Prospective study of epilepsy with generalized tonic-clonic seizures alone: Clinical features, response to treatment, and likelihood of medication withdrawal.

OBJECTIVE: This prospective study aimed to delineate the demographics, natural progression, and treatment response of patients newly diagnosed with epilepsy with generalized tonic-clonic seizures alone (EGTCA). Furthermore, our objective includes assessing the seizure recurrence rate post antiseizure medication (ASM) discontinuation within this cohort, alongside exploring predictive factors for seizure relapse. METHODS: The study cohort, derived from an ongoing, prospective, multicenter investigation on children and adults with new-onset unprovoked seizures, included consecutive patients enrolled between March 2010 and March 2020, and meeting mandatory ILAE criteria for EGTCA diagnosis. Participants underwent a 3-h sleep-deprived video-EEG recording along with an epilepsy protocol brain magnetic resonance imaging (MRI) with repeat EEG at each follow-up. Cumulative time-dependent probabilities of seizure recurrence were calculated using Kaplan-Meier survival analysis. Logistic regression identified variables associated with seizure recurrence following ASM taper. RESULTS: Eighty-nine patients with a median age of 16 years were included, constituting 31% of those diagnosed with an idiopathic generalized epilepsy. Regarding the circadian distribution of seizures, 59.6% of patients exclusively experienced diurnal seizures, 12.4% exclusively nocturnal, and 28.1% experienced both diurnal and nocturnal seizures. Generalized spike-wave discharges (GSWD) were present in the initial EEG of 88% of patients. A GTC recurred in 14% of patients treated with ASM compared with 73% of untreated patients (p < 0.00001). ASM discontinuation was attempted in 50 patients after a median treatment duration of 3 years, with 44% experiencing a recurrence. Patient-initiated taper and a mixed circadian seizure pattern independently predicted a higher likelihood of recurrence post-ASM discontinuation. SIGNIFICANCE: Our findings underscore the importance of prompt treatment upon the diagnosis of EGTCA. Notably, lifelong treatment may not be imperative; patients seizure-free for at least 2 years, with the absence of GSWD on EEG, often maintained seizure freedom after ASM withdrawal, especially with physician-initiated tapering. PLAIN LANGUAGE SUMMARY: Seizures in individuals diagnosed with "epilepsy with generalized tonic-clonic seizures alone" (EGTCA) typically start during adolescence and often respond well to antiseizure medications. An electroencephalogram, which measure brain waves, will show abnormal discharges in most patients with EGTCA. Lifelong treatment with antiseizure medication is not necessary for everyone with EGTCA; approximately, 40% can successfully stop treatment without facing seizure recurrence. Patients who stop medication on their own have a higher risk of seizures returning compared with those who undergo cessation under a doctor's supervision.

Early predictors of remission in children and adolescents with new-onset epilepsy: A prospective study.

PURPOSE: This study aims to identify predictive factors of a two-year remission (2YR) in a cohort of children and adolescents with new-onset seizures based on baseline clinical characteristics, initial EEG and brain MRI findings. METHODS: A prospective cohort of 688 patients with new onset seizures, initiated on treatment with antiseizure medication was evaluated. 2YR was defined as achieving at least two years of seizure freedom during the follow-up period. Multivariable analysis was performed and recursive partition analysis was utilized to develop a decision tree. RESULTS: The median age at seizure onset was 6.7 years, and the median follow-up was 7.4 years. 548 (79.7%) patients achieved a 2YR during the follow up period. Multivariable analysis found that presence and degree of intellectual and developmental delay (IDD), epileptogenic lesion on brain MRI and a higher number of pretreatment seizures were significantly associated with a lower probability of achieving a 2YR. Recursive partition analysis showed that the absence of IDD was the most important predictor of remission. An epileptogenic lesion was a significant predictor of non-remission only in patients without evidence of IDD, and a high number of pretreatment seizures was a predictive factor in children without IDD and in the absence of an epileptogenic lesion. CONCLUSION: Our results indicate that it is possible to identify patients at risk of not achieving a 2YR based on variables obtained at the initial evaluation. This could allow for a timely selection of patients who require close follow-up, consideration for neurosurgical intervention, or investigational treatments trials.

Risk of recurrence in patients with an unprovoked tonic-clonic seizure and generalized epileptiform discharges on EEG.

OBJECTIVE: The decision to initiate treatment in patients with a first unprovoked seizure remains controversial. Studies have reported a recurrence rate ranging from 21%-50%, but most have included patients with different etiologies, electroencephalography (EEG) findings, and seizure types. This study aimed to determine the risk of recurrence in patients with a first unprovoked generalized tonic-clonic (GTC) seizure with evidence of generalized spike-wave discharges (GSWDs) on EEG and compare the efficacy of antiseizure medications (ASMs) in preventing recurrence. METHODS: This prospective study included consecutive patients who presented with a single GTC seizure, evidence of GSWDs on EEG, and a follow-up period of at least 1 year. All patients underwent extensive evaluation, including a 3-hour sleep-deprived video-EEG recording and an epilepsy protocol brain magnetic resonance imaging (MRI). Treatment with ASMs was recommended for all patients. The decision regarding the specific ASM to be used was left to the treating physician's discretion. RESULTS: A total of 57 patients with a median age of 19 years were included. A total of 41 patients agreed to be started on an ASM while 16 declined. Seizure recurred in 6 of 41 patients (14.6%) in the treated group compared to 11 of 16 (68.8%) in the untreated group (p = .00006). Valproate was significantly more efficacious than levetiracetam or lamotrigine (p = .04). Of the 15 patients who discontinued ASM treatment after remaining seizure-free for an average of 30 months, 6 (40%) experienced a seizure recurrence. SIGNIFICANCE: Patients with a first unprovoked GTC seizure and evidence of GSWDs on EEG have a high risk of recurrence if left untreated. Valproate is the most efficacious ASM for preventing recurrence in this population. A sizeable proportion of patients can be successfully tapered off medication after a period of seizure freedom. This study provides valuable information for guiding treatment decisions in this patient population.

Acute Disseminated Encephalomyelitis Following COVID-19 Infection.

Acute disseminated encephalomyelitis (ADEM) is a relatively rare, post-inflammatory, immune-mediated demyelinating central nervous system disease that is predominantly reported in pediatric populations. Following the emergence of severe acute respiratory syndrome coronavirus 2, cases of ADEM are being reported following infection with this virus. Our case report describes a male patient in his early 40s who developed severe coronavirus disease 2019 (COVID-19) that rapidly progressed to a critical disease requiring invasive mechanical ventilation and high positive end-expiratory pressure, which was complicated by extensive neurological involvement and quadriplegia. MRI of the brain showed characteristic demyelinating lesions, suggestive of ADEM. As other entities were ruled out, our patient was treated using pulse steroids and intravenous immunoglobulins. The patient showed a good response to treatment and had an overall good prognosis, despite the severity of his condition. ADEM following COVID-19 is a rare entity worldwide.

The importance of acknowledging diagnostic uncertainty in patients with new-onset paroxysmal spells.

OBJECTIVE: The aims of this study were to evaluate the frequency of paroxysmal spells of indeterminate nature (PSIN) in a large cohort of children and adults with suspected new-onset seizures, to evaluate the reasons for including patients in this category, and to calculate the rate of erroneous diagnoses if the epileptologists were compelled to label those events as epileptic seizures or nonepileptic paroxysmal spells. METHODS: Patients identified for this study participated in a prospective study evaluating patients with suspected new-onset unprovoked seizures. The workup included a detailed history and a thorough description of the spells, a 3-hour video EEG recording, and an epilepsy protocol brain MRI. Based exclusively on a detailed description of the ictal events, two epileptologists were asked to independently classify each patient into those with a definite diagnosis of unprovoked seizures or a definite diagnosis of a nonepileptic paroxysmal spells (group 1) and those with PSIN (group 2). RESULTS: A total of 1880 consecutive patients were enrolled with 255 (13.6%) included in the PSIN group. Patients with PSIN were significantly younger than those with a definite diagnosis, and PSIN were significantly more frequent in children with developmental delay. The most common reason for including patients in the PSIN group was the inability to categorically discriminate between a seizure and a nonepileptic mimicker. When the raters were compelled to classify the spells in the PSIN group, the frequencies of erroneous diagnoses ranged between 32% and 38%. The final diagnoses on those patients were made based on the results of the EEG, MRI, and follow-up visits. SIGNIFICANCE: Our data indicate that a diagnostic category of PSIN should be recognized and ought to be used in clinical practice. Acknowledging this uncertainty will result in lower frequencies of erroneous diagnoses, possible stigma, and potential exposure to unnecessary antiseizure medications.

Depression and anxiety in patients from Lebanon with new onset functional seizures.

OBJECTIVES: To prospectively compare the frequencies of depression and anxiety in patients with new onset functional seizures versus two age and gender-matched control groups consisting of patients with new onset epileptic seizures and normal individuals. METHODS: Consecutive patients, 16 years and older, enrolled in a prospective study for suspected new onset epileptic seizures and diagnosed with documented functional seizures were included. We compared the depression and state and trait anxiety scores using the Beck Depression Inventory (BDI) and the State Trait Anxiety Inventory (STAI) between patients with functional seizures and the other two control groups. RESULTS: The 33 patients with functional seizures had significantly higher depression and anxiety scores compared to those with epileptic seizures and normal controls. Twenty patients (60.6%) in the functional seizures group scored in the "depression" range compared to 5/33 (15.2%) in the epileptic seizures and 1/33 (3%) in the control groups. In the functional seizures group, 14/33 (42.4%) had scores in the "state anxiety" range compared to 6/33 (18.2%) and 2/33 (6.1%) in the epileptic seizures and normal control groups, respectively. Similarly, 15/33 (51.5%) of patients in the functional seizures group had scores in the "trait anxiety" range compared to 4/33 (12.1%) and 1/33 (3%) in the epileptic seizures and normal control groups, respectively. CONCLUSIONS: Our results indicate that patients with new onset functional seizures frequently suffer from depression and anxiety at the time of their initial evaluation. These findings underscore the importance of screening for depression and anxiety in that patient population.

Frequency and Stratification of Epileptogenic Lesions in Elderly With New Onset Seizures.

Objective: To evaluate prospectively the frequency of epileptogenic lesions in a consecutive cohort of elderly patients presenting with new onset unprovoked seizures, and who underwent a complete evaluation including dedicated epilepsy protocol MRI. Methods and materials: We included all consecutive patients 60 years or older who participated in a prospective study on new onset epilepsy. The work-up included the acquisition of a dedicated epilepsy protocol MRI and a 3 h video/EEG recording. We evaluated the frequency and types of epileptogenic lesions in the whole cohort and stratified those variables by age, gender, types and number of seizures at presentation. We also correlated the EEG findings with the clinical characteristics and neuroimaging results. Results: Of the 101 patients enrolled in the study and who underwent an epilepsy protocol MRI, an epileptogenic lesion was identified in 67% of cases. The most common etiologies were vascular events, followed by tumoral causes and traumatic brain injuries. Epileptogenic lesions were more likely to be identified in patients who presented with only focal aware and impaired awareness seizures. In addition, patients with tumoral epilepsy were significantly more likely to only experience those seizure types compared to patients with other pathological substrates. Interictal/ictal discharges were detected in the EEG of 21% of patients. Epileptiform discharges were significantly more frequent in patients with an epileptogenic lesion on brain MRI, especially in those with a brain tumor. Conclusions: Our results stress the importance of obtaining a dedicated epilepsy protocol MRI in elderly patients with new onset seizures. An epileptogenic lesion will be identified in approximately two thirds of patients with important implications regarding initiation of treatment. In addition, the data underscore the value of distinguishing the types of seizures experienced at presentation as this will apprise the treating physician on the likelihood of identifying an epileptogenic lesion and on the probable etiologies.

Association of cardiovascular risk factors and troponin elevation after generalized tonic-clonic seizures.

PURPOSE: Troponins are very sensitive biomarkers of myocardial injury. Conflicting data regarding elevation of troponin levels following a generalized tonic-clonic (GTC) seizure have been reported. In this study we hypothesized that troponin elevation after a GTC seizure occurs more frequently in patients with cardiovascular risk factors. METHODS: Patients who presented to the ER after a single GTC seizure with troponin levels assessed by cardiac troponin T (cTnT) and drawn within 12h of the GTC seizure were included. Patients with cardiac symptoms, elevated CPK levels or renal insufficiency were excluded. The frequency and risk factors for elevated cTnT levels were analyzed. RESULTS: Fourteen patients with a mean age of 54 years (range: 19-87 years) were included. Four patients (28.6%) had elevated cTnT levels (mean=0.06µg/L; range: 0.035-0.076µg/L). Patients with elevated cTnT levels were significantly older than those with normal levels (77.5 years vs. 45.5 years; P=0.03). Of the eight patients 60 years of age and older, four (50%) had elevated cTnT levels. The coronary heart disease (CHD) score was significantly higher in patients with elevated cTnT levels compared to those with normal levels (13.5 vs. 9.75, P=0.012). CONCLUSIONS: Elevated troponin levels can occur after a GTC seizure. Patients at risk are the elderly and those with cardiovascular risk factors. Our results suggest that elevation of troponin levels after a GTC seizure reflects a minor ischemic cardiac injury related to the demand ischemia during the sympathetic overactivity that accompanies a GTC seizure.

Astasia-abasia and psychogenic tremor post-temporal lobectomy.

Temporal lobectomy can be complicated by somatoform disorders, psychiatric illnesses and non-epileptic psychogenic seizures. We report a woman who developed astasia-abasia and psychogenic tremor following temporal lobectomy for refractory epilepsy. To our knowledge, this type of conversion reaction following temporal lobectomy has not been previously reported.

Multi-organ damage induced by anabolic steroid supplements: a case report and literature review.

INTRODUCTION: The use of anabolic supplements and other related drugs for body building and to enhance athletic performance is nowadays widespread and acutely pervasive all around the world. This alarming increase in the use of anabolic and amino acid supplements has been linked to a diverse array of pathologies. As previously reported, the abuse of androgenic steroids is not without severe physiological, psychiatric and physical costs. The case we report here describes multi-organ damage resulting from the abuse and uncontrolled use of anabolic steroid supplements, mainly testosterone. CASE PRESENTATION: A 24-year-old white man presented with abdominal pain concomitant with nausea and vomiting. Laboratory analysis revealed hypercalcemia, elevated liver enzymes and high levels of amylase, lipase and creatine protein kinase. CONCLUSION: Amino acid as well as anabolic supplements may lead to abnormal functioning of many organs, which could be fatal in some instances. This mandates worldwide and concerted efforts to educate the public, especially the youth, about the dangers of these increasingly abused drugs.