A cognitive rehabilitation program to improve hot and cool executive dysfunction in juvenile myoclonic epilepsy: Preliminary findings.

OBJECTIVE: Executive and attentional deficits are often described in Juvenile Myoclonic Epilepsy (JME). We aimed to evaluate the short-term impact of rehabilitation developed for the most frequent cognitive deficits of persons with JME. METHODS: Thirty-three patients entered this study which consisted of 12 individual sessions once a 60-minute week, divided into planning/organization, attention, and impulsivity. Twenty-seven patients finished the protocol, and all patients had pre-and-post evaluations from neuropsychological tests and self-rating questionnaires. Generalized Estimating Equations (GEE) inferential statistics were used to verify the protocol's effect, and a 95% confidence interval was adopted. RESULTS: We found significant improvement in selective attention (TMT A [p < 0.01] and Stroop test 2 [p = 0.03]), inhibitory control (Stroop test 3 [p = 0.02], FAS [p < 0.01], CPT commissions [p < 0.01]), mental flexibility [WCST categories p < 0.01] and implicit decision making (IGT blocks A [p < 0.01], B [p = 0.02], C [p < 0.01] and D [p < 0.01]). All components of the Behavioral Rating Index of Executive Functions metacognition index and the general quotient had significant improvement (initiative [p ≤ 0.01], working memory [p ≤ 0.01], planning and organization [p ≤ 0.01], task monitor [p = 0.02] and organization of materials [p = 0.02]). Regarding the Behavioral Regulation Index, the "Emotional Control" was improved [p = 0.03]. The attentional component and general scores of the Adult Self-Report Scale for Adults also changed significantly [p ≤ 0.01]. SIGNIFICANCE: Executive function and attention had an improvement in objective and subjective tests. The context-dependent reactive mechanism of impulsivity improved in instruments based on the ecological evaluation. Our findings, though preliminary due to a lack of controls and practice effect corrections, support that cognitive rehabilitation may be a valuable resource to alleviate cognitive deficits in patients with JME.

Prevalence, nature, and severity of the psychiatric comorbidities and their impact on quality of life in adolescents with Juvenile myoclonic epilepsy.

INTRODUCTION: Adults with Juvenile myoclonic epilepsy (JME) are at increased risk for psychiatric comorbidities, personality traits, and abnormality in executive function. But studies on adolescents and their impact on quality of life are scarce in the literature. MATERIALS AND METHODS: This cross-sectional study was performed between August 2019 and October 2022 to compare the prevalence of psychiatric comorbidities in adolescents with JME and age and gender-matched healthy controls. After completing DSM-5 Structured Clinical Interview (SCID-5) initially in all patients, we measured the severity of individual psychiatric problems like anxiety, depression, and somatic symptoms by using an appropriate psychometric scale. We also measured both groups' intelligence quotient (IQ), executive function, and quality of life. RESULTS: One hundred patients with JME (14.3 ± 2.5 years, 48 boys) and 100 controls were enrolled. Psychiatric disorders were observed in 46% of JME and 6% of controls (p < 0.01). Psychiatric comorbidities noted in the patients with JME were: somatic symptom and related disorders(n = 14), anxiety (n = 13), adjustment disorders (n = 12), depression (n = 11), oppositional defiant disorder (n = 6), conduct disorder (n = 5), anorexia nervosa (n = 3), narcissistic (n = 3), histrionic (n = 1), substance-related disorder (n = 1), borderline (n = 2) and antisocial personality disorder (n = 2). The prevalence of depressive disorders, anxiety disorders, adjustment disorders, somatic symptoms, related disorders, and any personality disorder was significantly more in the JME group (p < 0.01 for all). Female gender, higher Epilepsy Stigma Scale score, and lower Epilepsy Outcome Expectancy Scale were significantly associated with depressive disorders (p = 0.04, 0.03, 0.03 respectively). Similarly, for anxiety, only female gender and lower Epilepsy Outcome Expectancy Scale were significant associated factors (p = 0.03, 0.02 respectively). CONCLUSIONS: Psychiatric disorders like anxiety, depression, and personality disorders are more frequent in adolescents with JME than in controls.

Executive functions and personality traits of juvenile myoclonic epilepsy patients: a single-center experience of 23 cases.

BACKGROUND: Juvenile myoclonic epilepsy (JME), which is a fairly common form of generalized epilepsy syndrome has attracted attention by providing focal findings in some electrophysiological, neuropsychological, and neuroimaging studies. These findings are considered to be based on frontal lobe dysfunction. Furthermore, it is known that Cluster B personality disorders that are related to impulsive behavior are frequently seen in JME patients. METHODS: In this study, 23 JME patients and 20 healthy control subjects were included. All subjects were assessed using neuropsychological tests for executive functions and the Temperament and Character Inventory (TCI) for personality traits. RESULTS: JME patients performed poorly in the digit span test and the Stroop Color and Word Interference Test. When the TCI scores were compared, there was no significant difference between the patients and the control subjects compatible with the literature. In addi-tion, cooperativeness-character dimension (C1-social acceptance) scores were significantly lower in the patient group. DISCUSSION: Our findings support that JME patients have frontal lobe dysfunction. Although several studies are available in the literature, no significant results related to personality traits were detected.

Trait impulsivity in Juvenile Myoclonic Epilepsy.

OBJECTIVE: Impulsivity is a multidimensional construct that can predispose to psychopathology. Meta-analysis demonstrates an association between response impulsivity and Juvenile Myoclonic Epilepsy (JME), a common genetic generalized epilepsy. Here, we test the hypotheses that trait impulsivity is (i) elevated in JME compared to controls; (ii) moderated by specific seizure characteristics; and (iii) associated with psychiatric adverse effects of antiepileptic drugs (AEDs). METHODS: 322 participants with JME and 126 age and gender-matched controls completed the Barratt's Impulsiveness Scale (BIS-brief) alongside information on seizure history and AED use. We compared group BIS-brief scores and assessed associations of JME BIS-brief scores with seizure characteristics and AED adverse effects. RESULTS: The mean BIS-brief score in JME was 18.1 ± 4.4 compared with 16.2 ± 4.1 in controls (P = 0.0007). Elevated impulsivity was associated with male gender (P = 0.027), frequent absence seizures (P = 0.0004) and lack of morning predominance of myoclonus (P = 0.008). High impulsivity significantly increased the odds of a psychiatric adverse event on levetiracetam (P = 0.036), but not any other psychiatric or somatic adverse effects. INTERPRETATION: Trait impulsivity is elevated in JME and comparable to scores in personality and neurotic disorders. Increased seizure frequency and absence of circadian seizure pattern moderate BIS score, suggesting disruption of both cortico-striatal and thalamocortical networks as a shared mechanism between seizures and impulsivity in JME. These findings warrant consideration of impulsivity as a distinct target of intervention, and as a stratifying factor for AED treatment in JME, and perhaps other types of epilepsy. The role of impulsivity in treatment adherence and psychosocial outcome requires further investigation.

Impulsiveness, personality traits and executive functioning in patients with juvenile myoclonic epilepsy.

PURPOSE: To evaluate impulsiveness in adult patients with JME and its relationship with personality traits and executive functioning. METHODS: Patients completed psychiatric evaluation (DSM IV), Barratt Impulsiveness Scale (BIS-11), Neo Revised Personality Inventory (NEO PI-R) and executive functioning evaluation comprising Controlled Oral Word Association (COWA), Digit Span, Trail Making Tests (TMT), Stroop Test (ST) and Wisconsin Card Sorting Test (WSCT). Healthy controls (63 % female, mean age 35.7 yrs. (±8.37)) were examined to allow calculation of z-scores. RESULTS: 50 patients (70 % female; mean age 32.5 yrs. (±9.2)) presented higher scores of Total (z=-0.37; p = 0.005) and Motor Impulsiveness (z=-0.79; p < 0.001) on BIS-11. Motor Impulsiveness was associated with higher rates of mild psychiatric disorders (depression and anxiety) (p = 0.035) and worse myoclonic seizure control (p = 0.007). NEO PI-R showed differences on Neuroticism (z=-0.60; p < 0.001), Openness (z = 0.38; p = 0.043), Agreeableness (z=-0.38; p = 0.033) and Conscientiousness (z=-0.53; p = 0.003). There were positive correlations between BIS-11 and Neuroticism with Total, Motor and Non-Planning Impulsiveness, on the other hand, Conscientiousness was negatively correlated with these as well as with Attentive Impulsiveness. Patients performance was worse than that of controls on COWA (z=-0.43; p = 0.009) and WCST's Total Number of Completed Categories (z=-2.08; p = 0.005), Trials Taken to Complete First Category (z=-1.56; p = 0.013), Percentage of Total Errors (z=-1.56; p < 0.001), Perseverative Errors (z=-0.73; p = 0.002), Non-Perseverative Errors (z=-1.05; p = 0.003) and Conceptual Level Responses (z=-1.52; p < 0.001). Non-Planning Impulsiveness correlated with Performance (ST and TMT). CONCLUSION: Patients with JME present with impulsive behavior, personality features and executive dysfunction which are linked and may lead to lack of commitment in treatment and affect other aspects of life.

Do interictal EEG findings reflect cognitive function in juvenile myoclonic epilepsy?

PURPOSE: This study investigated the relationship between frontal lobe cognitive function and frontal focal electroencephalography (EEG) findings in patients with juvenile myoclonic epilepsy (JME). METHODS: The study enrolled 60 patients diagnosed with JME and followed at the Epilepsy Outpatient Clinic of the University of Health Sciences, Bakirkoy Psychiatric Hospital, and 30 healthy volunteers. Demographic and clinical features were recorded. Frontal lobe cognitive functions were tested in both groups. Video-EEG recordings of patients with JME were evaluated. The presence and duration of generalized discharges, the presence and lateralization of focal findings, and the presence of generalized discharges during hyperventilation and photic stimulation were recorded during EEG. Cognitive function test results were compared between the two groups, and the relationship between the EEG findings and cognitive function was investigated. RESULTS: The study included 35 (58.3%) female and 25 (41.6%) male patients and 17 (56.7%) female and 13 (43.3%) male healthy controls. The mean ages of the group with JME and controls were 28.3 ±â€¯8.6 (16-50) and 31.3 ±â€¯7.9 (17-45) years, respectively. Patients with JME performed more poorly on the frontal lobe cognitive tests than controls (p < 0.05). Patients whose generalized discharges were longer than 1 s performed more poorly on tests evaluating attention and made more perseverative errors (p < 0.05). There was no significant correlation between the presence of focal EEG findings and the scores on frontal lobe cognitive functions tests in the group with JME (p > 0.05). CONCLUSION: Frontal lobe cognitive functions are affected in patients with JME. The cognitive effects were more pronounced in patients with prolonged generalized discharges on EEG.

The association between temperament features and childhood traumas in patients with juvenile myoclonic epilepsy

Background/aim: Epilepsy is a common chronic neurological problem that impairs daily activities, functionality, and quality of life. Childhood traumas (CTs) are known to be critical factors in the onset or development of many psychiatric and medical disorders. They also play a critical role in the development of temperament and personality. This study aimed to investigate the association between CTs and common temperament patterns and features seen in epilepsy patients. Materials and methods: The study included 38 patients who were diagnosed with juvenile myoclonic epilepsy (JME) and volunteered to participate in the study. In addition to the sociodemographic form and questions on disease features, Structured Clinical Interview for DSM-IV Axis I Disorders, Temperament Evaluation of Memphis, Pisa, Paris and San Diego Questionnaire (TEMPS-A), Childhood Trauma Questionnaire (CTQ), Beck Depression Inventory (BDI), and Beck Anxiety Inventory (BAI) were administered to all participants. In the present study, a cut-off value of 35 was used for the CTQ scale. The patients with CTQ scores lower than 35 (50%, n = 19, Group 1) and the patients with CTQ scores above 35 (50%, n = 19, Group 2) were compared. Results: The comparison of TEMPS-A and its subscale scores in the JME patients in the groups with CTQ scores above or below a cut-off value detected significant differences between the groups in depressive and irritable temperament scores. The mean BDI scores were also different between the two groups. Furthermore, a significant positive correlation was detected between the disease duration, anxiety, and depression scores in the JME patients. A significant relationship was detected between the emotional neglect subscale score of the JME patients and the BDI scores. A significant positive correlation was found between the total disease duration, BDI, and BAI. Significant moderate-level relationships were found between the BDI score and irritable, depressive, cyclothymic, and anxious temperaments and between the BAI score and irritable, depressive, cyclothymic, and anxious temperaments. Conclusion: Several temperamental features of JME patients are related to CTs. More depressive symptoms are seen in JME patients with higher disease durations.

Motor hyperactivation during cognitive tasks: An endophenotype of juvenile myoclonic epilepsy.

OBJECTIVE: Juvenile myoclonic epilepsy (JME) is the most common genetic generalized epilepsy syndrome. Myoclonus may relate to motor system hyperexcitability and can be provoked by cognitive activities. To aid genetic mapping in complex neuropsychiatric disorders, recent research has utilized imaging intermediate phenotypes (endophenotypes). Here, we aimed to (a) characterize activation profiles of the motor system during different cognitive tasks in patients with JME and their unaffected siblings, and (b) validate those as endophenotypes of JME. METHODS: This prospective cross-sectional investigation included 32 patients with JME, 12 unaffected siblings, and 26 controls, comparable for age, sex, handedness, language laterality, neuropsychological performance, and anxiety and depression scores. We investigated patterns of motor system activation during episodic memory encoding and verb generation functional magnetic resonance imaging (fMRI) tasks. RESULTS: During both tasks, patients and unaffected siblings showed increased activation of motor system areas compared to controls. Effects were more prominent during memory encoding, which entailed hand motion via joystick responses. Subgroup analyses identified stronger activation of the motor cortex in JME patients with ongoing seizures compared to seizure-free patients. Receiver-operating characteristic curves, based on measures of motor activation, accurately discriminated both patients with JME and their siblings from healthy controls (area under the curve: 0.75 and 0.77, for JME and a combined patient-sibling group against controls, respectively; P < .005). SIGNIFICANCE: Motor system hyperactivation represents a cognitive, domain-independent endophenotype of JME. We propose measures of motor system activation as quantitative traits for future genetic imaging studies in this syndrome.

The role of cortisol in trust behavior: Results from an experimental study on healthy controls and patients with juvenile myoclonic epilepsy.

Trust is one of the foundations of human society and pervades all aspects of human live. Research on humans focused primarily on identifying the biological basis of trust behavior in healthy subjects, and this evidence hints to certain brain areas, hormones, and genetic factors to be fundamentally involved. The contribution of cortisol in trust has not yet elicited much attention in research, especially when specifically examined at basal cortisol levels. Trust has been previously studied in some neurological diseases but not in patients with epilepsy, and the influence of hormones on trust in these diseases remains yet unknown. Against this background, we designed an experimental study with a group of patients with juvenile myoclonic epilepsy and a group of healthy controls to compare trust behavior and plasma cortisol levels between the two groups. This economic game is frequently used in research to operationalize trust behavior. All participants further underwent neuropsychological assessment. Our results showed that there was no significant difference in trust behavior during the trust game, but a trend toward lower trust in patients. Furthermore, there was a significant difference in cortisol levels between groups with lower levels in patients. Interestingly, cortisol levels correlated with trust only in the patient group, but not in the control group. Future studies should specifically differentiate the effect of induced cortisol increases (e.g., acute stress) versus the effect of basal cortisol levels reflecting homeostasis or chronic stress on trust behavior and leverage the potential of comparison between patients and healthy controls.

Meta-analysis of response inhibition in juvenile myoclonic epilepsy.

BACKGROUND: Patients with juvenile myoclonic epilepsy (JME) show evidence of cognitive impulsivity that may be linked to later adverse psychosocial outcomes. Here, we quantify the strength of association and estimate effect size (ES) of response inhibition by pooling available evidence in a meta-analysis. METHODS: We conducted a systematic review of the literature using Ovid MEDLINE and Ovid EMBASE databases (covering 2001-2019) with a search strategy using combinations of the specific Medical Subject Headings (MeSH) terms 'juvenile myoclonic epilepsy, cognitive impulsivity, response inhibition, Stroop, cognition, personality, traits' using the 'explode' feature where possible. We also searched within references of retrieved articles. We included studies reporting ESs describing established measures of response inhibition in teenage and adult patients with JME. RESULTS: Using the ESs pooled from 16 studies comprising 1047 patients and controls, we found ESs for response inhibition to be homogeneous with a significant moderate mean ES of d = 0.50 (95% confidence interval [CI]: 0.37-0.63). CONCLUSIONS: We confirm that reduced response inhibition is a consistently observed homogeneous trait in patients with JME.

Cognitive functioning in new-onset juvenile myoclonic epilepsy.

PURPOSE: Juvenile myoclonic epilepsy (JME) is a common genetic generalized epilepsy syndrome. Adult patients with JME have shown a neuropsychological profile suggestive of subtle frontal dysfunction, but studies of cognitive functioning in the early phases of JME are rare. We analyzed the cognitive performance data of 18 patients who had undergone a neuropsychological assessment either at the time of JME diagnosis and before the initiation of an antiepileptic drug (AED) treatment (11 patients) or during the first 6 years after JME diagnosis (seven patients). METHODS: The cognitive performance of the18 patients with JME (mean age: 18.1, range: 15-33 years) and 18 healthy controls (mean age: 18.7, range: 15-25 years) was compared in a retrospective study. The assessed cognitive domains were visuomotor speed, attention, executive function, and verbal memory. RESULTS: The patients with JME and the healthy controls did not differ in any of the assessed cognitive domains. The clinical variables did not correlate to cognitive performance. Furthermore, cognitive performance did not differ between the patients evaluated at the time of diagnosis and before the initiation of AEDs and the patients evaluated during the first 6 years after diagnosis and with an AED treatment. CONCLUSIONS: The cognitive performance of patients with new-onset JME was similar to healthy controls. We could not detect the frontal dysfunction that has been suggested to be associated with JME. Patients were in adolescence or early adulthood with a short duration of epilepsy, which may have contributed to the discovery of no cognitive impairments.

Dysfunctional personality beliefs and executive performance in patients with juvenile myoclonic epilepsy.

BACKGROUND: This article intends to verify the association of dysfunctional beliefs of personality disorders with the executive performance in people with juvenile myoclonic epilepsy (JME). METHODS: Fifty-two patients (35 women, 67.3%) with JME aged 18-50 yrs. (32.3 ±â€¯9.7) were evaluated between May 2017 and April 2018 and compared with controls. All subjects were submitted to the Personality Beliefs Questionnaire (PBQ) (Beck & Beck, 1991; Savoia et al., 2006), Dysexecutive Questionnaire (DQ; Wilson et al., 1996; Macuglia et al., 2016), estimated intelligence quotient (IQ) using Vocabulary and Block Design tests, attention and executive functions evaluation (Controlled Oral Word Association (COWA), Digit Span, Trail Making Tests (TMT) A and B, Stroop and Wisconsin Card Sorting Test (WCST)). The inclusion criteria were as follows: diagnosis of JME (ILAE, 1989); age ≥18 yrs., schooling ≥ 11 yrs. and IQ ≥70. The inclusion criteria for the control group were the same except diagnosis of epilepsy. RESULTS: Compared with controls, patients presented higher scores in PBQ for personality disorders, namely Narcissistic (z = -0.79; p < 0.001), Borderline (z = -0.58; p = 0.002), Paranoid (z = -0.43; p = 0.017), and Histrionic (z = -0.39; p = 0.041). Executive functions were impaired when compared with controls in TMT A (z = -0.97; p = 0.038), TMT B (z = -0.65; p = 0.023), and COWA (z = -0.51; p = 0.001). Patients showed higher WCST scores for Errors (z = -1.62; p ≤ 0.001), Perseverative Errors (z = -0.77; p = 0.001), Non-Perseverative Errors (z = -1.01; p = 0.001), Conceptual Level Response (z = -1.56; p ≤ 0.001), Completed Categories (z = -2.12; p = 0.002), and Failure to Maintain Context (z = -0.49; p = 0.015). Personality Beliefs Questionnaire results showed correlation with lower values in TMT A, Antisocial (r = -0.298; p = 0.032), Narcissistic (r = -0.303; p = 0.029), Schizoid (r = - 0.410; p = 0.003), Histrionic (r = -0.341; p = 0.013), Passive-aggressive (r = -0.341; p = 0.015), and Obsessive-compulsive (r = -0.319; p = 0.021); TMT B results showed a trend for Obsessive-compulsive traits (r = -0.261; p = 0.052); COWA was correlated to Dependent (r = 0.319; p = 0.021); and Digit Span to Passive-aggressive (r = 0.287; p = 0.039). On WCST, Failure to Maintain Context was correlated to Avoidant (r = 0.335; p = 0.017). The DQ was not correlated with PBQ. CONCLUSION: People with JME presented dysfunctional beliefs of personality disorder that were correlated with executive dysfunction. These findings reinforce the need for psychological rehabilitation in these patients.

[Clinical and psychosocial factors associated with seizure control in patients with juvenile myoclonic epilepsy]. / Factores clínicos y psicosociales asociados al control de crisis en pacientes con epilepsia mioclónica juvenil.

AIM: To identify the clinical and psychosocial factors of a Colombian cohort of patients with juvenile myoclonic epilepsy and its relation to crisis control. PATIENTS AND METHODS: Retrospective cohort study of patients over 14 years of age with a diagnosis of juvenile myoclonic epilepsy, with 12 months of follow-up in an epilepsy program, where the sociodemographic and clinical characteristics and the survey for the assessment of relapse conditioners. A bivariate and multivariate analysis was performed using binary logistic regression. RESULTS: Clinical records between November 2014 and December 2016 where 145 patients with a diagnosis of juvenile myoclonic epilepsy who met the selection criteria were evaluated. The factors associated with crisis control were: health plan, accessibility, personal attitudes, history of consumption, drug resistance, and crisis number per month; of these, health plans, personal attitudes, and crisis number per month persist with statistical significance after the logistic regression analysis. CONCLUSION: Psychosocial factors not only impact on crisis control, added to clinical factors explain 40% of crisis control in patients with juvenile myoclonic epilepsy and therefore affect their long-term prognosis.

TITLE: Factores clínicos y psicosociales asociados al control de crisis en pacientes con epilepsia mioclónica juvenil.Objetivo. Identificar los factores clínicos y psicosociales de una cohorte colombiana de pacientes con epilepsia mioclónica juvenil y su relación con el control de las crisis. Pacientes y métodos. Estudio de cohortes retrospectivo de pacientes mayores de 14 años con diagnóstico de epilepsia mioclónica juvenil, con 12 meses de seguimiento en un programa de epilepsia, donde se evaluaron las características sociodemográficas y clínicas y la encuesta para la valoración de los condicionantes de recaída. Se realizó un análisis bivariado y multivariado mediante regresión logística binaria. Resultados. Registros de historias clínicas entre noviembre de 2014 y diciembre de 2016, donde se evaluó a 145 pacientes con diagnóstico de epilepsia mioclónica juvenil que cumplieron los criterios de selección. Los factores asociados al control de las crisis fueron: plan de salud, accesibilidad, actitudes personales, antecedente de consumo, farmacorresistencia y número mensual de crisis; de éstos, plan de salud, actitudes personales y número mensual de crisis persisten con significación estadística tras el análisis de regresión logística. Conclusión. Los factores psicosociales no sólo influyen en el control de las crisis sino que, sumados con algunos factores clínicos, podrían explicar el 40% del control de las crisis en los pacientes con epilepsia mioclónica juvenil y, por ende, influir en su pronóstico a largo plazo.

Dissociation between decision making under ambiguity and risk in patients with juvenile myoclonic epilepsy.

INTRODUCTION: Decision making (DM) is one aspect of impulsivity that can be defined by the ability to decide between two or more options in a given situation. To date, there are at least two types of DM that differ in the level of uncertainty, and how much information about consequences is provided. In this study, we aimed to evaluate the two domains of DM - under risk and ambiguous - with a comprehensive evaluation in a group of patients with juvenile myoclonic epilepsy (JME), and correlate with patients' characteristics, clinical variables, and neuropsychological evaluation for executive functions. METHODS: We evaluated 35 patients with JME and 39 healthy controls using the Iowa Gambling Task for DM under ambiguity and the Game Dice Task for DM under risk. We assessed the performance in Iowa Gambling Task and Game Dice Task through net scores, safe and risky choices, besides the type of decisions across time. RESULTS: Patients with JME had a higher number of risky choices compared to controls in the Game Dice Task. There was no significant difference between patients and controls in the Iowa Gambling Task. However, patients with higher seizure frequency had worse scores on decks C and D (safe choices) from the Iowa Gambling Task. CONCLUSION: Patients with JME have worse performance on DM under risk. The same was not observed for DM under ambiguity. Epilepsy-related factors and the presence of psychiatric disorders, but not executive dysfunction, were associated with a lower tendency for safe choices. These findings showed a dissociation between DM processes in patients with JME and a tendency to make disadvantageous decisions with measurable risks.

Revisiting personality in epilepsy: Differentiation of personality in two epilepsies starting in adolescence.

PURPOSE: The purpose of this study was to investigate personality characteristics and clinical parameters in two well-defined epilepsies: mesial temporal lobe epilepsy related to hippocampal sclerosis (MTLE/HS) and juvenile myoclonic epilepsy (JME) through NEO Revised Personality Inventory (NEO-PI-R) and Neurobehavior Inventory (NBI) standardized instruments. METHODS: One hundred patients undergoing corticoamygdalohippocampectomy (CAH), 100 patients with JME, and 100 control subjects answered the personality measures. Clinical parameters such as psychiatric symptoms, seizure frequency, duration of epilepsy, and side of the lesion in MTLE/HS group were investigated. Statistical analysis consisted of the mean and standard deviation (SD) of each variable. Student's t-test or Fisher exact test were used according to the variable studied. RESULTS: The three groups were within the average range of NEO-PI-R and NBI, although 'tendencies' and differences were demonstrated. The MTLE/HS and control subjects had a similar profile: low scores in Neuroticism and high in Conscientiousness (r = -0.330; p < 0.001/r = -0.567; p < 0.001, respectively) in opposition to what occurred in JME, low in Conscientiousness and high in Neuroticism (r = -0.509; p = 0.005). The NBI 'sense of personal destiny' trait was higher (3.15; p = 0.003) in MTLE/HS than in JME and controls. The JME 'law and order' scores were lower than in other groups (p = 0.024). A tendency towards specific NBI traits differentiates MTLE/HS (Factor 3) from JME (Factor 1) groups. Psychiatric symptoms and seizure frequency were correlated with worse scores in NBI and, especially, in Neuroticism domain of NEO-PI-R. CONCLUSION: Specific personality features were linked to each epileptic disease. These findings highlight the importance of considering unique features linked to epilepsy conditions in daily clinical observation to develop support programmes.

Psychosocial complications in juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy (JME) constitutes about 10% of all epilepsies. Because of executive dysfunction, people with JME may be prone to impulsivity and risk-taking behavior. Our aim was to investigate whether psychosocial issues associated with impulsivity are more prominent in people with JME than in those with other types of genetic generalized epilepsy (GGE). Patients with GGE were recruited retrospectively through the Drammen Hospital records in Buskerud County, Norway, 1999-2013. They were invited to a semi-structured interview, either at the hospital or at home. Ninety-two patients with JME and 45 with other types of GGE were interviewed. Variables were evaluated in terms of their association with JME versus other GGE diagnosis using a logistic regression model. Juvenile myoclonic epilepsy was associated with use of illicit recreational drugs and police charges, although with borderline significance (odds ratio [OR] 3.4, p = 0.087 and OR 4.2, p = 0.095); JME was also associated with being examined for attention-deficit hyperactivity disorder (ADHD) in females (OR 15.5, p = 0.015), a biological parent with challenges like addiction or violent behavior (OR 3.5, p = 0.032), and use of levetiracetam (OR 5.1, p = 0.014). After controlling for group differences, we found psychosocial complications to be associated with JME, potentially influencing the lives of the individuals and their families to a greater extent than the seizures per se. Thus, JME should be considered a disorder of the brain in a broader sense than a condition with seizures only.

The cognitive phenotype of idiopathic generalized epilepsy.

OBJECTIVE: Dysexecutive traits have been described in idiopathic generalized epilepsy (IGE), but studies mainly focused on juvenile myoclonic epilepsy (JME). To better understand the neuropsychology of IGE, more research is needed on syndromes other than JME, controlling potential confounding factors as the cognitive effects of valproate and epileptic discharges (ED). We describe the neuropsychological profile of a group of patients with different syndromes of IGE including simultaneous video electroencephalography (EEG). METHODS: We performed a comprehensive cognitive and neuropsychiatric evaluation with video-EEG on 61 adults with IGE (JME 19; IGE with generalized tonic-clonic seizures [GTCS] alone [IGE-GTCS] 22; childhood absence epilepsy [CAE] or juvenile absences epilepsy [JAE] persisting in adulthood 20). We compared results between patients (globally and by syndrome) and a control group of 21 individuals (similar age, educational level); p-values were adjusted for multiple testing according to a 0.05 false discovery rate. RESULTS: Patients obtained significantly lower results than controls on visuospatial working memory, processing speed, cognitive flexibility and strategy, abstract visuospatial reasoning, arithmetic, and acquired knowledge. While CAE/JAE showed the lowest scores on cognitive assessment and highest anxiety index, IGE-GTCS showed the most favorable scores. Most tests were not influenced by valproate intake, and the dose did not correlate with cognitive performance in the test that yielded differences between patients and controls. Epileptic discharges during assessment were not frequent (10 patients, 1-4 tests). SIGNIFICANCE: Our findings suggest that patients with IGE have significantly lower abilities in various executive functions and acquired knowledge, compared to population of same age and education. The low frequency of ED on simultaneous video-EEG and absence of correlation of scores with valproate dose reinforce that the obtained results are due to a cognitive phenotype in IGE. This phenotype may be influenced by syndrome, and patients with CAE/JAE persisting in the adult may have a wider neuropsychiatric impairment.

Frontal lobe cognitive functions and electroencephalographic features in juvenile myoclonic epilepsy.

PURPOSE: The study aimed to examine the relationship between frontal lobe functions and interictal electroencephalography (EEG) discharge characteristics of patients with juvenile myoclonic epilepsy (JME). METHOD: Thirty patients with JME who had EEG with asymmetrical generalized discharge (aEEG), 15 patients with JME who had EEG with symmetrical generalized discharge (sEEG), and 15 healthy controls were included in the study. To evaluate attention, the digit span and Corsi block tests were used; to evaluate memory, we applied verbal and visual memory tests; to evaluate frontal lobe functions, we used clock drawing, verbal fluency, the Stroop test, trail making, mental control, and antisaccadic eye movement tests as well as the continuous performance (CPT) tests. ETHICAL CONSIDERATIONS: The research was approved by the Research Ethics Committee of the Bakirkoy Research and Training Hospital for Psychiatry, Neurology, Neurosurgery, with protocol number: 41340010/4891-262, date: 05.02.2013. RESULTS: The mean age of the 45 patients with JME was 22.89 ±â€¯6.77 years, and 34 (75.6%) were female. The age at onset of seizures and disease duration of the patients with JME was 15.56 ±â€¯4.06 years (range, 9-26 years) and 7.20 ±â€¯5.59 years (range, 1-25 years), respectively. All patients were under valproate (VPA) treatment, and the mean VPA dosage was 783.33 ±â€¯379.14 mg/day. Patients with JME scored worse than the control group in attention, memory, and frontal lobe functions. In patients with aEEG, scores of attention, memory, and frontal lobe function tests were lower than in patients with sEEG; however, with the exception of CPT, they were not statistically significant. CONCLUSION: Cognitive functions in JME have been shown to be impaired. Furthermore, we concluded that the frontal lobe cognitive functions may be worse in patients with aEEG than in patients with sEEG. Further studies in patients with JME with aEEG abnormalities may lead to a better understanding of the pathophysiology of JME.

Higher IQ in juvenile myoclonic epilepsy: Dodging cognitive obstacles and "masking" impairments.

Executive deficits and impulsiveness are extensively reported in juvenile myoclonic epilepsy (JME). Previous literature suggests that intelligence may mediate these deficits. In this study, we evaluated and compared the performance of adults with JME with high and low intelligence quotient (IQ) and controls on tasks for executive function (EF) and impulsive traits. We investigated the neuropsychological performance of 53 adults with JME and below average IQ (57% women; 26.9 [±7.88] years; mean IQ: 89.8 [±5.1]), 26 adults with JME and average or above average IQ (53.8% women; 28.2 [±9.33] years; mean IQ: 110.7 [±8.3]), 38 controls with below average IQ (55% women; 28.4 [±8.4] years; mean IQ: 90.1 [±5.8]), and 31 controls with average or above average IQ (61.3% women; 32.20 [±11.3] years; mean IQ: 111.6 [±10.5]) with a comprehensive battery of neuropsychological tests that measure executive/attentional function. Impulsive traits were assessed using the Cloninger et al.'s Temperament and Character Inventory (novelty seeking (NS) domain). The group with JME with higher IQ presented worse performance compared with controls with higher IQ on Controlled Oral Word Association (COWA) and Wisconsin Card Sorting Test (WCST) (errors). This group showed worse performance than controls with lower IQ on Stroop Color-Word Test (SCT) 1, Trail Making (TM) A, COWA, and WCST (errors). Patients with lower IQ showed worse performance than controls with higher IQ on Digit Span Forward (DSF), Digit Span Backward (DSB), SCT1, SCT2, SCT3, TM A, COWA, and WCST (errors and failure to maintain set). Patients with lower IQ showed worse performance than controls with lower IQ on DSF, DSB, SCT1, SCT2, SCT3, TM A, TM B, COWA, and WCST (errors and failure to maintain set). Patients from groups with low and high IQ showed higher scores than controls with higher and lower IQ on impulsivity for NS1 and NS2 (except for patients with higher IQ versus controls with lower IQ). Adults with JME and higher IQ show less evidence of EF deficits compared with those with JME and below average IQ, suggesting that a higher degree of intellectual efficiency may act as a compensatory mechanism. However, it does not minimize some aspects of impulsive traits. Patients with JME and higher cognitive reserve may create strategies to dodge their cognitive obstacles. In this context, intelligence may protect and, at the same time, "mask" impairments that could be detected earlier.

Praxis-induced myoclonia: From the neurophysiologist to the patient perspective.

PURPOSE: To characterize semiology and EEG features of praxis-induced (PI) myoclonia and to describe the subjective perception of juvenile myoclonic epilepsy (JME) patients with this reflex trait. METHODS: Patients with JME who presented myoclonia during a Video-EEG Neuropsychological Protocol were selected. We analyzed the semiology of upper limbs myoclonia and the ictal EEG patterns on Video-EEG. We explored the subjective aspects of PI by performing a semi-structured interview to each patient. RESULTS: 15 patients experienced 59 upper limbs myoclonia. Jerks were more frequently asymmetric or unilateral (32/59); a bilaterally symmetric pattern of all myoclonia was observed in only five patients. Ictal pattern was polyspike-wave (PSW) in 31/59 myoclonic seizures and spike-and-wave (SW) in 28/59. Six patients started perceiving myoclonia while learning a new skill or practicing a previously learned skill in a more stressful context. For most patients, PI-myoclonia were a source of anxiety. PI persisted despite antiepileptic medications in 10 patients. CONCLUSIONS: Electroclinical features of PI-myoclonia were more heterogeneous than traditionally described. Ictal pattern of SW was almost as frequent as classical PSW. Patients described the influence of learning new skills and anxiety on PI. Their subjective perception let us understand the impact of this reflex trait.