Inhibitory motor seizures: correlation with centroparietal structural and functional abnormalities

Six adults and 2 children with focal inhibitory motor seizures (ictal paralysis) were evaluated during a 4-year period. Paresthesias at seizure onset occurred during some seizures in all patients, and focal clonic activity followed paralysis in 4. EEG-CCTV recordings of the seizures in 2 patients showed that ictal paralysis coincided with an ictal discharge starting in one centroparietal area. MRI showed centroparietal structural lesions in six patients. One patient with a normal MRI scan had right centroparietal hypometabolism on PET. Inhibitory motor seizures must be differentiated from transient ischemic attacks and migraine. In our patients a centroparietal epileptogenic focus was suggested by neuroimaging studies, and in 2 instances by ictal EEG.     

Seizures and syncope:

Abstract Although pathophysiologically distinct, syncope and seizures share clinical characteristics which may make diagnosis difficult. Adding to diagnostic complexity are the facts that seizures and syncope may coexist in the same patient, syncope may be associated with seizure-like motor manifestations, and seizures may be complicated by cardiac arrhythmia and syncope. Combined EEG/ECG telemetry is sometimes necessary to establish the correct diagnosis. These techniques also provide an opportunity to study the role of certain cortical regions in the modulation of cardiac function. There is an increasing understanding of the central autonomic pathways involved in the genesis of the cardiovascular changes that occur during epileptic seizures. This article reviews the use of EEG/ECG telemetry in the evaluation of syncope and seizures, and the neuroanatomic circuitry involved in the production of the cardiovascular manifestations of seizures.     

Interictal change in cardiac autonomic function associated with EEG abnormalities and clinical symptoms: a longitudinal study following acute deterioration in two patients with temporal lobe epilepsy

The purpose of the present study was to investigate the ictal and interictal changes in cardiac autonomic function (CAF), and the relationship between the interictal change in CAF to the electroencephalogram (EEG) and clinical findings. In two patients with temporal lobe epilepsy (TLE) showing acute deterioration, a quantitative evaluation of their interictal CAF based on heart rate variability and their EEG using spectral analysis was conducted, and the findings compared with repeated clinical evaluations during the recovery period. The ictal heart rate changes and their temporal relationship to ictal discharge were investigated using simultaneous EEG/electrocardiogram (ECG) monitoring in one of the patients. Interictal parasympathetic function was decreased during the period of acute deterioration, but was increased in association with improvements in the EEG and clinical findings. In contrast, the sympathetic function showed no specific changes. The ictal discharges were preceded by a brief bradycardia, with a long delay of up to 40s. The results demonstrated that this decrease in parasympathetic function was closely related to the interictal changes in central nervous system function. On the other hand, the ictal discharges in one of the patients were thought to have caused a transient elevation of parasympathetic function. It is strongly suggested that patients with TLE have interictal as well as ictal changes in CAF that are mediated mainly through the parasympathetic nervous system.     

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.     

Video-EEG monitoring in patients with hippocampal atrophy

OBJECTIVES: To analyze the value of ictal EEG recordings in patients with unilateral magnetic resonance imaging (MRI)-identified hippocampal atrophy and concordant interictal epileptiform discharges (IEDs). MATERIAL AND METHODS: The ictal EEG patterns in 84 patients with pharmaco resistant epilepsy undergoing an anterior temporal lobectomy between 1992 and 1995 were reviewed. The concordance between the ictal EEG and MRI and the IEDs was examined. RESULTS: Two-hundred-and-thirty seizures (76.4%) were concordant and 11 seizures (3.7%) were discordant with the atrophic temporal lobe. Sixty seizures (19.9%) were indeterminate in localization. Sixty-three of the 69 patients (91.3%) with confirmatory and 14 of the 15 patients (93%) with non-confirmatory ictal EEG recordings, respectively, experienced an excellent operative outcome (P=0.629, Fisher's exact test). CONCLUSIONS: Patients with unilateral hippocampal atrophy and concordant IEDs are excellent surgical candidates even when video-EEG monitoring shows discordant or non-localizing seizures.     

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.     

Frontal lobe epileptic seizures are accompanied by elevated pitch during verbal communication

The objective of our study was to assess alterations in speech as a possible localizing sign in frontal lobe epilepsy. Ictal speech was analyzed in 18 patients with frontal lobe epilepsy (FLE) during seizures and in the interictal period. Matched identical words were analyzed regarding alterations in fundamental frequency (?o) as an approximation of pitch. In patients with FLE, ?o of ictal utterances was significantly higher than ?o in interictal recordings (p = 0.016). Ictal ?o increases occurred in both FLE of right and left seizure origin. In contrast, a matched temporal lobe epilepsy (TLE) group showed less pronounced increases in ?o, and only in patients with right‐sided seizure foci. This study for the first time shows significant voice alterations in ictal speech in a cohort of patients with FLE. This may contribute to the localization of the epileptic focus. Increases in ?o were interestingly found in frontal lobe seizures with origin in either hemisphere, suggesting a bilateral involvement to the planning of speech production, in contrast to a more right‐sided lateralization of pitch perception in prosodic processing.     

Epileptic seizures induced by sexual abuse. Pathogenic and pathoplastic factors

Previous sexual abuse is now thought to be a common cause of non-epileptic seizures (pseudoseizures). However, since sexual abuse is common, a previous history of sexual abuse may also occur in people with actual epilepsy. We present six patients in all of whom sexual abuse may, by acting as a stressor in the already predisposed, have precipitated epilepsy and in some of whom the abuse may have affected the actual experiences of the epilepsy itself: all but one of the patients had partial seizures.     

The self-induction of epileptic seizures by eye closure

Of 22 consecutive epileptic patients sensitive to intermittent photic stimulation, 7 were found to induce paroxysmal activity and/or seizures by a slow closure of the lids accompanied by eye rolling, which in 6 of the 7 was totally different from the movement normally carried out on voluntary eye closure to command. To identify the syndrome it may be necessary to monitor the EEG and oculogram for some 10 min or longer with the eyes open in a well-lit environment.     

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.     

Focal seizures with affective symptoms are a major feature of PCDH19 gene–related epilepsy

Purpose: Mutations of the protocadherin19 gene (PCDH19) cause a female‐related epilepsy of variable severity, with or without mental retardation and autistic features. Despite the increasing number of patients and mutations reported, the epilepsy phenotype associated with PCDH19 mutations is still unclear. We analyzed seizure semiology through ictal video–electroencephalography (EEG) recordings in a large series of patients. Methods: We studied 35 patients with PCDH19 gene–related epilepsy and analyzed clinical history and ictal video‐EEG recordings obtained in 34 of them. Key Findings: Clusters of focal febrile and afebrile seizures had occurred in 34 patients, at a mean age of 10 months. The predominant and more consistent ictal sign was fearful screaming, occurring in 24 patients (70.5%); it was present since epilepsy onset in 12 and appeared later on, during the course in the remaining 12 patients. In infancy, fearful screaming mainly appeared within the context of seizures with prominent hypomotor semiology, whereas during follow‐up it was associated with prominent early motor manifestations. In 16 patients, seizures were video‐EEG recorded both at onset and during follow‐up: in five patients (31%) seizure semiology remained identical, in 7 (44%) semiology varied and in four patients it was unclear whether ictal semiology changed with age. Three patients (9%) had both focal and generalized seizures, the latter consisting of absences and myoclonus. Ictal EEG during focal seizures showed a prominent involvement of the frontotemporal regions (22 patients). About 45% of patients had an alternating EEG pattern, with the ictal discharge migrating from one hemisphere to the contralateral during the same ictal event. Status epilepticus occurred in 30% of patients. Cognitive impairment occurred in 70%, ranging from mild (42%) to moderate (54%) and severe (4%); autistic features occurred in 28.5%. Direct sequencing detected 33 different heterozygous candidate mutations, 8 of which were novel. Mutations were missense substitutions (48.5%), premature termination (10 frameshift, 4 nonsense, and 2 splice‐site mutations; 48.5%), and one in‐frame deletion. Thirty candidate mutations (91%) were de novo. No specific genotype–phenotype correlation could be established, as missense and truncating mutations were associated with phenotypes of comparable severity. Significance: Most patients with PCDH19 mutations exhibit a distinctive electroclinical pattern of focal seizures with affective symptoms, suggesting an epileptogenic dysfunction involving the frontotemporal limbic system. Awareness of this distinctive phenotype will likely enhance recognition of this disorder.     

Syncope

Syncope is a common presenting symptom, and is often a challenging diagnostic dilemma because of its various underlying causes. A careful initial clinical assessment with directed investigations is crucial in arriving at a presumptive diagnosis. Prolonged cardiac monitoring technologies have improved diagnostic accuracy in the more difficult cases.     

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.     

Interictal and ictal dipole modelling in patients with refractory partial epilepsy

Fifteen patients (7 men, 8 women) with mean age of 34 years and mean duration of refractory partial seizures of 17 years were included in a presurgical evaluation protocol. Neuroimaging (CAT, 1.5 T MR) demonstrated intracranial structural lesions (space-occupying: n = 9; atrophic: n = 6) and video-EEG monitoring showed complex partial seizures in all patients. Four patients underwent additional intracranial EEG monitoring that demonstrated hippocampal seizure onset in all. Voltage topography and spatiotemporal dipole mapping of interictal epileptic discharges revealed two distinct dipole types. Patients with lesions in the medial (and lateral) temporal lobe uniformly presented with a negative voltage field with a steep gradient over the inferior temporal area and a stable, combined dipole that consisted of a radial and a tangential component with a high degree of elevation relative to the axial plane. Patients with extratemporal lesions had a more diffuse, less dipolar voltage field and a corresponding dipole which was less stable and had a predominant radial component. Dipole modelling of epochs of early ictal discharges revealed a striking correspondence with the interictal findings in individual patients. Interictal spike voltage topography and corresponding dipole mapping provided additional and reliable information that was relevant in surgical candidates for refractory partial epilepsy, e.g. by suggesting in some patients that the medial temporal structures were not primarily involved. Ictal dipole modelling revealed concordant results with interictal data. It shows promising but needs further confirmation and validation in a larger patient population with intracranial EEG recordings. Despite intrinsic limitations, spike voltage topography and dipole mapping contributes to a better localisation of the underlying brain source of epileptic discharges.     

Comparing spiking and slow wave activity from invasive electroencephalography in patients with and without seizures

Objectives

To develop quantitative measures for estimating seizure probability, we examine intracranial EEG data from patient groups with three qualitative seizure probabilities: patients with drug resistant focal epilepsy (high), these patients during cortical stimulation (intermediate), and patients who have no history of seizures (low).

Role of I-123-Iomazenil SPECT imaging in drug resistant epilepsy with complex partial seizures

Fifteen patients with therapy resistant partial complex seizures with no structural lesions were examined interictally with 123-I-IOMAZENIL SPECT for measurement of benzodiazepine receptor distribution and with 99m-Tc-HMPAO SPECT for measurement of cerebral blood flow distribution. Regional abnormalities were correlated with the seizure onset patterns in EEG later recorded with implanted subdural strips. SPECT scans were made immediately after and at 1 and 2 h after intravenous injection of 123-I-Iomazenil. During that time there was a continuous change from an immediate flow-related distribution toward a more specific receptor distribution. The decay of radioactivity of I-123 in the brain was linear over time. Two patients on benzodiazepine treatment showed much faster elimination and showed no focal abnormalities. Eight patients with clear-cut unifocal seizure onset showed concordant focal benzodiazepine defects. These patients showed a progressive focus/homotopic non-focus enhancement over time much larger than the HMPAO scans in the same patients. Also the estimated focal area of abnormality was more restricted in the Iomazenil scans than in HMPAO scans. Five patients had more complex seizure onset patterns. In these patients a mismatch between the locations of abnormalities in Iomazenil and HMPAO scans were often found but benzodiazepine receptor abnormalities were more circumscribed also in these patients. The results suggest that 123-I-Iomazenil SPECT is more useful than 99m-Tc-HMPAO SPECT when applied interictally in patients with partial complex epilepsy, since in addition to demonstrate the hemispheric laterality of the epileptogenic zone, 123-I-Iomazenil appears to indicate its anatomical location with higher confidence, which could be of practical value for positioning of intracranial EEG electrodes.