Marcelo E. Lancman

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.

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.

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).

Number 20 Ring Chromosome: A Case With Complete Seizure Control

quency increased to a daily basis. These seizures were associated with a clonic component. Phenytoin was added, and he continued to have one to two seizures per month for the next year. At age 3 years, the seizure frequency again increased, and staring spells were also noted. A genetic study was done with cells obtained from peripheral blood, cultured, and harvested. A total of 35 cells were ex-

Paroxysmal pain as sole manifestation of seizures

A developmentally normal 4-year-old white female who presented with pain in the right hand as the only manifestation of epilepsy is reported. Two years later, she developed complex partial seizures following right-hand pain. Computed tomography and magnetic resonance imaging were unremarkable. Prolonged ambulatory electroencephalography (EEG) as well as video-EEGs with ictal pain episodes failed to reveal abnormalities. Only a full night video-EEG performed after antiepileptic drug withdrawal demonstrated 2 right-hand pain episodes followed by a complex partial seizure with ictal epileptiform activity on the scalp EEG in the left parasagittal area, rapidly generalized and interictal discharges in the C3-P3 area. This patient had a very unusual presentation of epilepsy.

Startle epileptic seizures: Clinical and imaging findings

Abstract Twenty-six cases of startle epileptic seizures (SES) were studied. The mean age at seizure onset was 5.1 years, with six patients starting immediately after birth. Severe brain damage was observed in 17 patients (65%). Epilepsy was severe and intractable in 21 patients (80.7%). Imaging studies (magnetic resonance imaging and computed tomography) were abnormal in 17 cases, although no single pattern of lesion was found. Most of these patients had large brain lesions, either unilateral (n = 7) or bilateral (n = 10). The corpus callosum was affected in five cases (19.2%). We conclude that SES is a heterogeneous entity that could be associated with different types of seizures and epilepsies. SES is more frequently associated with severe brain damage and intractable epilepsy. No clear-cut imaging pattern was found in association with SES.