsy: An underdiagnosed syndrome

Abstract Since the first description of juvenile myoclonic epilepsy (JME) in the English literature in 1984, the delay in correct diagnosis of JME remains unchanged. Ninety patients with JME were studied. Median delay in diagnosis was 9 years (range 0–52 years). Reasons for delay in diagnosis were analyzed. Failure to recognize the myoclonic seizures (MS) was the most common element contributing to the delay. In some cases, MS were not reported by the patients; in other cases, MS were confused with nervousness or clumsiness and not interpreted as epileptic in nature by the referring physicians. Clinical and EEG asymmetries were the second most important cause for misdiagnosis. In 53.7% of these cases, asymmetries were interpreted as partial features. The third cause of misdiagnosis was the lack of recognition of the MS associated with normal interictal EEGs. These cases were interpreted as nonepileptic seizures. It is important to be aware of this frequent epileptic syndrome, in which nearly 90% of cases can be controlled with adequate treatment (valproate, VPA).