An infant with multiple cavernous angiomas presenting with frequent epileptic seizures - detection of epileptic focus by magnetoencephalography

We report on a six-year-old girl with frequent partial seizures secondary to multiple cavernous angiomas (CAs) since the age of 17 months. MRI showed two CAs in the left parietal and right frontal lobes. Ictal scalp video EEG demonstrated complex partial seizures of left hemispheric origin, indicating that the left parietal CA was the epileptogenic lesion. Ictal SPECT showed extensive hyper-perfusion in the left frontal and parietal lobes, indicating the left hemispheric focus. Magnetoencephalography (MEG) showed clustered equivalent current dipoles of interictal spikes in the left parietal cortex adjacent to the left parietal CA. We performed lesionectomy of the left parietal CA at 19 months old. The patient became seizure-free for four years. Postoperative MEG yielded no residual interictal spikes. Our study suggests that early surgical intervention of CA may prevent from further development of epileptic seizures. MEG can identify both the epileptogenic zone and lesion underlying the multiple CAs in the infants with catastrophic partial seizures.     

Benign epileptiform discharges in Rolandic region with mesial temporal lobe epilepsy: MEG, scalp and intracranial EEG features

AIM OF THE STUDY: To report benign epileptiform discharges (BEDs) in the Rolandic region, coexisting in a pediatric patient with intractable localization-related epilepsy, secondary to hippocampal sclerosis. METHODS: We describe the clinical features, MRI, scalp video EEG, magnetoencephalography (MEG) and intracranial video EEG findings, and surgical outcome in a 9-year-old boy with BEDs and intractable complex partial seizures. RESULTS: MRI showed left hippocampal sclerosis. Scalp video EEG interictally demonstrated left temporal spike and sharply contoured slow waves, and right fronto-centro-temporal spike and waves. Ictal scalp video EEG showed left temporal rhythmic sharp waves after the clinical onset of epigastric aura, followed by staring. MEG showed interictal dipoles in the bilateral Rolandic regions with a uniform orientation and right hemispheric predominance. Intracranial video EEG, with bilateral mesial temporal depth and fronto-temporo-parietal strip electrodes, interictally showed polyspikes and slow waves with superimposed low-amplitude fast waves in the left mesial and posterior lateral temporal regions, and spike and waves in the bilateral fronto-parietal regions. Ictal onset was marked by low-amplitude fast waves in the left mesial and posterior lateral temporal regions. He underwent left anterior temporal lobectomy with hippocampectomy. Pathology was hippocampal sclerosis. Predominant right fronto-centro-temporal spike and waves and MEG right Rolandic dipoles persisted after surgery. He was seizure-free 14 months after surgery. CONCLUSION: This is the first report on MEG and intracranial video EEG features of BEDs in the Rolandic region, coexisting with hippocampal sclerosis. Persistence of contralateral benign MEG Rolandic dipoles after surgery indicates that BEDs are coincidental in mesial temporal lobe epilepsy. MEG identified Rolandic dipoles, although was unable to localize the deep and focal epileptogenic dipoles from the hippocampal sclerosis.     

Gelastic seizures due to right temporal cortical dysplasia.

Gelastic seizures are an uncommon seizure type. They are most frequently observed in patients with hypothalamic hamartoma. Their association with other types of cerebral lesions is rare. Depending on the location of the lesion, gelastic seizures may or may not be accompanied by a subjective feeling of mirth. The pathophysiological mechanisms of this type of seizure are still undefined, and little is known about which pathways promote laughter and its emotional content, mirth. We present a young man with drug-resistant, gelastic seizures due to focal cortical dysplasia of the right inferior temporal gyrus. The lesion was evident on cranial MRI. Interictal EEG displayed a right temporal focus, whereas ictal EEG was not informative. Ictal loss of consciousness precluded reporting of any possible emotional experience. The patient underwent surgical resection of the lesion and has been seizure-free with anti-epileptic medication for two years. Although various anatomical regions may elicit laughter, in view of the current literature it seems that the anterior cingulate region is involved in the motor aspects of laughter, while the basal temporal cortex is involved in the processing of mirth. The fact that the present case exhibited gelastic seizures stresses once more the importance of the baso-lateral temporal cortex in the genesis of this type of seizures.[Published with video sequences].     

Gelastic seizures involving the left parietal lobe

Gelastic seizures have been described in various epilepsies arising from the temporal or frontal lobes, although the most commonly encountered form is related to the presence of a hypothalamic hamartoma. We describe a patient with gelastic seizures involving the left parietal lobe. Our patient, an 8-year-old girl, underwent interictal video/EEG monitoring and MRI. The seizures consisted of brief staring followed by smiling and laughing. Electroencephalography during the gelastic seizures showed rhythmic spikes and waves in the left parietal lobe. MRI revealed the characteristic features of focal cortical dysplasia. Our findings suggest that the left parietal lobe may actively participate in the particular epileptogenic network generating gelastic seizures.     

Magnetoencephalographic study of rhythmic mid-temporal discharges in non-epileptic and epileptic patients.

To evaluate the source location and clinical significance of rhythmic mid-temporal theta discharges (RMTD) by MEG in non-epileptic and epileptic patients, we conducted simultaneous MEG and EEG recordings with a whole-scalp 306-channel neuromagnetometer in three patients: one with right temporal lobe epilepsy (TLE), one with right frontal lobe epilepsy (FLE), and one with tension headache. We visually detected the RMTD activity and interictal spikes, and then localised their generators by MEG source modelling. We repeated MEG measurement 3 months after right anterior temporal lobectomy (ATL) in the TLE patient; 3 months after anticonvulsant medication in the FLE patient. In epileptic patients, RMTD activities were found during drowsiness over the left temporal channels of both MEG and EEG recordings, and their generators were localised to the left posterior inferior temporal region. In the patient with tension headache, RMTD was localised in the right inferior temporal area. When the epileptic patients became seizure free with disappearance of epileptic spikes, RMTD was still found over the left temporal channels. Besides, some bursts of RMTD appeared also in the right temporal channels in our TLE patient after ATL. Our results indicate that the source of RMTD activity is located in the fissural cortex of the posterior inferior temporal region. As a physiologic rhythm related to dampened vigilance, RMTD has no direct relation to epileptogenic activity.     

Characterizing magnetoencephalographic spike sources in children with tuberous sclerosis complex

PURPOSE: Tuberous sclerosis complex (TSC) often causes medically intractable seizures. Magnetoencephalography (MEG) localizes epileptiform discharges. To evaluate the use of MEG spike sources (MEGSSs) for localizing epileptic zones in TSC patients, we characterized MEGSSs and correlated them to EEG and magnetic resonance imaging (MRI) results. METHODS: We analyzed data from seven children who underwent prolonged video-EEG, MEG, and MRI. We classified MEGSSs as clusters (six or more spike sources, 1 cm between sources regardless of number of sources). RESULTS: A single, unilateral cluster with additional scatters occurred in two patients; these predominantly lateralized dipoles correlated to prominent tubers on MRI and ictal/interictal EEG zones. Bilateral clusters with scatters existed in two patients; cluster locations partly overlapped multiple prominent tubers. These patients also had bilateral or diffuse interictal discharges, bilateral or generalized seizures, and changing seizure types and EEG findings. Only bilateral scatters occurred in three patients; scatters partly overlapped EEG interictal/ictal-onset regions; one patient had coexisting generalized seizures. In one patient with equally bilateral scatters, scatters overlapped a prominent tuber and interictal/ictal-onset zones in the right frontal region. CONCLUSIONS: MEG contributes to information from EEG and MRI for localizing epileptogenic zones in children with TSC. A single cluster with scatters in a unilateral hemisphere predicts a primary epileptogenic zone or hemisphere; bilateral or multiple clusters indicate bilateral primary or potential epileptogenic zones; and bilateral scatters without clusters may indicate epileptogenic zones that are hidden within extensive areas of scattered MEGSSs.     

Efficacy of Thyrotropin-Releasing Hormone in the Treatment of Intractable Epilepsy

Purpose : The ictal EEG and magnetoencephalogram (MEG) for gelastic seizures were recorded in a 4-year-old girl with tuberous sclerosis. The sites of origin for the seizure activities were investigated by using an equivalent current dipole (ECD) with the MEG.
Methods : EEG and MEG were recorded simultaneously under the administration of diazepam (DZP). The MEG recording was performed on a system consisting of an array of 64 sensors uniformly distributed over the patient's whole head (CTF, Canada), and the estimated ECDs were superimposed on the magnetic resonance imaging (MRI) images (Siemens, 1.5 Tesla).
Results : Two laughing attacks lasting 5 s each were documented. The ictal EEG showed gradually increasing 11–Hz rhythmic α activities with dominance over the frontocentral areas bilaterally, followed by irregular spike-and-wave discharges. The ictal MEG detected bilateral frontal rhythmic sharp waves before the appearance of the activities on the EEG. The estimated ECDs were localized in the deep white matter of the right frontal lobe on the MRI. However, those dipoles did not coincide with the locations of her cortical tubers.
Conclusions : Although gelastic seizures accompanied with hypothalamic hamartomas are well known, several reports have suggested a temporal or frontal lobe origin for gelastic seizures. In this patient, the ECD indicated that the seizures originated in the frontal lobe, although ictal scalp EEG recordings could not determine the precise focus. Thus, in cases in which the use of ictal scalp EEG fails to show the sites of origin for the seizures, it is recommended that the origins be estimated by using the non-invasive method of ictal MEG analysis.     

A case with frontal lobe epilepsy presenting with absence seizures as cardinal manifestation: ictal EEG findings]

We report here a 9-year-old boy presenting with absence and complex partial seizures. Absence seizures occurred several times a day, with sudden arrest of speech and gesture, alteration of consciousness, myoclonus of unilateral or bilateral angles of the mouth, occasional simple automatism and brisk recovery of consciousness. Complex partial seizures occurred once to three times a month with loss of consciousness, salivation, deviation of the head and eyes toward the left, elevation of upper limbs and tonic convulsion of the left upper and lower limbs. Interictal EEG showed right frontal pole-dominant high-voltage slow waves or spike-and-waves. Ictal simultaneous video-EEG recordings of absence seizures revealed a frontal dominant 3-3.5 Hz spike-wave burst lasting several seconds. A partial seizure never preceded the absence seizure. Transverse topographical analysis revealed that the first spike component of the spike-wave burst of absence seizure always showed phase reversal on the right anterior temporal electrode. The following ones, however, showed phase reversal on the left anterior temporal electrode. Ictal EEG of the complex partial seizure could not be detected because it rarely occurred. There was no abnormal finding on brain MRI. Interictal single photon emission tomography (SPECT) indicated hypoperfusion of the dorsal and medial cortex of the right middle frontal lobe. Interictal positron emission tomography (PET) also indicated hypometabolic areas in the dorsal and medial cortex of the right frontal lobe, together with those in the right temporal and parietal cortex. EEG evolution and neuroimaging studies suggested that the epileptic focus of the absence seizure might have originated at the dorsal cortex of the right middle frontal lobe and immediately spread to the medial cortex. Both the seizures were well controlled by the combination of phenytoin and high dose sodium valproate.     

A pediatric case of intractable complex partial seizures associated with mesial temporal lobe astrocytoma: usefulness of interictal epileptiform discharges in the present case]

The patient was a 10-year-old male with normal developmental milestones. He had medically intractable complex partial seizures since the age of 7 years. At the age of 10 years, he had focal motor seizures of the right face, and a head CT scan showed a calcified lesion in the left mesial temporal region. The tumor exhibited low intensity on T 1-weighted and high intensity on T 2-weighted MR images, and was not enhanced by gadolinium-diethylenetriamine pentaacetic acid. Interictal SPECT showed hypoperfusion in the left temporal region. One-day video/EEG monitoring revealed very frequent epileptiform discharges which occurred only during sleep period exclusively in the left anterior-to-middle temporal region. The patient underwent lesionectomy with the guidance of electrocorticography. The histological study of the resected tissue showed astrocytoma. After surgery he has had no seizures for 10 months. It was concluded that very frequent interictal epileptiform discharges strictly localized to the temporal lobe at which MRI-identified tumor was present could be predictive of epileptogenic zone in the present patient in whom clinical symptoms and the results of other studies were also concordant.     

A case of frontal lobe epilepsy presenting with gelastic seizures]

We described a 9-year-old boy with frontal lobe epilepsy presenting with gelastic seizures. CT-scan showed mild widening of the left sylvian fissure. Abnormal findings in the left frontal operculum were detected by both MRI and SPECT. Attacks mainly consisted of gelastic seizures with comfortable feeling followed by screaming with fear. Administration of anticonvulsants resulted in reducing the frequency and severity of seizures. Finally the patient had brief laughter attacks only. In the present case, the clinical course suggests that the gelastic seizures does not occur by way of the spreading of epileptic discharges to the temporal or hypothalamic region; rather it might occur as a focal symptom of the frontal region.     

Magnetoencephalographic localization of peritumoral temporal epileptic focus previous surgical resection.

Magnetoencephalography (MEG) is suggested as a localizing technique of epileptogenic areas in drug-resistant seizure patients due to intracraneal lesions. A male 42-year-old patient who begins at 26 with partial complex drug-resistant seizures is put forward. MRI shows a 9 mm diameter lesion located in left superior temporal gyrus which seems compatible with cavernoma. Both conventional and sleep deprivation EEGs have proved normal. Sleep EEG shows sharp waves in left temporal region. MEG helps to localize interictal spike and spike-wave activity, as well as wide slow wave (2-7 Hz) activity areas. Craniotomy under analgesia and aware sedation conditions is carried out. Intrasurgery cortical electric stimulation assisted by neuronavigator causes a limited partial complex seizure which the patient recognizes to be exactly like his. Thus, MEG localization of the epileptogenic area is confirmed. Surgical resection of both the lesion and the epileptogenic area is carried out. The patient remains free from seizures 9 months after surgery. A control MEG study reveals no epileptogenic nor slow wave activity. CONCLUSION: in this particular case, MEG has proven to be a useful presurgical evaluation technique to localize epileptogenic activity, validated by intrasurgical cortical stimulation.     

Magnetoencephalographic Analysis of Secondary Bilateral Synchrony

To assess the clinical value of magnetoencephalography (MEG) in investigating the origin of secondary bilateral synchrony (SBS) in patients with partial epilepsy. MEG and simultaneous electroencephalography (EEG) were recorded with a 204-channel whole-head MEG system in 2 patients. The equivalent current dipoles (ECDs) for epileptic discharges on MEG were calculated according to a single dipole model. In patient 1, the ictal EEG showed bursts of bilateral synchronous 3-Hz spike-and-slow-wave complexes. ECDs obtained from the ictal MEG localized to the right medial frontal lobe. On the second patient's MEG recordings, epileptic discharges corresponding to prolonged EEG bursts of bilateral synchronous spike-and-slow-wave complexes were obtained. ECDs calculated from the prolonged bursts were clustered in the left medial frontal lobe. MEG detected the sources of SBS in the medial frontal lobe. MEG is extremely useful for the identification of the source of SBS.     

Neuromagnetic separation of secondarily bilateral synchronized spike foci: report of three cases.

To demonstrate the high spatiotemporal resolution of magnetoencephalography (MEG), we report three cases with focal epilepsy that exhibited bilateral synchronized spikes on simultaneous scalp EEG and MEG recording. Constant time lags (19.4 +/- 3.0 ms and 20.0 +/- 5.5) between the leading and the following contralateral spikes were noted on MEG and the current dipole sources were localized in the bilateral homotopic regions symmetrically in Cases 1 and 3. In Case 2, MEG indicated leading spikes in the left frontal region, with a time lag of 42.3 +/- 8.4 ms to reach the contralateral frontal and bilateral temporal regions as well. Chronic subdural EEG recording in Cases 1 and 2 confirmed that the leading spike focus in MEG was close to the seizure onset zone in cortical EEG. Spatio-temporal analysis of MEG spikes may be useful to identify the primary epileptic region in patients with synchronized bilateral epileptiform discharges.     

Functional brain mapping of ictal activity in gelastic epilepsy associated with hypothalamic hamartoma: A case report

Hypothalamic hamartomas (HHs) have been demonstrated as the cause of gelastic epilepsy, both by intracranial electrodes and functional imaging. The neocortex becomes secondarily involved, through poorly characterized propagation pathways. The detailed dynamics of seizure spread have not yet been demonstrated, owing to the limited spatial–temporal resolution of available functional mapping. We studied a patient with epilepsy associated with HH and gelastic epilepsy. Simultaneous electroencephalography (EEG) and functional magnetic resonance imaging (fMRI) of several seizure events were obtained, with blood oxygen level dependent (BOLD) activation of the hamartoma, and left hemisphere hypothalamus, hippocampus, parietal–occipital area, cingulate gyrus, and dorsal–lateral frontal area. Integration of regional BOLD kinetics and EEG power dynamics strongly suggests propagation of the epileptic activity from the HH through the left fornix to the temporal lobe, and later through the cingulate fasciculus to the left frontal lobe. The EEG/fMRI method has the spatial–temporal resolution to study the dynamics of seizure activity, with detailed demonstration of origin and propagation pathways.     

Temporo-spatial analyses define epileptogenic and functional zones in a case of Dyke–Davidoff–Masson syndrome

Dyke–Davidoff–Masson syndrome (DDMS) is a rare epilepsy syndrome that is characterized by cerebral hemiatrophy, homolateral skull hyperplasia, hyperpneumatization of the paranasal sinuses, seizures with or without mental retardation, and contralateral hemiparesis. We describe a case of DDMS in a 40-year-old female who had complex partial seizures with occasional secondary generalization since the age of 4 years. Her seizure frequency was 10–20 seizures/month even though she took four antiepileptic drugs. We applied magnetic resonance imaging (MRI), positron emission tomography (PET), functional MRI, and invasive electroencephalography (EEG) to define her epileptogenic and functional zones. Brain MRI showed prominent atrophy in the left frontal dorsal and lateral regions and mild atrophy of the left superior temporal gyrus and left parietal gyri. Interictal PET revealed decreased glucose metabolism in the atrophic regions. Functional MRI demonstrated that the inferior frontal and inferior parieto-occipital regions of the right hemisphere were activated by language testing. Invasive EEG revealed that the left lateral temporal lobe was the sole source of her seizures. Our results imply that the “metabolic border zone” rather than the atrophic region plays an important role in seizure activity, and that reorganization of functional zones occur after cerebral damage early in life.     

Restricted frontomesial epileptogenic focus generating dyskinetic behavior and laughter

PURPOSE: Substantial data are missing about the anatomic location of frontal regions supporting gelastic seizures. METHODS: We report the results of stereo-electro-encephalographic recordings performed over several distinct functional premotor and executive fields in a patient whose seizures were characterized by dyskinetic behavior and ictal laughter, in the absence of cerebral MRI abnormalities. RESULTS: The epileptogenic zone was circumscribed in the anterior and ventral part of the supplementary motor area and the underlying dorsal cingulate cortex. There were no or little spreading to cortical neighboring areas. The patient is seizure-free (follow-up of 27 months) after a stereotactic electric radiofrequency lesion of the epileptogenic focus. CONCLUSION: The present data suggest that pericingulate premotor areas are involved in the triggering of the motor component of laughter. In this case, the coexistence of paroxysmal dyskinesias during laughter might reflect the involvement of specific compartment(s) of the basal ganglia.     

Two magneto-encephalographic epileptic foci did not coincide with the electrocorticographic ictal onset zone in a patient with temporal lobe epilepsy.

To evaluate the usefulness and limitations of magneto-encephalography (MEG) for epilepsy surgery, we compared 'interictal' epileptic spike fields on MEG with ictal electrocorticography (ECoG) using invasive chronic subdural electrodes in a patient with intractable medial temporal lobe epilepsy (MTLE) associated with vitamin K deficiency intracerebral hemorrhage. A 19-year-old male with an 8-year history of refractory complex partial seizures, secondarily generalized, and right hemispheric atrophy and porencephaly in the right frontal lobe on MRI, was studied with MEG to define the interictal paroxysmal sources based on the single-dipole model. This was followed by invasive ECoG monitoring to delineate the epileptogenic zone. MEG demonstrated two paroxysmal foci, one each on the right lateral temporal and frontal lobes. Ictal ECoG recordings revealed an ictal onset zone on the right medial temporal lobe, which was different from that defined by MEG. Anterior temporal lobectomy with hippocampectomy was performed and the patient has been seizure free for two years. Our results indicate that interictal MEG does not always define the epileptogenic zone in patients with MTLE.     

Bilateral symmetric tonic posturing suggesting propagation to the supplementary motor area in a patient with precuneate cortical dysplasia.

We report a patient manifesting seizures with bilateral symmetric tonic posturing, which were markedly reduced after resection of the left precuneus. A 16-year-old man had sudden onset, complex partial seizures with bilateral symmetric tonic posturing since the age of eight years. Magnetic resonance fluid-attenuated inversion-recovery imaging revealed a hyperintense lesion in left precuneus. In almost all focal seizures recorded during an invasive EEG evaluation, ictal onset was detected from the inferomesial aspect of the lesion, but fast paroxysmal discharges from the ipsilateral supplementary motor area (SMA) were observed just before the clinical onset. After surgical excision of the EEG onset zone, including the lesion, seizure frequency was markedly (> 95%) reduced. By the 20th month after surgery, there were only brief nocturnal seizures involving slight elevation of both shoulders and slight abduction of both arms, with preservation of consciousness occurring once every few days. Invasive EEG findings and surgical outcome suggested that the epileptic activity originating from the epileptogenic zone may have propagated to the symptomatogenic zone including mainly the ipsilateral SMA. In summary, we report an interesting case of bilateral symmetric tonic posturing suggesting propagation to the SMA. MRI and invasive EEG confirmed the epileptogenic focus as a precuneate cortical dysplasia lesion.[Published with video sequences].     

Gelastic seizures and the anteromesial frontal lobe: A case report and review of intracranial EEG recording and electrocortical stimulation case studies

Symptomatogenic areas for ictal laughter have been described in the frontal and temporal lobes. Within the frontal lobe, gelastic seizures have been recorded from the cingulate gyrus. Electrocortical stimulation of the cingulate gyrus as well as the superior frontal gyrus induced laughter. We describe a patient whose gelastic seizures were associated with electrographic ictal activity in the mesial aspect of the right anterior frontal gyrus. The symptomatogenic area for ictal laughter in the frontal lobe may reside in the superior frontal gyrus.     

颞叶占位性病变伴癫痫患者的手术治疗研究

目的探讨颞叶占位性病变伴癫痫患者手术治疗的疗效,分析多种致痫灶定位技术的联合应用对手术疗效的影响。方法回顾性分析31例颞叶占位性病变伴癫痫患者术前脑电图及术后随访资料,其中囊性占位7例,海绵状血管瘤6例,胶质瘤16例,其他病变2例;应用MRI、视频脑电图、脑磁图行致痫灶定位,术中应用皮质脑电图再次精确致痫灶范围后行前颞叶联合海马切除术,术后随访评估疗效。结果长程VEEG监测中,20例患者均有惯常发作和发作间期痫样放电,14例(14/20)患者发作间期痫样放电位于单侧颞叶及海马区,其中合并同侧额区放电5例。6例(6/20)患者发作间期放电位于双侧颞叶,其中合并单侧额区放电2例。8例(8/20)起源于左侧颞叶及海马区,12例(12/20)起源于右侧。MEG检查20例患者发作间歇期皆有痫样放电,检出率为100%,17例(17/20)患者单侧颞叶放电,其中合并同侧额区放电8例;3例(3/20)患者双颞放电。术后随访12~24个月:16例患者Ⅰ级,3例Ⅱ级,1例Ⅲ级,手术有效率100%,效果良好率95%。结论颞叶占位性病变伴癫痫患者的手术治疗疗效好,多种致痫灶定位技术的联合应用可提高手术疗效并有效减少术后并发症。