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].     

Tilt table testing in patients with suspected epilepsy

Background –  Approximately 20–30% of patients with epilepsy are misdiagnosed and syncope often seems to be the mistaken cause. We re-evaluated patients referred to an epilepsy clinic where suspicion of neurally mediated (reflex) syncope were raised using tilt table testing (HUT).
Methods –  HUT laboratory results and medical records of 120 consecutive patients were reviewed retrospectively over a period of 27 months.
Results –  HUT was positive in 59 (49%) patients. Seventeen of 38 (45%) patients previously diagnosed with epilepsy and taking antiepileptic drugs were found to be misdiagnosed. Four of 21 patients with epilepsy (19%) had dual diagnoses of reflex syncope and epilepsy.
Conclusion –  HUT is an informative investigation when suspicions of reflex syncope are raised in patients referred to an epilepsy clinic. Reflex syncope is an important and common differential diagnosis of epilepsy.     

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 comparison of self-reported quality of life between patients with epilepsy and neurocardiogenic syncope

Epilepsy and neurocardiogenic syncope share a final common pathway of loss of consciousness and consequent social disruption. We compared 52 patients with syncope, 96 with epilepsy and 100 controls. Epilepsy and syncope patients expressed significantly higher levels of anxiety and depression and reported significantly less good quality of life (QoL) compared with controls. There were no significant differences on any of the QoL parameters measured between the syncope and epilepsy patients. These findings suggest the main contributor to poor QoL in epilepsy may be the unpredictable loss of control that is the hallmark of the condition.     

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.     

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.     

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.     

Severe cardioinhibitory vasovagal syncope in sleep and supine posture

Severe cardioinhibitory vasovagal syndrome is characterised by syncope accompanied by cardiac asystole which may lead clinically to seizure‐like motor activity. Vasovagal syncope usually occurs in erect posture and is often provoked by emotional or physical triggers. We report two patients who presented with severe cardioinhibitory vasovagal syncope accompanied by cardiac asystole resulting in seizure‐like motor manifestations in sleep and supine posture. Both cases were initially diagnosed as epilepsy and treated with antiepileptic drugs. We discuss the putative mechanisms of this rare condition and its potential for misdiagnosis as epilepsy. [Published with video sequences]     

Usefulness of ocular compression during electroencephalography in distinguishing breath-holding spells and syncope from epileptic seizures

Episodes of syncope or breath-holding spells are often misdiagnosed as epileptic events. The purpose of this study was to assess the usefulness of an electroencephalogram (EEG) with ocular compression to distinguish breath-holding spells and syncope from epileptic seizures. A retrospective analysis was performed on the EEG records of all children on whom ocular compression was performed from 2000 to 2003. Data from 116 patients with a clinical diagnosis consistent with either syncope or breath-holding spells were compared with a group of 46 patients with epilepsy. The RR interval during ocular compression was significantly higher in syncope patients compared with patients with epilepsy (P < .005). Using 2 seconds of asystole as the cutoff, the sensitivity of ocular compression was 26%, with 100% specificity. The change in RR interval from baseline to ocular compression also distinguished patients with breath-holding spells and syncope from patients with epilepsy. Even a small increase of 0.5 seconds in the RR interval demonstrated a sensitivity of 46%, with a specificity of 98%. Ocular compression performed during an EEG is useful in distinguishing patients with breath-holding spells and syncope from those with epileptic seizures. A requirement of a 2-second period of asystole with ocular compression excludes many patients. Our data indicate that an RR interval increase of 0.5 seconds over baseline identifies additional patients with increased vagal tone. Prompt and accurate diagnosis of the etiology of loss of consciousness might preclude the need for further extensive and expensive evaluation and reduce patient and parental distress.     

Unrecognized paroxysmal ventricular standstill masquerading as epilepsy: a Stokes-Adams attack.

Recognition of cardiac syncope masquerading as epilepsy may be difficult in the Emergency Department. We report a middle-aged man with recent onset convulsions who posed a diagnostic puzzle before it was found that he had paroxysmal ventricular standstill with complete atrioventricular block: he made a complete recovery after temporary pacemaker insertion. The main lessons from this case were (1) a convulsive seizure of only seconds duration and with an abrupt return of consciousness suggests syncope not epilepsy, (2) repeated, convulsive syncopes without provocation suggest cardiac syncope, (3) a 12-lead ECG should be recorded as soon as possible after such a series of episodes and should not be discontinued until an event is captured, and (4) Emergency Department clinicians should be familiar with any automatic gain on their ECG machine, lest fast, atrial activity be mistaken for narrow complex tachycardia. In summary, a good clinical history is of prime importance in differentiating convulsive syncope from epilepsy, and a simple, non-invasive cardiovascular evaluation may help to diagnose the condition as cardiac syncope.     

Significance of ambulatory electroencephalography for differential diagnosis of epilepsy and syncope★

BACKGROUND： Twenty-four hour ambulatory electroencephatography （AEEG） provides advantages for continuous electroencephalogram, monitoring brief loss of consciousness complicated by suspect or mild limb spasm. OBJECTIVE： To explore the significance of AEEG for differentially diagnosing epilepsy and syncope, compared to EEG. DESIGN, TIME AND SETTING： Sixty patients with brief loss of consciousness, complicated by suspect or mild limb spasm, were selected from Suqian People＇s Hospital between January 2006 and June 2007. PARTICIPANTS： Sixty participants comprised 34 males and 26 females, aged 13-64 years. According to clinical symptoms prior to the study, 36 patients were initially diagnosed with epilepsy and 24 with syncope. METHODS AND MAIN OUTCOME MEASURES： Abnormalities and epileptiform discharge were detected using EEG and AEEG, and the diagnostic value of the two methods for epilepsy and syncope was compared. RESULTS： A total of sixty patients were included in the final analysis. Abnormal AEEGs were observed in 37 cases （62%） and epileptiform discharge AEEGs in 23 cases （38%）, both of which were significantly greater than EEGs [37% （22/60）, 18% （11/60）, respectively, P 〈 0.01, 0.05]. The detection rate of abnormal AEEG and epileptiform discharge in the epilepsy group [75% （27/36）, 47% （17/36）, respectively was significantly greater than in the syncope group [42% （10/24）, 25% （6/24）, respectively, P 〈 0.01, 0.05]. CONCLUSION： AEEG can improve detection probability of epileptiform discharge and exhibits significant differences in the differential diagnosis of epilepsy and syncope.     

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.     

Seizure clusters in drug‐resistant focal epilepsy

We investigated clinical factors associated with seizure clustering in patients with drug‐resistant focal epilepsy and any association between seizure clustering and outcome after surgery. We performed a retrospective study including patients with a diagnosis of drug‐resistant focal epilepsy who underwent epilepsy surgery. Patients were prospectively registered in a database from 1986 until 2015. Seizure cluster was defined as two or more seizures occurring within 2 days. Potential risk factors for seizure clustering were assessed. To investigate any potential association between seizure clusters and seizure outcome after surgery, time to event analysis was used to produce a Kaplan‐Meier estimate of seizure recurrence. We studied 764 patients. Seizure clusters were reported in 23.6% of patients with temporal lobe epilepsy (TLE) and 16.9% of extratemporal patients (p = 0.2). We could not identify any significant clinical factors associated with seizure clustering. Among patients with TLE, those who had history of seizure clusters fared better after surgery (p < 0.01). We found that seizure clusters relate to prognosis after temporal lobe surgery in drug‐resistant TLE. These data may provide added value for surgical prognostication when combined with other data types. A better understanding of the neurobiology underlying seizure clusters is needed.     

Syncope as a first sign of seizure disorder

We studied the long-term follow-up of patients with the diagnosis of "syncope of unknown origin," and their progression to epilepsy to gain a better understanding of the relationship between syncope and epilepsy, and to determine whether findings of the first syncopal attack have prognostic significance in relationship to the onset of epilepsy or not. Eighteen patients with the diagnosis of syncope of unknown origin were evaluated for the possibility of becoming epileptic during a 4-year period, and four patients showed characteristic seizure disorder. There were no clinical or laboratory features that differentiated them from the nonepileptic group, except that they were all girls. The interval between the first syncopal attack and the typical epileptic seizure ranged between 7 and 19 months. Syncope of unknown origin could be the first sign of an epileptic disorder, especially in girls. Long-term follow-up extending up to 1 year is necessary to disclose the risk of becoming epileptic.     

Ictal asystole in temporal lobe epilepsy before and after pacemaker implantation.

Events of ictal bradycardia or asystole may be of relevance in epilepsy patients presenting with ictal falls, and are a potential contributor to SUDEP. The literature on ictal bradycardia or asystole is anecdotal and consists of case reports and small case series. There are no guidelines for the care of patients with ictal arrhythmias. Insertion of cardiac pacemakers may prevent life-threatening cardiac arrest, syncope and trauma.We report the case of a 41-year-old man who presented with refractory partial seizures resulting in syncope leading to severe head trauma, as the only ictal semiology. During presurgical video-EEG monitoring, two episodes of ictal bradycardia followed by asystole and syncope were recorded. A cardiac pacemaker was implanted. At the nine-month follow-up, the patient reported no overt seizures, syncopes or traumatic falls. Our case demonstrates that implantation of a cardiac pacemaker while continuing AEDs may render a patient free from ictal symptoms and prevent ictal syncope and subsequent trauma. [Published with video sequences].     

Video-EEG in syncopal attack due to ocular compression in an adolescent mistreated for epilepsy.

Vasovagal syncope can be confused with epileptic seizure. In this situation, eye compression may be performed during EEG. We present a video-EEG of a patient in whom eye compression produced a typical syncope. Before this recording, the patient had been treated for five years with sodium valproate because of a misdiagnosis of epilepsy. The diagnosis of neurally-mediated syncope was not difficult on the basis of the clinical history, but the reproduction of syncope reinforced the correct diagnosis. Ocular compression is now only indicated in exceptional circumstances and should be performed with precautions. The video of this episode in an adolescent should be of considerable educational value to those who are confronted to diagnostic work-up of transient loss of consciousness.     

Familial vasovagal syncope and pseudosyncope: Observations in a case with both natural and adopted siblings

This report describes an 11-year-old girl with recurrent syncope beginning at the age of 2 1/2 years. Her paternal grandmother, father, and three of her five natural siblings had similar symptoms, often linked to emotional upsets. There were three adopted children from a single family, and none of these had syncope. Prior to referral there was an increase in attacks, some with convulsions, but with no other features of epilepsy. Vasovagal syncope was confirmed. However, continuous electroencephalogram, blood pressure, and heart rate recordings during attacks indicated that in some episodes there was neither cardiovascular change nor epileptiform activity, implying feigned syncope (pseudosyncope) with pseudoseizures. A psychological origin was sought, found, and in part rectified. The separation of vasovagal syncope from pseudosyncope, in the context of the family history, is discussed.     

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.     

Spécificités des crises d’épilepsie chez le sujet âgé : proposition d’un score électro-radioclinique d’orientation

Diagnosis of epileptic seizure may be difficult in older patients because seizure manifestations are often unusual: confusion, paresis… and because there are multiple differential diagnoses (syncope, transient ischemic attack, transient global amnesia…). To promote and facilitate the diagnosis of seizures in the elderly, neurologists and gerontologists must work together and focus their strategy on two points: firstly, the knowledge of the specific presentation of seizures in elderly patients, and secondly, the adoption of a reasoning based on seizures and not epileptic syndromes. A multidisciplinary group worked on epilepsy of the elderly to elaborate an electro-clinical score which aims to help establish the diagnosis of epilepsy in elderly patients in different clinical settings. This electro-clinical score is based on a systematic review of scientific literature and the recommendations are explicitly linked to supporting evidence. Further, clinical validation of the electro-clinical score is required.