A Rare Presentation of Sick Sinus Syndrome: Generalized Seizures.

Cardiac and neurological disorders are the main broad etiologies for loss of consciousness. Ictal bradycardia syndrome refers to epileptic discharges that profoundly disrupt normal cardiac rhythm, resulting in cardiogenic syncope during the ictal event. Convulsive syncope is a well-described phenomenon in both adults and children in which abrupt cerebral hypoperfusion leads to brief extensor stiffening and non-sustained myoclonus. Sick sinus syndrome or tachycardia bradycardia syndrome is a common cause of arrhythmias in the elderly secondary to sinus node dysfunction. We present a case of a 91-year-old male who presented with generalized seizure with associated bradyarrhythmias with telemetry showing sinus rhythm, followed by severe bradycardia, followed by Ventricular tachycardia, followed by an episode of asystole, which likely precipitated seizures as a result of cerebral hypoperfusion. The patient had a permanent dual-chamber pacemaker. He was discharged on antiepileptics as his EEG was abnormal, which might indicate an underlying predisposition.

Autonomic nerve activity and cardiovascular changes during discrete seizures in rats.

Activity in both divisions of the autonomic nervous system (ANS) can increase during seizures and result in tachy- or bradyarrhythmias. We sought to determine the patterns of ANS activity that led to heart rate (HR) changes and whether the character of ANS and HR changes can impact the seizures themselves. Simultaneous recordings of vagus nerve and cervical sympathetic ganglionic or nerve activity, EEG, ECG, and blood pressure were acquired from 16 urethane-anesthetized rats that received systemic kainic acid to induce seizures. After initial continuous seizure activity, discrete seizures were observed in 11/16 rats. Individual seizures were classified based on HR changes as tachycardic (n = 3), bradycardic (n = 17), or one of two more severe categories in which (a) the seizure appeared to be terminated by severe bradyarrhythmia (n = 5) or (b) the animal died (n = 6). Interestingly, even simple bradycardic seizures had episodes of dramatically increased respiratory effort, which we interpret as evidence of airway occlusion based on muscle artifacts in the recordings with transient blood pressure decreases. In the severe outcomes, ANS activity increased during seizures until it caused a drastic HR reduction (>50%), in which case seizure and ANS activity decreased dramatically. Sympathetic activity during this late vulnerable period was important for survival. We conclude that individual seizures produce (a) stereotypical changes in autonomic activity and HR, (b) persistence of sympathetic tone helps to protect against death, and (c) bradycardic seizures may indicate increased risk for seizure-associated obstructive apnea.

Syncope, Epilepsy and Ictal Asystole: A Case Series and Narrative Review.

IMPORTANCE: Syncope is a common presentation to emergency departments, and cardiac and neurological aetiologies are the predominant causes. Ictal asystole is a rare cardio-neural phenomenon seen in epilepsy syndromes whereby a seizure causes asystole (≥3 s) leading to syncope. OBSERVATIONS: We present three cases of ictal asystole, together with a narrative review of the literature to assess the prevalence of the condition and review the pathophysiology, diagnosis and management. Our review of the literature has shown that ictal asystole is an unlikely contributor to sudden unexplained death in epilepsy (SUDEP). Pacemaker insertion may limit morbidity from trauma related to syncopal episodes but does not impact mortality. CONCLUSIONS AND RELEVANCE: Patients with ictal asystole should be diagnosed with concurrent electroencephalogram-electrocardiograph (EEG-ECG) monitoring, have their anti-epileptic drugs optimised and be considered for epilepsy surgery if feasible. The use of longer term ECG monitoring may be used as a diagnostic aid if ictal asystole is suspected. If there are ongoing syncopal episodes with associated ictal asystole ≥6 seconds, particularly despite medical therapy, a permanent pacemaker may be considered to reduce morbidity. Current guidelines should be updated to reflect the increasing knowledge of this condition.

Head Over Heart: An Atypical Presentation of Temporal Lobe Epilepsy.

Ictal asystole is a long-documented medical condition that causes pauses during epileptic episodes. This condition has garnered attention due to resulting accidents. The mechanism of action and treatment guidelines are not well established. We present a case of a 39-year-old male truck driver who presented with dizziness, lightheadedness, confusion, and amnesia with a history of two motor vehicle accidents within one week. During his second hospitalization he underwent pacemaker placement due to presumed sinus node dysfunction. The patient returned with recurrent symptoms and was found to have epileptic focus of the left anterior temporal lobe on electroencephalogram and was thought to have ictal asystole. In this report, we focus on the importance of evaluating for neurogenic cause of cardiac arrythmias.

Ictal asystole with intercurrent cardiopathy: a complex combination leading to delayed diagnosis.

Ictal asystole is a rare epileptic phenomenon, though usually self-limiting, which has been associated with an increased risk of sudden death in epileptic patients. Although early recognition is desirable, the diagnosis can be delayed until prolonged video-EEG monitoring is completed. We report a case of ictal asystole in a 74-year-old patient with co-morbid cardiac conditions leading to a delay in diagnosis of approximately 10 years. Indeed, multiple cardiac investigations and EEGs failed to reveal that the recurrent syncope was seizure induced. A detailed description of the case with an emphasis on the prolonged diagnostic process, as well as the clinical and EEG findings, is provided. This case highlights the complexity of the diagnosis of some ictal asystole cases and the need to perform continuous video-EEG monitoring for confirmation. [Published with video sequence].

Resolution of ictal bradycardia and asystole following temporal lobectomy: A case report, and review of available cases using pacemakers.

Ictal bradycardia (IB) and ictal asystole (IA) are uncommonly recognized phenomena that increase morbidity in patients with epilepsy by causing syncope and seizure-related falls. These arrhythmias are also suspected to be involved in the pathophysiology of sudden unexpected death in epilepsy (SUDEP). We report a case of a 57-year-old male with left temporal lobe epilepsy who experienced both IB and IA. This patient was initially managed with pacemaker implantation, prior to undergoing left temporal lobectomy. Following surgery, the patient had no ongoing IB or IA on his pacemaker recordings, and his seizure control was greatly improved. His pacemaker was removed approximately one year post-operatively and he continued treatment with anti-seizure drugs (ASDs). A literature review of cases of IB and IA that were managed with pacemakers was performed. Pacemaker implantation appears to be quite effective for reducing seizure-related syncope and falls in the setting of IB/IA. Epilepsy surgery also seems to be an effective treatment option for IB/IA, as many patients are able to have their pacemakers removed post-operatively. Further investigations into the pathophysiology of IB and IA and long-term outcomes using different treatment modalities are clearly needed to help formulate treatment guidelines and, potentially, to reduce the occurrence of SUDEP in these patients.

Generalized tonic seizures with autonomic signs are the hallmark of SCN8A developmental and epileptic encephalopathy.

Developmental and epileptic encephalopathy (DEE) due to SCN8A gene variants is characterized by drug-resistant early onset epilepsy associated with severe intellectual disability. Different seizure types have been reported, and a sequence of autonomic manifestations such as brady-/tachycardia, irregular breathing, and cyanosis. Nevertheless, an exhaustive video-polygraphic documentation is still lacking. In this study, we reviewed the ictal electroencephalograms (EEGs) of five patients with SCN8A-DEE followed-up at the Neuroscience Department at Bambino Gesù Children's Hospital in Rome. We identified generalized tonic seizure as the major seizure type at epilepsy onset. Seizure severity could vary from subtle to marked clinical manifestations, depending from the extent and groups of muscles involved and association with autonomic modifications. We found autonomic signs in 80% of seizures in our cases, and we were able to identify a stereotyped sequence of ictal events for most of seizures. Autonomic signs occurred in rapid sequence: flushing of the face, sometimes associated with sialorrhea, bradycardia, and hypopnea appeared within the first 1-2 s. Tachycardia, polypnea, perioral cyanosis, and pallor occurred later in the course of the seizure. Generalized tonic seizures are rarely described in other genetic epileptic conditions of early infancy because of ion channel mutations, such as in DEE due to KCNQ2 or SCN2A gene mutations, where seizures are most frequently reported as focal to bilateral tonic. Therefore, generalized symmetric tonic seizures with autonomic signs can be considered a clinical hallmark for diagnosis of SCN8A-related DEE and relevant for therapeutic implications.

Impact of repeated kindled seizures on heart rate rhythms, heart rate variability, and locomotor activity in rats.

Although an impact of epilepsy on circadian rhythmicity is well-recognized, there are profound gaps in our understanding of the influence of seizures on diurnal rhythms. The effect on activity levels and heart rate is of particular interest as it might contribute to the disease burden. The kindling model with telemetric transmitter implants provides excellent opportunities to study the consequences of focal and generalized seizures under standardized conditions. Data from kindled rats with generalized seizures revealed an increase in activity and heart rate during the resting phase. Total and short-term heart rate variabilities were not affected by electrode implantation or seizure induction. Ictal alterations in heart rate associated with generalized seizures were characterized by a biphasic bradycardia with an immediate drop of heart rate followed by a transient normalization and a second more steady decrease. In conclusion, the findings demonstrate that once daily generalized seizures can exert significant effects on heart rate rhythms. Respective alterations in patients would be of relevance for patient counselling and therapeutic management. Occurrence of biphasic bradycardia associated with seizure induction suggests that the kindling model is suitable to study the consequences and the prevention of ictal bradycardia, which may pose patients at risk for sudden unexpected death.

Ictal asystole with isolated syncope: A case report and literature review.

Ictal syncope is a rare phenomenon that occurs in association with 0.002-0.4% of seizures. In the absence of other symptoms, seizures presenting with syncope may be challenging to diagnose. We report a case of a previously healthy male who developed recurrent episodes of syncope with postictal confusion and was later diagnosed with temporal seizures. The patient was successfully treated with anti-seizure drugs and placement of a cardiac pacemaker. In a systematic review of literature, we summarize the clinical characteristics of patients with ictal asystole and isolated syncope. Seizures should be considered in patients with syncope of uncertain etiology.

Epileptic seizures associated with syncope: Ictal bradycardia and ictal asystole.

INTRODUCTION: Heart rate decrease during epileptic seizures is rare and should be considered in patients with unusual or refractory episodes of syncope or in patients with a history suggestive of both epilepsy and syncope. We systematically reviewed the literature to better understand the clinical signs and risk factors of ictal heart rate decreases. MATERIAL AND METHODS: We performed a literature-search on "ictal bradycardia" and "ictal asystole" in Pubmed and added papers from the references and personal archives. Articles relating to animal studies, seizures without ictal decrease of heart rate, cases without simultaneous electroencephalography (EEG) and electrocardiography (ECG), convulsive syncopes, or cases with bradycardia before seizure onset and articles written in other languages than English, Dutch, German, French, or Spanish were excluded. Full texts of the remaining articles were screened for cases of ictal bradycardia or ictal asystole. Cases were selected on the basis of a self-designed quality score. The relationship of RR wave interval of at least 5 s, signs of syncope, and EEG signs of ischemia were analyzed with chi-square test and identifying 95% confidence intervals. RESULTS: Ictal bradycardia and ictal asystole predominantly occurred during focal seizures with loss of awareness (proportion in the combined group of bradycardia and asystole (p1 + 2) = 0.85) in people with mainly left lateralized (p1 + 2 = 0.64; p = 0.001) temporal lobe seizures (p1 + 2 = 0.91). Seizures with ictal asystole typically started with a heart rate decrease. During ictal asystole in the majority of cases, not only the clinical signs of syncope occurred (change of proportion (Δp) = 0.67; 95% CI: 0.48-0.86; p < 0.0001), i.e., interrupting the seizure semiology, but also the characteristic EEG signs of ischemia (Δp = 0.50; 95% CI: 0.26-0.74; p < 0.001). We found a statistically significant relation between signs of syncope and EEG signs of ischemia (Δp = -0.37; 95% CI: (-0.64)-(-0.10); p < 0.01) but not between duration of asystole (5 s) and either signs of syncope (Δp = -0.36; 95% CI: (-0.77)-0.05; p = 0.03) or EEG signs of ischemia (Δp = -0.37; 95% CI: (-1.07)-0.33; p = 0.16). CONCLUSION: In the ictal bradycardia syndrome, signs of syncope disrupt the semiology of ongoing seizures and are associated with EEG signs of brain ischemia and the duration of the cardiac arrhythmia.

Ictal Asystole Induced by Right Posterior Quadrant Epilepsy: Report of a Radically Treated Case.

BACKGROUND: Ictal asystole (IA) and ictal bradycardia (IB) are mainly seen with temporal or frontal lobe epilepsy. Many patients with these conditions undergo cardiac pacemaker therapy but not epilepsy surgery. CASE DESCRIPTION: We report the case of a 15-year-old boy with IA and IB secondary to right posterior quadrant epilepsy (PoQE) who underwent right posterior quadrant disconnection, but not cardiac pacemaker implantation. He has remained free from daily epileptic seizures, IA, and IB for more than 6 months postoperatively. This is the first report of a radically treated case with IA and IB caused by PoQE. CONCLUSIONS: Both temporofrontal lobe epilepsy and PoQE caused the IA and IB. Because a cardiac pacemaker only addresses arrhythmia, not epileptic seizures, radical treatment for both epilepsy and arrhythmia may be warranted for patients with medically intractable epilepsy.

Ictal Asystole Caused by Epileptic Seizure due to Brain Metastases

<p>Bradycardia or asystole during epileptic seizure are referred to as ictal bradycardia syndrome. Ictal asystole is very rare, and there is no report about ictal bradycardia syndrome caused by brain metastases. A 62-year old man was diagnosed as having lung cancer and had multiple brain metastases. The patient had no history of epilepsy and syncope. The patient developed cardiac asystole with sinus arrest for up to 16 seconds. The bradycardia was associated with other signs and symptoms, including abdominal pain, nausea, low blood pressure, sinus arrest, decreased level of consciousness, and staring at a single point. Electroencephalograms showed multiple sharp waves. Repeated seizures, ictal asystole, and coexisting symptoms disappeared after improved treatment of brain metastases by radiation therapy. Therefore, a diagnosis of ictal asystole caused by brain metastases was made. There is no recommended treatment for ictal bradycardia syndrome. However, in the case of ictal bradycardia syndrome caused by brain metastases, treatment of the metastatic tumor might be useful. When patients with cancer present with syncope or sick sinus syndrome, we should consider the possibility of cardiac arrest caused by an epileptic seizure.</p>

The ictal bradycardia syndrome: A case report.

We report a case of a 56-year-old man affected by frontal lobe seizures who has developed bradycardia followed by asystole. The patient had a positive family history for epilepsy. In fact, the mother, brothers, and one sister had epilepsy. Furthermore, the patient's two brothers suddenly died of unspecified heart disease at the ages of 26 and 53, respectively. The patient also experienced syncope once or twice a year. Three similar epileptic seizures, without the recurrence of asystole, were registered after pacemaker implantation.

Ictal 99mTc-Ethyl Cysteinate Dimer SPECT Findings of a Girl With Refractory Localization-Related Epilepsy Who Developed Transient Ictal Bradycardia.

Ictal bradycardia, which is considered to be one of the causes of sudden unexplained death in epilepsy, is rare. A 10-year-old girl with focal cortical dysplasia in her right centroparietal region developed transient ictal bradycardia during cluster seizures. Brain magnetic resonance imaging demonstrated a high signal intensity lesion adjacent to the focal cortical dysplasia lesion. Ictal 99mTc-ethyl cysteinate dimer single-photon emission computed tomography (SPECT) detected hyperperfusion in an area containing the high signal intensity lesion, which was located close to the insular cortex. Since the hyperperfusion zone observed on SPECT was considered to reflect seizure propagation, it is possible that the ictal bradycardia experienced in the present case was caused by the following mechanism: The repetitive seizure activity caused the high-intensity lesion seen on MRI to expand into the right insular cortex, which controls cardiac rhythm, resulting in ictal bradycardia.

Ictal asystole as the first presentation of epilepsy: A case report and systematic literature review.

We report the case of a 69-year-old woman who presented with recurring episodes of mental confusion/dizziness followed by loss of consciousness, intense pallor, and sweating. Cardiologic investigations were unremarkable. The electroencephalogram recorded during one typical episode allowed the demonstration of a right frontotemporal seizure with progressive bradycardia leading to a 9-second asystole. Following levetiracetam treatment up to 2500 mg/day, seizures with ictal asystole (IA) recurred. An MRI compatible pacemaker was then implanted. At 26-month follow-up, the patient has not had further episodes of loss of consciousness. A systematic review (1950-Apr 2014) searching for cases in which IA was an early manifestation of epilepsy led to the observation of 31 cases. The time lag between the first seizures and the correct diagnosis of IA was long (average: 27 months; median: 12 months). Clinical history alone was not sufficient to prompt a correct diagnosis of IA, and only 11 out of 31 cases presented with symptoms suggestive of a seizure disorder. The majority of patients had a frontotemporal epilepsy with a slight prevalence of left-side involvement (19 out of 31). Ictal bradycardia-asystole is an important condition that should be recognized by epileptologists, neurologists, as well as emergency department physicians. It is important to underscore that IA not only can occur in patients with drug-resistant epilepsy but also may be the first manifestation of the patient's epilepsy.

Asystole in course of epileptic episode.

A subgroup of patients with epilepsy die suddenly and ictal bradycardia (IB) has been implicated. We describe a 63-year-old man with diagnosis of refractory epilepsy. The patient had long video and EEG/ECG recordings (48 hours) of wakefulness and abnormal sleep due to the presence of seven ictal events, which are associated with complex partial seizures that start on the temporal region. The patient presented a severe dysautonomia ictal, at 10-15 seconds of the start of the event is observed bradycardia and after 25 seconds occurs asystole for 15 seconds. Dual-chamber pacemaker was implanted with improvement.