Asymmetric Tonic Seizures Research Articles

Long-term follow-up of vagus nerve stimulation in drug-resistant KCNT1-related epilepsy: a case presentation

BackgroundThe KCNT1 gene encodes a Na+-activated K+ channel. Gain-of-function mutations of KCNT1 lead to autosomal dominant sleep-related hypermotor epilepsy, early-onset epileptic encephalopathy, focal epilepsy and other epileptic encephalopathies. In this paper, we report a boy carrying a KCNT1 gene mutation, who presented with drug-resistant focal-onset seizures. He had decreased seizure frequency and improvement of background changes in electroencephalography (EEG) after vagus nerve stimulation (VNS).Case presentationThe case was a nonverbal 9-year-old male who presented with drug-resistant focal-onset seizures since age 3 and had underwent VNS therapy for 2 years. He had hypermotor symptoms, automatism and bilateral asymmetric tonic seizures with cognitive decline and aphasis from age 3. The patient had a variety of seizure types that only occurred at night. The most common seizure type was automatisms, and ictal video EEG showed high-amplitude delta waves, followed by a fast rhythmic sharp activity in the mesial frontal and bitemporal regions. The patient was diagnosed with KCNT1-related epilepsy, epileptic encephalopathy and cognitive disorder. He was refractory to multiple anti-seizure medicines (ASM) and ketogenic diet. After VNS treatment at age 7, the frequency of seizures was reduced significantly and EEG was improved in background slowing.ConclusionsChildren with KCNT1-related epilepsy usually have early onset of disease, are nonverbal, and are refractory to ASM. This boy with drug-resistant KCNT1-related epilepsy showed significantly reduced seizure frequency after VNS. This report may provide reference for management of cases of KCNT1-related epilepsy.

Bilateral asymmetric tonic seizure in insulo-opercular epilepsy: an anatomo-electro-clinical study

BackgroundInsulo-opercular seizures are highly heterogeneous in seizure semiology and electrical features. Bilateral asymmetric limb posturing, as a classical pattern of supplementary sensorimotor area (SMA) seizure, also occurs in insulo-opercular epilepsy. This study was aimed to study the anatomo-electro-clinical correlations in bilateral asymmetric tonic seizures (BATS), in order to advance the understanding of insulo-opercular epilepsy.MethodsEight patients with insulo-opercular epilepsy as confirmed by stereoelectroencephalography (SEEG) and manifesting BATS as the major ictal motor sign, in Guangdong Sanjiu Brain Hospital Epilepsy Center from 2014 to 2018, were employed in this study. The BATS of the patients were evaluated, and the semiologic features and concomitant intracerebral EEG changes were quantified. Then the variables were examined with Cluster Analysis, and the semiologic features were correlated with anatomic localization using the Kendall correlation test.ResultsOf the 8 patients, the most frequent initial motor sign was bilateral asymmetric tonic posturing (62.5%). Facial tonic-clonic sign also had a high prevalence in the evolution of seizures (87.5%). The results of Cluster Analysis showed that the semiologic features were subdivided into two main groups, one group comprising exclusively BATS and the other including signs of focal tonic seizure, aura, focal limb tonic-clonic seizure (TCS), facial TCS, hypermotor behavior, eye movement, autonomic changes and generalized TCS. The BATS was strongly associated with the posterior long gyrus (PLG) of insula (t = 0.732) and parietal operculum (t = 1.000); the hypermotor behaviors were associated with the anterior long gyrus (ALG) (t = 0.770); and the autonomic changes were associated with the anterior limiting sulcus (ALS) (t = 0.734) and middle short gyrus (MSG) (t = 0.700).ConclusionsThe seizure semiology of insulo-opercular epilepsy is characterized, in temporal order, by BATS, with or without simultaneous hypermotor behaviors, and frequently ends up with facial tonic-clonic signs, which is different from that of the SMA seizure. The early spread network involving the posterior insular lobe and parietal operculum may contribute to this pattern of manifestation.

Clinical and electroencephalographic characteristics of neurodegeneration with brain iron accumulation type 5 in children on the example of 5 cases

Neurodegenerative disease with brain iron accumulation type 5 (OMIM: 300894) manifests itself with early-onset epilepsy, mental retardation with stereotypies that resemble Rett syndrome, and motor disorders under the mask of cerebral palsy in childhood; since adolescence, patients usually have aggravation of parkinsonism and develop complications, such as torsion dystonia. We analyzed medical records of 5 female patients aged between 2.5 and 6 years. There were no family relationships between patients’ families. All children had problems with their motor skills: 4 out of 5 patients could only crawl; none of them could walk independently. We also observed severe speech disorders in these patients: they had no expressive speech along with reduced understanding of speech. Their behavior was characterized by contact disorders and multiple stereotypies. All children had no self-service skills. The assessment of neurological status demonstrated uniform symmetrical paresis (score 3–4; 100 % of cases), increased muscle tone of the extrapyramidal type (40 % of cases), and diffuse muscle hypotension with ataxia (40 % of cases). One patient (with autistic-like behavior) had no motor disorders. Magnetic resonance imaging showed non-specific changes in 100 % of cases (diffuse cortical / subcortical atrophy, secondary hydrocephalus ex vacuo). One patient was found to have hypointense signal in the substantia nigra and globus pallidus on follow-up T2‑weighted magnetic resonance images obtained at the age of 6 years. All patients presented with epilepsy: West syndrome (n = 1), Lennox–Gastaut syndrome (n = 1), focal epilepsy with asymmetric tonic seizures (n = 1), and focal epilepsy with febrile generalized tonic-clonic seizures (n = 2). Remission for more than 1 year was achieved in 4 out of 5 patients. The following electroencephalographic patterns were identified before treatment initiation: hypsarrhythmia with transformation into epilepsy with a pattern of continued spike-and-wave activity during sleep (n = 1), multi-regional epileptiform activity with a tendency to diffuse spread and predominance in the frontal region (n = 3), and regional epileptiform activity in the frontocentral area (n = 1). The initial therapy with first-line drugs was highly effective and ensured remission in 3 patients; one patient had remission in response to hormone therapy; one patient continued to have seizures despite polytherapy with antiepileptic drugs. Interictal epileptiform activity was completely blocked by treatment in 2 cases; the rest of the patients had transformation of epilepsy into benign epileptiform discharges of childhood (n = 3).

Age-dependent semiology of frontal lobe seizures

IntroductionFrontal lobe epilepsy is the second most frequent origin of focal epilepsy. Various studies have discussed localizing aspects of ictal signs in frontal lobe epilepsy; the effect of age on semiological manifestations has, however, not been analyzed so far. Material and methodsWe retrospectively analyzed video-documented semiological signs in a cohort of 122 consecutive patients aged 0–70 years (mean age: 24.9 years) with EEG/MR evidence for frontal lobe epilepsy undergoing video-EEG telemetry assessment between 1999 und 2016. ResultsIn this patient cohort, most common etiologies were focal cortical dysplasia (48%) and tumors (16%). Most frequent ictal manifestations overall were impaired language comprehension (60.3%), unilateral tonic posturing (58.9%), unilateral cloni (46.6%), versive movements (44.5%), vocalization (42.5%) and impaired reactivity to non-verbal stimuli (40.4%). With increasing age, sign of four (p = 0.019), dystonic posturing (p = 0.026), changes in heart rate (p = 0.014) and impaired reactivity to non-verbal stimuli (p = 0.009) occurred significantly more frequently. In contrast, myoclonic components were significantly less frequent observed in the higher age group (p = 0.037). ConclusionsFrontal lobe seizures can be categorized into different behavioral manifestations related to involved symptomatic brain regions, including clonic, bilateral asymmetric tonic seizures and complex motor phenomena. In this cross-sectional study, we found age-related changes in the frequency of both, motor and non-motor semiological elements. Especially simple lateralized motor signs like dystonic posturing, sign of four and version were more common with increasing age. Age-dependent alterations in phenomenology may reflect maturation in connectivity and seizure propagation within and beyond the frontal lobe, and affect the localizing and lateralizing value of ictal phenomena.

Electroclinical characteristics of seizures arising from the precuneus based on stereoelectroencephalography (SEEG)

BackgroundSeizures arising from the precuneus are rare, and few studies have aimed at characterizing the clinical presentation of such seizures within the anatomic context of the frontoparietal circuits. We aimed to characterize the electrophysiological properties and clinical features of seizures arising from the precuneus based on data from stereoelectroencephalography (SEEG).MethodsThe present retrospective study included 10 patients with medically intractable epilepsy, all of whom were diagnosed with precuneal epilepsy via stereoelectroencephalography (SEEG) at Yuquan Hospital and Xuan Wu Hospital between 2014 and 2016. Clinical semiology, scalp electroencephalography (EEG) findings, magnetic resonance images (MRI), and positron emission tomography (PET) images were analyzed during phase I preoperative evaluations. Following electrode implantation, the semiological sequence, ictal SEEG evolution, and anatomy of the relevant brain structures were analyzed for each seizure.ResultsSeven of ten patients reported auras, including body image disturbance (2/7), vestibular responses (2/7), somatosensory auras (1/7), visual auras (1/7), and non-specific auras (1/7). Primary motor manifestations included bilateral asymmetric tonic seizures (BATS) (7/10) and hypermotor seizures (HMS) (3/10). In one patient, epileptiform discharge on interictal EEG occurred ipsilateral to the side of the epileptogenic zone (EZ). Discharge was non-lateralized in the remaining nine patients. In six patients, interictal EEG signals were primarily localized in the temporal–parietal–occipital area. In two patients, ictal onset occurred ipsilateral to the EZ, which was mainly located in the temporal–parietal–occipital area. Two patterns of seizure spread were observed. The first pattern was characterized by BATS activity with ictal spread to the supplementary motor area (SMA), paracentral lobule (PCL), precentral gyrus (PrCG), or postcentral gyrus (PoCG). The second pattern was characterized by HMS activity with ictal spread to middle cingulate cortex (MCC) and posterior cingulate cortex (PCC).ConclusionAura type (e.g., body image disturbance and vestibular response), BATS, and HMS are the main indicators of precuneal epilepsy. Scalp EEG is of little use when attempting to localize precuneal seizures. Our findings indicate that the clinical characteristics of precuneal epilepsy vary among patients, and that the final electro–clinical phenotype depends on the pattern of seizure spread.

Electroclinical semiology of the bilateral asymmetric tonic seizures observed in patients with supplementary sensorimotor area epilepsy confirmed by pre- and post-operative MRI.

To retrospectively reappraise characteristics of the electro-clinical seizure semiology of the bilateral asymmetric tonic seizure (BATS) in the patients with supplementary sensorimotor area (SSMA) epilepsy. From the retrospective analysis of the pre- and post-operative Magnetic Resonance Imaging (MRI) data involving 386 patients who received epilepsy surgery, 123 BATS were identified meeting the clinical criteria and included in the study. For comparison in four extremities involvement, limbs were paired and comparatively evaluated between the contralateral and ipsilateral sides, proximal and distal segments, and upper and lower limbs. For evaluation of sequential events, each tonic phase of the BATS was chronologically divided into 10 equal epochs. In each epoch, distribution of tonic events in 4 extremities and axes was visually evaluated and comparatively analyzed. Asymmetric tonic posturing was the most constant findings in 6 patients, whose upper limbs contralateral to epileptogenic cortex were kept in abduction in all 123 (100%) seizures and extension in 118 (95.9%) seizures. This type of asymmetry became visible and remained stable in the initial three epochs of the tonic phase in 107 out of 123 (87.0%) seizures. In each epoch, especially the initial one, the contralateral upper limbs were involved more frequently than those ipsilateral to the epileptogenic cortex (p < 0.05). By pairwise comparison, an earlier involvement of the contralateral side to epileptogenic cortex was visually observed in 53 out of 280 (18.9%) limb pairs, in which the ipsilateral limbs were preceded by the contralateral ones 4.6 (0.1-16.0) seconds earlier. Both of the proximal and distal segments were simultaneously involved in 260 out of 298 (87.2%) limb pairs, although the former were 4.3 (0.5-16.0) earlier than the latter in 34 out of 298 (11.4%) limb pairs. This study demonstrated that by studying the restricted epileptogenic lesion limited to pure SSMA, unilateral extension and abduction posturing in upper limb were the most prominent and valuable sign for the lesion lateralization in SSMA neurosurgery decision-making.

A case with status epilepticus and cardiac arrest after bupropion overdose

A case with status epilepticus and cardiac arrest after bupropion overdose Bupropion is an atypical antidepressant used for the treatment of severe depression and smoking cessation. Hallucinations, agitation, and seizures are the commonest central nervous system effects after a bupropion overdose. Cardiac manifestations are uncommon. We present a 19-year-old female who presented to the emergency department with asymmetric tonic seizures and then went into cardiac arrest. She was intubated and mechanically ventilated and then admitted to intensive care unit. Brain computerized tomography and magnetic resonance imaging scans were normal at admission. Clinical features resolved completely with symptomatic treatment. She had no previous history of epilepsy, head trauma, infection, family history, and no risk factors for developing epilepsy. There was no history of alcohol abuse, suicide attempt, or depression. She only was a heavy smoker. When she completely regained consciousness, she said to have ingested 15 tablets of bupropion XL (4.5g), which belonged to her mother, with the intention of suicide. Overdose of bupropion may become more common with increasing therapeutic use and may cause life-threatening conditions. Patients receiving high doses of bupropion should be closely monitored cardiologically and neurologically. Patients should receive cardiac monitoring until the accompanying tachycardia has abated and any QRS or QTc interval prolongation has been excluded.

Insular and insulo-opercular epilepsy in childhood: An SEEG study

PurposeIn recent years, there have been series analysing the electro-clinical correlations of insular epilepsy in adult populations. In contrast, the ictal semiology in children with insular epilepsy is poorly described. Considering that early and successful surgery may greatly improve the cognitive outcome and quality of life, it is worthwhile to deepen our knowledge of insular epilepsy in children. MethodsWe retrospectively evaluated ten children with drug-resistant focal insular epilepsy who had been consecutively explored with stereoelectroencephalography (SEEG), followed by individually tailored resective surgery that included part of the insula in all cases. A detailed anatomo-electro-clinical analysis of non-invasive EEG and SEEG data was performed. At least one of the electrodes explored the insular cortex. SEEG analysis confirmed that the insular cortex was included in the ictal onset zone. ResultsEpilepsy onset was mostly during the first year of life, characterized by subtle seizures as well as spasms and myoclonic seizures. Later on, neurovegetative signs and asymmetric tonic and hypermotor seizures (HMS) dominated the ictal semiology. The epileptogenic zone was frequently wider than insular with frontal and central predominance. In eight patients, the tailored resection included a lesion. In seven patients, an Engel class 1 outcome as well as neuropsychological and behavioural improvement was obtained. ConclusionsSEEG is feasible and useful in children with drug-resistant insular epilepsy which is often characterized by autonomic symptoms as the initial symptoms and should be suspected in cases with HMS, asymmetric tonic seizures and even asymmetric spasms. Early propagation is mostly frontal and central. Analysis of a larger population is required to refine these findings.

Atypical multifocal Dravet syndrome lacks generalized seizures and may show later cognitive decline

To show that atypical multifocal Dravet syndrome is a recognizable, electroclinical syndrome associated with sodium channel gene (SCN1A) mutations that readily escapes diagnosis owing to later cognitive decline and tonic seizures. Eight patients underwent electroclinical characterization. SCN1A was sequenced and copy number variations sought by multiplex ligation-dependent probe amplification. All patients were female (age range at assessment 5-26y) with median seizure onset at 6.5 months (range 4-19mo). The initial seizure was brief in seven and status epilepticus only occurred in one; three were febrile. Focal seizures occurred in four patients and bilateral convulsion in the other four. All patients developed multiple focal seizure types and bilateral convulsions, with seizure clusters in six. The most common focal seizure semiology (six out of eight) comprised unilateral clonic activity. Five also had focal or asymmetric tonic seizures. Rare or transient myoclonic seizures occurred in six individuals, often triggered by specific antiepileptic drugs. Developmental slowing occurred in all: six between 3 years and 8 years, and two around 1 year 6 months. Cognitive outcome varied from severe to mild intellectual disability. Multifocal epileptiform discharges were seen on electroencephalography. Seven out of eight patients had SCN1A mutations. Atypical, multifocal Dravet syndrome with SCN1A mutations may not be recognized because of later cognitive decline and frequent tonic seizures.

Posterior cingulate epilepsy: clinical and neurophysiological analysis

ObjectivePosterior cingulate epilepsy (PCE) is misleading because the seizure onset is located in an anatomically deep and semiologically silent area. This type of epilepsy is rare and has not been...

The significance of clinical feature in the source localization of epileptogenic zone in children with frontal lobe epilepsy

Objective To investigate the significance of clinical feature in the source localization of epileptogenic zone in children with frontal lobe epilepsy. Methods Thirty children who were diagnosed frontal lobe epilepsy consecutively admitted to the pediatric department of Shengjing hospital from Oct 2010 to Jun 2011 were recruited for retrospective study. All the cases were subject to video-electroencephalogram monitoring. Results Seventy-six episodes with clear origins of the electroencephalogram were monitored in 30 cases,including orbital frontal attack 11 times （14.5%）,dorsolateral frontal attack 33 times （43.4%）,mesial frontal attack 32 times （42. 1% ）. The correlation analysis showed that the head and eye deviation （47 times,61.8% ） ,asymmetric tonic seizures （32 times,42. 1% ） and parakinesia seizures （29 times,38. 2% ） prompted the focus was located in the dorsolateral frontal and mesial frontal. But there was no significant difference between the two areas （X2 = 10. 58, 9. 67,11.55 ,P 〉0. 05）. The vocalization,manual pedal automatism and emotional seizure prompted the focus was located in the dorsolateral frontal and orbitofrontal ;and the emotional seizure was significant differences between the two areas（ X2 = 38. 68 ,P 〈0. 001 ）. Emotional seizure was the characteristic of orbitofrontal attack. Conclusion The clinical features have important significance in the judgment of the source localization of epileptogenic zone in children with frontal lobe epilepsy. The emotional seizure could be considered as the characteristic attack of orbitofrontal. Key words: Frontal lobe epilepsy; Video-electroencephalogram; Epileptic seizure; Children

Functional neuroimaging in startle epilepsy: Involvement of a mesial frontoparietal network

Startle epilepsy is a rare form of epilepsy with seizures triggered by unexpected stimuli. Previous studies have suggested the participation of several brain regions, such as the supplementary motor area (SMA) or the mesial aspect of the frontal and parietal lobes in the generation of startle epilepsy. However, how these brain regions interact with each other during seizures remains largely unknown. The aim of this study was to get insight into brain structures involved in startle-induced seizures using an approach with functional neuroimaging. Four patients with startle epilepsy secondary to unexpected sounds were studied. All of them underwent a presurgical evaluation including ictal-single-emission computed tomography/subtraction ictal SPECT coregistered to MRI (magnetic resonance imaging) (SPECT/SISCOM). We searched for areas with ictal changes of perfusion higher than two standard deviations (2 SD) above the reference. In one patient, a fluorodeoxyglucose-positron emission tomography (FDG-PET) and an ictal electroencephalography-functional MRI (EEG-fMRI) were also performed. In this patient, the results of FDG-PET and sequential analysis of EEG-fMRI were compared to SISCOM. All the patients had their typical startle-induced seizures, consistent with bilateral asymmetric tonic seizures. Ictal-EEG pattern was located over the mesial centroparietal region in all of them. In three of four patients, a significant hyperperfusion over the mesial frontocentral region was seen, involving the SMA, the perirolandic region, and the precuneus. In one patient, who had a congenital bilateral perisylvian polymicrogyria, it was located over the lateral perirolandic region. 18F-FDG-PET results in the patient in whom it was done, were concordant with SISCOM findings. Ictal EEG-fMRI showed an initial activation located over the precuneus, SMA, cingulate gyrus, and the precentral/perirolandic area. By using a functional neuroimaging approach we have found that startle-induced seizures could be generated by the interaction of a frontoparietal network located over the mesial surface of the brain.

Statistical analysis of transcallosal propagation of spikes arising from the mesial frontal area

To study the connections between bilateral mesial frontal (MF) regions. We evaluated synchrony of spikes, recorded by subdural electrodes, arising from bilateral MF regions using cross correlation in MF epilepsy. A seven-year-old boy with intractable daily bilateral asymmetric tonic seizures and a normal MRI was investigated. To confirm the lateralization of epileptogenicity, subdural electrodes were implanted bilaterally. Only spikes of an amplitude of 400 μV or more were analyzed. Of 92.4% (194/210) of the left MF spikes recorded for 30 minutes, an approximately synchronous spike was also detected in the right MF region. Cross correlation analysis demonstrated that for 88.7% of the bilateral MF spikes (172/194, 88.7%) the left MF spike led the contralateral spikes with relative fixed peak-to-peak intervals (18.9 ± 11.1 ms) and high cross correlation values (0.81 ± 0.10). An estimated conduction velocity of 7.2 ± 9.8 m/sec was calculated (assuming no synaptic delay). After a second period of more extended invasive EEG monitoring, a left partial frontal lobectomy was performed and the patient immediately had a few brief seizures before remaining seizure-free for a follow-up period of 14 months. We conclude that the propagation of spikes between bilateral MF regions most likely occurs mainly through myelinated callosal fibres. In addition, this cross correlation method showed that the left MF spikes, most of the time, preceded the right MF spikes suggesting that the epileptogenic zone was localized in the left MF region.

Brain activation patterns of versive, hypermotor, and bilateral asymmetric tonic seizures

Patients who have seizure onset from different brain regions can produce seizures that appear clinically indistinguishable from one another. These clinically stereotypic manifestations reflect epileptic activation of specific networks. Several studies have shown that ictal perfusion single photon emission computed tomography (SPECT) can reveal propagated ictal activity. We hypothesize that the pattern of hyperperfusion may reflect neuronal networks that generated specific ictal symptomatology. All patients were identified who were injected with (99m)Tc-hexamethyl-propylene-amine-oxime (HMPAO) during versive seizures (n = 5), bilateral asymmetric tonic seizures (BATS; n = 5), and hypermotor seizures (n = 7) in the presurgical epilepsy evaluation between 2001 and 2005. The SPECT ictal–interictal difference image pairs of each subgroup were compared with image pairs of 14 controls using statistical parametric mapping (SPM 2) to identify regions of significant hyperperfusion. Hyperperfused regions with corrected cluster-level significance p < 0.05 were considered significant. We have identified a distinct ictal perfusion pattern in each subgroup. In versive seizure subgroup, prominent hyperperfusion was present in the frontal eye field opposite to the direction of head version. In addition, there was associated caudate and crossed cerebellar hyperperfusion. The BATS subgroup showed pronounced hyperperfusion supplementary sensorimotor area ipsilateral to the epileptogenic region, bilateral basal ganglia, and contralateral cerebellar hemisphere. The hypermotor seizure subgroup demonstrated two clusters of significant hyperperfusion: one involving bilateral frontomesial regions, cingulate gyri, and caudate nuclei, and another involving ipsilateral anteromesial temporal structures, frontoorbital region, insula, and basal ganglia. We have identified distinct hyperperfusion patterns for specific ictal symptomatology. Our findings provide further insight into understanding the anatomic basis of seizure semiology.

Familial frontal lobe epilepsy and its relationship with other nocturnal paroxysmal events

Familial frontal lobe epilepsy and its relationship with other nocturnal paroxysmal events

Presurgical evaluation in refractory epilepsy secondary to meningitis or encephalitis: bilateral memory deficits often preclude surgery

We investigated the clinical features and surgical outcome of 17 patients with refractory epilepsy secondary to CNS infection who were referred to a tertiary center for presurgical evaluation. Six patients had a history of meningitis and 11 patients had a history of encephalitis. Median age at infection was three years (40 days-40 years). Time to seizure onset was shorter in the encephalitis group (median of 0.9 years versus 5.9 years in the meningitis group). MRI showed unilateral mesial temporal sclerosis (MTS) in all but one patient with meningitis (5/6). MRI in the encephalitis group showed unilateral MTS (four patients), bilateral MTS (three), porencephalic cysts (one) or no significant findings (three). Seizure semiology, following analysis of 127 seizures, included automotor seizures, complex motor/hypermotor seizures, dialeptic seizures and bilateral asymmetric tonic seizures. Neuropsychological assessment in patients with MTS frequently showed bilateral memory impairment (7 out of 12 MTS-patients), even in 4 patients with unilateral MTS, precluding epilepsy surgery. Six patients (two meningitis and four encephalitis patients) underwent a temporal lobe resection. All patients are either seizure-free (Class 1a) or are having only auras after surgery. One patient from the meningitis group underwent functional hemispherectomy and he is also seizure-free. In our series, MTS was the most common finding in refractory epilepsy after CNS infections. Bilateral memory deficits were often encountered in patients with MTS, even when unilateral, these deficits being a limiting factor for surgery. Good surgical outcome can be expected in selected patients with unilateral MTS and congruent memory deficits.

Interobserver Reliability of Video Recording in the Diagnosis of Nocturnal Frontal Lobe Seizures

Nocturnal frontal lobe seizures (NFLS) show one or all of the following semeiological patterns: (1) paroxysmal arousals (PA: brief and sudden recurrent motor paroxysmal behavior); (2) hyperkinetic seizures (HS: motor attacks with complex dyskinetic features); (3) asymmetric bilateral tonic seizures (ATS: motor attacks with dystonic features); (4) epileptic nocturnal wanderings (ENW: stereotyped, prolonged ambulatory behavior). To estimate the interobserver reliability (IR) of video-recording diagnosis in patients with suspected NFLS among sleep medicine experts, epileptologists, and trainees in sleep medicine. Sixty-six patients with suspected NFLS were included. All underwent nocturnal video-polysomnographic recording. Six doctors (three experts and three trainees) independently classified each case as "NFLS ascertained" (according to the above specified subtypes: PA, HS, ATS, ENW) or "NFLS excluded". IR was calculated by means of Kappa statistics, and interpreted according to the standard classification (0.0-0.20 = slight agreement; 0.21-0.40 = fair; 0.41-0.60 = moderate; 0.61-0.80 = substantial; 0.81-1.00 = almost perfect). The observed raw agreement ranged from 63% to 79% between each pair of raters; the IR ranged from "moderate" (kappa = 0.50) to "substantial" (kappa = 0.72). A major source of variance was the disagreement in distinguishing between PA and nonepileptic arousals, without differences in the level of agreement between experts and trainees. Among sleep experts and trainees, IR of diagnosis of NFLS, based on videotaped observation of sleep phenomena, is not satisfactory. Explicit video-polysomnographic criteria for the classification of paroxysmal sleep motor phenomena are needed.

Analysis of risk factors for prognosis of frontal lobe epilepsy

To analyze the risk factors for the prognosis of frontal lobe epilepsy in order to predict the refractory frontal lobe epilepsy early, so as to provide theoretic basis for rational therapy early. Forty-seven patients with medically refractory frontal lobe epilepsy and 92 patients with medically controllable epilepsy, totally 139 patients, underwent epidemiological savery, cranial CT or MRI, and video-electroencephalography (VEEG). Single factor analysis and Logistic stepwise regression analysis were done to analyze the relationship between the influential factors and the prognosis of disease. Single factor analysis showed that the factors including earlier onset age, longer duration before taking reasonable medicine, more frequent seizure (> 1 time/day), asymmetric tonic seizures, seizures with hypermotor automatism, secondary generally tonic-clonic seizures, and a past history of taking medicine, exerted adverse effects on the prognosis of frontal lobe epilepsy (all P < 0.05). Logistic stepwise regression analysis screened out such factors including duration before taking reasonable medicine (OR = 1.384, 95% CI 1.133 - 1.689) and seizure frequency (OR = 6.512, 95% CI 1.637 - 25.911) as independent predictors for the prognosis of frontal lobe epilepsy. Long duration before taking reasonable medicine and frequent seizure onset are important risk factors for refractory frontal lobe epilepsy. It is possible to improve the prognosis of frontal lobe epilepsy by making a definite diagnosis and beginning rational therapy early.

Monday July 3, 2006 15:00-17:00 Hall 5B Discussion Group Session From functional brain anatomy to clinical seizure semiology

Abstract 1 C. Baumgartner ( 1 Department of Neurology, Medical University of Vienna, Austria ) Motor manifestations during epileptic seizures include focal clonic motor seizures, seizures with asymmetrical tonic posturing and seizures with hypermotor automatisms. Focal clonic motor seizures are generated by epileptic activity in the primary motor cortex and consist of unilateral, jerky, usually rhythmic movements, frequently also tonic components like tonic postural signs and tonic face contractions can be observed. Most frequently they start in the face or hand due to their large representation in primary motor cortex and due to a lower seizure threshold of these cortical regions. Asymmetric tonic seizures or supplementary motor area (SMA) seizures are mediated by epileptic activity in the supplementary sensorimotor area or the premotor cortex, but can also be generated by epileptic activity in other frontal lobe regions. Because frequently somatosensory auras like feelings of pulling, pulsing, heaviness, numbness or tingling which can occur ipsi-, contra- or bilaterally occur the term “supplementary sensorimotor seizures” has been introduced. Asymmetric tonic seizures are characterized by bilateral, asymmetric, tonic or dystonic posturing of the extremities and facial grimacing resulting in tonic posturing. Vocalizations frequently occurring during asymmetrical tonic seizures can be a reactive phenomenon, can be caused by a tonic contraction of the diaphragm and laryngeal muscles and finally can be a consequence of an activation of Broca's area or SMA. While hyperkinetic or ‘hypermotor’ automatisms are not associated with a seizure onset in any specific region within the frontal lobes. They are characterized by prominent and complex, coordinated, purposeless or semipurposefull movements (trembling, waving, rotating, stepping, cycling, rocking, hopping etc.) with an agitated or frenetic appearance. Vocalizations often are explosive and pronounced which seems to be unique for frontal lobe seizures with hypermotor automatisms. Seizures with hypermotor automatisms usually are associated with a loss of consciousness and therefore also are referred to as frontal lobe complex partial seizures.

Surgery: All Ages

Surgery: All Ages