Clinical diagnosis of syncopes (including so-called breath-holding spells) without electroencephalography or ocular compression

A recent article in this journal suggested that ocular compression during electroencephalography was useful in distinguishing "breath-holding spells and syncope" from epileptic seizures. The method proposed involved measurement of the RR interval on the simultaneously recorded electrocardiographic trace and determining both the absolute RR lengthening and the change in RR interval as compared with the baseline value. It is argued by the present author that this is not an appropriate way to come to a diagnosis in episodic loss of consciousness in children. It is pointed out that so-called "breath-holding spells" are reflex syncopes and that the diagnosis of reflex syncopes should be by clinical history, even if this means delaying the diagnosis until a future consultation. Published evidence on the nature and clinical diagnosis of reflex syncopes in infants and children is reviewed in depth. It is concluded that routine electroencephalography is not an appropriate investigation when the diagnosis of episodic loss of consciousness is in doubt and has the implicit danger of false positive "abnormality". Aside from scientific exploration of the developing autonomic nervous system, the only current indication for diagnostic ocular compression is to induce a syncope so that its nature may be better understood. Such a circumstance might be a history of an apparent reflex syncope but with atypical features, including prolonged post-syncopal unconsciousness such as might indicate epileptic absence status. Several additional investigations of a primarily cardiological nature may be indicated in some cases, but a wait-and-see policy is usually to be preferred.     

Bradycardia and asystole during generalised interictal EEG discharges

Few studies have investigated the effects of interictal epileptic discharges on the cardiac autonomic system. This study reports the case of a 37‐year‐old man with refractory generalised epilepsy, who recently reported an increase in frequency of nocturnal tonic‐clonic seizures, not responding to treatment. During the nocturnal video study, in non‐rapid eye movements sleep, we recorded 106 generalised sharp‐ and polyspike‐waves lasting for 3 to 7 seconds, associated with bradycardia and asystole, without behavioural changes and without increase in deltoid muscle activity. The asystole had a duration of between 3 and 8 seconds. In one case, a 7 second asystole was associated with a tonic‐clonic generalised seizure. A 24‐hour electrocardiographic study revealed a bradycardia and a Wenckebach atrioventricular block. Heart rate analysis at the time of the interictal epileptic discharges revealed an abrupt increase in the RR interval, occurring simultaneously with the onset of interictal epileptic discharges and followed by a return to values below baseline value. A cardiac pacemaker was installed with a reduction of asystole length during the interictal epileptic discharges. Our findings indicate, for the first time, the role of interictal generalised discharges in EEG‐related asystole and bradycardia. These data support the hypothesis that some patients with epilepsy may be predisposed to disturbances of the autonomic cardiac system.     

Panayiotopoulos syndrome: A case series from Turkey

The aim of the study was to evaluate the demographic, clinical, and EEG characteristics of patients with Panayiotopoulos syndrome (PS) and the course of their illness.Thirty-eight patients followed up with a diagnosis of PS between January 2011 and December 2013 were evaluated.We found high rates of personal history of febrile convulsions, breath-holding spells, and family history of febrile convulsions, afebrile convulsion/epilepsy, migraine, and breath-holding spells. Seizures started before the age of eight in 87% of the patients, and the mean age at seizure onset was 4.6 years. Seizures were sleep-related in 81.5%, and autonomic status was seen in a third of the patients. The number of seizures was between 2 and 10 in 66% of the patients. The most common symptoms were ictus emeticus, eye/head deviation, and altered consciousness. Rolandic features were seen in 26% of the patients, and visual symptoms in 5%. Multifocal epileptiform discharges on EEG were identified in 84% of the patients. Two or more antiepileptic drugs were required in only 13% of the patients. Evolution to electrical status epilepticus in sleep and Gastaut-type epilepsy were seen in patients with more than ten seizures.The high rates of febrile convulsions, afebrile convulsions/epilepsy, migraine, and breath-holding spells in the patients and families suggest the importance of genetic factors and, perhaps, a common pathogenesis. However, the high rates of febrile convulsions and breath-holding spells in patients can be related to a misdiagnosis because of the similar symptoms. Despite its disturbing symptoms, PS is a benign epileptic syndrome requiring multiple antiepileptic drug use only in a small proportion of patients.     

Ictal asystolia in the differential diagnosis of dizziness

Frequently, epileptic seizures are accompanied by changes in the heart rate. They are usually transient and irrelevant for the patient's symptoms and the patient recorders his usually baseline rhythm in second or a few minutes. In this report we present a case of a patient diagnosed previously of epilepsy who presented episodes of dizziness and presyncope. During the video-EEG study we recorded one of these spells. The diagnosis of this spell was a focal seizure associated with asystole: the seizure was almost asymptomatic, and only when the asystole developed (40 seconds after the EEG ictal changes) the patient complained about dizziness. Recovery was fast, but treatment with pacemaker must be considered.     

Differenzialdiagnostische Kl?rung durch implantierten ?Loop“-Rekorder

It is not yet sufficiently clarified which patients who suffer from repetitive sudden losses of consciousness (SLOC) and for whom the differential diagnosis between epileptic seizure and syncope is open would benefit from the use of an implantable loop recorder (ILR). This article describes the case of a patient who suffered from infrequent SLOC and who had been referred in order to improve the treatment of the suspected drug-resistant epilepsy. The patient had shown normal results during extensive cardiological work-up and presented with normal neurological, magnetic resonance imaging (MRI) and routine electrocardiogram (EEG) findings. Long-term video EEG also did not reveal any abnormalities. Typical examples of syncope, epileptic and psychogenic non-epileptic seizures had been shown to the husband as an eyewitness but he was still not able to help with the diagnosis. An ILR was implanted and the typical attacks could be recorded and were diagnosed as asystole. The patient was treated with a pacemaker and has remained free from attacks with a follow-up of 4 years. It can be concluded that in adult patients with infrequent SLOC in spite of normal neurological, neuroimaging and long-term EEG results and in spite of normal cardiological findings cardiac syncope is more probable than epileptic seizures. Implantation of an ILR offers a chance to find the correct diagnosis and is therefore recommended if such clinical circumstances exist.     

Severe pallid breath-holding spells treated with low-dose theophylline

BackgroundThe medical treatment for severe pallid breath-holding spells accompanied with severe bradycardia or transient cardiac arrest is controversial. Although various medications have been reported to be effective, patients treated with pacemaker insertion are not always evaluated for pharmacological therapy beforehand.Case reportA 9-month-old boy developed pallid breath-holding spells. At 15 months of age, a Holter electrocardiogram revealed 12 s of asystole during a breath-holding spell. Treatment with low-dose theophylline sustained-release dry syrup (5.3 mg/kg/day) led to complete control of the spells. The peak concentration of theophylline was 4.4 μg/mL which was below the therapeutic range for bronchial asthma. When he turned 3 years and 5 months of age, theophylline treatment was discontinued without recurrence of pallid breath-holding spells.DiscussionTheophylline is now infrequently used to treat pediatric bronchial asthma due to its limited effect coupled with its side effects, which include headache, digestive symptoms, and theophylline-associated convulsions. The effectiveness of theophylline as a treatment for pallid breath-holding spells has been reported in several reports. In our case, the theophylline dosage was approximately half the amount described in previous reports.ConclusionsIn this case, low-dose theophylline was adequate in controlling the pallid breath-holding spells. Because theophylline-associated seizures are a major concern, we suggest an evaluation of low-dose theophylline for treating patients with severe pallid breath-holding spells without febrile convulsions or epilepsy before proceeding with permanent pacemaker insertion. Further development of preventive strategies for theophylline-associated seizures and characterization of patients who respond well to theophylline treatment is required.     

Comparison of clinical and electrophysiological characteristics between ictal and cardiac asystole encountered during video-EEG monitoring

Aims. Differentiation between syncope secondary to epileptic seizures and cardiac disease in patients displaying transient loss of consciousness associated with convulsive movements is a diagnostic challenge both for neurologists and cardiologists. In such patients, prolonged video-EEG monitoring not only helps in identifying asystole as the cause of syncope, but also in categorizing asystole as primarily cardiac in origin (cardiac asystole) and secondary to epileptic seizures (ictal asystole). We carried out this study to ascertain the prevalence of asystole in an epilepsy monitoring unit, and to contrast the clinical and electrophysiological characteristics between ictal asystole and cardiac asystole. Methods. Through a retrospective search, we identified patients who were shown to have had asystole using a database of patients who underwent prolonged video-EEG monitoring during a 68-month period. We compared the data of 18 consecutive patients; five with ictal asystole and 13 with cardiac asystole, with 121 and 64 events recorded from them, respectively. Results. Of the 10,096 patients who underwent prolonged video-EEG monitoring during the study period, we identified 18 (0.17%) patients with asystole. Cardiac asystole was 2.6 times more frequent than ictal asystole. Older age at onset, heralding symptoms of presyncope, occurrence during wakefulness, and brief duration of the events supported the diagnosis of cardiac asystole. Ictal asystole events were more protracted, and prolonged asystole more frequently occurred in patients with extratemporal seizures compared to temporal lobe seizures. Asystole occurred in only half of the recorded seizures. Conclusions. The accurate categorization of asystole as seizure-related or heart disease-related has huge implications for management strategy and outcome. The necessity of permanent pacemaker implantation is more frequent and urgent in patients with cardiac asystole because of the greater risk of sudden death. Hence, in patients with an ominous diagnosis of cardiac asystole, a thorough cardiac evaluation should surpass neurological evaluation.     

Clinical evaluation of the patients with epilepsy following febrile convulsions

We studied clinical, EEG and developmental features of 46 epileptic children following febrile convulsions. Incidence of developing epilepsy was 9.9 percent. Eleven patients (group G) out of 46 had generalized epileptic seizures, and 34 patients (group P) had partial seizures. Febrile convulsions of early onset, partial seizures and postictal neurological symptoms were more striking in group P (p less than 0.05), whereas febrile convulsions of late onset and prolonged seizures were slightly dominant in group G. And EEG abnormalities were more frequent in group P (p less than 0.05). Group P patients had significant number of risk factors (complex features of febrile convulsions) than group G patients (p less than 0.01). The interval between the last febrile convulsion and subsequent epileptic seizures was shorter in group G (p less than 0.01). Although subsequent epileptic seizures were well controlled in the both groups (91% in group G and 82% in group P), intractable seizures were recognized in 9% of group P patients. The patients who had risk factors of prolonged seizures, postictal neurological symptoms and early onset manifested poor controlled epileptic seizures (p less than 0.01). Motor or mental deficits were more frequently associated with group P: in some patients they had been observed before the onset of febrile convulsions. These results suggest that pathogenesis of epilepsy following febrile convulsions may be different among various seizure types of subsequent epilepsy. And the risk factors during febrile convulsions may be related to the prognosis of subsequent epileptic seizures as well as the incidence of developing epilepsy.     

Syncope,seizure-induced bradycardia and asystole: Two cases and review of clinical and pathophysiological features

Episodes of transient loss of consciousness (TLOC) are commonly due to syncope or epileptic seizures. The distinction between both entities on clinical grounds and eyewitness accounts can be challenging and is often hampered by similar clinical features. We briefly summarize syncope-related symptoms and present the case of a female patient who suffered from TLOC episodes due to both reflex syncope and epileptic seizures. Seizure-induced syncope is a rare complication particularly of non-generalized temporal lobe seizures and may be suspected in people with epilepsy who report new semiological features with sudden onset of atonia, TLOC and seizure-related falls. We review epidemiological, clinical and electroencephalographic aspects of seizure-related asystole and syncope and discuss their clinical relevance. The implantation of a cardiac pacemaker appears to efficiently prevent seizure-related falls and consecutive injuries and is an important treatment option if full seizure-control cannot be achieved in these patients. We describe a second case of a patient with refractory temporal lobe epilepsy and seizure-related syncope which ceased after the implantation of a cardiac pacemaker.     

Over-interpretation of electroclinical and neuroimaging findings in syncopes misdiagnosed as epileptic seizures.

Syncope and epileptic seizures share some common clinical characteristics that may complicate the diagnostic process. In clinical practice, syncope is frequently misdiagnosed as an epileptic seizure and consequently treated with antiepileptic drugs. In this study, we identified 57 patients with syncope (diagnosis based on accepted criteria) who had come to our unit with a previous diagnosis of definite epilepsy in 30 cases (syncope misdiagnosed as epileptic seizures, SMS), or suspected epilepsy in the remaining 27 cases (unrecognized syncope, US). We attempted to identify factors underlying misdiagnosis by reviewing clinical findings, particularly potentially confounding features, and EEG/neuroimaging data. Finally, we compared these two groups of patients to search for crucial elements that had led to misdiagnosis. Although some clinical elements were found to be confounding in both groups, it was the interpretation of the EEG and MRI findings, particularly when combined with the confounding clinical features that constituted the main reasons for misdiagnosis.     

Cardiac asystole in epilepsy: clinical and neurophysiologic features

PURPOSE: Cardiac asystole provoked by epileptic seizures is a rare but important complication in epilepsy and is supposed to be relevant to the pathogenesis of sudden unexplained death in epilepsy (SUDEP). We sought to determine the frequency of this complication in a population of patients with medically intractable epilepsy and to analyze the correlation between EEG, electrocardiogram (ECG), and clinical features obtained from long-term video-EEG monitoring. METHODS: Retrospective analysis of the clinical records of hospitalized patients from May 1992 to June 2001 who underwent long-term video-/EEG monitoring. RESULTS: Of a total of 1,244 patients, five patients had cardiac asystole in the course of ictal events. In these patients, 11 asystolic events, between 4 and 60 s long in a total of 19 seizures, were registered. All seizures had a focal origin with simple partial seizures (n = 13), complex partial seizures (n = 4), and secondarily generalized seizures (n = 2). One patient showed the longest asystole ever reported (60 s) because of a seizure. Cardiac asystole occurred in two patients with left-sided temporal lobe epilepsy (TLE) and in three patients with frontal lobe epilepsy (FLE; two left-sided, one bifrontal). Two patients reported previous cardiac disease, but only one had a pathologic ECG by the time of admission. Two patients had a simultaneous central ictal apnea during the asystole. None of the patients had ongoing deficits due to the asystole. CONCLUSIONS: These findings confirm that seizure-induced asystole is a rare complication. The event appeared only in focal epilepsies (frontal and temporal) with a lateralization to the left side. A newly diagnosed or known cardiac disorder could be a risk factor for ictal asystole. Abnormally long postictal periods with altered consciousness might point to reduced cerebral perfusion during the event because of ictal asystole. Central ictal apnea could be a frequent associated phenomenon.     

Clinical correlate of EEG rhythmicity.

The widespread use of the routine EEG in clinical practice was a major development in the treatment of patients with ill-defined spells thought to be epileptic. Not every finding on the EEG is suggestive of epilepsy, and the EEG is subject to over-interpretation, which may lead to misdiagnosis and incorrect treatment. Although supplemented by other procedures, the EEG remains a cost-effective and noninvasive way to diagnose spells. To enhance further the diagnostic use of the EEG, it is important to determine how strongly patterns are correlated with clinical seizures. The authors studied one EEG pattern, lateralized bursts of theta, and found the rhythmicity of the pattern to be most strongly correlated with seizures.     

Ictal asystole mimicking seizure deterioration in temporal lobe epilepsy

We report on a patient with temporal lobe epilepsy, secondary to a left lateral temporal cavernoma, in whom the change in seizure semiology suggested recurrence of secondary generalized seizures. Anticonvulsive medication previously controlled secondary generalized seizures over a period of years but focal seizures continued at a lower rate. Continuous video‐EEG monitoring revealed ictal asystole associated with myoclonic syncope and falls during focal seizures arising from the left temporal lobe. After implantation of a cardiac pacemaker, no more falls occurred during the focal seizures. In conclusion, recurrence of seizure‐associated falls is typically attributed to recurrence of secondary generalized seizures, however, ictal asystole should be considered in selected epilepsy patients as a differential diagnosis of falls. [Published with video sequence]     

Prolonged generalized epileptic seizures triggered by breath-holding spells

We report a 3-year-old female with anoxic-epileptic seizures. Beginning at 11 months of age, she had repeated breath-holding spells with transition into generalized tonic-clonic seizures or status epilepticus. Interictal electroencephalography exhibited no abnormalities. A multidisciplinary diagnostic approach revealed a severely disturbed mother-daughter relationship that was the trigger of the breath-holding spells. Psychotherapy for the mother and daughter led to cessation of the breath-holding spells and, consequently, of the anoxic-epileptic seizures. Her further development was largely normal. We discuss the etiology and treatment of anoxic-epileptic seizures. This case is the first reported case of anoxic epileptic seizures that responded to psychologic rather than antiepileptic treatment. We advocate an initial psychologic assessment to help determine the appropriate treatment in children with recurrent anoxic-epileptic seizures.     

Temporal lobe seizures triggering recurrent syncope by ictal asystole

Described here is a case of a patient with left temporal lobe epilepsy and recurrent complex partial seizures associated with asystole. The posturing and myoclonus caused by the nonepileptogenic syncope during the asystole gave the appearance of secondary generalization prompting a surgical evaluation. A distinct atonic phase that interrupts the transition from a complex partial semiology to generalization is a critical clue to the presence of ictal asystole and is easily detected by video/EEG monitoring.     

Comparison of heart rate variability parameters during complex partial seizures and psychogenic nonepileptic seizures

Purpose: Psychogenic nonepileptic seizures (PNES) superficially resemble epileptic seizures. Little is known about ictal autonomic nervous system (ANS) activity changes in epilepsy and PNES. This study compares ictal heart rate variability (HRV) parameters as a reflection of ANS tone in epileptic seizures and PNES, and explores differences between interictal and ictal ANS tone in both patient groups. Methods: Ictal HRV parameters were extracted from single‐lead electrocardiography (ECG) data collected during video–electroencephalography (EEG) recordings of 26 patients with medically refractory temporal lobe epilepsy and 24 age‐ and sex‐matched patients with PNES. One seizure per patient in a resting, wake, supine state was analyzed. Interictal ECG data were available for comparison from 14 patients in both groups. HRV parameters in time and frequency domains were analyzed (low frequency [LF], high frequency [HF], standard deviation of all consecutive normal R wave intervals [SDNN], square root of the mean of the sum of the squares of differences between adjacent normal R wave intervals [RMSSD]). CVI (cardiovagal index), CSI (cardiosympathetic index), and ApEn (approximate entropy) were calculated from Lorenz plots. Key Findings: There were significant differences between ictal HRV measures during epileptic and nonepileptic seizures in the time and frequency domains. CSI (p < 0.001) was higher in epileptic seizures. Time interval between two consecutive R waves in the ECG (RR interval) (p = 0.002), LF (p = 0.02), HF (p = 0.003), and RMSSD (p = 0.003) were significantly lower during epileptic seizures. Binary logistic regression yielded a significant model based on the differences in CSI classifying 88% of patients with epilepsy and 73% of patients with PNES correctly. The comparison between resting and ictal states in both seizure disorders revealed significant differences in RR interval (epilepsy p < 0.001, PNES p = 0.01), CSI (epilepsy p < 0.001, PNES p = 0.02), HF (epilepsy p = 0.002, PNES p = 0.03), and RMSSD (epilepsy p = 0.004, PNES p = 0.04). In patients with epilepsy there were also significant differences in ictal versus interictal mean values of ApEn (p = 0.03) and LF (p = 0.04). Although CSI was significantly higher, the other parameters were lower during the seizures. Stepwise binary regression in the 14 patients with epilepsy produced a significant model differentiating resting state from seizures in 100% of cases. The same statistical approach did not yield a significant model in the PNES group. Significance: Our results show greater ANS activation in epileptic seizures than in PNES. The biggest ictal HRV changes associated with epileptic seizures (CSI, HF, and RMSSD) reflect high sympathetic system activation and reduced vagal tone. The reduced ApEn also reflects a high sympathetic tone. The observed ictal alterations of HRV patterns may be a more specific marker of epileptic seizures than heart rate changes alone. These altered HRV patterns could be used to detect seizures and also to differentiate epileptic seizures from PNES. Larger studies are justified with intergroup and intragroup comparisons between ictal and resting states.     

Cardiac asystole associated with epileptic seizures: a case report with simultaneous EEG and ECG.

Cardiac arrhythmias occurring in association with epileptic seizures are a potential source of diagnostic confusion and a possible cause of sudden unexpected death in epilepsy. A case is described in which simultaneous ambulatory electroencephalography and electrocardiography revealed periods of asystole coinciding with epileptic seizures. The aystole appeared to precede obvious changes in the scalp recorded electroencephalogram (EEG), but clinical attacks and EEG seizure activity were not altered by pacemaker correction of the cardiac arrhythmias.     

24小时动态脑电图癔病诱发试验对癔病与癫痫的鉴别诊断价值

目的寻找鉴别癔病性发作与癫痫发作的有效方法。方法对９６例发作性疾病患者进行２４小时动态脑电图监测及癔病诱发试验（ＡＥＥＧ－ＨＰＴ）研究。结果难治性癫痫疑伴癔病组（１８例）、癔病或癫痫发作诊断不定组（３６例）、癫痫组（１８例）、癔病组（２４例）４组中癔病诱发试验阳性率分别为７７．８％、６６．７％、５．６％、９１．７％；记录到非诱发下自发发作（癫痫或癔病）分别为３、７、３、１例；记录到发作间期癫痫性放电分别为１６、１８、１７、２例。９６．０％的患者明确诊断。结论ＡＥＥＧ－ＨＰＴ是鉴别癔病与癫痫发作的有效方法之一。     

Identifying patients with epilepsy at high risk of cardiac death: signs,risk factors and initial management of high risk of cardiac death

People with epilepsy have a three‐fold increased risk of dying prematurely, and a significant proportion is due to sudden cardiac death or acute myocardial infarctions. The causes of increased cardiovascular morbidity and mortality in epilepsy are manifold and include acute or remote effects of epileptic seizures, the longstanding epilepsy itself or antiseizure treatments. Seizure‐related cardiac arrhythmias are common and comprise bradyarrhythmia and asystole, atrial fibrillation and ventricular tachycardia. The most frequent clinically relevant seizure‐related arrhythmia is ictal asystole that may require implantation of a cardiac pacemaker, whereas seizure‐related ventricular tachycardias are only rarely reported. Takotsubo cardiomyopathy and myocardial infarction are rare complications and predominantly described in association with tonic‐clonic seizures. Epilepsy‐related cardiac complications include a disturbed cardiac autonomic nervous system and acquired dysfunction of the heart (recently defined as ‘epileptic heart’), probably contributing to the abnormalities of cardiac repolarisation and elevated risk of sudden cardiac death in people with epilepsy. If successful, the use of antiseizure medication prevents seizure‐related cardiac arrhythmias and remote cardiac complications. However, enzyme‐inducing antiseizure medications have a negative impact on cardiovascular risk factors, which may further be aggravated by weight gain linked to specific antiseizure drugs. Given the severe consequences of cardiac risks, the aim of this educational review is to explain the many facets of cardiac complications and their underlying causes, and to enable the reader to recognize and manage these risks with the goal to mitigate the cardiac risks in people with epilepsy. Features of syncope are explained in detail, as syncope of all origins can be mistaken as epileptic seizures in people with or without epilepsy, and ictal syncope (i.e. seizure‐induced syncope) can easily be ignored.     

Wavelet analysis for neonatal electroencephalographic seizures

Electroencepholographs (EEGs) of neonatal seizures differ from those of children and adults. This study evaluated whether wavelet transform analysis, a nonstationary frequency analysis of EEG, can recognize and characterize neonatal seizures. Twenty-second segments were analyzed from 69 EEG seizures in 15 neonatal patients whose seizures lasted 10 seconds or longer. The wavelet transform results were examined, as were EEG seizure durations and dominant frequencies. The wavelet transform results were correlated with the occurrence, after an 18-month follow-up, of postneonatal seizures. Wavelet transform analysis identified 40 seizures (58%) with a "sustained dominant frequency component" that lasted 10 seconds or longer and 29 seizures without a sustained dominant frequency component. The mean seizure duration of the 40 seizures with sustained dominant frequency components was 63.3 seconds, longer than the mean duration (33.6 seconds) of the seizures without sustained dominant frequency components, P < 0.01. Eleven patients manifested postneonatal epileptic seizures. Fifty-two EEG seizures in these 11 patients revealed more sustained dominant frequency components (74%) than 17 seizures in the 4 patients without postneonatal seizures (only 12%), P < 0.05. Wavelet transform analysis can identify neonatal EEG seizures and characterize their epileptic components. The presence of sustained dominant frequency components may predict postneonatal epileptic seizures.