Journal of Epileptology最新文献

{"title":"Epileptic seizure propagation from the second somatic sensory area to the fronto medial region, by insular redistribution - A case report and a connectome description","authors":"A. Balogh, P. Halász, D. Fabó, L. Erőss","doi":"10.1515/JOEPI-2015-0023","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0023","url":null,"abstract":"Introduction. The seizure propagation phenomenon by inducing remote symptoms brings several difficulties in finding the seizure onset and delineating the epileptic network which should be taken into consideration in epilepsy surgery. By demonstrating a difficult (MRI negative) epilepsy surgery case explored with invasive presurgical evaluation we highlight the importance to recognise the secondary sensory area and to explore the the parieto-opercular-insular-medial frontal network in certain cases. A further conclusion is the consideration of the redistributory role of the insula as a special structure in the cerebral connectome, having a role in epileptic network organisation. Aims. To support the role of the insula in the organisation of an opercular – medial frontal epileptic network and to confirm Penfield’s the “second somatic sensory leg area” by way of a case report. We try to give an up to date exploration of our patient’s remote epileptic seizures by way of a connectome. Methods. The epileptic disorder was studied with intensive video EEG monitoring and two times 3T MRI. Interictal FDG (fluorodeoxyglucose) PET was also undertaken. Beside the scalp EEG and computerized frequency analysis, the evaluation was performed by invasive EEG with 2 grids and 2 strips and an insular deep electrode in addition. Electrical cortical stimulation and cortical mapping were also undertaken. Results. The video-EEG study revealed the complex seizure semiology. The left sided global somatosesensory aura in the leg, followed supplementary motor area manifestations represented a remote seizure. The seizure onset zone and the symptomatogenic zone were localised by the invasive electrophysiology. With the insular deep electrode we succeeded to explore the propagation of ictal activity to the insula and later to frontal medial surface. The PET, the negative 3T MRI results and the postprocessing morphometry confirmed the lesional origin and localised the epileptogenic area to the second somato-sensory field where a dysgenesis was located. Conclusions. By preoperative invasive video-EEG evaluation, the second somato-sensory leg area was delineated as the seizure onset zone. The resection of this area by IIb type cortical dysgenesis, resulted in a complete relief of the seizures. The invasive video-EEG revealed the peculiar role of the insula in the propagation of the epileptic seizure from the second sensory leg area to the ipsilateral fronto-medial supplemetary motor area. Our results, confirm, that the insula has a relay or node function on the parietal opercular-fronto-medial epileptic network. The connectome of the insula is a further additive of the scale-free features of the remote epileptic networks.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"7 1","pages":"61-67"},"PeriodicalIF":0.0,"publicationDate":"2015-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76051795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The pathophysiology of Lennox-Gastaut syndrome – a review of clinico-electrophysiological studies","authors":"K. Yagi","doi":"10.1515/JOEPI-2015-0021","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0021","url":null,"abstract":"Summary Introduction Lennox-Gastaut syndrome (LGS) is a type of therapy-resistant epileptic syndrome. Since the establishment of our Epilepsy Center in 1975 we have performed many studies to assess the clinical symptoms, seizure manifestations, sleep and long-term follow-up of the clinical course and changes on electroencephalographs (EEGs) in patients with LGS. Aim To review the updated pathophysiology of LGS based on our own clinico-electrophysiological data referring to recent advances in brain research. Methods All of our previously published and unpublished data were reviewed in order to investigate the pathophysiology of LGS and using PubMed database for relevant literature. Results and Discussion While LGS usually occurs in infancy, it has become apparent that there is a form of late-onset LGS (L-LGS) that may occur at age eight or older. L-LGS often occurs when there is a history of encephalitis/encephalopathy or status epilepticus. The long-term progression of LGS includes mainly tonic seizures that persist and are the basis of LGS. In approximately 30% of cases, the basic symptoms of LGS remain 10 years or longer after long-term progression, while the rest lose their characteristics, although the condition is residual in 60% of cases and remission occurs in fewer than 10%. Among the characteristic seizures associated with LGS, atypical absence seizures occur in response to a diverse range of EEG features; wherein, while they are mostly short, they are accompanied by a state of enervation along with a tendency for it to be unclear when the seizure has ended. Drop attacks can in fact be categorized into those in which the subject falls over due to hypertonia in the muscles used to maintain body posture and those in which the subject falls over due to loss of tension in the posture-retaining muscles. Tonic seizures range from those manifesting in the form of extremely mild axial muscle tonicity, open eyes and respiratory changes, accompanied by high voltage, fast rhythm (averaging 14 ± 0.4 Hz), or tonicity from axorhyzomelic muscles to the peripheral muscles, accompanying global tonic seizures, and EEG features showing low voltage fast activity (averaging 22 ± 0.6 Hz) from desynchronization. A total of 1191 clinical seizures were recorded upon overnight polysomnography and videotape, and seizure symptoms and their ictal EEGs were analyzed. In LGS, seizure activity increases during slow wave sleep, inhibiting progression into the further sleep stages but falls significantly during rapid eye movement (REM) sleep. Conclusions From the research into seizure symptoms, clinical progression, sleep and seizures during sleep, it was believed that in LGS epileptic native lesions occur due to mesencephalic reticular formation, in the thalamic reticular system and, as a result of recent of brain physiology research, it is considered that LGS is an epileptic reticulo-thalamo-cortical system disorder. This has been supported by EEG-fMRI findings (Siniat","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"60 1","pages":"7 - 23"},"PeriodicalIF":0.0,"publicationDate":"2015-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74110783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cognitive functions in myoclonic epilepsy with ragged red fibres – a case report","authors":"M. Domańska, E. Sitek, M. Schinwelski, M. Mazurkiewicz-Bełdzińska, A. Matheisel, J. Sławek","doi":"10.1515/JOEPI-2015-0026","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0026","url":null,"abstract":"Summary Introduction Myoclonic epilepsy with ragged red fibers (MERRF) is a rare, progressive mitochondrial disease affecting multiple systems, including the central nervous system. Typical MERRF symptoms include: myoclonus, epileptic seizures, ataxia and cognitive decline. In mitochondrial diseases selective cognitive impairment or generalized decline, called mitochondrial dementia, is usually diagnosed. Description of case We present the case of an 18-year-old patient with progressive neurological symptoms such as multifocal myoclonus, cerebellar syndrome (gait impairment, intention tremor, ataxia and dysmetria). The diagnosis of MERRF was confirmed at the age of 16. Neuropsychological examination showed slowing of verbal learning and deficient spontaneous recall with improvement on recognition as well as low verbal fluency. Discussion The authors discuss differential diagnosis of mitochondrial diseases (MIDs) in respect to cognitive function impairment and, in particular, to dementia: MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes), KSS (Kearns-Sayre syndrome), NARP syndrome (neuropathy, ataxia, and retinitis pigmentosa and ptosis). The authors emphasize importance of comprehensive neuropsychological assessment in differential diagnosis of MIDs. Conclusion Mild and selective cognitive impairment was identified. The type and degree of cognitive function impairment is not sufficient to diagnose dementia in this particular case of MERRF. Comprehensive neuropsychological assessment is crucial in MID in order to provide the patient with useful recommendations for education planning.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"28 1","pages":"69 - 74"},"PeriodicalIF":0.0,"publicationDate":"2015-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80996174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Failure of a first regimen of monotherapy to control the newly diagnosed epilepsies. What to do next?","authors":"J. Pimentel","doi":"10.21307/joepi-2015-0018","DOIUrl":"https://doi.org/10.21307/joepi-2015-0018","url":null,"abstract":"SUMMARY Background Monotherapy is the choice regimen to treat newly diagnosed epilepsies. However, if it fails, several strategies may be followed. Aim To discuss the treatment options when an initial monotherapy regimen fails. Methods We reviewed the relevant literature on the topic by using PubMed. Review and Discussion Approximately 64% of people with epilepsy (PWE) de novo are free of seizures with the first appropriate antiepileptic drug (AED) in monotherapy. The type (first versus second generation) of the first AED to use depends on the physician’s personal choice provided that it is a first-line AED. There is a tendency to prefer a substitution rather than a combination of a failed first AED when it was produced associated with an idiosyncratic reaction, was poorly tolerated at a moderate dose, or produced no improvement in seizure control. In contrast, there is some evidence to prefer secondary polytherapy whenever the PWE tolerate its first AED but with a suboptimal response. In this case, and particularly mainly if a first generation AED was used as a first-line treatment, I prefer to choose a new generation AED given their more favourable pharmacokinetic and pharmacodynamic profiles. A very often used strategy is transitional polytherapy between two regimens of monotherapy. Conclusion Any therapeutic decision should take into account factors such as seizure type or syndrome, possibility of drug side effects, comorbidities, comedications, age, teratogenic potential, and compliance. Whatever the option to be taken, the PWE, his family or the caregivers should take part in the decision making.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"24 1","pages":"109 - 112"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82544595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"20th Annual Meeting of the German-Austrian-Swiss Epilepsy Working Group","authors":"H. Stefan","doi":"10.1515/JOEPI-2015-0020","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0020","url":null,"abstract":"SUMMARY The stated purpose of the meeting was to summarize ,,current, new and proven approaches in diagnostics and epilepsy therapy” to define optimal clinical practice and scientific research areas, highlight advances in clinical and basic science and to identify future approaches or directions in epilepsy treatment.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"80 1","pages":"123 - 125"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83829303","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Failure of a first regimen of monotherapy to control the newly diagnosed epilepsies. What to do next","authors":"J. Pimentel","doi":"10.1515/JOEPI-2015-0018","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0018","url":null,"abstract":"Background. Monotherapy is the choice regimen to treat newly diagnosed epilepsies. However, if it fails, several strategies may be followed. Aim. To discuss the treatment options when an initial monotherapy regimen fails. Methods. We reviewed the relevant literature on the topic by using PubMed. Review and Discussion. Approximately 64% of people with epilepsy (PWE) de novo are free of seizures with the first appropriate antiepileptic drug (AED) in monotherapy. The type (first versus second generation) of the first AED to use depends on the physician’s personal choice provided that it is a first-line AED. There is a tendency to prefer a substitution rather than a combination of a failed first AED when it was produced associated with an idiosyncratic reaction, was poorly tolerated at a moderate dose, or produced no improvement in seizure control. In contrast, there is some evidence to prefer secondary polytherapy whenever the PWE tolerate its first AED but with a suboptimal response. In this case, and particularly mainly if a first generation AED was used as a first-line treatment, I prefer to choose a new generation AED given their more favourable pharmacokinetic and pharmacodynamic profiles. A very often used strategy is transitional polytherapy between two regimens of monotherapy. Conclusion. Any therapeutic decision should take into account factors such as seizure type or syndrome, possibility of drug side effects, comorbidities, comedications, age, teratogenic potential, and compliance. Whatever the option to be taken, the PWE, his family or the caregivers should take part in the decision making.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"2 1","pages":"109-112"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81859945","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Encephalopathy with Electrical Status Epilepticus in Slow Wave Sleep – a review with an emphasis on regional (perisylvian) aspects","authors":"P. Halász, M. Hegyi, Z. Siegler, A. Fogarasi","doi":"10.1515/JOEPI-2015-0015","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0015","url":null,"abstract":"SUMMARY Aim The aim of this article is to review criticaly the Electrical Status Epilepticus in Slow Sleep (ESES) phenomenon from a neurophysiological mechanisms aspect as well as terminological and classification issues. Methods The review includes all the relevant papers published during the last 43 years on the subject of ESES and Continous Spike – Wave in Sleep (CSWS).These papers were identified in various large databases via the internet. Rewiev and remarks ESES/CSWS phenomena can be held as a common final pathway originating from different etiologies, including patients with early brain damage (probably involving thalamic structures), but also patients without structural pathology as in atypical evolution of idiopathic regional childhood hyperexcitability syndromes (with Rolandic epilepsy as a prototype). There are hints that genetic predisposition might be an important factor in the development of this process. The two large patient groups (lesional and non-lesional) show the same EEG evolution and encephalopathic cognitive consequences. The sleep EEG activation can be held as a common endophenotype. ESES represents an extreme sleep activation/potentiation of the local/regional interictal discharges, enhancing them in frequency, territorial extension, intra and trans-hemispherial propagation, synchrony and continuity. This process is most probably not identical with the development of bilateral spike-wave pattern in ,,generalized” epilepsies which involves primarily or secondarily the thalamocortical system as it had been explored by Gloor (1979) for idiopathic generalized rpilepsy and Steriade and Amzica (2003) for different types of generalized spike and wave discharges. Conclusions and syndromological embedding of ESES In an overwhelming majority of the investigated cases, the maps of the single discharges constituting sleep activation are identical; with focal/regional interictal spikes followed by slow closing wave, as it is seen in childhood regional age dependent hyperexcitability syndromes (prototype of the centro-temporal spikes of Rolandic epilepsy). The main mechanism of the developing cognitive impairment is most probably the consequence of interference with plastic function of slow wave sleep by obliterating synaptic decline during sleep. Presently, the consensus and co-operative research is highly obstacled by the terminological chaos, the controversial definitions and views around this still striking and enigmatic phenomenon.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"689 1","pages":"71 - 87"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91445014","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ictal Forced Repetitive Swearing in Frontal Lobe Epilepsy: Case report and review of the literature","authors":"Marjan Dolatshahi, A. Yankovsky","doi":"10.1515/JOEPI-2015-0019","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0019","url":null,"abstract":"SUMMARY Introduction Dominant presentation of ictal forced repetitive swearing has been rarely addressed and could be misdiagnosed. Case report We report a 45-year-old man with a long history of right frontal lobe epilepsy (FLE) who developed forced repetitive swearing during hypermotor seizures. His seizures were refractory to different antiepileptic drugs (AEDs). Scalp video-EEG telemetry suggested a right frontal epileptic focus. Magnetic resonance imaging (MRI) suggested focal cortical dysplasia (FCD) in the right mesial frontal lobe. Intracranial implantation with video-EEG recordings confirmed seizures originating from the MRI lesion. Patient underwent right frontal lobe resection followed by seizure freedom in the last five years on a single AED. Neuropathology confirmed FCD type IIB. Discussion The following aspects of the case are discussed: FLE and ictal vocalization, swearing, FLE and aggression. We emphasize the differences among ictal vocalisation, verbal automatism and ictal speech. We propose that ictal swearing might fit a verbal automatism definition. Conclusion Ictal forced repetitive swearing can be a manifestation of hypermotor seizures in FLE and should not be misdiagnosed.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"35 1","pages":"113 - 119"},"PeriodicalIF":0.0,"publicationDate":"2014-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85612049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recent advances in pathophysiology studies and treatment of epilepsy in neurocutaneous disorders","authors":"K. Sadowski, S. Jóźwiak","doi":"10.1515/JOEPI-2015-0017","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0017","url":null,"abstract":"SUMMARY Introduction Epilepsy that is associated with neurocutaneous disorders seriously deteriorates quality of life and cognitive outcome of affected children. Recent advances in epilepsy pathophysiology raise hopes for better treatment results in this difficult group of patients. Aim The aim of this review is to present recent treatment recommendations as well as current research progress in the most frequent neurocutaneous disorders. Material and methods We analyzed PubMed database to select the most prominent and recent (up to 2014 year) publications on the treatment and mechanisms of epilepsy in selected neurocutaneous disorders. We aimed to emphasize evidence-based medicine recommendations as well as basic experimental studies dealing with molecular mechanisms of epileptogenesis. Discussion and conclusions Recent advances in disease-modifying treatment options such as mTOR inhibitors in patients with tuberous sclerosis complex open up new perspectives for neurologists. Traditional resective surgery has still a major role as a treatment of choice in carefully selected cases.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"6 1","pages":"99 - 108"},"PeriodicalIF":0.0,"publicationDate":"2014-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72725507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cognitive impairment predicts social disability in persons with epilepsy","authors":"K. Bujarski, Gabriella H. Wozniak, E. Kobylarz","doi":"10.1515/JOEPI-2015-0016","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0016","url":null,"abstract":"SUMMARY Introduction Cognitive dysfunction is one of the main comorbidities of epilepsy which co-exists with seizures and contributes to the adverse impact of the disease on employment, education and interpersonal relationships. A fundamental question regarding cognitive dysfunction in epilepsy goes as follows: in comparison to seizures, what role does cognitive dysfunction play in causing social disability? The purpose of this review was to evaluate our understanding of the role cognitive impairment plays in social disability in persons with epilepsy (PWE). We systematically searched the medical literature and identified studies which assessed the impact of seizures and cognitive function on some aspect of social disability in PWE. Results and Discussion We identified 12 studies which adequately measured all variables in non-surgical cohorts, and 9 studies of cohorts following epilepsy surgery. We found evidence from non-surgical and from surgical series that cognitive variables strongly correlate with levels of social disability. Conclusions We conclude that efforts to better understand the origins of cognitive dysfunction in epilepsy and subsequently at developing treatment modalities will be needed in order to reduce the degree of social disability caused by the condition.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"6 1","pages":"89 - 97"},"PeriodicalIF":0.0,"publicationDate":"2014-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91336710","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}