Focal tonic seizures with asymmetrical posturing could allow voluntary movements: A lesson to not be misled for a non-epileptic event.

This report documents the clinical features of supplementary motor area seizures with voluntary movements in two patients. The first case describes a 13-year-old boy with a 2-year history of nocturnal seizures, characterized by an asymmetrical brief tonic posture followed by bilateral rapid hand shaking, but without impaired awareness. Magnetic resonance imaging revealed no abnormalities. Video electroencephalogram indicated interictal focal spikes and ictal activity 2 s before clinical onset in the frontal midline area. The patient stated that he purposely shook his hands to lessen the seizure-induced upper limb stiffness. The second case describes a 43-year-old man with a 33-year history of nocturnal seizures, characterized by an asymmetric brief tonic posture, with the right hand grabbing to hold this posture, but without impaired awareness. Video electroencephalogram indicated that he voluntarily moved his right hand during the latter part of the seizures; however, no clear ictal electroencephalogram change was noted. Magnetic resonance imaging revealed a mass lesion in the right medial superior frontal gyrus. Fluorodeoxyglucose-positron emission tomography and ictal single-photon emission computed tomography indicated ictal focus in the mesial frontal area, as confirmed by invasive electroencephalogram and seizure freedom after surgery. Both patients had typical supplementary motor area seizures, except they could perform voluntary movements in the body parts. The co-occurrence of supplementary motor area seizures and voluntary movements is clinically useful, as it may help avoid the inaccurate and misleading diagnosis of non-epileptic events such as psychogenic non-epileptic seizures.

Derivation and initial validation of a surgical grading scale for the preliminary evaluation of adult patients with drug-resistant focal epilepsy.

OBJECTIVE: Presently, there is no simple method at initial presentation for identifying a patient's likelihood of progressing to surgery and a favorable outcome. The Epilepsy Surgery Grading Scale (ESGS) is a three-tier empirically derived mathematical scale with five categories: magnetic resonance imaging (MRI), electroencephalography (EEG), concordance (between MRI and EEG), semiology, and IQ designed to stratify patients with drug-resistant focal epilepsy based on their likelihood of proceeding to resective epilepsy surgery and achieving seizure freedom. METHODS: In this cross-sectional study, we abstracted data from the charts of all patients admitted to the New York University Langone Medical Center (NYULMC) for presurgical evaluation or presented in surgical multidisciplinary conference (MDC) at the NYU Comprehensive Epilepsy Center (CEC) from 1/1/2007 to 7/31/2008 with focal epilepsy, who met minimal criteria for treatment resistance. We classified patients into ESGS Grade 1 (most favorable), Grade 2 (intermediate), and Grade 3 (least favorable candidates). Three cohorts were evaluated: all patients, patients presented in MDC, and patients who had resective surgery. The primary outcome measure was proceeding to surgery and seizure freedom. RESULTS: Four hundred seven patients met eligibility criteria; 200 (49.1%) were presented in MDC and 113 (27.8%) underwent surgery. A significant difference was observed between Grades 1 and 3, Grades 1 and 2, and Grades 2 and 3 for all presurgical patients, and those presented in MDC, with Grade 1 patients having the highest likelihood of both having surgery and becoming seizure-free. There was no difference between Grades 1 and 2 among patients who had resective surgery. SIGNIFICANCE: These results demonstrate that by systematically using basic information available during initial assessment, patients with drug-resistant epilepsy may be successfully stratified into clinically meaningful groups with varied prognosis. The ESGS may improve communication, facilitate decision making and early referral to a CEC, and allow patients and physicians to better manage expectations.

Vagoglossopharyngeal Neuralgia Occurred Concomitantly with Ipsilateral Hemifacial Spasm and Versive Seizure-Like Movement: A First Case Report.

Vagoglossopharyngeal neuralgia (VGPN) is a very rare condition. VGPN with convulsive like attack is even rarer All of the cases had their head turned to the opposite side of facial pain. Hemifacial spasm occurring concurrently with VGPN has never been reported. Herein, we present the first case of VGPN that had ipsilateral hemifacial spasm and versive seizure-like movement to the same side of facial pain. We reported a 71-year-old man presenting with multiple episodes of intermittent sharp shooting pain arising on the right middle neck, followed by hemifacial spasm on right face. Then the patient became syncope while his head and gaze turned to the same side of the painful neck. Electrocardiography showed sinus arrest. Interictal Electroencephalography was normal. This patient initially responded to pregabalin for two weeks, then the symptoms became worse. Microvascular decompression and carbamazepine resulted in the complete remission of all symptoms after six months of follow-up. We could not explain the pathophysiology of unilateral versive seizure like movement.

Surgical treatment of patients with drug-resistant hypermotor seizures.

PURPOSE: To describe the clinical, electrophysiologic, neuroradiologic, and histologic findings in our patients with drug-resistant hypermotor seizures (HMSs) and to evaluate the outcome of their surgical treatment. METHODS: Twenty-three patients were identified by criteria for drug-resistant HMS. Surgical treatment and presurgical evaluation modalities including semiology, magnetic resonance imaging (MRI), interictal/ictal scalp video-EEG (electroencephalography), and intracranial recording were analyzed retrospectively. RESULTS: The common seizure frequency of 60-300 per month was observed among 15 patients. Sixteen patients (69.6%) experienced auras such as fear and palpitation. Marked agitation was observed in 12 patients and mild agitation in 11 patients. Groaning/shouting and asymmetric posturing were common accompanying symptoms. Asymmetric posturing was observed more often in patients with mild agitation than in those with marked agitation (p = 0.027). MRI detected focal abnormalities in six patients. Intracranial recording was conducted in 16 patients. The origins of seizures were localized in mesial frontal cortex in four patients, dorsolateral frontal cortex in four patients, and mesial temporal cortex in two patients. The epileptogenic zones (EZs) were resected from the frontal lobe in 21 patients and from the temporal lobe in 2 patients. The follow-up ranged from 12-60 months. Seventeen patients (73.9%) had been seizure-free, 11 of whom had presented with marked agitation (11 of 12) and 6 with mild agitation (6 of 11) (p = 0.069). Histologic examinations demonstrated focal cortical dysplasia (FCD) in 18 patients. DISCUSSION: The HMSs can originate from both the mesial and dorsolateral frontal cortex, and occasionally from the temporal lobe. Patients with drug-resistant HMSs should be recommended for resective surgical treatment.

Double-blind, randomized controlled pilot study of bilateral cerebellar stimulation for treatment of intractable motor seizures.

PURPOSE: The efficacy and safety of cerebellar stimulation (CS) was reevaluated in a double-blind, randomized controlled pilot study on five patients with medically refractory motor seizures, and especially generalized tonic-clonic seizures. METHODS: Bilateral modified four-contact plate electrodes were placed on the cerebellar superomedial surface through two suboccipital burr holes. The implanted programmable, battery-operated stimulator was adjusted to 2.0 microC/cm(2)/phase with the stimulator case as the anode; at this level, no patient experienced the stimulation. Patients served as their own controls, comparing their seizure frequency in preimplant basal phase (BL) of 3 months with the postimplant phases from 10 months to 4 years (average, eight epochs of 3 months each). During the month after implantation, the stimulators were not activated. The patient and the evaluator were blinded as to the next 3-month epoch, as to whether stimulation was used. The patients were randomized into two groups: three with the stimulator ON and two with the stimulator OFF. After a 4-month postimplantation period, all patients had their stimulator ON until the end of the study and beyond. Medication was maintained unchanged throughout the study. EEG paroxysmal discharges also were measured. RESULTS: Generalized tonic-clonic seizures: in the initial 3-month double-blind phase, two patients were monitored with the stimulation OFF; no change was found in the mean seizure rate (patient 1, 100%, and patient 5, 85%; mean, 93%), whereas the three patients with the stimulation initially ON had a reduction of seizures to 33% (patient 2, 21%; patient 3, 46%; patient 4, 32%) with a statistically significant difference between OFF and ON phase of p = 0.023. All five patients then were stimulated and monitored. At the end of the next 6 months of stimulation, the five patients had a mean seizure rate of 41% (14-75%) of the BL. The second patient developed an infection in the implanted system, which had to be removed after 11 months of stimulation; the seizures were being reduced with stimulation to a mean of one per month from a mean of 4.7 per month (BL level) before stimulation. At the end of 24 months, three patients were monitored with stimulation, resulting in a further reduction of seizures to 24% (11-38%). Tonic seizures: four patients had these seizures, which at 24 months were reduced to 43% (10-76%). Follow-up surgery was necessary in four patients because of infection in one patient and lead/electrode displacement needing repositioning in three patients. The statistical analysis showed a significant reduction in tonic-clonic seizures (p < 0.001) and tonic seizures (p < 0.05). CONCLUSIONS: The superomedial cerebellar cortex appears to be a significantly effective and safe target for electrical stimulation for decreasing motor seizures over the long term. The effect shows generalized tonic-clonic seizure reduction after 1-2 months and continues to decrease over the first 6 months and then maintains this effectiveness over the study period of 2 years and beyond.

[Supplementary sensory-motor seizures--symptomatology, etiology, and surgical management with illustrative case reports]. / A szupplementer szenzomotoros rohamok tünettana, kóreredete és mutéti kezelhetosége, illusztratív esetismertetésekkel.

In the past decade, owing to the advance of epilepsy surgery, growing knowledge has accumulated on the role of the supplementary motor area, described by Penfield and coworkers in the early fifties, in movement regulation and on the characteristics of seizures involving this area. In the Hungarian neurological literature this topic--despite its neurophysiological and practical clinical importance--has been hardly touched. The authors, based on their own experience obtained from surgeries performed within the framework of the "Co-operative Epilepsy Surgery Program", describe the electrophysiological features of this area, its role in movement regulation and the symptoms of epileptic seizures stemmed from or spread onto this area. Using cases as illustrations, they demonstrate the reasoning and various algorithms of the multidisciplinary examination necessary to explore the seizure onset zone and the pathways of seizure spread. Details of the surgical solution are also described.

Electroencephalographic dipoles of spikes with and without myoclonic jerks caused by epilepsia partialis continua.

We used electroencephalographic (EEG) dipole analysis to investigate the generators of spikes with and without myoclonic jerks in a 12-year-old patient with epilepsia partialis continua secondary to left parietal cortical dysplasia. We recorded EEG and right wrist extensor electromyography (EMG) and collected 42 spikes with jerks (jerking spikes) and 42 spikes without jerks (nonjerking spikes). We applied a single moving dipole model to the individual and averaged spikes. Dipoles at the negative peak of individual jerking and nonjerking spikes were localized in the dysplastic area. At the onset of the averaged jerking spike that preceded the EMG discharges by 20 ms, the dipole was in the motor cortex, whereas for the averaged nonjerking spike, the dipole was in the sensory cortex. The dipole moment at averaged jerking spike onset was twice that of the averaged nonjerking spike. Electroencephalographic dipole analysis of averaged spikes differentiated the generator of jerking and nonjerking spikes in epilepsia partialis continua. Individual dipoles demonstrated the area of epileptogenic cortical dysplasia.

Ictal brain hemodynamics in the epileptic focus caused by a brain tumor using functional magnetic resonance imaging (fMRI).

Using functional magnetic resonance imaging (fMRI) we were able to observe, in detail, ictal brain hemodynamics during epileptic seizure caused by a brain tumor. A 53-year-old man was experencing partial motor seizures of the left side of his face and neck. In a brain MR image a mass lesion was found in the subcortical area of the right frontal lobe. We found focal spikes in his right hemisphere, though dominantly in C4 and T4 regions. fMRI investigations were carried out at 1.5 T (GE Signa Horizon) using gradient-echo echo-planar neuroimaging. We were able to perform the ictal examination twice. The activated regions were focalized and clearly found only on the lateral side of the tumor base. The region was in agreement with the epileptic focus examined using an electrocorticogram (ECOG). The signal intensity in the seizure focus rapidly increased 30 seconds before the convulsion was observed. After the end of the convulsion it also took 30 seconds to restore the signal intensity to the baseline value. fMRI is a very useful tool for various studies such as the identification of the epileptic focus, the mechanism of epileptic seizure, and so on.

Epilepsy and perisylvian lipoma/cortical dysplasia complex.

Intracranial lipomas are congenital malformations composed of mature adipocytes. They are usually located in the midline, particularly in the pericallosal region, a hemispheric location accounting for only 3 to 7% of cases. Review of the literature found 21 previous cases of hemispheric lipoma. Although hemispheric cerebral lipomas are rare, association with epilepsy appears to be frequent. We have recently studied two patients in whom epilepsy was the first clinical manifestation of hemispheric cerebral lipoma in the sylvian region. The patients presented with simple motor partial seizures as the first manifestation of the lesion. Neurological examination was normal. MRI disclosed in both cases a lesion involving the sylvian fissure with characteristics of the lipid signal. MRI also demonstrated abnormalities involving the cerebral cortex in the vicinity of the lesion (pachygyria-like aspect). Partial excision of the lesion was achieved in one patient but was followed by a worsening of seizures and neurological condition (hemiparesis). According to the literature, the prognosis for epilepsy in patients with hemispheric lipoma appears good. Several other arguments support non-surgical management: the lesion is benign and can be identified with a high degree of certainty by imaging; surgery is technically difficult due to adherence to adjacent vascular and cerebral structures and hypervascularity; location near functional brain tissue increases the risk of postoperative sequelae. In addition, mechanisms of epilepsy probably involve vascular and cortical dysplasic abnormalities. In consideration of the complexity of the lesion, hemispheric lipomas are more appropriately classified with localized cortical malformations rather than as simple extracerebral malformations.