Mirian Salvadori Bittar Guaranha

Hyperventilation Revisited: Physiological Effects and Efficacy on Focal Seizure Activation in the Era of Video‐EEG Monitoring

Summary:  Purpose: Hyperventilation is an activation method that provokes physiological slowing of brain rhythms, interictal discharges, and seizures, especially in generalized idiopathic epilepsies. In this study we assessed its effectiveness in inducing focal seizures during video‐EEG monitoring.

Magnetic resonance spectroscopy reveals an epileptic network in juvenile myoclonic epilepsy

Purpose:  To investigate the cerebral metabolic differences between patients with juvenile myoclonic epilepsy (JME) and normal controls and to evaluate to what extent these metabolic alterations reflect involvement of an epileptic network.

Prognosis of juvenile myoclonic epilepsy is related to endophenotypes

PURPOSE To investigate if phenotypic variations have prognostic implications in juvenile myoclonic epilepsy (JME). METHODS Sixty-five consecutive JME patients had video-EEG recording and were followed for at least three years. Reflex traits were defined as seizures and/or EEG discharges induction by eye-closure, photic stimulation, language, praxis or calculation. Patients had psychiatric evaluation and answered to STAI (State-Trait Anxiety Inventory). Seizure control was classified according to Prasad et al. (2003).(24) Patients were divided into Group 1: good seizure control and Group 2: moderate or poor seizure control. Video-EEG/EEG evaluation was repeated in 21 patients. RESULTS Forty of 65 (61.5%) patients reached good seizure control, 25 (38.5%) of whom became seizure free. Group 2 patients had longer epilepsy duration (13.9±9.0 vs. 8.7±8.2; p=0.019); higher prevalence of the combination of all three seizure types (72.0% vs. 30.0%; p=0.003); discharges in baseline EEG (56.0% vs. 22.5%; p=0.008); seizure recording (68% vs. 20%; p<0.001) and sensitivity to praxis (63.6% vs. 29.6%; p=0.023). Compared to seizure-free patients, those with persistent seizures presented younger age at epilepsy onset (12.6±3.33 years vs. 15.4±5.47 years; p=0.015); higher prevalence of personality disorders (25% vs. 4%; p=0.029); higher scores in STAI-T (45.9±11.31 vs. 36.6±11.43; p=0.011) and higher incidence of sensitivity to praxis (58.6% vs. 25.0%; p=0.04) and to language (53.8 vs. 16.7%; p=0.026) tasks. Repetition of EEG/video-EEG revealed a parallel evolution of reflex traits disappearance and seizure control. DISCUSSION Clinical features and reflex traits have prognosis implications in JME.

Provocative and inhibitory effects of a video-EEG neuropsychologic protocol in juvenile myoclonic epilepsy

Purpose:  Studies suggest that higher cognitive functions could precipitate seizures in juvenile myoclonic epilepsy (JME). The present study aimed to analyze the effects of higher mental activity on epileptiform discharges and seizures in patients with JME and compare them to those of habitual methods of activation.

Modulation of epileptiform EEG discharges in juvenile myoclonic epilepsy: An investigation of reflex epileptic traits

Purpose:  Previous studies have suggested that cognitive tasks modulate (provoke or inhibit) the epileptiform electroencephalography (EEG) discharges (EDs) in patients with juvenile myoclonic epilepsy (JME). Their inhibitory effect was found to be especially frequent (64–90%). These studies arbitrarily defined modulation as a >100% increase or >50% decrease of the EDs compared with baseline, which may not sufficiently distinguish from spontaneous fluctuations. The aim of our study was to assess the modulation of EDs and the precipitation of myoclonic seizures by cognitive tasks and by conventional provocation methods, taking into account also the spontaneous fluctuation of EDs.

Are personality traits of juvenile myoclonic epilepsy related to frontal lobe dysfunctions? A proton MRS study

Purpose:  Personality traits characterized by emotional instability and immaturity, unsteadiness, lack of discipline, hedonism, frequent and rapid mood changes, and indifference toward one’s disease have been associated with patients who have juvenile myoclonic epilepsy (JME). Literature data demonstrate worse seizure control and more psychosocial dysfunctions among patients with JME who have those traits. In this controlled study we performed a correlation analysis of psychiatric scores with magnetic resonance spectroscopy (MRS) values across JME patients, aiming to verify the existence of a possible relation between frontal lobe dysfunction and the prevalence of personality disorders (PDs) in JME.

Physiological and electroencephalographic responses to acute exhaustive physical exercise in people with juvenile myoclonic epilepsy

Although the available evidence suggests that exercise may positively affect epilepsy, whether this effect is applicable to different types of epilepsy has not been established. Physiological responses during rest, acute physical effort, and a recovery period were studied by concomitant analysis of cerebral electric activity using EEGs in subjects with juvenile myoclonic epilepsy (JME) and healthy controls. In addition, level of habitual physical activity, body composition, and 1 week of actigraphy monitoring data were evaluated. Twenty-four subjects (12 with JME and 12 controls) participated in this study. Compared with the control group, the JME group had a significantly lower V˙O(2) at rest (13.3%) and resting metabolic rate (15.6%). The number of epileptiform discharges in the JME group was significantly reduced during the recovery period (72%) compared with the resting state. There were no significant differences between the JME and control groups in behavioral outcomes and sleep parameters evaluated by actigraphy monitoring. The positive findings of our study strengthen the evidence for the benefits of physical exercise for people with JME.

Blinking and eyelid myoclonia: Characteristics and correlations of eyelid movements

PURPOSE Eyelid myoclonia (EM) is considered a seizure type and has been described in several epileptic conditions. Previous studies reported that EM are precipitated only by slow eye closure, but little is known about the characteristics of blinking in patients with EM seizures and differences in precipitation of EM by different kinds of eye closure. We analyzed by video-EEG the characteristics of blinking and eye closure in these patients. METHOD Twenty patients with EM had a video-EEG protocol with eyelid sensors. Semiology and rate of blinking and EM were analyzed. Statistical analysis was performed and p-values <0.05 were considered significant. RESULTS Seventeen cases (mean age 20.7, range 3-35) were women, 10 had EM as the main seizure type and between the others, all, but one, had criteria to Juvenile Myoclonic Epilepsy. All patients presented EM, 18 spontaneously during awake, 10 on eye closure and one only during intermittent photic stimulation. EM assumed the form of flicker, flutter or jerk, accompanied by generalized discharges, spiky posterior alpha, theta rhythm or absence of any EEG abnormality. Analysis of the characteristics of blinking had no statistics differences between patients and healthy subjects. The rate of blinks and EM increased during speech and decreased during reading. EM never occurred during blinking or in the dark. CONCLUSIONS Despite normal physiology of blinking, EM can manifest as jerk, flicker or flutter, with or without EEG abnormalities and independently of IPS, suggesting that eye closure sensitivity seems to include both, a motor and a visual component.

Adherence to treatment in patients with juvenile myoclonic epilepsy: correlation with quality of life and adverse effects of medication

OBJETIVOS: Este estudo teve como objetivo avaliar a adesao ao tratamento com drogas antiepilepticas (DAEs) em pacientes com Epilepsia Mioclonica Juvenil (EMJ) e correlacionar com a Qualidade de Vida (QV) e com os efeitos adversos a medicacao. METODOLOGIA: A amostra foi composta de 43 pacientes com diagnostico clinico e eletrografico (EEG/Video-EEG) de EMJ (ILAE,1989), em tratamento regular no Hospital Sao Paulo, UNIFESP, Brasil. Todos os pacientes responderam a um questionario de adesao ao tratamento (escores de 0-100), em que escores mais elevados evidenciavam uma pobre adesao ao tratamento. Para avaliar a QV foi utilizada a versao brasileira validada do Quality of Life in Epilepsy Inventory 31 (QOLIE-31); os efeitos adversos das DAEs foram avaliados atraves do Adverse Events Profile (AEP), escores de 19 a 76, no qual escores ≥45 indicam toxicidade. Foram considerados significantes os valores de p<0,05. RESULTADOS: Dezesseis pacientes (37,2%) estavam em monoterapia e 26 (60,4%), em politerapia; 22 (48%) tiveram uma crise nos ultimos tres meses antes da entrevista. A media de adesao ao tratamento foi 68.5. Foram observados escores ≤45 em 38 (88.3%) no AEP e 29 (67.4%) apresentaram queixas espontâneas em relacao ao uso das DAEs. Os efeitos adversos mais comuns foram sonolencia em 11 (13,8%) e inquietacao em 7 (8,8%). A maior media do QOLIE-31 foi 79,0 (Funcionamento Social), e a mais baixa 33,0 (Preocupacao com as Crises). A adesao ao tratamento apresentou correlacao estatistica com valores melhores na QV enquanto valores elevados no AEP indicaram pior adesao (Pearson <0,05). CONCLUSAO: A adesao ao tratamento mostrou alta correlacao com uma melhor QV. A presenca de efeitos adversos foi negativamente associada com a adesao ao tratamento.

Phenotyping juvenile myoclonic epilepsy. Praxis induction as a biomarker of unfavorable prognosis

PURPOSE Juvenile myoclonic epilepsy (JME) is a heterogeneous syndrome with seizures presenting typical fluctuation in diurnal cycle and relation with awakening. Few publications have approached clinical expressions of praxis induction (PI) in the nosology of JME as well as its impact on outcome. The aim of this study is to characterize PI as the only reflex trait in JME and its relation with prognosis. METHOD JME with PI reported on a questionnaire and confirmed by video-EEG testing (Group 1, 20 patients) were compared with JME without any reflex epileptic trait (Group 2, 25 patients) and followed for a mean of 7.82 years (SD=3.98). Circadian distribution and frequency of seizures were assessed in a diary. Patients also had psychiatric evaluation. RESULTS Prevalence of PI was 20/133 (15%) JME patients, and was predominant in males (1.5 male: 1 female; OR 13; p=0.042). Among Group 1 patients, only 2/20 presented seizures exclusively in the morning (p=0.013), and none, exclusively on awakening (p<0.001). PI patients had worse prognosis regarding control of myocloni (p=0.02) and absences (p=0.01); only 7/20 (35.0%) could be treated with VPA in monotherapy (p=0.01). At the last follow-up, 2/20 (10.0%) of Group 1 and 10 (40.0%) of Group 2 patients were free of all three seizure types (p=0.02). Even though relative risk of stress as a precipitant of seizures increased 3.82 times in Group 1, psychiatric comorbidities were not different between groups. CONCLUSION PI reflex trait in JME is related to seizures without preferential circadian occurrence and reduced response to antiepileptic drugs.