Continuous cardiac monitoring in epilepsy: an implantable loop manual activation algorithm for improving ECG signal acquisition accuracy.

BACKGROUND: The muscle artifacts, caused by prominent muscle contractions, mimicking cardiac arrhythmias, might compromise the ECG signal quality and the implantable loop recorder memory capacity in patients with epilepsy. We developed an epileptic seizures clinical pattern-based implantable loop recorder manual activation algorithm, presenting its real-world efficacy here. METHODS: One hundred ninety-three patients (18-60 years) with drug-resistant focal epilepsy were consecutively enrolled and underwent a subcutaneous loop recorder implantation. Patients with focal onset-aware seizures and patients with focal impaired awareness seizures /bilateral tonic-clonic seizures without aura were recommended to use the activator once - just after the episode. Patients with focal impaired awareness seizures/bilateral tonic-clonic seizures with aura, the caregivers of patients experiencing status epilepticus, were advised to use the activator twice - during the aura and after the episode/ regaining consciousness. RESULTS: Six thousand four hundred ninety-four ECG traces (4826 - auto-triggered events, 1668 - person-activated events) were recorded and analyzed. The rate of true positive events in the person-activated group was statistically higher than in the autoactivation group (72.5% vs.19.4%, p < 0.0001). Person-activated false-positive events were observed in 30.5% of patients with focal impaired awareness seizures and 27.7% in patients with bilateral tonic-clonic seizures. The highest rate of false-positive events (61.5%) was detected in patients undergoing epileptic status, and the lowest rate (3.8%) - was in patients with focal onset aware seizures. The rate of false-positive events was significantly higher in patients with impaired awareness seizures without aura both in focal impaired awareness (45.5% vs. 19.3%, p < 0.0001) and bilateral tonic-clonic seizure groups (38.8% vs. 5.9%, p < 0.0001). CONCLUSIONS: Arrhythmias with varying clinical outcomes are expected in epilepsy patients and have been monitored continuously. The specified loop recorder external activation algorithm can improve the clinically relevant cardiac arrhythmia detection accuracy in epilepsy patients and the value of future studies.

Idiopathic generalized epilepsy with phantom absences, absence status, and generalized tonic-clonic seizures: A case report.

RATIONALE: Phantom absences refer to mild and short-lasting absence seizures, which are usually accompanied by infrequent generalized tonic-clonic seizures and absence status. Generally, phantom absences do not impair the individual neurological functions. Herein, we report the case of a young woman with idiopathic generalized epilepsy, phantom absences, absence status, and generalized tonic-clonic seizures. PATIENT CONCERNS: A 31-year-old woman presented with a 16-year history of paroxysmal convulsions. DIAGNOSES: Electroencephalogram (EEG) showed recurrent universal and synchronized 3~4 Hz spike waves and spike-slow waves in the interictal phase with normal background activity. During the ictal phases, EEG revealed bursts of 3~4 Hz spike waves and spike-slow waves that were universal, synchronized, and symmetrical. Additionally, there was 1 seizure episode induced by a 3-Hz flash in the current case. Based on these findings, a diagnosis of idiopathic generalized epilepsy was made. INTERVENTIONS: The patient was treated with oral sodium valproate, and the epileptic seizures were controlled. OUTCOMES: The frequency of absence seizures was significantly reduced and there were no generalized tonic-clonic seizures. LESSONS: Idiopathic generalized epilepsy with phantom absences, absence status, and generalized tonic-clonic seizures is an extremely rare condition. EEG is the exclusive method for diagnosis. Antiepileptic drugs are effective for controlling epileptic seizures in this disease.

Frequency of non-generalized tonic clonic seizures in a referral population of dogs.

Absence seizures are a type of generalized onset seizure associated in humans with brief activity interruptions, unresponsiveness and staring. Absence seizures are infrequently reported in veterinary patients, visually indistinguishable from focal seizures, and so may be grouped as non-generalized tonic clonic seizures (non-GTCS). The objective of this retrospective study was to provide a preliminary understanding of the frequency of non-GTCS in dogs and estimate its prevalence by evaluating the distribution of seizure types presented to a referral hospital over 4 years (May 2017-April 2021), as determined from the medical record history and electroencephalography (EEG) diagnostic testing where available. A total of 528 cases were included via a medical record search for dogs with epilepsy and/or seizures presented to the neurology or emergency services. Cases were categorized into seizure types based on reported clinical signs. Each year, 53-63 % of seizure cases were described as generalized tonic clonic seizures (GTCS), 9-15 % GTCS with additional events and 29-35 % suspected non-GTCS. EEG confirmed absence seizures in 12 of 44 EEGs, 5 cases having a history of GTCS and seven without prior GTCS. This preliminary study suggests that non-GTCS may be relatively common as one third of seizure cases in the referral population presented with non-GTCS clinical signs. Prospective studies using EEG are merited to definitively determine the prevalence of these different seizure types in dogs. Acknowledging the impact of these seizures will improve awareness, aiding veterinarians in their recognition, diagnosis and potential treatment options.

Automated detection of nocturnal motor seizures using an audio-video system.

BACKGROUND: Unsupervised nocturnal tonic-clonic seizures (TCSs) may lead to sudden unexpected death in epilepsy (SUDEP). Major motor seizures (TCSs and hypermotor seizures) may lead to injuries. Our goal was to develop and validate an automated audio-video system for the real-time detection of major nocturnal motor seizures. METHODS: In this Phase-3 clinical validation study, we assessed the performance of automated detection of nocturnal motor seizures using audio-video streaming, computer vision and an artificial intelligence-based algorithm (Nelli). The detection threshold was predefined, the validation dataset was independent from the training dataset, patients were prospectively recruited, and the analysis was performed in real time. The gold standard was based on expert evaluation of long-term video electroencephalography (EEG). The primary outcome was the detection of nocturnal major motor seizures (TCSs and hypermotor seizures). The secondary outcome was the detection of other (minor) nocturnal motor seizures. RESULTS: We recruited 191 participants aged 1-72 years (median: 20 years), and we monitored them for 4183 h during the night. Device deficiency was present 10.5% of the time. Fifty-one patients had nocturnal motor seizures during the recording. The sensitivity for the major motor seizures was 93.7% (95% confidence interval: 69.8%-99.8%). The system detected all 11 TCS and four out of five (80%) hypermotor seizures. For the minor motor seizure types, the sensitivity was low (8.3%). The false detection rate was 0.16 per h. CONCLUSION: The Nelli system detects nocturnal major motor seizures with a high sensitivity and is suitable for implementation in institutions (hospitals, residential care facilities), where rapid interventions triggered by alarms can potentially reduce the risk of SUDEP and injuries.

Can semiology differentiate between bilateral tonic-clonic seizures of focal-onset and generalized-onset? A systematic review.

OBJECTIVE: Bilateral tonic-clonic seizures are encountered in both focal and generalized epilepsies. We reviewed the literature regarding the presence of focal signs in generalized-onset tonic-clonic seizures (GOTCS) and the utility of semiology in differentiating those from focal to bilateral tonic-clonic seizures (FBTCS). METHODS: We conducted a comprehensive literature search using four electronic databases (Medline, Embase, Web of Science, and Psychinfo) and constructed a systematic review in keeping with the Preferred Items for Systematic Reviews and Meta-analyses guidelines. RESULTS: We included 13 studies on focal semiological features of GOTCS. These studies included a total of 952 participants. The key focal signs described in GOTCS included: early head version, figure of four sign, asymmetric seizure termination, and a multitude of auras as well as automatisms. Additionally, we reviewed five studies that investigated the use of semiology to differentiate GOTCS from FBTCS; these studies had a total of 289 participants. Asymmetry in clonic phase, side-to-side axial movements, asymmetrical seizure termination, figure of four sign, index finger pointing, and fanning posture of the hand were found to be significantly more frequent in FBTCS compared with GOTCS. Furthermore, combinations of focal semiological features occurring in a single seizure were found to be suggestive of FBTCS rather than GOTCS. CONCLUSION: Focal signs are often evident in GOTCS. Though the observation of multiple focal signs within a given seizure may be in favor of an FBTCS, our findings caution against differentiating between the two seizure types based on semiology alone due to considerable overlap in focal features.

A case report of several intraoperative convulsions while using the Narcotrend monitor: Significance and predictive use.

INTRODUCTION: Intraoperative seizures under general anesthesia are rare and our observation is the first to demonstrate a distinct electroencephalogram (EEG) pattern on the Narcotrend monitor. PATIENT CONCERNS: We present the case of a 30-year-old man undergoing craniotomy for glioblastoma resection under general anesthesia who suffered tonic-clonic seizures captured in a specific pattern by the intraoperative EEG. DIAGNOSES: Our depth of anesthesia monitor recorded, before the seizure, a widening of the beta-wave performance in a distinct "triangular-shaped" pattern. This pattern was repeated before the second seizure. The patient had no previous history of seizures and following surgery no further seizures were recorded. INTERVENTIONS: A spectrogram analysis showed a distinct increase in mean absolute beta power immediately prior to the first seizure. The EEG immediately prior to the second seizure was characterized by broadband noise. Both seizures were characterized by increased mean absolute delta, theta, and beta power. OUTCOMES: The increase in EEG beta activity seen before the tonic-clonic movements may represent cortical irritability secondary to surgical manipulation, induced by electrical stimulation, reflecting progressive brain over-arousal. The attentive analysis of the relative beta power may have helped forecast the occurrence of the second seizure. LESSONS: We report the use of a simple, inexpensive, and portable EEG-based monitoring device to assist seizure detection and decision making.

[ECG pearl: tonic-clonic seizure detected by implantable loop recorder]. / EKG-gyöngyszem: tónusos-clonusos epilepsziás roham kimutatása implantábilis loop recorderrel.

A 52-year-old woman is presented with repetitive transient loss of consciousness. Implantable loop recorder (ILR) recorded muscle artifacts during the generalized tonic-clonic seizures. Seizure was diagnosed and antiepileptic drug was started. The patient has been asymptomatic for 9 months. Orv Hetil. 2019; 160(29): 1143-1145.

Calcificaciones cerebrales en el diagnóstico precoz del síndrome de DiGeorge sin cardiopatía / Brain calcifications in the early diagnosis of DiGeorge's syndrome without heart disease

Introducción: Las calcificaciones cerebrales son un hallazgo frecuente y no siempre tienen un significado patológico. El diagnóstico diferencial incluye diversas entidades en la población pediátrica, como tumores cerebrales, infecciones connatales y perinatales, síndromes neurocutáneos, enfermedades autoinmunes (p. ej., celiaquía) y, con mayor frecuencia, trastornos del metabolismo del calcio, entre los que se incluye el hipoparatiroidismo. Caso clínico: Varón de 12 años de edad que ingresa en la unidad de pediatría tras haber presentado 2 crisis tónico-clónicas generalizadas, sin relajación de esfínteres. Estuvo afebril en todo momento. Durante el ingreso presentó un estatus convulsivo con características similares a las crisis previas, acompañado de espasmos carpopedales. En las pruebas complementarias realizadas durante el ingreso destacaba la presencia de una calcemia de 5,3 mg/dL, un calcio iónico de 0,84 mmol/L y un fósforo de 6,9 mg/dL. El paciente se encontraba en fase de seguimiento debido a un retraso psicomotor, y 2 años antes, a raíz de otro episodio comicial, se habían detectado calcificaciones cerebrales bilaterales en la resonancia magnética realizada. Conclusiones: En el amplio abordaje de las calcificaciones cerebrales, es obligado realizar un estudio del metabolismo del calcio, aun en el caso de que éstas sean asintomáticas y se consideren un hallazgo casual

Introduction: Cerebral calcifications are a frequent finding and do not always have a pathological significance. The differential diagnosis in the pediatric population is large and includes entities such as brain tumors, connatal and perinatal infections, neurocutaneous syndromes, autoimmune diseases, such as celiac disease, and more frequently calcium metabolism disorders, including hypoparathyroidism. Case report: We present a 12-year-old male admitted to the pediatric unit after having presented two generalized tonic-clonic seizures without sphincter relaxation. No fever at all times. During admission, he presented convulsive status with characteristics similar to previous crises and accompanied by carpopedal spasms. In the complementary tests performed during admission, the presence of a 5.3 mg/dL calcemia, an ionic calcium of 0.84 mmO/L, and a phosphorus of 6.9 mg/dL was noteworthy. The patient was in follow-up due to psychomotor retardation and two years earlier, following another seizure episode, bilateral cerebral calcifications had been detected in magnetic resonance. Conclusions: In the large approach of cerebral calcifications, the study of calcium metabolism is mandatory, even if they are asymptomatic and are considered a casual finding

Non-electroencephalography-based seizure detection.

PURPOSE OF REVIEW: There is need for automated seizure detection using mobile or wearable devices, for objective seizure documentation and decreasing morbidity and mortality associated with seizures. Due to technological development, a high number of articles have addressed non-electroencephalography (EEG)-based seizure detection. However, the quality of study-design and reporting is extremely heterogeneous. We aimed at giving the reader a clear picture on the current state of seizure detection, describing the level of evidence behind the various devices. RECENT FINDINGS: Fifteen studies of phase-2 or above, demonstrated that non-EEG-based devices detected generalized tonic-clonic seizures (GTCS) with high sensitivity (≥90%) and low false alarm rate (FAR) (down to 0.2/day). We found limited evidence for detection of motor seizures other than GTCS, mostly from subgroups in larger studies, targeting GTCS. There is little evidence for non-EEG-based detection of nonmotor seizures: sensitivity is low (19-74%) with extremely high FAR (50-216/day). SUMMARY: Detection of GTCS is reliable and there are several, validated devices on the market. However, detection of other seizure types needs further research.

Time to response and patient visibility during tonic-clonic seizures in the epilepsy monitoring unit.

OBJECTIVE: There is a high cost associated with recording quality video and electroencephalography (EEG) data in National Association of Epilepsy Center (NAEC) level IV epilepsy monitoring units (EMU). This study considers potential quality measures in EMUs for generalized tonic-clonic (GTC) seizures: types of safety signals, response time, and visibility of patient's limbs for semiology. These quality measures have been summarized across 12 EMUs to estimate response times to GTC seizures and the quality of video data that is captured during admissions. METHODS: Video electroencephalographies (vEEGs) from two prospective regulatory studies for the Brain Sentinel device were reviewed. A total of 232 subjects with a history of GTC seizures underwent routine clinical EMU stays. Fifty-four of the study subjects had 96 GTC seizures. The vEEG of events were reviewed for safety signal used, response time, and visibility of patient's limbs. RESULTS: The average response time from members of the hospital team was 22 s from electrographic generalization (minimum -37 s, maximum 111 s, two no response). For caregivers, average response was 11 s (minimum -15 s, maximum 33 s, 45 not present/no response). In 73% of events, the patient visibility was limited at seizure onset. In 55% of events with limited limb visibility, the visibility was improved (by removing sheets or improving camera angle) >30 s after start of the event. The primary safety signals were as follows: an alert from outside the patient room (54%), button press (23%), hospital team present at seizure start (14%), caregiver vocal alert (6%), and no response (2%). SIGNIFICANCE: The average response time of caregivers was twice as fast as the hospital team, underscoring the importance of having a person in the room during onset of a GTC seizure. Diagnostic yield could be improved with more timely removal of patient coverings. It was observed that when patients experienced a GTC seizure, 40% were fully or partially obscured for more than 30 s during the event, compromising the ability of epileptologists to evaluate semiology during seizure onset. Automated seizure alarms may help staff get to patients more quickly and improve diagnostic characterization.

The discriminative value of blood gas analysis parameters in the differential diagnosis of transient disorders of consciousness.

AIM: The differentiation between epileptic and non-epileptic episodes can be challenging. Our aim was to compare lactate, anion gap (AG), bicarbonate and the Denver Seizure Score (DSS) as point-of-care test (POCT) markers for episodes of transient alterations of consciousness. METHODS: The blood serum parameters were drawn at arrival in the emergency department (ED) within 2 h of the episode. After calculating AG and DSS values, the four parameters were compared retrospectively between patients with generalized tonic-clonic seizures (GTCS) (n = 165) and patients with other disorders of consciousness [syncopes (n = 43), and psychogenic non-epileptic seizures (n = 15)]. Additionally, we compared all values among men and women. RESULTS: In GTCS patients, all four parameters differed significantly compared to non-epileptic episode patients (p < 0.001). Serum lactate showed significant additional benefit over the remaining values, with an AUC of 0.947 (95% CI 0.92-0.975) and a high sensitivity and specificity for an optimal cut-off value of 2.45 mmol/l. For DSS, the AUC was 0.857 (95% CI 0.808-0.906; cut-off: 0.35), and for AG 0.836 (95% CI 0.783-0.889; cut-off: 12.45 mmol/l). In the case of serum bicarbonate, the AUC was 0.831 (95% CI 0.775-0.886; cut-off: 22.75 mmol/l). In the sex-dependent comparison, the results were similar. Men showed more significant differences in the compared values than women. CONCLUSIONS: Serum lactate is best suited as POCT marker in the differential diagnosis of epileptic and non-epileptic episodes and is superior to AG, DSS and bicarbonate. The differences among sexes may pose a challenge in their implementation and interpretation.

Clinical spectrum of Dyke-Davidoff-Masson syndrome in the adult: an atypical presentation and review of literature.

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition usually diagnosed in paediatric patients with clinical features of hemiparesis, seizures, mental retardation and contralateral cerebral hemiatrophy on neuroimaging. This report follows the case of a 22-year-old man presenting with seizures and hemiatrophy and hemiparesis. On review of cases the most common neuroimaging findings were cerebral hemiatrophy (100%) followed by hemicalvarial thickening (71.4%) and hyperpneumatisation of sinuses (71.4%). Apart from our patient, all nine cases with data on epilepsy control had drug-resistant epilepsy. The onset of seizures in adulthood, block vertebra, short stature, absence of mental retardation and well-controlled epilepsy on monotherapy makes our case exceptional-even bringing to mind the possibility of a DDMS variant. This report exhaustively reviews the wide range of clinical and radiological manifestations of DDMS in the adult, thereby adding to the literature on an unusual syndrome that causes significant neurological morbidity.

Epilepsy under my skin?

A 44-year-old male patient was admitted to the hospital for observation after an unwitnessed syncope. Physical examination revealed skin purpura and bilateral tongue haematoma. Laboratory studies were unremarkable. Radiological imaging showed no abnormalities of the vasculature, signs of thrombosis or brain anomalies. Biopsy of a purpuric lesion revealed extravasation of erythrocytes. After excluding several causes of both syncope and purpura, the typical location of these thoracocervicofacial purpura, the tongue haematoma and an elevated prolactin level (which came back later) led to the diagnosis of an epileptic seizure. The patient was referred to the neurology department for follow-up. Within 3 weeks, the purpura were completely resolved, and the patient remained free of seizures during follow-up. In case of an unwitnessed syncope, an epileptic seizure should be carefully considered and thoracocervicofacial purpura can be the pivotal manifestation leading to this diagnosis.

Oxcarbazepine oral suspension in young pediatric patients with partial seizures and/or generalized tonic-clonic seizures in routine clinical practice in China: a prospective observational study.

BACKGROUND: This study aimed to assess efficacy and safety of oxcarbazepine (OXC) oral suspension in pediatric patients aged 2-5 years with partial seizures (PS) and/or generalized tonic-clonic seizures (GTCS) in real-world clinical practice in China. METHODS: This 26-week, prospective, single-arm, multicenter, observational study recruited pediatric patients aged 2-5 years with PS or GTCS suitable for OXC oral suspension treatment based on physicians' judgments from 11 medical centers in China. Enrolled subjects started OXC oral suspension treatment as monotherapy or in combination with other antiepileptic drugs. Primary efficacy outcome was the percentage of pediatric subjects achieving ≥ 50% seizure frequency reduction at the end of the 26-week treatment. Secondary efficacy-related parameters and safety parameters such as adverse events (AEs) and serious AEs (SAEs) were also monitored during the 26-week treatment period. RESULTS: Six hundred and six pediatric patients were enrolled and 531 (87.6%) completed the study. After 26 weeks of treatment, 93.3% subjects achieved ≥ 50% seizure frequency reduction, and 81.8% achieved 100% seizure frequency reduction compared to baseline. Among different seizure types, OXC was effective in all subjects with simple PS and in > 90% of subject with other type of seizure present in the study. AEs were observed in 49 (8.1%) subjects. Only three subjects experienced SAE. Rash (n = 18, 2.97%) was the most common AE. Only 17 subjects discontinued due to AEs. CONCLUSION: This study, reporting the real-world data, further confirms the efficacy and good safety profile of OXC oral suspension in Chinese pediatric patients aged 2-5 years with PS and/or GTCS.

[Neurological manifestations in atypical Kawasaki disease]. / Manifestaciones neurológicas en la enfermedad de Kawasaki atípica.

BACKGROUND: Kawasaki disease (KD) is a type of systemic vasculitis of unknown etiology. Atypical Kawasaki disease is defined as that where there are signs and symptoms not corresponding to the classical criteria for this nosological entity. Children with atypical Kawasaki disease may present with acute abdominal symptoms, meningeal irritation, pneumonia or renal failure. CLINICAL CASES: We describe 4 children with ages ranging from 2 to 12 years who had atypical Kawasaki disease, with neurological and gastrointestinal symptoms as part of the systemic presentation of the disease. Treatment consisted of immunoglobulin and corticosteroids with good evolution. CONCLUSIONS: KD is a systemic vasculitis that can involve many territories. Atypical manifestations can mislead the clinician and delay diagnosis. Pediatricians and sub-specialists should be aware of these neurological manifestations in order to provide adequate and opportune treatment.

Antecedentes: La enfermedad de Kawasaki es una vasculitis sistémica de etiología desconocida. La modalidad atípica se define como aquella en la cual hay signos y síntomas que no corresponden a los criterios clásicos de esta entidad nosológica. Los niños con enfermedad de Kawasaki atípica pueden presentar síntomas abdominales agudos, irritación meníngea, neumonía o falla renal. Casos clínicos: Describimos 4 niños con edades que oscilaron entre los 2 y 12 años que presentaron enfermedad de Kawasaki atípica, con síntomas neurológicos y gastroinstetinales como parte de la presentación sistémica de la enfermedad. El tratamiento se llevó a cabo con corticosteroides e inmunoglobulina, con los cuales los pacientes evolucionaron satisfactoriamente. Conclusiones: La enfermedad de Kawasaki es una vasculitis sistémica que puede involucrar numerosos aspectos. Las manifestaciones atípicas pueden confundir al clínico y retrasar el diagnóstico. Los pediatras y subespecialistas deben estar conscientes de estas manifestaciones neurológicas, con el fin de proporcionar tratamiento adecuado y oportuno.

Detection of generalized tonic-clonic seizures using surface electromyographic monitoring.

OBJECTIVE: A prospective multicenter phase III trial was undertaken to evaluate the performance and tolerability in the epilepsy monitoring unit (EMU) of an investigational wearable surface electromyographic (sEMG) monitoring system for the detection of generalized tonic-clonic seizures (GTCSs). METHODS: One hundred ninety-nine patients with a history of GTCSs who were admitted to the EMU in 11 level IV epilepsy centers for clinically indicated video-electroencephalographic monitoring also received sEMG monitoring with a wearable device that was worn on the arm over the biceps muscle. All recorded sEMG data were processed at a central site using a previously developed detection algorithm. Detected GTCSs were compared to events verified by a majority of three expert reviewers. RESULTS: For all subjects, the detection algorithm detected 35 of 46 (76%, 95% confidence interval [CI] = 0.61-0.87) of the GTCSs, with a positive predictive value (PPV) of 0.03 and a mean false alarm rate (FAR) of 2.52 per 24 h. For data recorded while the device was placed over the midline of the biceps muscle, the system detected 29 of 29 GTCSs (100%, 95% CI = 0.88-1.00), with a detection delay averaging 7.70 s, a PPV of 6.2%, and a mean FAR of 1.44 per 24 h. Mild to moderate adverse events were reported in 28% (55 of 199) of subjects and led to study withdrawal in 9% (17 of 199). These adverse events consisted mostly of skin irritation caused by the electrode patch that resolved without treatment. No serious adverse events were reported. SIGNIFICANCE: Detection of GTCSs using an sEMG monitoring device on the biceps is feasible. Proper positioning of this device is important for accuracy, and for some patients, minimizing the number of false positives may be challenging.

Oxcarbazepine oral suspension in pediatric patients with partial seizures and/or generalized tonic-clonic seizures: a multi-center, single arm, observational study in China.

BACKGROUND: To assess efficacy and safety of oxcarbazepine (OXC) oral suspension in pediatric patients aged 2-16 years with partial seizures (PS) and/or generalized tonic-clonic seizures (GTCS) in real-world clinical practice in China. METHODS: This 26-week, single arm, multicenter and observational study recruited patients aged 2-16 years with PS or GTCS suitable for OXC oral suspension treatment. Enrolled patients received OXC oral suspension treatment for 26 weeks. Primary endpoints included mean seizure frequency at the end of the treatment and mean seizure frequency reduction at the end of the treatment vs. baseline. Secondary efficacy-related endpoints and safety parameters were also assessed. RESULTS: Nine hundred and eighty-seven pediatric patients were enrolled and 912 (92.4%) completed the study. The mean seizure frequencies at baseline and the end of week 26 were 13.40±64.92 and 1.62±19.47 times/ month, respectively. The mean seizure frequency reduction was 10.03±63.67 times/month and the mean seizure frequency reduction percentage was 90.02%±5127.0% (P<0.0001). After 26 weeks of treatment, 82.36%, 7.24% and 3.86% of the patients became controlled, significantly improved and improved, respectively. Adverse events (AEs) were reported in 74 (7.65%) patients. Rash was the most common AE. The efficacy of OXC was not affected by seizure types, age or gender. CONCLUSIONS: This study confirms the efficacy and good safety profile of OXC oral suspension in Chinese pediatric patients aged 2-16 years with PS and/or GTCS.

Long-term outcome in adolescent-onset generalized genetic epilepsies.

OBJECTIVE: Until now, it has been unclear if the three subsyndromes of adolescent-onset generalized genetic epilepsy (GGE) differ in long-term prognosis. Therefore, this study aimed to compare long-term seizure outcome in juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and epilepsy with generalized tonic-clonic seizures alone (EGTCS). METHODS: This retrospective study is based on the archive of an institutional tertiary care outpatient clinic for adult patients with epilepsy. Charts of 870 epilepsy outpatients were reviewed among whom 176 had adolescent-onset GGE (53 JAE, 66 JME, 57 EGTCS). Median patient age at investigation was 60 years; median follow-up time was 42.5 years. If possible, GGE patients were additionally interviewed on psychosocial and clinical variables. RESULTS: Age at first seizure was significantly higher in EGTCS patients (median 18 years) than in patients with JAE or JME (14 years each; p ≤ 0.001). Long-term seizure outcome hardly differed between the three subsyndromes. At the end of follow-up, 60% of all patients were in 5-year terminal seizure remission, and in 14%, epilepsy even had resolved (>10 years without seizures, >5 years without pharmacotherapy). Twenty percent of patients had persistent seizures during the last year of follow-up. Across all patients, 23% reported a psychiatric comorbidity, 87% had married, and 57% had achieved university entrance qualification. SIGNIFICANCE: Long-term outcome was shown to be highly similar across all subsyndromes of adolescent-onset GGE. Even in a selection of difficult-to-treat epilepsy patients still attending an adult epilepsy clinic, most become seizure-free. To confirm these findings, prospective studies are needed.