Usefulness of the Head-Upright Tilt Test for Distinguishing Syncope and Epilepsy in Children

PURPOSE: Episodic loss of consciousness in children, whether or not associated with hypertonia or short-duration clonic movements, presents a diagnostic challenge to the pediatrician and child neurologist. We provide some evidence of the usefulness of the head-upright tilt test for investigating the causes of transient loss of consciousness in children, and for distinguishing between syncope, convulsive syncope, and epilepsy. METHODS: We studied nine children previously diagnosed as epileptic on the basis of compatible clinical events and epileptiform findings in routine EEGs who were treated over the long term with antiepileptic drugs, but whose clinical records suggested syncope or convulsive syncope rather than epilepsy on reevaluation. All subjects underwent head-upright tilt testing. RESULTS: The tilt-test result was positive in all nine cases, with the patients reporting the same symptoms as in the previously considered epileptic attacks. CONCLUSIONS: Inadequate histories and misuse/overinterpretation of EEG results often lead to misdiagnosis of epilepsy in children. The head-upright tilt test is a useful and reliable diagnostic technique, allowing syncopal events to be induced and evaluated under controlled conditions. In a subset of patients, it may help to distinguish epilepsy from simple or convulsive syncope.     

A simultaneous electrocardiogram is important when electroencephalography is used in the evaluation of loss of consciousness

This study examined the use of electroencephalograms (EEGs) in 2 groups of patients with loss of consciousness (LOC), and the importance of a simultaneous electrocardiogram (ECG). Each group consisted of 75 patients referred for EEG. Group 1 patients (LOC without an established cause) had no clear history of convulsive disorder, while group 2 patients (LOC secondary to epilepsy) did. In all patients, it was ascertained if any previous cardiac investigations had been performed. All patients had an EEG performed with a simultaneous ECG. In addition to reporting the EEG, the neurologist reviewed the ECG rhythm and QRS morphology from the strip recording in an attempt to use this as a cardiac screening test. A cardiologist with the availability of all strip recordings and a subsequent 12 lead ECG reviewed his findings.The ECG was classified as significantly abnormal (potentially capable of causing syncope) in 17% of group 1 patients and 5% of group 2. The neurologist, using the simultaneous ECG strip recording only, correctly identified 14 of the 17 significantly abnormal ECGs (84%) and 127 of the 133 normal cases. In most cases, this was the only ECG evaluation as the EEG was performed without any prior cardiac investigation in 53% of group 1 patients and 92% of group 2.In conclusion, the EEG was often used as an initial investigation in these patients with LOC. A simultaneous ECG strip enabled the neurologist to detect most patients with significantly abnormal ECGs or rhythm. This will allow appropriate early cardiac review of these patients.     

A case of Brugada syndrome with convulsive seizure during antidepressant administration: relation of antidepressant agents and arrhythmia leading to sudden death]

Brugada syndrome is an arrhythmogenetic disease characterized by electrocardiographic ST segment elevation in right precordial leads, which is called "coved type", and an increased risk of sudden death as the result of ventricular fibrillation. We presented a case of Brugada syndrome with a convulsive seizure, during administration of a tricyclic antidepressant for the treatment of a depressive state. A 43-year-old man with bipolar II disorder and obsessive-compulsive disorder was admitted to our hospital for the treatment of a depressive state. There was no medical history of heart failure. Nortriptyline was effective for his depressive as well as for obsessive symptoms. During the treatment, however, he presented a convulsive syncope. Electrocardiography (ECG) showed "coved type" ST segment elevation, and the patient consulted a cardiologist. Electrophysiological study revealed Brugada syndrome, and an implantable cardioverter defibrillator was placed. An overdose of antidepressants has been reported to produce a Brugada-type ECG because of its Na channel antagonism. However, in the present case, the abnormal ECG findings occurred following a usual dosage of nortriptyline. Thus, it is suggested that Brugada syndrome is related to a susceptibility to antidepressants in the present case. Every psychiatrist managing antidepressant therapy should be aware of Brugada syndrome and this ECG pattern, which may be a marker of sudden death.     

NATURAL HISTORY OF MICTURITION SYNCOPE

A follow-up study was performed on 10 patients with micturition syncope 5–15 years after their initial attack. Three patients had died of cardiac or pulmonary diseases (at age 61, 69 and 82 years). One had developed focal motor epilepsy which was considered to be unrelated to his previous attack of micturition syncope. Only (age 60) developed a hemianopsia in connection with his micturition syncope. Only one patient had had a new attack of micturition syncope which had occurred during a febrile illness. Two often sit down prophylactically during micturition. It is concluded that micturition syncope most often is a benign phenomenon which should be distinguished from epilepsy. The patient may be advised to sit down during micturition, especially at night, but treatment is otherwise not necessary.     

Cardiogenic syncope diagnosed as epileptic seizures: the importance of ECG during video‐EEG recording

We describe four patients with a previous diagnosis of epilepsy. After reviewing the ECG during the video‐EEG recording, cardiogenic syncope, resulting from a cardiac arrhythmia, was identified as the cause of the seizures. Epileptic disorders and cardiogenic syncope may both manifest with convulsions, loss of consciousness, and loss of postural tone, leading to a high level of misdiagnosis. The one‐lead ECG during video‐EEG monitoring is a key component, which may allow correct diagnosis and treatment.     

Differenzialdiagnose paroxysmaler Bewusstseinsstörungen

Paroxysmal cardiac arrhythmias are an important differential diagnosis of epileptic seizures. Their clinical diagnosis is of particular importance as cardiac syncope is associated with a higher mortality. Therefore an actual surface ECG should be carefully examined and further cardiological diagnostics should be considered.We report on a patient with torsade de pointes tachycardia due to a long QT syndrome. Representative episodes were recorded during video EEG monitoring, as a diagnosis of epilepsy was assumed.     

Effects of seizure repetition on postictal and interictal neurocardiac regulation in the rat

PURPOSE: Human epilepsy is associated with abnormalities in cardiac regulation, as measured by reductions of heart rate variability (HRV) and approximate entropy (ApEn), but it is not known how these abnormalities are related to seizure experience. METHODS: Baseline electrocardiogram (ECG) was recorded from seizure-naive rats. They were subjected daily to maximal electroshock (MES), which induced tonic seizures with hindlimb extension, for a total of 10 days. ECG was obtained for 30 min before and after the first and last seizure. R-R variability, spectral variability, and ApEn were calculated to determine changes in pre- and postictal cardiac regulation. Before the last seizure, interictal parameters were compared with baseline values to determine changes in interictal HRV as a consequence of seizure repetition. Postictal values obtained after the last seizure were compared with the initial postictal data to look for changes in postictal cardiac regulation. RESULTS: During the postictal state, a mild, but significant, loss of ApEn was present after either the first or last seizure. Seizure repetition induced loss of R-R variability and high-frequency spectral band, which was present both interictally and postictally. CONCLUSIONS: The results suggest that convulsive seizures are associated with an immediate reduction of the complexity of cardiac rhythm regulation, as reflected by reductions of ApEn. Seizure repetition may induce long-term neural abnormalities in neurocardiac regulatory systems, especially parasympathetic, which limit appropriate autonomic responses. These acquired abnormalities may, in turn, predispose individuals to cardiac arrhythmia and sudden unexpected death in epilepsy.     

Cough syncope mimicking epilepsy in asthmatic children

Cough syncope is a more common complication of childhood asthma than formerly recognized. We report twelve children with typical cough syncope who were identified in a pediatric clinic over a period spanning seven years. The condition may be confused with epilepsy because of frequently associated brief clonic convulsive movements during the height of the cerebral anoxia. Cough syncope is readily distinguished from epilepsy by a thorough history. The management and prevention of cough syncope is directed at the aggressive control of bronchospasm in children with asthma.     

Cardiogenic syncope. Seizure versus syncope

Our recommendations for the evaluation of the patient with suspected cardiogenic syncope are as follows: An initial thorough history and physical examination of the patient will suggest the diagnosis of cardiogenic syncope in at least 50 per cent of patients. A sudden occurrence of syncope, or "drop attack", is most suggestive of an arrhythmic cause for syncope. If there is no evidence of neurologic, metabolic, or obvious cardiac (for example, aortic stenosis) cause for syncope, a 24-hour ambulatory ECG should be performed. If the patient has had repeated syncopal episodes or if the patient has had seizures that are difficult to control with anticonvulsant therapy, ambulatory ECG monitoring may reveal an arrhythmia in these preselected patients. If the initial 24-hour ambulatory ECG is negative and if the patient has had multiple episodes of syncope, multiple days of recording will be necessary before typical symptoms occur that correlate with arrhythmias. Seventy-two hours of ambulatory ECG monitoring should be sufficient in most instances to establish an arrhythmic cause for syncope. Echocardiography and exercise stress testing are not routinely indicated in the evaluation of syncope unless the clinical history and examination suggest valvular or coronary disease. Rarely, an atrial myxoma will be visualized by echocardiography that is not apparent clinically. Those patients who still have unexplained recurrent syncopal episodes, despite all noninvasive studies, can benefit from invasive electrophysiologic studies, although the expected yield from electrophysiologic testing is low.     

Psychogenic pseudosyncope: an underestimated and provable diagnosis

OBJECTIVE: The goal of this study was to estimate the frequency of psychogenic pseudosyncope in patients with "syncope of unknown origin." Twenty to thirty percent of patients referred to epilepsy centers for refractory seizures have psychogenic seizures. With syncope, about 20-30% of the cases remain unexplained after a complete evaluation, but, unlike in seizures, a psychogenic etiology is not usually investigated. METHODS: We prospectively evaluated patients referred to our epilepsy center for evaluation of recurrent syncope-like episodes, that is, limp, motionless fainting. All patients had a negative syncope workup. We performed EEG-video monitoring with activation by suggestion ("induction"), similar to what is used for diagnosis of psychogenic seizures. Activation was performed with patients standing or sitting up. The diagnosis of psychogenic pseudosyncope required: (1) an activation procedure that triggered the habitual event; (2) a clinical event of loss of postural tone and limp, motionless unresponsiveness with eyes closed; (3) normal EEG before, during, and after the clinical event, that is, no epileptiform abnormalities, a normal alpha rhythm during unresponsiveness, and no suppression of background or slowing as is typically seen in syncope. RESULTS: Ten patients were recruited over an 18-month period. Habitual syncope-like episodes were triggered in 9 of 10 (90%) patients, and all 9 were shown to have psychogenic pseudosyncope (eyes closed, motionless, unresponsive with normal EEG including normal alpha rhythm). In one patient, no episode was triggered, so a diagnosis could not be made. Among the 9 patients for whom episodes were recorded, age ranged from 21 to 60 (mean=36). Five were women. Duration of symptoms ranged from 6 months to 15 years (mean=4.2 years). Event frequency ranged from four per day to two per month. Prior evaluations for syncope included ECG in all patients, two-dimensional echocardiogram in three, Holter monitoring in two, and tilt-table test in five. Four patients had undergone cardiac catheterization, and one had received a pacemaker. Neurologic tests included CT of the head in seven and MRI of the brain in eight. CONCLUSION: Many patients with "syncope of unknown origin" may have psychogenic pseudosyncope, but most such patients do not undergo EEG-video monitoring, which is the only way to demonstrate a psychogenic etiology. Psychogenic pseudosyncope is not simply a diagnosis of exclusion, and can be firmly diagnosed. As is usually recommended for seizure-like events, patients with syncope-like events and a negative evaluation should undergo EEG-video monitoring with induction, specifically looking for a possible psychogenic etiology.     

A 5-year-old boy with nonconvulsive status epilepticus induced by theophylline treatment

A normally developed 5-year-old boy with no history of convulsive disorder presented with a generalized tonic-clonic convulsion on the second day of oral theophylline therapy. Although the convulsive seizure was abolished by an intravenous bolus of diazepam, he did not regain consciousness. Emergency electroencephalography disclosed continuous irregular seizure activity, occurring predominantly over the right hemisphere, indicating a diagnosis of nonconvulsive status epilepticus (NCSE). The serum theophylline concentration was 19.7 microg/mL. Treatment with an initial intravenous bolus of midazolam (0.26 mg/kg) largely restricted seizure activity to the right hemisphere, with activity then diminishing to continuous right occipital spikes. An additional 0.24 mg/kg bolus followed by continuous infusion at a rate of 0.20 mg/kg/hr completely abolished electrical status. Our case suggests that theophylline treatment can provoke NCSE even after successful control of a brief convulsion by initial antiseizure treatment in some patients predisposed toward localization-related epilepsy. This possibility should be kept in mind in any child receiving theophylline who presents with sustained unconsciousness.     

急诊晕厥患者常见原因分析及治疗对策

目的分析急诊患者晕厥的常见原因及治疗对策。方法选择2013-05—2015-05我院就诊的急诊晕厥患者68例,对其临床资料进行回顾性分析,总结急诊晕厥患者的常见原因,提出相关对策。结果所有68例急诊晕厥患者中,神经介导性晕厥患者28例(41.18%),心源性晕厥23例(33.82%),脑源性晕厥8例(11.76%),直立性低血压性晕厥6例(8.82%),其他原因晕厥3例(4.41%),其中,神经介导性晕厥的发病人群主要以中青年为主,而心源性晕厥的好发人群主要以老年人为主。结论神经介导性晕厥是中青年急诊患者最常见的晕厥原因,而老年急诊患者最常见的晕厥原因则是心源性晕厥,急诊工作中医护人员应根据患者临床表现和体征及时查明并确认晕厥原因,并给予合理有效的对症治疗和处理措施,以改善预后,保障患者的生命安全和生活质量。     

Recurrent Takotsubo Cardiomyopathy Triggered by Convulsive Status Epilepticus

Introduction  Takotsubo cardiomyopathy can complicate several conditions including neurological emergencies. A few recurrent cases associated with seizures have been reported, but none of the patients had status epilepticus. The pathophysiology of takotsubo syndrome, although debated, may involve stunning of the myocardium by a catecholamine storm triggered by stress. Patients with epilepsy may be at increased risk for takotsubo syndrome, which may occur repeatedly. Methods  We report on a postmenopausal woman with symptomatic epilepsy who experienced recurrent takotsubo cardiomyopathy triggered by convulsive status epilepticus. Brief seizures were not associated with takotsubo syndrome. The relevant literature was reviewed. Results  Over a 1-year period, she experienced two episodes of convulsive status epilepticus with complete neurological recovery after treatment. Echocardiography showed latero-septo-apical hypokinesia and apical ballooning. The cardiac abnormalities resolved fully and she recovered her baseline level of self-sufficiency. During the same period, she experienced several brief seizures, with no cardiac manifestations. Conclusion  The occurrence of takotsubo cardiomyopathy in association with convulsive status epilepticus, but not with brief seizures, supports neurogenically mediated myocardial stunning related to direct toxicity of endogenous catecholamines. Neuro-intensivists must be aware of this potentially fatal but fully reversible cardiac complication, which may be among the causes of death in patients with status epilepticus.     

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.     

Management and long-term outcome in patients presenting with ictal asystole or bradycardia

Purpose: Ictal asystole (IA) and ictal bradycardia (IB) are rare autonomic symptoms during epileptic seizures and may be potentially life‐threatening. Guidelines for the care of these patients are missing. The aim of this multicenter study was to evaluate the management and long‐term outcome in patients with IA and IB. Patients and Methods: All patients with IA and IB were included from four epilepsy centers (Bielefeld, Kork, Marburg, and Zürich) from 2002 until 2009. Using a standardized assessment form, clinical data, treatment decisions, and outcomes were extracted from patient charts and simultaneous electroencephalography/electrocardiography (EEG/ECG) recordings. Key Findings: Seizures with IA or IB were identified in 16 patients. In all patients an associated temporal seizure pattern was recorded and in 15 patients, sudden falls, fainting, or trauma was previously reported or recorded during the monitoring. In three patients (18.8%) diagnosis of focal epilepsy was newly established and anticonvulsive treatment was initiated. Two patients with refractory epilepsy underwent epilepsy surgery. In seven patients (43.8%) a cardiac pacemaker was implanted. In 14 of 16 treated patients, seizure freedom (n = 5) or absence of sudden falls, fainting, or trauma (n = 9) could be achieved. Two patients denied epilepsy surgery as well as a pacemaker and continue to have frequent falls and trauma. Significance: Our study demonstrates that epilepsy surgery and antiepileptic drugs may lead to sustained freedom of seizures as well as ictal syncope. In drug‐resistant patients not suitable for epilepsy surgery, implantation of a cardiac pacemaker may prevent sudden falls as well as trauma. Based on our results and previously reported cases we propose a treatment algorithm.     

Syncope: case studies

In this series of clinical vignettes, the authors have attempted to provide a "feel" for the varied causes of syncope. The neurologist should be able to diagnose most causes of syncope using a simple algorithmic approach. Initial evaluation includes detailed clinical history, physical examination, and 12-lead ECG. Following initial evaluation, the cause of syncope is usually immediately apparent (typical story for vasovagal syncope, clinically demonstrable autonomic failure, long QT), strongly suspected (syncope preceded by chest pain or palpitations), or uncertain. In the latter group of patients, further workup will depend on the suspicion or documented presence of heart disease. In those with a single episode of syncope and no evidence of heart disease, further workup may not be necessary. In patients over 60 years of age with recurrent episodes and no cardiac history or abnormal ECG, tilt-table testing and carotid sinus massage may be diagnostic. If no diagnosis is found, an implantable loop monitor may be needed. Patients with heart disease will need the most comprehensive evaluations, possibly including exercise testing, cardiac electrophysiology, and tilt-table testing. As better understanding of pathophysiology and epidemiology emerge, under-standing of the diagnosis and treatment of syncope will improve. In the meantime, there is no substitute for astute clinical acumen.     

Autonomic seizures versus syncope in 18q- deletion syndrome: a case report

PURPOSE: The 18q- deletion syndrome (18qDS) is frequently associated with cardiac anomalies. Patients with this syndrome may also have epilepsy, which presents certain diagnostic difficulties. This case report aims to illustrate these diagnostic problems, document the usefulness of heart rate-based seizure detection algorithms in this setting, and define the epilepsy syndrome associated with 18qDS. METHODS: Closed-circuit video electroencephalogram (EEG) monitoring using a heart rate-based seizure detection software was used to identify the event in question and to establish the diagnosis of epilepsy. Chromosomal analysis and magnetic resonance imaging (MRI) were used to further define the epilepsy syndrome. RESULTS: We report on a patient with an atrial septal defect, enlargement of the right heart, and incomplete right bundle branch block, who developed episodes of tachycardia, loss of consciousness, and pallor, for which he was amnesic. Chromosomal analysis demonstrated karyotype 46,XY,del(18)(q21.3). ish del(18)(wcp18+,D18Z1+) with a loss of the gene for myelin basic protein. MRI revealed multifocal dysmyelination. Video-EEG monitoring using an electrocardiogram (ECG)-triggered seizure detection software proved to be indispensable in detecting an autonomic seizure and establishing the correct diagnosis; the procedure also allowed for the definition of the epilepsy syndrome. The patient was treated with carbamazepine and remained seizure-free. CONCLUSIONS: Video-EEG monitoring using a heart rate-based seizure detection software can be helpful in diagnostically differentiating autonomic seizures from syncope. Dysmyelination due to loss of the myelin basic protein gene on 18q and cortical dysgenesis may be of pathogenic relevance.     

Vasovagal syncope misdiagnosed as epilepsy for 17 years: prime importance of clinical history.

Distinguishing epilepsy from syncope often can be challenging. We report a case of a 20-year-old patient with presumed refractory epilepsy since the age of 3 years. Although the clinical suspicion of syncope was raised at the age of 9 years, key historic features were not identified, cardiac work-up was not pursued and despite lack of electrographic evidence of epilepsy, he received anticonvulsant treatment. During his presurgical evaluation for "refractory epilepsy", one typical event was captured that was associated with asystole and normal electroencephalogram. The diagnosis of vasodepressor syncope was made and anticonvulsant medication was discontinued. With this case report, we would like to emphasize the importance of a meticulous history and the need to perform continuous video electroencephalographic with simultaneous electrocardiographic recordings in the evaluation of paroxysmal events with atypical presentation. [Published with video sequences].     

Syncope and seizures

Abstract. Syncope and seizures are common conditions and pose diagnostic challenges for the clinician. The cumulative lifetime incidence of epilepsy is 3%, and the incidence of a first attack of syncope is 6.2 per 1000 person-years in the general population. Although the underlying pathophysiological processes are distinct, seizures and syncope share some clinical characteristics which may lead to diagnostic confusion. Confounding matters further is the fact that seizures and syncope may coexist in a given patient. For example, some conditions such as prolonged QT syndrome may result in both arrhythmia-associated syncope and seizures, and in some cases, seizures may result in cardiac arrhythmia and syncope. The clinical history, examination, electrocardiography, and combined EEG/ECG telemetry are all helpful in the diagnosis of seizures and syncope.     

Multidisciplinary approach for diagnosing syncope: a retrospective study on 521 outpatients

OBJECTIVES: To describe causes of syncope in outpatients in whom structural heart disease was ruled out as a cause, and to analyse the role of a multidisciplinary approach in a syncope unit for the diagnosis of patients with syncope of unknown origin. METHODS: Cardiovascular autonomic nervous system (ANS) function was evaluated extensively in 521 outpatients by careful history, physical examination including orthostatic blood pressure measurement and standard ECG, and tilt testing. RESULTS: Causes of syncope remained unknown in 29.2% of cases. ANS dysfunction was found in 58.6% of those presenting with either neurally mediated syncope (53.6%) or chronic autonomic failure (5%); 3.8% of the patients suffered from syncope of cardiogenic origin (2.5%) or non-neurogenic hypotension (1.3%), and 8.4% had loss of consciousness of non-syncopal origin. Loss of consciousness was confirmed as being related to seizures in under 30% of patients initially diagnosed as having epilepsy. CONCLUSIONS: Neurally mediated syncope represents the commonest type of syncope. ANS evaluation including tilt testing should be considered as preliminary screening in patients with syncope in the absence of definite heart abnormalities. Neurologists should consider syncope from ANS failure as a comorbid factor in patients with seizures where the clinical characteristics are not straightforward.