Electroclinical aspects and therapy of Han patients with juvenile myoclonic epilepsy in northern China

ObjectiveThe objective of this study was to assess the electroclinical aspects and treatment of Han patients with juvenile myoclonic epilepsy (JME) in northern China.MethodsOne hundred fifty-six outpatients with JME from six epilepsy centers, between January 2011 and June 2012, were followed up for at least two years. They underwent twenty-four-hour video-EEG recording. Brain imaging was performed using magnetic resonance imaging (MRI). Clinical aspects, electroencephalographic (EEG) features, and antiepileptic drugs (AEDs) received were reviewed.ResultsGeneralized tonic–clonic seizures (GTCS) were found in 150/156 patients. Delay of diagnosis was 4.60 ± 9.92 years. Photosensitivity was more common in eye closure condition during IPS in patients with JME; in addition, patients with JME with myoclonic seizures (MS) and GTCS as seizure types were likely to present photoparoxysmal responses (PPRs). The 82 nontreated patients showed a median latency to first interictal or ictal generalized spike–wave discharge (GSWD) of 50 min (IQR: 22–102 min). The first GSWDs were recorded in 63%, 76%, 90%, and 98% patients within one, two, three, and 4 h, respectively; only 2% of patients had first GSWDs after 4 h. One hundred eleven patients (111/156) chose extended-release valproate (VPA) at daily doses ≤ 1000 mg. The percentages of seizure-free patients among MS, GTCS, and absence seizure (AS) groups were 88.3%, 99.0%, and 94.9%, respectively.ConclusionPhotoparoxysmal responses were more common in patients with JME with MS and GTCS and rare in patients with JME with MS and AS in northern Chinese Han patients. Most patients with JME in northern China chose VPA as first therapeutic choice, and low dose (500 to 1000 mg daily) of extended-release VPA may be an optimal choice for them. Video-EEG monitoring for at least 4 h may be helpful in detecting the first interictal or ictal GSWD in patients with potential JME. Moreover, video-EEG monitoring performed at about 9 o'clock in the morning with patients in the awake state might be useful to find the first GSWD. For JME diagnosis, Class II criteria are more helpful than Class I counterparts, the latter yielding more missed diagnoses.     

Cortical processing during smartphone text messaging

ObjectiveThe objective of this study was to report the EEG features of text messaging using smartphones.MethodsOne hundred twenty-nine patients were prospectively evaluated during video-EEG monitoring (VEM) over 16 months. A reproducible texting rhythm (TR) present during active text messaging with a smartphone was compared with passive and forced audio telephone use, thumb/finger movements, cognitive testing/calculation, scanning eye movements, and speech/language tasks in patients with and without epilepsy. Statistical significance was set at p < 0.05.ResultsTwenty-seven patients with a TR were identified from a cohort of 129 (93 female, mean age: 36; range: 18–71) unselected VEM patients. Fifty-three out of 129 patients had epileptic seizures (ES), 74/129 had nonepileptic seizures (NES), and 2/129 were dual-diagnosed. A reproducible TR was present in 27/129 (20.9%) specific to text messaging (p < 0.0001) and present in 28% of patients with ES and 16% of patients with NES (p = NS). The TR was absent during independent tasks and audio cellular telephone use (p < 0.0001). Age, gender, epilepsy type, MRI results, and EEG lateralization in patients with focal seizures were unrelated (p = NS).ConclusionsOur results suggest that the TR on scalp EEG represents a novel technology-specific neurophysiological alteration of brain networks. We propose that cortical processing in the contemporary brain is uniquely activated by the use of PEDs.SignificanceThese findings have practical implications that could impact industry and research in nonverbal communication.     

High-dose versus low-dose valproate for the treatment of juvenile myoclonic epilepsy: Going from low to high

Juvenile myoclonic epilepsy (JME) is a genetic generalized epilepsy accounting for 3–12% of adult cases of epilepsy. Valproate has proven to be the first-choice drug in JME for controlling the most common seizure types: myoclonic, absence, and generalized tonic–clonic (GTC). In this retrospective study, we analyzed seizure outcome in patients with JME using valproate monotherapy for a minimum period of one year. Low valproate dose was considered to be 1000 mg/day or lower, while serum levels were considered to be low if they were at or below 50 mcg/dl. One hundred three patients met the inclusion criteria. Fifty-six patients (54.4%) were female. The current average age was 28.4 ± 7.4 years, while the age of epilepsy onset was 13.6 ± 2.9 years. Most patients corresponded to the subsyndrome of classic JME. Forty-six (44.7%) patients were free from all seizure types, and 76 (73.7%) patients were free from GTC seizures. No significant difference was found in seizure freedom among patients using a low dose of valproate versus a high dose (p = 0.535) or among patients with low blood levels versus high blood levels (p = 0.69). In patients with JME, it seems appropriate to use low doses of valproate (500 mg to 1000 mg) for initial treatment and then to determine if freedom from seizures was attained.     

Duration of electroencephalographic recordings in patients with epilepsy

PurposePrevious studies have demonstrated different diagnostic yields with electroencephalography (EEG). Due to the small sample sizes or different patient populations (outpatients or inpatients only) in these previous studies, the clinical use of routine EEG and outpatient/inpatient video-EEG monitoring (VEM) needs further clarification. In this study, we investigated EEGs obtained from patients referred by epileptologists; by comparing the results of different EEG methods, we sought to determine the optimal durations and specific types of EEG recordings for different clinical situations.MethodsThe data from 335 routine EEGs, 281 3 h outpatient VEMs, and 247 inpatient VEMs (>48 h) were reviewed. We analyzed the latency to the first epileptiform discharge or clinical event.ResultsIn patients undergoing outpatient VEMs, 48% of the first epileptiform discharges appeared within 20 min, and 64% appeared within 30 min. In patients undergoing inpatient VEMs, 21.2% had their first attack within 3 h. The second peak of event occurrence was during the 33rd–36th h. Only 3.5% of the seizures were recorded after 57 h. The detection rate of epileptiform discharges was higher for 3 h outpatient VEM than for routine EEG (54.1% versus 16.4%, p < 0.01). Epileptic and/or nonepileptic events were recorded in 45.8% of the inpatient VEMs, the diagnostic yield of which was higher than for outpatient VEMs (p < 0.01). Since the patients in this study had been selected to limit the bias between each group, the diagnostic yield of EEGs in this study are likely to have been higher than those found in routine practice. Patients with generalized epilepsy had a shorter latency to the first epileptiform discharge compared to patients with localization-related epilepsy (mean, 22.1 min versus 33.9 min, p < 0.05).ConclusionsTwo-thirds of epileptiform discharges were detected within 30 min of VEM. A 30-min recording is recommended for routine EEG examinations that aim to detect epileptiform discharges. A 3 h outpatient VEM is a reasonable option when a routine EEG fails to detect epileptiform discharges. The latency to the first epileptiform discharge was shorter in patients with generalized epilepsy than in patients with localization-related epilepsy. 48 h of inpatient VEM might be adequate for detecting the target events.     

Evaluating the use of prolonged video-EEG monitoring to assess future seizure risk and fitness to drive

ObjectiveThe use of prolonged video-electroencephalography monitoring (VEM), rather than routine electroencephalography (EEG), in predicting the risk of future seizures in patients with epilepsy is not well studied. A longer period of monitoring could be more likely to capture either ictal or interictal epileptiform activity. This information may better assist clinical decision making on driving fitness. The goal of this study was to evaluate the use of 6-hour prolonged VEM versus routine EEG in the assessment of future seizure risk and driving fitness for patients with epilepsy.MethodsData on consecutive patients referred for 6-hour prolonged VEM were retrospectively analyzed. Criteria were developed that combined EEG findings and clinical factors to determine each patient's fitness to drive. Seizure relapse outcomes were followed over 2 years.ResultsOf 34 patients, 27 were considered safe to drive following prolonged VEM. Five (19%) of these 27 patients had seizure relapses; all had an obvious precipitant(s) identified including sleep deprivation, excessive alcohol, and missed medication doses. Seven of the 34 patients were deemed unsafe to drive. All seven (100%) had seizure relapses, with unprovoked seizures in four patients. The relative risk of seizure in patients deemed unfit to drive was 5.4 (P = 0.00015). If only the routine EEG component of the recordings were used with the criteria, the relative risk would have been 3.4 (P = 0.037), with nearly double the number of active drivers having seizures. The majority of patients (76%) in this study had idiopathic generalized epilepsy, with a relative seizure risk of 4.0 (P = 0.002) for patients deemed unfit to drive in this subgroup. The focal epilepsy group was small (eight patients) and did not quite achieve statistical significance.ConclusionSix-hour VEM improves the evaluation of driving fitness by better predicting the risk of subsequent seizure relapse for idiopathic generalized epilepsy and possibly focal epilepsy. Prolonged monitoring is superior to routine EEG. Ongoing avoidance of seizure-provoking factors remains paramount to driving safety.     

Electroencephalographic features of benign adult familial myoclonic epilepsy

ObjectiveTo investigate electroencephalographic (EEG) features of benign adult familial myoclonic epilepsy (BAFME).MethodsWe reviewed interictal EEG features in patients with BAFME treated between April 2005 and November 2012 at a tertiary referral center. The diagnostic criteria for BAFME were the presence of infrequent generalized tonic–clonic seizures, myoclonus or myoclonic seizures, and autosomal dominant inheritance. Interictal EEG findings of epilepsy with generalized tonic–clonic seizure only (EGTCS) were reviewed for comparison. We randomly selected 10 generalized spike/polyspike and wave complexes (GSW) for each BAFME patient and measured the duration of them. Photic stimulation and hyperventilation were performed in all.ResultsNineteen (eight men, 11 women) patients with BAFME were included in this study. The mean frequency of GSW was 4.3 ± 1.0 Hz (mean ± SD, n = 14) in BAFME and 3.2 ± 0.8 Hz (n = 10) in EGTCS. There was a statistically significant difference (p = 0.008) between the two. Photoparoxysmal responses (PPR) were noted in 18 (95%) patients with BAFME but 1 (10%) with EGTCS.ConclusionFaster frequency of GSW, compared with that in EGTCS, accompanied by PPR may be characteristic EEG features of BAFME.SignificanceThese findings may lead the diagnosis of BAFME.     

Factors associated with lack of response to valproic acid monotherapy in juvenile myoclonic epilepsy

PurposeTo determine factors associated with lack of response to valproic acid (VPA) in juvenile myoclonic epilepsy (JME).MethodRetrospective analysis of clinical and EEG data of 201 patients with JME who had at least 3 years follow up was performed. Psychiatric evaluation was performed using ICD-10 by structured clinical interview. Patients were divided into two groups: VPA responders (seizure free for 2 or more years) and those with lack of response to VPA. Effect size for non-response and correlations for variables significantly different between the groups was performed, the findings were confirmed by ROC curves.ResultsThe mean duration of follow up was 7.75 (range 3–12) years; 55.2% were males. Focal semiologic features were noted is 16%. EEG was abnormal in 67%; focal EEG abnormalities were noted in 32.8%. Coexisting psychiatric disorders (PDs) were found in 33.3%. Lack of response to VPA was noted in 19%. Diagnosis of PDs and focal EEG abnormalities significantly increased the risk of VPA non-responsiveness by 5.54 (95% CI of 2.60–11.80; p < 0.0001) and 3.01 times respectively (95% CI of 1.40–6.47; p < 0.008). Diagnosis of PDs showed significant correlation (r = 0.332; p < 0.0001) and association (AUC 0.700; p < 0.0001) with lack of response to VPA. Though focal EEG abnormalities increased the chances, it did not correlate with lack of response to VPA.ConclusionLack of response to VPA was noted 19% of patients with JME. Coexisting PDs showed significant correlation and association with lack of response to VPA.     

Distinct domains of impulsivity are impaired in juvenile myoclonic epilepsy but not in temporal lobe epilepsy

ObjectiveThe Barratt Impulsiveness Scale (BIS-11) is the most widely used questionnaire to study impulsivity in persons with psychiatric disorders, but it has rarely been applied to persons with epilepsy. The present study aimed to evaluate the usefulness of BIS-11 as a tool to explore impulsivity in two distinct epilepsy syndromes.MethodThe BIS-11 was applied to 20 patients with juvenile myoclonic epilepsy (JME) (32.5 ± 8.95 years old), 20 patients with temporal lobe epilepsy (TLE) (37.7 ± 13.25 years old), and 26 healthy controls (31.86 ± 11.25 years old). The scores in motor, attentional, and lack of planning impulsivity were compared between groups.ResultsPatients with JME showed higher scores than patients with TLE and controls in all domains: motor (JME vs TLE: 28.60 vs 13.25 (mean score), p < 0.001 and JME vs controls: 28.60 vs 14.12, p < 0.001), attentional (JME vs TLE: 21.55 vs 13.45, p < 0.001 and JME vs controls: 21.55 vs 14.88, p < 0.001) and nonplanning (JME vs TLE: 28.05 vs 13.10, p < 0.001 and JME vs controls: 28.05 vs 16.15, p < 0.001).ConclusionHigher BIS-11 scores in all domains of impulsivity [i.e., motor, attentional, and lack of planning] corroborated previous findings described in patients with JME. On the other hand, BIS-11 could not demonstrate problem solving and inhibitory control deficits related to impulsive behavior, which were described in patients with TLE. Other behavioral measures may be more sensitive to some aspects of impulsivity in TLE. Our results reinforce the concept that distinct epileptic syndromes require different neuropsychological approaches, especially considering a complex construct such as impulsivity.     

Duration of focal complex,secondarily generalized tonic–clonic,and primarily generalized tonic–clonic seizures — A video-EEG analysis

IntroductionIdentifying seizures with prolonged duration during video-electroencephalographic (EEG) monitoring is of importance to inform clinicians when to start emergency treatment of seizures to prevent status epilepticus. The aims of this study were to assess the clinical and EEG seizure duration (SD) in consecutive patients with epilepsy who underwent prolonged video-EEG monitoring and to identify a seizure type-dependent time point to start emergency treatment based on the likelihood that seizures will not stop spontaneously. Furthermore, we sought to determine predictors of SD and explored the relationship between antiepileptic drug (AED) serum levels and SD.Material and methodsWe retrospectively analyzed 1796 seizures in 200 patients undergoing video-EEG monitoring between January 2006 and March 2008.ResultsFocal simple seizures lasted significantly shorter (clinical SD: 28 s, EEG SD: 42 s) compared with focal complex seizures (clinical SD: 64 s, EEG SD: 62 s), and both seizure types lasted significantly shorter compared with secondarily generalized tonic–clonic seizures (GTCSs; clinical SD: 90 s, EEG SD: 96 s). There was no difference between the duration of the convulsive phase of primary GTCSs (defined as nonfocal) and that of secondarily GTCSs (each 65 s). Cumulative clinical SD (99%) was 7 min in focal complex seizures and 11 min in focal simple seizures. Mixed linear regression model demonstrated that history of status epilepticus (P = 0.034), temporal lobe seizure onset (P = 0.040), and MRI lesions (P = 0.013) were significantly associated with logarithmic EEG SD in focal epilepsies recorded with scalp electrodes. We found significant negative correlations between the AED serum level and the EEG SD in patients treated with monotherapy: carbamazepine (P < 0.001), levetiracetam (P = 0.001), oxcarbazepine (P = 0.001), and valproic acid (P = 0.038) but not with lamotrigine monotherapy and EEG SD.DiscussionBased on the results of this study, we propose 2 min of convulsive seizure activity (irrespective of focal or generalized onset) as a prolonged seizure, which could serve as a time point to consider treatment to prevent status epilepticus. In focal complex seizures, we suggest an upper limit of 7 min, and in focal simple seizures 11 min, as definition of prolonged seizures. History of status epilepticus, temporal seizure onset, and lesional MRI findings are factors associated with significantly longer SD. Negative correlations of carbamazepine, levetiracetam, oxcarbazepine, and valproic acid serum levels and SD suggest a prolonging effect on seizures during withdrawal of these AEDs during video-EEG monitoring sessions.This article is part of a Special Issue entitled “Status Epilepticus”.     

A case–control study of wicket spikes using video-EEG monitoring

PurposeTo investigate clinical characteristics associated with wicket spikes in patients undergoing long-term video-EEG monitoring.MethodsA case–control study was performed in 479 patients undergoing video-EEG monitoring, with 3 age- (±3 years) and gender-matched controls per patient with wicket spikes. Logistic regression was utilized to investigate the association between wicket spikes and other factors, including conditions that have been previously associated with wicket spikes.ResultsWicket spikes were recorded in 48 patients. There was a significantly higher prevalence of dizziness/vertigo (p = 0.002), headaches (p = 0.005), migraine (p = 0.015), and seizures (p = 0.016) in patients with wickets. The majority of patients with wicket spikes did not exhibit epileptiform activity on EEG; however, patients with history of seizures were more likely to have wickets (p = 0.017). There was no significant difference in the prevalence of psychogenic non-epileptic seizures between the groups. Wickets were more common on the left, during sleep, and more likely to be first recorded on day 1–2 of monitoring.ConclusionsPatients with wicket spikes are more likely to have dizziness/vertigo, headaches, migraine, and seizures. Patients with history of seizures are more likely to have wickets. The prevalence of psychogenic non-epileptic seizures is not significantly higher in patients with wickets.     

Local brain activity persists during apparently generalized postictal EEG suppression

ObjectivesPostictal generalized EEG suppression (PGES) frequently occurs after generalized convulsive seizures (GCS) and may be involved in the pathophysiology of sudden unexpected death in epilepsy (SUDEP). It is usually determined using conventional scalp EEG which is likely to miss cerebral activity in deeper brain structures. Here, we examined intracranial EEG activity after GCS to unravel the pattern and extent of local brain activity during apparent PGES on scalp EEG (s-PGES).MethodsWe retrospectively reviewed electroencephalographic data of people with chronic epilepsy who had GCS during presurgical video-EEG monitoring using simultaneous intracranial and scalp EEG (10–20 system) electrodes.ResultsTwenty-five GCS (20 with s-PGES) of 15 patients with an average number of 88 ± 42 intracranial electrode contacts were included. The majority of GCS with s-PGES (18 of 20) displayed persisting or reemerging intracranial EEG activity during apparent PGES on scalp EEG. Three patterns were identified: Pattern 1 (11 GCS, 6 patients) consisted of continuous local interictal activity; Pattern 2 (5 GCS, 5 patients) displayed suppressed EEG activity at all intracranial contacts in the early phase of s-PGES, but reemerging local brain activity before s-PGES dissolved; and Pattern 3 (2 GCS, 2 patients) showed persistent local ictal activity during s-PGES. Persisting intracranial EEG activity at PGES onset on scalp EEG was present in 10 ± 14% (range: 0 to 42%) of all intracranial contacts and mostly in the temporal lobe.ConclusionsOur results reveal that, during apparently generalized postictal EEG suppression, local brain activity persists or reemerges in most GCS. Possible implications of this localized neuronal activity in the context of SUDEP are discussed in the paper.     

Clinical heterogeneity of juvenile myoclonic epilepsy: Follow-up after an interval of more than 20 years

PurposeThe view that juvenile myoclonic epilepsy (JME) is a uniform and life-long disorder is currently being challenged. The aim of this study was to assess the seizure and psychosocial outcome of JME at least 20 years after onset.MethodsIn 1992, 42 patients with JME were identified. In 2012, 37 agreed to a semi-structured interview. In the remaining five, only medical records were available.ResultsOf 40 patients with known seizure outcome, 21 were in remission for >5 years. Seven were off antiepileptic drugs (AEDs), four being seizure free for >10 years. Myoclonic seizures (MC) evolving to generalized tonic–clonic seizures (GTC) were associated with seizure persistence (p = 0.013), whereas >1 year between MC and GTC onset was associated with a trend to GTC remission (p = 0.069). Of 19 patients with uncontrolled seizures, eight experienced remission with second generation AEDs.Favorable psychosocial outcome by interview was found in a third, whereas another third had psychiatric comorbidity, seven with substance or alcohol abuse. Psychosocial and seizure outcome did not correlate.ConclusionThis study corroborates the heterogeneity of JME in terms of seizure and psychosocial outcome, but without a clear association between the two. It confirms that seizure control may persist after AED withdrawal in some and supports MC evolving to GTC as a predictor of seizure persistence. Moreover, it suggests that newer broad spectrum AEDs may improve the prognosis of JME; their impact should be focus of prospective studies.     

Efficacy of dietary therapy for juvenile myoclonic epilepsy

Juvenile myoclonic epilepsy (JME) is often successfully managed with anticonvulsants; however, some patients may have medically resistant seizures. The modified Atkins diet (MAD) has been reported as effective for idiopathic generalized epilepsy and is increasingly being used in adolescents and adults. Since 2006, 8 adolescents and adults have been started on the MAD for JME at Johns Hopkins Hospital. Of these 8 patients, 6 (75%) were female, with a mean age at seizure onset of 10.5 years (range: 6–13 years) and 24.3 years (range: 15–44 years) at MAD initiation. After 1 month, 6 (75%) patients had > 50% seizure reduction, and after 3 months, 5 (63%) patients had > 50% improvement. Several patients found the MAD difficult to adhere to, including 3 patients who reported temporarily increased seizures during periods of noncompliance. In this limited experience, the modified Atkins diet was an efficacious adjunctive therapy for young adults with very medically resistant JME.     

Comparing sleep profiles between patients with juvenile myoclonic epilepsy and symptomatic partial epilepsy: Sleep questionnaire-based study

ObjectivesPatients with epilepsy commonly report excessive daytime sleepiness and daytime fatigue, which may be attributed to the direct effect of seizures, a side effect of antiepileptic drugs or a combination of the two. The aim of the study was to compare sleep profiles in patients with juvenile myoclonic epilepsy (JME) and symptomatic partial epilepsy (PE) in drug naïve and treated patients using standardized sleep questionnaires.MethodsThree study groups: - 1) juvenile myoclonic epilepsy (N = 40) [drug naïve (N = 20); On sodium valproate (SVA) (N = 20)]; 2) symptomatic partial epilepsy (N = 40) [drug naïve (N = 20); On carbamazepine (CBZ) (N = 20)]; 3) healthy controls (N = 40) completed 3 standardized sleep questionnaires – Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index, and NIMHANS Comprehensive Sleep Disorders Questionnaire. Scores were compared using t-test and Chi-squared tests (P ≤ 0.005).ResultsThe mean PSQI scores as well as the proportion of subjects with abnormal PSQI scores were higher in patients with JME and PE compared to controls. Although the mean ESS scores were comparable between patients with epilepsy and controls, the percentage of patients with partial epilepsy having abnormal ESS scores was higher. No significant differences were present between drug naïve and treatment monotherapy groups. Excessive daytime somnolence was reported more often by patients with JME compared to patients with partial epilepsy and controls.ConclusionThis study found that patients with epilepsy have a higher prevalence of poor sleep quality compared to controls. Moreover, a significantly higher percentage of patients with partial epilepsy had higher ESS scores compared to healthy controls. However, there was no difference between ESS and PSQI scores between drug naïve and treated patients with JME or PE.SignificancePoor sleep quality is more prevalent in patients with epilepsy irrespective of the use of antiepileptic medications. Excessive daytime somnolence is more commonly seen in patients with partial epilepsy when compared to the general population.     

Surgical treatment of hypermotor seizures originating from the temporal lobe

PurposeTo describe the characteristics of electroclinical manifestations in patients with hypermotor seizures (HMSs) originating from the temporal lobe.MethodsWe retrospectively reviewed the data of patients who underwent surgical treatments for seizure to identify patients with HMSs of temporal origin. We systematically reviewed patient seizure histories, imaging reports, video-EEG monitoring data, operative records and pathological findings.ResultsEight of the 9 patients reported auras. The ictal behavior included marked agitation in 5 patients and mild agitation in 4 patients. All of the 9 patients exhibited stiffness or dystonia of the upper limb or contralateral limbs during ictus. Seven of the 9 patients completed intracranial recording and at least 3 seizures were recorded for each patient. The intracranial recordings showed ictal activity originating from mesial temporal lobe in 6 patients and the lateral temporal lobe in 1 patient. The time interval of ictal propagation from the temporal to frontal lobe was 15.0 ± 8.3 s. While the time interval from EEG origination to the beginning of hypermotor behavior was 21.0 ± 8.1 s. Brain MRIs revealed hippocampal sclerosis in 3, neoplastic lesion in 1, and normal images in the remaining 5 patients. Patients were followed for 1–5 years after the anterior temporal lobectomy; 7 patients remained seizure-free throughout follow-up.ConclusionSome HMSs can originate from the temporal lobe. In carefully selected patients, surgical resection may lead to good outcomes.     

A randomized open-label observational study to compare the efficacy and tolerability between topiramate and valproate in juvenile myoclonic epilepsy

Juvenile myoclonic epilepsy (JME) is managed with valproate in most patients; however, valproate is an antiepileptic drug that has relatively severe adverse effects, especially in women. We performed a prospective, open-label, randomized observational study for comparison of efficacy and tolerability between topiramate and valproate in patients with JME. The inclusion criteria were patients with newly diagnosed JME or previously diagnosed JME with a history of a poor response or adverse effects to other antiepileptic drugs. The primary endpoint of this study was percentage of patients who were free of myoclonic seizures for 24 weeks in the two groups. The frequency and severity of adverse effects were also assessed. Sixteen patients were randomized to topiramate and 17 to valproate. In the topiramate arm, 11 of 16 patients (68.9%) completed 24-week maintenance therapy and seven of the 11 (64%) were seizure-free. In the valproate arm, 16 of 17 patients (94.1%) completed 24-week follow-up and nine of 16 (56%) were seizure-free. The difference (64% topiramate versus 56% valproate) did not reach statistical significance in this study group (p = 0.08, Fisher’s exact test). However, the severity of adverse effects was significantly different. Only 1 of 10 adverse effects from topiramate was ranked moderate-to-severe (10%), in comparison with severe rankings for 10 of 17 adverse effects from valproate (59%) (p = 0.018, Fisher’s exact test). In summary, the efficacy of topiramate and valproate was not different, but the severity of adverse effects was favourable for topiramate. Our findings suggest that valproate may be replaced with topiramate, especially for the patients with JME who do not tolerate valproate.     

Psychiatric disorders among 165 patients with juvenile myoclonic epilepsy in India and association with clinical and sociodemographic variables

ObjectiveThe current study evaluated the association between clinical variables and psychiatric disorders (PDs) in patients with juvenile myoclonic epilepsy (JME).MethodsConsecutive patients with JME who had at least two years of regular follow-up from May 2011 to April 2014 formed the study population. The association between clinical and sociodemographic data with psychiatric evaluation on structured clinical interview and quality of life in epilepsy — 31 (QOLIE-31) was evaluated using logistic regression analysis.ResultsOut of 165 patients in the current study, 77 (46.6%) patients were diagnosed with PDs; while 50 were categorized to having anxiety disorders, 27 patients had depressive disorders. The mean age of the study population was 25.35 ± 7.6 years with 37.52% women. Patients with PDs had lower overall QOLIE score (55.84 ± 13.07 vs 68.70 ± 11.23, p < 0.001) and lower social function score (80.95 ± 19.22 vs 91.09 ± 14.74, p < 0.001). Being married was the strongest predictor of depressive disorders (β = 8.59; 95% CI, 1.44–51.28; p = 0.018); whereas, lower emotional well-being (β = 0.942; 95% CI, 0.907–0.978; p = 0.002) was the only variable associated with anxiety disorders. Patients with depressive disorders had longer duration of PDs (11.85 ± 8.68 years vs 7.75 ± 6.70 years, p = 0.039), and a majority of them were married (66.7% vs 26.0%, p = 0.001). Patients with depressive disorders scored low on emotional well-being (50.81 ± 14.62 vs 61.02 ± 13.05, p = 0.002), energy levels (52.78 ± 11.71 vs 62.80 ± 10.84, p < 0.001), and social function (70.96 ± 20.69 vs 86.34 ± 16.16, p = 0.001). Depressive disorders were more prevalent among married patients above 35 years of age (5.2% vs 36.8%, p = 0.042).SignificanceNearly half of the patients with JME had coexisting PDs. The psychological profile of anxiety disorders was different from depressive disorders in patients with JME. Depressive disorders were more prevalent among older patients with JME, and marriage was strongly associated with depressive disorders.     

Diagnostic yield of short-term video-EEG monitoring for epilepsy and PNESs: A European assessment

IntroductionAlthough long-term video-EEG monitoring (LVEM) is routinely used to investigate paroxysmal events, short-term video-EEG monitoring (SVEM) lasting < 24 h is increasingly recognized as a cost-effective tool. Since, however, relatively few studies addressed the yield of SVEM among different diagnostic groups, we undertook the present study to investigate this aspect.MethodsWe retrospectively analyzed 226 consecutive SVEM recordings over 6 years. All patients were referred because routine EEGs were inconclusive. Patients were classified into 3 suspected diagnostic groups: (1) group with epileptic seizures, (2) group with psychogenic nonepileptic seizures (PNESs), and (3) group with other or undetermined diagnoses. We assessed recording lengths, interictal epileptiform discharges, epileptic seizures, PNESs, and the definitive diagnoses obtained after SVEM.ResultsThe mean age was 34 (± 18.7) years, and the median recording length was 18.6 h. Among the 226 patients, 127 referred for suspected epilepsy — 73 had a diagnosis of epilepsy, none had a diagnosis of PNESs, and 54 had other or undetermined diagnoses post-SVEM. Of the 24 patients with pre-SVEM suspected PNESs, 1 had epilepsy, 12 had PNESs, and 11 had other or undetermined diagnoses. Of the 75 patients with other diagnoses pre-SVEM, 17 had epilepsy, 11 had PNESs, and 47 had other or undetermined diagnoses. After SVEM, 15 patients had definite diagnoses other than epilepsy or PNESs, while in 96 patients, diagnosis remained unclear. Overall, a definitive diagnosis could be reached in 129/226 (57%) patients.ConclusionsThis study demonstrates that in nearly 3/5 patients without a definitive diagnosis after routine EEG, SVEM allowed us to reach a diagnosis. This procedure should be encouraged in this setting, given its time-effectiveness compared with LVEM.     

Subcortical structural abnormalities in juvenile myoclonic epilepsy (JME): MR volumetry and vertex based analysis

PurposeImaging studies in juvenile myoclonic epilepsy (JME) have shown abnormalities of the thalamus and frontal cortex. The purpose of this study was to systematically investigate the morphological changes in the deep gray matter (GM) structures using techniques of voxel based morphometry (VBM), MR volumetry and shape analysis.MethodologyThe study included 40 patients with JME (M:F = 21:19; age 22.8 ± 5.3 years) and 19 matched controls (M:F = 13:6; age 24.5 ± 4.2 years). All subjects underwent MRI using standard protocol that included T1-3D TFE (Turbo Field Echo) images with 1 mm thickness. VBM analysis and MR volumetry were performed. The volumes of deep subcortical GM structures were extracted and vertex-wise shape analysis was performed using FSL-FIRST (FSL-Integrated Registration and Segmentation Toolbox) software.ResultsVBM analysis with a thalamic mask revealed focal thalamic alterations in the anteromedial aspect of the thalamus (p < 0.05, false discovery rate (FDR) corrected) which remained significant after adjusting for age, gender and intracranial volume (ICV). Significant volume loss was noted in both the thalami. Vertex-wise shape analysis showed significant focal surface reductions in the thalami bilaterally in patients that were predominantly seen in the medial as well as lateral aspects of the thalamus (p < 0.05, FDR corrected). The disease duration correlated with left hippocampus volume while age of onset correlated with right hippocampus volume.ConclusionsThis study confirms the presence of thalamic alterations in patients with JME. Shape analysis technique provided complementary information and disclosed the presence of focal atrophic changes in patients’ thalami. The striatum and hippocampus did not show any significant alterations.     

Prediction of rhythmic and periodic EEG patterns and seizures on continuous EEG with early epileptiform discharges

BackgroundContinuous EEG (cEEG) is necessary to document nonconvulsive seizures (NCS), nonconvulsive status epilepticus (NCSE), as well as rhythmic and periodic EEG patterns of ‘ictal–interictal uncertainty’ (RPPIIU) including periodic discharges, rhythmic delta activity, and spike-and-wave complexes in neurological intensive care patients. However, cEEG is associated with significant recording and analysis efforts. Therefore, predictors from short-term routine EEG with a reasonably high yield are urgently needed in order to select patients for evaluation with cEEG.ObjectiveThe aim of this study was to assess the prognostic significance of early epileptiform discharges (i.e., within the first 30 min of EEG recording) on the following: (1) incidence of ictal EEG patterns and RPPIIU on subsequent cEEG, (2) occurrence of acute convulsive seizures during the ICU stay, and (3) functional outcome after 6 months of follow-up.MethodsWe conducted a separate analysis of the first 30 min and the remaining segments of prospective cEEG recordings according to the ACNS Standardized Critical Care EEG Terminology as well as NCS criteria and review of clinical data of 32 neurological critical care patients.ResultsIn 17 patients with epileptiform discharges within the first 30 min of EEG (group 1), electrographic seizures were observed in 23.5% (n = 4), rhythmic or periodic EEG patterns of ‘ictal–interictal uncertainty’ in 64.7% (n = 11), and neither electrographic seizures nor RPPIIU in 11.8% (n = 2). In 15 patients with no epileptiform discharges in the first 30 min of EEG (group 2), no electrographic seizures were recorded on subsequent cEEG, RPPIIU were seen in 26.7% (n = 4), and neither electrographic seizures nor RPPIIU in 73.3% (n = 11). The incidence of EEG patterns on cEEG was significantly different between the two groups (p = 0.008). Patients with early epileptiform discharges developed acute seizures more frequently than patients without early epileptiform discharges (p = 0.009). Finally, functional outcome six months after discharge was significantly worse in patients with early epileptiform discharges (p = 0.01).ConclusionsEpileptiform discharges within the first 30 min of EEG recording are predictive for the occurrence of ictal EEG patterns and for RPPIIU on subsequent cEEG, for acute convulsive seizures during the ICU stay, and for a worse functional outcome after 6 months of follow-up.This article is part of a Special Issue entitled Status Epilepticus.