Analysis of clinical patterns and underlying epileptogenic zones of hypermotor seizures

Background: Hypermotor seizures (HMS) are characterized by complex movements involving the proximal segment of the limbs and trunk. Although they are primarily reported in mesial frontal or orbitofrontal epilepsies, they have also been described in patients with temporal or insular epilepsies, questioning the localizing value of HMS in patients contemplating epilepsy surgery. Furthermore, HMS can include different forms of HM behaviors. Whether these clinical variations may be systematized and may reflect different locations of the epileptogenic zone (EZ) has not been evaluated yet. Methods: We have retrospectively analyzed ictal signs and intracerebral EEG data in 11 patients presenting with HMS who became seizure free following epilepsy surgery with a minimum follow‐up of 24 months. Clinical phenomena were reviewed blinded to seizure onset zone. Results: Two types of HMS could be distinguished in this population: HMS1, observed in six patients, mainly consisted of marked agitation that either included body rocking, kicking, or boxing, associated with a facial expression of fear. HMS2, observed in the five other patients, mainly consisted of mild agitation that included either horizontal movements or rotation of trunk and pelvis while lying on the bed, usually associated with tonic/dystonic posturing. SEEG findings showed that the EZ associated with HMS1 was mainly centered on the ventromesial frontal cortex. Conversely, HMS2 was primarily associated with an EZ localized within the mesial premotor cortex. Conclusions: Although these findings remain to be confirmed by larger studies, they may help optimize the placement of intracerebral electrodes in patients contemplating epilepsy surgery.     

Seizure semiology of lesional frontal lobe epilepsies in children

The aim of the study was to analyse the semiology of seizures in children with frontal lobe epilepsy (FLE) and to compare them with other paediatric cohorts described in the literature as well as with adult counterparts. We analysed 174 registered seizures of 18 cases under 12 years with lesional epilepsy whose frontal origin was defined by the concordance of neuroimaging and ictal electrographic findings, and confirmed by surgery in the six cases operated on. Seizures were generally short, with a high daily frequency and usually related to sleep. The most characteristic semiological pattern consisted of complex motor seizures, particularly hypermotor. Often seizures corresponded to a mixture of different semiological patterns (tonic, gelastic, automotor, hypermotor, versive) presenting in the same seizure, often as a unique type in the same patient. With regard to several aspects the semiology of FLE in our cohort looks like that reported in adult series, in particular as to the frequency of complex motor seizures. However, our cohort was also characterised by a more protean array of seizure semiology, stressing the occurrence of seizures typically present in adults (versive and complex motor) and of some seizure patterns more characteristic in children such as epileptic spasms; moreover, the rare occurrence of secondarily generalised tonic clonic seizures (SGTCS) was confirmed.     

Frontal lobe epilepsy

About one-quarter of patients with refractory focal epilepsies have frontal lobe epilepsy (FLE). The typical seizure semiology for FLE includes unilateral clonic, tonic asymmetric or hypermotor seizures. Interictal electroencephalograms (EEG) usually reveal interictal epileptiform discharges and rhythmical midline theta, which has localizing value. The usefulness of ictal EEG recordings is limited by frequent muscle artifacts in motor seizures and because a large portion of the frontal lobe cortex is “hidden” to scalp electrodes. Ictal single photon emission CT and positron emission tomography are able to localize FLE in about one-third of patients only. A pre-surgical evaluation should include, whenever possible, a subclassification of FLE as dorsolateral frontal, mesial frontal or basal frontal lobe epilepsy to allow a minimal cortical resection. A review of the typical findings of seizure semiology, interictal and ictal EEG regarding the different FLE subtypes is given. Etiology, medical treatment and surgery are also discussed.     

Clinical and ictal characteristics of infantile seizures: EEG correlation via long-term video EEG monitoring

Purpose

The semiology of infantile seizures often shows different characteristics from that of adults. We performed this study to describe clinical and ictal characteristics of infantile seizures at less than two years of age.

Methods

A retrospective study was done for infants with epilepsy (ages: 1–24 months) who underwent long-term video electroencephalography (EEG) monitoring at Samsung medical center between November 1994 and February 2012. We analyzed the clinical and ictal characteristics of the 56 cases from 51 patients.

Results

In 69% of the patients, the seizure onset was before six months of age and the etiology was symptomatic in one third of the patients. Twelve seizure types were identified; spasms (24%), unilateral motor seizures (18%), and generalized tonic seizures (15%) were the three frequent types of seizure.All partial seizures were well correlated with the partial-onset ictal EEG, however 19.4% (7/36) of clinically generalized seizures revealed partial-onset ictal EEG. About one-thirds (4/11) of generalized tonic seizures had its ictal onset on unilateral or bilateral frontal areas and two out of seven generalized myoclonic seizures showed unilateral frontal rhythmic activities. Hypomotor seizures mainly arose from the temporal areas and hypermotor seizures from the frontal regions.

Conclusions

Even though most of the seizure semiology of infants is well correlated with ictal EEG, some of the generalized tonic seizures or myoclonic seizures revealed partial-onset ictal EEG suggesting localized epileptic focus. Accurate definition of seizures via video EEG monitoring is necessary for proper management of seizures in infancy, especially in some clinically generalized seizures.     

Drug-resistant temporal lobe epilepsy due to middle fossa meningoencephalocele in a child: A surgical case report

BackgroundMeningoencephalocele (ME) of the temporal lobe through a bone defect in the middle cranial fossa is a rare known cause of refractory temporal lobe epilepsy (TLE). ME-induced drug-resistant TLE has been described in adults; however, its incidence in children is very rare.Case reportA 7-year-old girl presented at our hospital with brief episodes of impaired consciousness and enuresis. Initial brain MRI results were interpreted as normal. Her seizures could not be controlled even with multiple anti-seizure medications. She was diagnosed with drug-resistant TLE, which presented with prolonged impaired awareness seizures for 30–60 s and secondary bilateral tonic seizures. At 9 years of age, brain MRI revealed a left temporal anteroinferior ME with a congenital bone defect in the left middle cranial fossa. She was referred for presurgical epilepsy evaluation. Long-term video electroencephalography (EEG) failed to reveal regional abnormality in the left temporal lobe; invasive evaluation using stereoelectroencephalography (SEEG) was thus indicated.Ictal onset SEEG was identified in the temporal pole near the ME which was rapidly propagated to the mesial temporal structures and other cortical regions. The left temporal pole including the ME was micro-surgically disconnected while preserving the hippocampus and amygdala. The patient’s seizures have been completely controlled for 1 year and 6 months post-operatively.ConclusionSEEG revealed rapid propagation of ictal activity in this patient’s case, confirming that the ME was epileptogenic. Since the majority of patients with refractory epilepsy caused by ME have favorable postoperative seizure outcomes, it is important to carefully check for ME in drug-resistant TLE patients with apparently normal MRI.     

Clinical manifestations of insular lobe seizures: a stereo-electroencephalographic study

PURPOSE: In this study, we report the clinical features of insular lobe seizures based on data from video and stereo-electroencephalographic (SEEG) ictal recordings and direct electric insular stimulation of the insular cortex performed in patients referred for presurgical evaluation of temporal lobe epilepsy (TLE). METHODS: Since our first recordings of insular seizures, the insular cortex has been included as one of the targets of stereo-electroencephalographic (SEEG) electrode implantation in 50 consecutive patients with TLE whose seizures were suspected to originate from, or rapidly to propagate to, the perisylvian cortex. In six, a stereotyped sequence of ictal symptoms associated with intrainsular discharges could be identified. RESULTS: This ictal sequence occurred in full consciousness, beginning with a sensation of laryngeal constriction and paresthesiae, often unpleasant, affecting large cutaneous territories, most often at the onset of a complex partial seizure (five of the six patients). It was eventually followed by dysarthric speech and focal motor convulsive symptoms. The insular origin of these symptoms was supported by the data from functional cortical mapping of the insula by using direct cortical stimulations. CONCLUSIONS: This sequence of ictal symptoms looks reliable enough to characterize insular lobe epileptic seizures (ILESs). Observation of this clinical sequence at the onset of seizures on video-EEG recordings in TLE patients strongly suggests that the seizure-onset zone is located not in the temporal but in the insular lobe; recording directly from the insular cortex should occur before making any decision regarding epilepsy surgery.     

The effect of age on seizure semiology in childhood temporal lobe epilepsy

PURPOSE: Complex partial seizure is the characteristic seizure type observed in epilepsy arising from temporal lobe structures. The seizure evolution in adult patients is quite stereotyped and well characterized, manifesting initially with an aura, behavioral arrest, and oroalimentary and gestural automatism. A greater variability of semiology including motor features with tonic or myoclonic components, as well as a paucity of automatism, has been reported in young children with temporal lobe epilepsy. The aim of our study was to examine in more detail the effects of age on individual ictal features to be able to determine the critical age when lesional temporal lobe seizure semiology undergoes transition from the pediatric to the more adult-type clinical pattern. METHODS: We performed a video analysis of 83 seizures from 15 children (aged 11-70 months) selected by post-temporal lobectomy seizure-free outcome, looking specifically at the motor and behavioral (nonmotor) manifestations in relation to age of the children. RESULTS: All of the children younger than 42 months had seizures with early and marked motor features, which included tonic and myoclonic components and epileptic spasms. Parallel with age, the frequency of these motor components decreased, and in five of 11 children older than 3 years, motor features were totally absent. Analyzed quantitatively, we saw a linear and inverse correlation of the ratio of motor components with age at monitoring. CONCLUSIONS: These findings support the hypothesis that events in brain maturation significantly affect clinical seizure semiology and may override the more typical localizing features seen in adult-type temporal lobe epilepsy. These findings are important to consider in the early diagnosis of childhood temporal lobe epilepsy.     

Complex Partial Seizures of Temporal Lobe Origin in Children of Different Age Groups

Summary: The semiology of complex partial seizures(CPS) of temporal lobe origin in adults is well known and is important in establishing seizure localization in patients considered for epilepsy surgery. In contrast, the behavioral features of temporal lobe seizures (TLS) in children described in the literature have not been consistent. In the present study, we investigated children with TLS to compare their attacks to TLS occurring in adults. The study was based on video recordings of 29 children with TLS aged 18 months to 16 years. Children were included, if they became seizure-free after temporal lobectomy (except 4 children with a marked reduction in seizure frequency and 1 with isolated auras), and if clear unitemporal seizure onset in ictal EEG-recordings, unilateral radiological lesions, and corresponding histopathological findings were detected. Children aged >6 years had TLS with features similar to those of adults. In younger children, typical semiology included symmetric motor phenomena of the limbs, postures similar to frontal lobe seizures in adults, and head nodding as in infantile spasms. We concluded that the clinical features of TLS in younger children can be misleading and should therefore be considered with caution in selecting patients for surgical procedures on the temporal lobe.     

Frontal lobe epilepsy manifesting with seizures consisting of isolated vocalization.

Vocalizations may occur in focal epileptic seizures, which typically arise from frontal and temporal regions. They are commonly associated with other motor phenomena such as automatisms, tonic posturing, or head version. We report on a patient whose seizures were documented by video-EEG monitoring, but in whom the observable ictal semiology consisted solely of a brief, monotonous vocalization. Ictal EEGs showed left frontal seizure patterns. Isolated vocalizations can constitute an ictal epileptic event and may be the only observable clinical manifestation of a left frontal lobe epilepsy. [Published with video sequences].     

Mesio-temporal ictal semiology as an indicator for surgical treatment of epilepsies with large multilobar cerebral lesions

PurposeMesio-temporal ictal semiology is sometimes observed in patients with large multilobar lesion. In this situation, surgery is often discarded because of the lesion size and/or suspicion of extended or multifocal epileptogenic areas. In this retrospective study we evaluated the surgical outcome of such patients in order to assess whether the electro-clinical presentation of seizures could be a prognostic marker of surgical outcome.MethodsAmong the temporal lobe epilepsy population explored in our department between 2000 and 2011 (240 patients), we identified 7 patients who presented an extensive lesion on brain Magnetic Resonance Imaging (MRI) (multilobar in four, hemispheric in two, and bilateral in one). All patients underwent ¹⁸Fluorodeoxyglucose Positron Emission Tomography, which showed large, hemispheric or multilobar, areas of glucose hypometabolism. Because of the large lesion size, all patients were explored by stereoelectroencephalography (SEEG) before taking a decision regarding surgical indication.ResultsSEEG confirmed the temporal origin of the seizures and discarded the possibility of multiple epileptogenic zones. A temporal lobectomy, tailored on the basis of SEEG data, was proposed to the seven patients. The seven patients are classified Engel class I after the surgery (mean follow-up: 37.4 ± 22.1 months).ConclusionOur data thus suggest that, even in the absence of hippocampal MRI abnormality, ictal symptoms compatible with a temporal origin of seizures should be considered as a reliable indicator for surgery eligibility regardless of MRI lesion size. On the basis of our findings, the mesio-temporal semiology of seizures appears as one of the most reliable markers of operability in patients with large MRI lesions. These patients should not be excluded a priori from invasive exploration and surgical treatment, even if a large portion of their lesion is likely to be left in place after surgery.     

Epileptic seizure semiology in different age groups

Aims. Seizure semiology provides information about the eloquent cortex involved during a seizure and helps to generate a hypothesis regarding the localization of the epileptogenic zone (EZ), a prerequisite for surgical management of epilepsy. We aimed to study the seizure semiology among all different age groups to better characterize semiological changes that occur with age. Methods. We performed a retrospective review of video‐EEG data in paediatric and adult patients admitted to the Epilepsy Monitoring Unit over a three‐year period. Authors independently reviewed and classified the seizure semiology while blinded to clinical, EEG, and neuroimaging data. A total of 270 patients were included in the study. Results. The most frequent EZ in patients who were one month to three years old was undetermined. Focal epilepsy became more frequent in patients older than 10 years. Among patients with focal epilepsy, a posterior quadrant EZ was most frequent in children younger than three years old, a temporal EZ between three and six years old, and a frontal EZ between six and 10 years old. The temporal lobe was the most frequent location for focal EZ in patients older than 18 years. Auras, automotor seizures, and generalized tonic‐clonic seizures were extremely infrequent in patients younger than 10 years old. The youngest patient with auras was 5.7 years old. The youngest patient with automotor seizures was 3.7 years old. We identified only three patients with generalized tonic‐clonic seizures who were younger than 10 years (aged six months, 6.6 years, and nine years, respectively). Patients younger than three years exhibited mostly generalized simple motor seizures and hypomotor seizures. Generalized epileptic spasms, generalized tonic seizures, and generalized clonic seizures were infrequent in patients older than 10 years. Conclusion. Seizure semiology and electroencephalographic changes most likely reflect the maturation of cortical functions.     

Correlation between provoked ictal SPECT and depth recordings in adult drug-resistant epilepsy patients

PURPOSE: To correlate ictal hyperperfusion single-photon emission computed tomography (SPECT) area during provoked seizures to the epileptogenic zone (EZ), as defined by depth recordings in adult drug-resistant patients. METHODS: We included in the study eight drug-resistant epilepsy patients, subjected to both noninvasive and invasive (stereo-electroencephalography, SEEG) presurgical evaluation in the Epilepsy Surgery Center of the Catholic University in Rome, from 2001 to 2003. All patients were subjected to interictal and provoked ictal SPECT scans during scalp video-EEG monitoring. The ictal hyperperfusion area assessed by visual image analysis and, when possible, by statistical parametric mapping (SPM), was compared with the EZ, as assessed by SEEG, to define whether they colocalized. RESULTS: For each provoked seizure, we obtained a SPECT hyperperfusion area. In five patients, the SPECT hyperperfusion area was included in the EZ as assessed by SEEG. The effectiveness of provoked SPECT was confirmed by comparison with SEEG data, SPM analysis (four patients), and spontaneous ictal SPECT (two patients). Our data were obtained in adult drug-resistant epilepsy patients whose EZ was either located in or extended to extratemporal regions in all but two patients. CONCLUSIONS: Provoked ictal SPECT confirmed its efficacy and accuracy in the presurgical evaluation because of the colocalization to the EZ. Although the low number of patients precluded any statistical correlation with the surgical outcome, it is worth pointing out that the five patients in whom the hyperperfusion area was included in the EZ showed very satisfactory results.     

Eating epilepsy characterized by periodic spasms

Seizures induced by eating are generally considered rare. Eating epilepsy is a kind of reflex epilepsy. We report two patients aged 11 and 16, with seizures provoked by eating. The eating-provoked seizures in both patients were a series of tonic spasms. Both ictal EEGs showed a periodic pattern characterized by diffuse high voltage slow wave. These findings were consistent with periodic spasms described by Gobbi et al. The ECD were widely distributed in both patients, although that of Patient 1 was partially clustered in the right frontal region. This is the first report of patients with periodic spasms induced by eating.     

A detailed analysis of frontal lobe seizure semiology in children younger than 7 years

PURPOSE: We sought to analyze semiology of seizure onset and evolution in young children with frontal lobe epilepsy (FLE), compare this with adult reports, and assess age-related differences. METHODS: We analyzed 111 videotaped seizures from 14 patients with FLE based on focal cortical dysplasia aged 3-81 months (mean, 30 months). Ictal events were categorized into behavioral, consciousness, autonomic, and sensory features, as well as motor patterns, which included tonic, clonic, epileptic spasm, and myoclonic seizure components. We developed a time-scaled datasheet to record each epileptic event as onset, very early, early, or late manifestation. RESULTS: Patients had a high seizure frequency with up to 40 attacks/day; half of them showed a cluster tendency. Forty-seven percent of the seizures started in sleep. Mean duration of seizures was short (29 s). Most common seizure components were motor manifestations, mostly tonic-clonic seizures, and epileptic spasms. Behavioral change was frequent, and hypermotor seizures were not seen. In five patients, the motor features were contralateral to the epileptic focus, including two children with asymmetric epileptic spasms. Secondarily generalized tonic-clonic seizures (SGTCSs) were not recorded, but had been reported in the history of two patients. Complex motor automatisms were not seen, whereas oral automatism appeared in three children. CONCLUSIONS: Motor features are common in young children with FLE, as reported in adults. The characteristics, however, differ. Epileptic spasms and subtle behavioral change were frequent. Hypermotor seizures and complex motor automatisms were not seen, and SGTCSs were unusual. Our results suggest that FLE in young children shows age-related features differing from those of adults.     

Insular-opercular seizures manifesting with sleep-related paroxysmal motor behaviors: a stereo-EEG study

Purpose: Sleep‐related complex motor seizures are a common feature of nocturnal frontal lobe epilepsy. Nevertheless, recent studies also suggest that sleep‐related hypermotor seizures can originate in the insula. The present study describes the electroclinical features of eight drug‐resistant epileptic patients with insular‐opercular seizures manifesting with nocturnal complex motor seizures. Methods: Patients underwent a comprehensive presurgical evaluation, which included history, interictal electroencephalography (EEG), scalp video‐EEG monitoring, high‐resolution magnetic resonance imaging (MRI), and intracerebral recording by stereo‐EEG. Key Findings: Almost all patients reported an initial sensation consisting of viscerosensitive or somatosensory symptoms. Ictal clinical signs were represented by tonic–dystonic asymmetric posturing and/or hyperkinetic automatisms, including bimanual/bipedal activity and ballistic movements. Some patients exhibited dysarthric speech, hypersalivation, and apnea. Interictal and ictal EEG provided lateralizing information in the majority of patients. In three patients, MRI showed a focal anatomical abnormality in the insular‐opercular region. Stereo‐EEG ictal recordings demonstrated that the epileptic discharge involved simultaneously the insular cortex and the opercular region. Complex motor manifestations appeared when the ictal discharge showed an extrainsular spreading to frontomesial regions (cingulum, superior frontal gyrus, and supplementary motor area) and/or to internal and neocortical temporal lobe structures. Six patients received an insular‐opercular cortical resection; three of them are seizure free (minimum follow‐up 24 months) and in one a marked reduction in seizure frequency was obtained. Two patients have been operated on recently. Histology revealed a focal cortical dysplasia in three patients. One patient excluded from surgery died for sudden unexpected death in epilepsy during sleep. Significance: Our data strengthen the concept that sleep‐related complex motor attacks can originate in the insula, and provide useful electroclinical information to differentiate this localization from those with similar clinical characteristics. Furthermore, this study indicates that in these drug‐resistant patients, surgical treatment represents a highly effective treatment option.     

Anxiety as the only manifestation of focal non-convulsive status epilepticus: case report and review of the literature

ABSTRACT

Psychiatric disorders represent common comorbidities in epileptic patients. Sometimes anxiety is part of the ictal semiology, especially during seizures arising from/involving frontal or temporal lobes. We describe a patient with focal epilepsy and recurrent hyperkinetic seizures who also presented prolonged episodes characterized by massive anxiety, alarm and fear. A Video-Electroencephalographic monitoring performed during one of these attacks revealed a continuous epileptiform activity over the right frontal regions, consistent with a focal non-convulsive status epilepticus accounting for the patient’s psychiatric symptoms. Our case confirms the complex relationship between epilepsy and anxiety. A review of the literature is also included.     

Ictal Coprolalia: A Case Report and Review of Ictal Speech as a Localizing Feature in Epilepsy

BackgroundRecognizing ictal semiology is an essential component to localization of seizure onset, especially in intractable epilepsy where surgical therapies may be beneficial. Ictal speech can be a common component of seizure semiology, but the various forms of ictal speech may have different lateralizing and localizing value. Coprolalia is a very rare form of ictal speech.MethodsWe present a 15 year old with medically intractable seizures characterized by agitation and coprolalia.ResultsThe patient underwent surgical evaluation including video EEG, MRI, and functional neuroimaging. These studies indicated onset within the dominant frontal lobe which was further localized using stereo-electroencephalography prior to focal cortical resection.ConclusionsIctal coprolalia is a rare presentation of ictal speech. We review the various forms of ictal speech and their value in localizing seizure onset.     

Ictal Patterns of Cerebral Glucose Utilization in Children with Epilepsy

Summary: To determine seizure propagation patterns, we analyzed ictal positron emission tomography (PET) studies of regional cerebral glucose utilization in 18 children (11 male and 7 female aged weeks to 16 years) with epilepsy (excluding infantile spasms IS). Three major metabolic patterns were determined based on degree and type of subcortical involvement: Nine children had type I; asymmetric glucose metabolism of striatum and thalamus. Of these, the 7 oldest children showed unilateral cortical hypermetabolism (always including frontal cortex) and crossed cerebellar hypermetabolism. Two infants (aged <1 year) had a similar ictal PET pattern but no cerebellar asymmetry, presumably owing to immaturity of corticopontocerebellar projections. Five children had type II, symmetric metabolic abnormalities of striatum and thalamus; this pattern was accompanied by hippocampal or insular cortex hypermetabolism, diffuse neocortical hypometabolism, and absence of any cerebellar abnormality. Four children had type III, hypermetabolism restricted to cerebral cortex. This classification can accommodate ictal PET and single photon emission computed tomography (SPECT) patterns described by other investigators. Future studies should be directed at the clinical relevance of this classification, particularly with regard to epilepsy surgery.