bluuebunny/crossref_metadata_2025_split huggingface dataset:
The response to ACTH is determined early in the treatment of infantile spasms
Authors: Mytinger John R , Weber Amanda , Heyer Geoffrey L 
Published: August 2019

AbstractAlthough adrenocorticotropic hormone is the most commonly used treatment for infantile spasms in the United States, the optimal regimen for this indication is not known. The purpose of this study was to elucidate the optimal adrenocorticotropic hormone treatment duration. We conducted a retrospective chart review of response to adrenocorticotropic hormone among all patients with infantile spasms managed at our institution from January 2009 to September 2013. Treatment response was defined as clinical remission for greater than or equal to 28 days starting at any point within the adrenocorticotropic hormone course and remission of hypsarrhythmia (or definite EEG improvement if hypsarrhythmia was absent at baseline). For responders, the diagnostic and post‐treatment EEG tracings were reviewed. Electroclinical remission was achieved in 21 of 39 patients (54%) receiving adrenocorticotropic hormone, including 11/25 (44%) receiving a long course (typically 12 weeks) and 10/14 (71%) receiving a short course (typically four weeks). The mean time to clinical remission was 5.8 days (median: 5 days; range: 1–20 days). Only one patient responded beyond two weeks of treatment. This study provides Class IV evidence that among patients with infantile spasms, the response to adrenocorticotropic hormone is most often determined early in the treatment course. Given the importance of rapid remission, clinicians should consider adding or changing treatment if infantile spasms do not resolve within two weeks of adrenocorticotropic hormone initiation. Further study is needed to determine the optimal adrenocorticotropic hormone regimen for infantile spasms.
Possible induction of multiple seizure foci due to parietal tumour and anti‐NMDAR antibody
Authors: Matsumoto Riki , Mikuni Nobuhiro , Tanaka Keiko , Usami Kiyohide , Fukao Kenjiro , Kunieda Takeharu , Takahashi Yukitoshi , Miyamoto Susumu , Fukuyama Hidenao , Takahashi Ryosuke , Ikeda Akio 
Published: August 2019

Abstract“Formes frustes” of encephalopathy associated with anti‐NMDAR antibody have been recently described in cases of chronic epilepsy. We report a young woman with a parietal lesion and anti‐NMDAR antibody who acquired bilateral, secondary epileptogenesis in the temporal lobes within a period as short as six years. Removal of the primary epileptogenic lesion of oligoastrocytoma in the right parietal lobe resulted in seizure freedom, disappearance of secondary foci, and substantial decrease of the antibody titre. Chronic exposure to anti‐NMDAR antibody, albeit at a low titre, may have resulted in a smoldering chronic course and relatively early acquisition of “reversible” secondary foci without development of a high degree of epileptogenicity and structural changes.
Long‐lasting seizure‐related anxiety in patients with temporal lobe epilepsy and comorbid psychiatric disorders
Authors: Boulogne Sebastien , Catenoix Hélène , Ryvlin Philippe , Rheims Sylvain 
Published: August 2019

AbstractIctal anxiety is a frequent epileptic symptom; it is usually brief, associated with objective clinical signs, and is not positively influenced by external factors, in contrast to psychiatric disorders. These criteria can, however, be misleading, especially in patients with psychiatric comorbidities. We report two patients with a history of drug‐resistant right temporal lobe epilepsy, who developed long‐lasting psychiatric symptoms, suggestive of exacerbation of their comorbid anxiety disorder. However, intracranial EEG data and [18F] FDG‐PET suggested that these symptoms were related to seizure activity, highlighting the difficulties in differentiating ictal symptoms from psychiatric episodes in some patients with epilepsy and comorbid psychiatric disorders. [Published with video sequence]
Antiepileptic drug withdrawal in medically and surgically treated patients: a meta‐analysis of seizure recurrence and systematic review of its predictors
Authors: Lamberink Herm J. , Otte Willem M. , Geleijns Karin , Braun Kees P.J. 
Published: August 2019

AbstractAim. Many seizure‐free patients consider withdrawal of antiepileptic drugs, both when seizure control is achieved by medication alone, or once they became seizure‐free following epilepsy surgery. The risk of recurrence is consequently of very important prognostic value. However, estimations of recurrence risks are outdated for both populations. In addition, although many publications have reported predictors of seizure relapse, no comprehensive overview of prognostic factors is available.Methods. A systematic review of the databases of PubMed and EMBASE was conducted, identifying articles on antiepileptic drug withdrawal in patient cohorts. Recurrence risk meta‐analyses were performed for both populations at one, two, three to four, and five or more years of follow‐up. Within the selected articles, studies presenting multivariable analysis of predictors were identified; all studied predictors were listed, as well as all significant independent predictors. The quality of separate analyses of predictors was assessed.Results. There was no significant difference of long‐term cumulative recurrence risk between surgical and medication‐only populations, with respectively 29% and 34% recurrences. In medication‐only treated patients, 25 factors have been reported as significant independent predictors; 12 have been reported in surgical cohorts. The quality of most analyses of predictors was low to moderate. No predictor was consistently found among all analyses, and for most predictors, study results were contradictory.Conclusion. No consistent set of predictors could be identified because a large number of variables have been identified in the literature, many studies reported contradicting results, study populations varied considerably, and the quality of the original studies was often low. Meta‐analysis of individual participant data is necessary, because it allows for (1) correction for differences in follow‐up duration between subjects and studies, (2) a study of interaction effects, (3) calculation of more accurate estimates valid across several populations, and (4) the assessment of each predictor's effect size.
Adult‐onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy
Authors: Koutroumanidis Michalis , Tsirka Vasiliki , Panayiotopoulos Chrysostomos 
Published: August 2019

AbstractTo evaluate the clinical associations of adult‐onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically‐induced seizures, while 15 had exclusively, or almost exclusively, reflex photically‐induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically‐induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first‐seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.
Interictal high‐frequency oscillations (HFOs) as predictors of high frequency and conventional seizure onset zones
Authors: Modur Pradeep , Miocinovic Svjetlana 
Published: August 2019

AbstractWe investigated the relationship between the interictal high‐frequency oscillations (HFOs) and the seizure onset zones (SOZs) defined by the ictal HFOs or conventional frequency activity (CFA), and evaluated the usefulness of the interictal HFOs as spatial markers of the SOZs. We analysed seizures showing discrete HFOs at onset on intracranial EEGs acquired at ≥1000‐Hz sampling rate in a training cohort of 10 patients with temporal and extratemporal epilepsy. We classified each ictal channel as: HFO+ (HFOs at onset with subsequent evolution), HFO‐ (HFOs at onset without evolution), CFA (1.6–70‐Hz activity at onset with evolution), or non‐ictal. We defined the SOZs as: hSOZ (HFO+ channels only), hfo+&‐SOZ (HFO+ and HFO‐ channels), and cSOZ (CFA channels). Using automated methods, we detected the interictal HFOs and extracted five features: density, connectivity, peak frequency, log power, and amplitude. We created logistic regression models using these features, and tested their performance in a separate replication cohort of three patients. The models containing the five interictal HFO features reliably differentiated the channels located inside the SOZ from those outside in the training cohort (p<0.001), reaching the highest accuracy for the classification of hSOZ. Log power and connectivity had the highest odds ratios, both being higher for the channels inside the SOZ compared with those outside the SOZ. In the replication cohort of novel patients, the same models differentiated the HFO+ from HFO‐ channels, and predicted the extents of the hSOZ and hfo+&‐SOZ (F1 measure >0.5) but not the cSOZ. Our study shows that the interictal HFOs are useful in defining the spatial extent of the SOZ, and predicting whether or not a given channel in a novel patient would be involved in the seizure. The findings support the existence of an abnormal network of tightly‐linked ictal and interictal HFOs in patients with intractable epilepsy.
Cerebrospinal fluid findings after epileptic seizures
Authors: Chatzikonstantinou Anastasios , Ebert Anne D. , Hennerici Michael G. 
Published: August 2019

AbstractWe aimed to evaluate ictally‐induced CSF parameter changes after seizures in adult patients without acute inflammatory diseases or infectious diseases associated with the central nervous system. In total, 151 patients were included in the study. All patients were admitted to our department of neurology following acute seizures and received an extensive work‐up including EEG, cerebral imaging, and CSF examinations. CSF protein elevation was found in most patients (92; 60.9%) and was significantly associated with older age, male sex, and generalized seizures. Abnormal CSF‐to‐serum glucose ratio was found in only nine patients (5.9%) and did not show any significant associations. CSF lactate was elevated in 34 patients (22.5%) and showed a significant association with focal seizures with impaired consciousness, status epilepticus, the presence of EEG abnormalities in general and epileptiform potentials in particular, as well as epileptogenic lesions on cerebral imaging. Our results indicate that non‐inflammatory CSF elevation of protein and lactate after epileptic seizures is relatively common, in contrast to changes in CSF‐to‐serum glucose ratio, and further suggest that these changes are caused by ictal activity and are related to seizure type and intensity. We found no indication that these changes may have further‐reaching pathological implications besides their postictal character.
Transcranial direct current stimulation improves seizure control in patients with Rasmussen encephalitis
Authors: Tekturk Pinar , Erdogan Ezgi Tuna , Kurt Adnan , Kocagoncu Ece , Kucuk Zeynep , Kinay Demet , Yapici Zuhal , Aksu Serkan , Baykan Betul , Karamursel Sacit 
Published: August 2019

AbstractAim. Rasmussen encephalitis is associated with severe seizures that are unresponsive to antiepileptic drugs, as well as immunosuppressants. Transcranial direct current stimulation (t‐DCS) is a non‐invasive and safe method tried mostly for focal epilepsies with different aetiologies. To date, there is only one published study with two case reports describing the effect of t‐DCS in Rasmussen encephalitis. Our aim was to investigate the effect of t‐DCS on seizures in Rasmussen encephalitis and to clarify its safety.Methods. Five patients (mean age: 19; three females), diagnosed with Rasmussen encephalitis were included in this study. Patients received first cathodal, then anodal (2 mA for 30 minutes on three consecutive days for non‐sham stimulations), and finally sham stimulation with two‐month intervals, respectively. Three patients received classic (DC) cathodal t‐DCS whereas two patients received cathodal stimulation with amplitude modulation at 12 Hz. Afterwards, all patients received anodal stimulation with amplitude modulation at 12 Hz. In the last part of the trial, sham stimulation (a 60‐second stimulation with gradually decreasing amplitude to zero in the last 15 seconds) was applied to three patients. Maximum current density was 571 mA/m2 using 70 mm × 50 mm wet sponge electrodes with 2‐mA maximum, current controlled stimulator, and maximum charge density was 1028 C/m2 for a 30‐minute stimulation period.Results. After cathodal stimulation, all but one patient had a greater than 50% decrease in seizure frequency. Two patients who received modulated cathodal t‐DCS had better results. The longest positive effect lasted for one month. A second trial with modulated anodal stimulation and a third with sham stimulation were not effective. No adverse effect was reported with all types of stimulations.Conclusion. Both classic and modulated cathodal t‐DCS may be suitable alternative methods for improving seizure outcome in Rasmussen encephalitis patients.
Electrophysiological findings in Rasmussen's syndrome
Authors: Gündüz Ayşegül , Kızıltan Meral E. , Coşkun Tülin , Delil Şakir , Yeni Naz , Özkara Çiğdem 
Published: August 2019

AbstractBackground. Rasmussen syndrome is a rare, inflammatory and probably autoimmune disease presenting with epilepsia partialis continua which is generally in the form of myoclonic jerks and involves the upper extremities with or without head involvement. We sought to demonstrate the electrophysiological features in patients with Rasmussen syndrome.Methods. We performed continuous electrophysiological recordings of involuntary movement, as well as recordings of startle responses and long latency reflex in three patients with a diagnosis of Rasmussen syndrome.Results. Positive and negative myoclonus were recorded. Startle responses were found to be suppressed. However, long latency reflexes were high in amplitude and one patient even had a C reflex.Conclusion. Stimulus‐sensitive positive and negative cortical myoclonus are typical in epilepsia partialis continua of Rasmussen syndrome and degeneration of brainstem and reticulospinal pathways may develop in Rasmussen syndrome.
Stimulation of the bilateral anterior nuclei of the thalamus in the treatment of refractory epilepsy: two cases of subcortical band heterotopia
Authors: Franco Ana , Pimentel José , Campos Alexandre Rainha , Morgado Carlos , Pinelo Sara , Ferreira António Gonçalves , Bentes Carla 
Published: August 2019

AbstractSubcortical band heterotopia is a neuronal migration disorder that may cause refractory epilepsy. In these patients, resective surgery has yielded inadequate results. Deep brain stimulation of the anterior nuclei of the thalamus has been used for the treatment of refractory epilepsy with good results. We describe the first two patients with subcortical band heterotopia who were submitted to deep brain stimulation of the anterior nuclei of the thalamus, with evaluation of seizure outcome after 12 and 18 months of follow‐up. At these times, both showed a >50% decrease in seizure frequency and an increase in seizure freedom. Both patients had a depressive syndrome after surgery that responded fully to anti‐depressive medication in one patient and partly in the other. In both, deep brain stimulation of the anterior nuclei of the thalamus was associated with good seizure outcome. This procedure can therefore be considered in the treatment of patients with subcortical band heterotopia and refractory epilepsy. Depression may be a transient adverse event of the surgery or stimulation, however, its aetiology is probably multifactorial.
Outcome of vagus nerve stimulation for drug‐resistant epilepsy: the first three years of a prospective Japanese registry
Authors: Kawai Kensuke , Tanaka Tatsuya , Baba Hiroshi , Bunker Mark , Ikeda Akio , Inoue Yushi , Kameyama Shigeki , Kaneko Sunao , Kato Amami , Nozawa Taneyoshi , Maruoka Eiji , Osawa Makiko , Otsuki Taisuke , Tsuji Sadatoshi , Watanabe Eiju , Yamamoto Takamichi 
Published: August 2019

AbstractAims. Vagus nerve stimulation (VNS) is an established option of adjunctive treatment for patients with drug‐resistant epilepsy, however, evidence for long‐term efficacy is still limited. Studies on clinical outcomes of VNS in Asia are also limited. We report the overall outcome of a national, prospective registry that included all patients implanted in Japan.Methods. The registry included patients of all ages with all seizure types who underwent VNS implantation for drug‐resistant epilepsy in the first three years after approval of VNS in 2010. The registry excluded patients who were expected to benefit from resective surgery. Efficacy analysis was assessed based on the change in frequency of all seizure types and the rate of responders. Changes in cognitive, behavioural and social status, quality of life (QOL), antiepileptic drug (AED) use, and overall AED burden were analysed as other efficacy indices.Results. A total of 385 patients were initially registered. Efficacy analyses included data from 362 patients. Age range at the time of VNS implantation was 12 months to 72 years; 21.5% of patients were under 12 years of age and 49.7% had prior epilepsy surgery. Follow‐up rate was >90%, even at 36 months. Seizure control improved over time with median seizure reduction of 25.0%, 40.9%, 53.3%, 60.0%, and 66.2%, and responder rates of 38.9%, 46.8%, 55.8%, 57.7%, and 58.8% at three, six, 12, 24, and 36 months of VNS therapy, respectively. There were no substantial changes in other indices throughout the three years of the study, except for self/family‐accessed QOL which improved over time. No new safety issues were identified.Conclusions. Although this was not a controlled comparative study, this prospective national registry of Japanese patients with drug‐resistant epilepsy, with >90% follow‐up rate, indicates long‐term efficacy of VNS therapy which increased over time, over a period of up to three years. The limits of such trials, in terms of AED modifications and during follow‐up and difficulties in seizure counting are also discussed.
Investigation of SLC2A1 gene variants in genetic generalized epilepsy patients with eyelid myoclonia
Authors: Altıokka‐Uzun Güneş , Özdemir Özkan , Uğur‐İşeri Sibel , Bebek Nerses , Gürses Candan , Özbek Uğur , Baykan Betül 
Published: August 2019

ABSTRACTAims. In addition to a complex inheritance pattern in genetic generalized epilepsy (GGE) syndromes, some studies have recently identified SLC2A1 variants which lead to glucose transporter type 1 (GLUT1) defects, in patients diagnosed with GGE. Here, we investigated the possible role of SLC2A1 variants in GGE patients with eyelid myoclonia (EM) which is a rare generalized seizure type associated with drug resistance and cognitive dysfunction.Methods. After polymerase chain reaction with designed primers, sequencing of all SLC2A1 exons was performed for 25 GGE‐EM patients, as well as a control group of 15 GGE patients with absence seizures.Results. Although various single nucleotide polymorphisms clustered in the ninth exon were detected, no variant was found in the two groups with GGE.Conclusions. Even though the patient number in this study is small, the data suggest that SLC2A1 variants do not play any causative role in GGE associated with EM.
Faciobrachial dystonic seizures expressed as epileptic spasms, followed by focal seizures in anti‐LGI1 encephalitis: a video‐polygraphic study
Authors: d’Orsi Giuseppe , Martino Tommaso , Lalla Alessandra , Claudio Maria Teresa Di , Carapelle Elena , Avolio Carlo 
Published: August 2019

ABSTRACTThe origin of faciobrachial dystonic seizures in anti‐LGI1 encephalitis is controversial due to a lack of neurophysiological characterization. We report a 68‐year‐old man with subacute anterograde memory loss and involuntary faciobrachial movements. Video‐polygraphic recordings disclosed repetitive events characterized by sudden, short contraction of the upper limbs and ipsilateral hemiface. A focal contralateral EEG slow wave from frontal or central electrodes was accompanied by increased muscle activity, often with a diamond‐shaped configuration, on the orbicularis oris muscle, deltoid muscle, and extensor muscle of the hand. This EEG/EMG pattern (resembling a tonic epileptic spasm) was always followed by oral and gestural automatisms with dystonic posturing of the upper limbs, compatible with a temporal lobe seizure. Brain MRI showed hyperintensity in the bilateral mesial temporal lobes, while 18FDG‐PET revealed basal ganglia hypermetabolism with extensive cortical hypometabolism. Serum and CSF were both positive for anti‐LGI antibodies. The patient was treated with intravenous methylprednisolone (1 g/day for five days) with seizure freedom within four days after initiation of the immunotherapy. In this case, a video‐EEG/polygraphic study disclosed that faciobrachial dystonic seizures may resemble epileptic spasms, and the occurrence in close temporal association with focal seizures as a single ictal event is suggestive of a peculiar cortical‐subcortical interaction. [Published with video sequence onwww.epilepticdisorders.com]
Lombalgia e alterações funcionais em feirantes: um estudo transversal
Authors: Rodrigues Roquenei da Purificação , Santos Kionna Oliveira Bernardes 
Published: August 2019

INTRODUÇÃO: A dor lombar atinge níveis epidêmicos na população em geral, sendo uma das causas de incapacidade funcional e motivo mais comum para a consulta médica. Sua etiologia é multifatorial e os fatores mais comuns para esta sintomatologia envolvem os elementos biomecânicos, ocupacionais e as características individuais. OBJETIVO: investigar a ocorrência das lombalgias e as repercussões funcionais entre os feirantes do setor de hortifruti (varejo). MÉTODOS: Foi realizado um estudo quantitativo, de natureza descritiva, com desenho de estudo transversal. Para tanto, aplicou-se o formulário de incapacidades Oswestry (modificado) e para a mensuração da dor,a Escala Visual Analógica. Os dados foram avaliados descritivamente, por meio de frequência absoluta e relativa. RESULTADOS: Foram entrevistados 100 feirantes, com a idade média de 43 anos. Destes, 73% apresentam alguma dor ou desconforto na região lombar e para minimizar tal ocorrência, 39,7% faziam uso de remédio oral/tópico sem orientação médica. Com relação às alterações funcionais, 14% apresentam incapacidade intensa. CONCLUSÃO: O estudo permitiu identificar, que os indivíduos com lombalgia, possuíam algum grau de incapacidade que repercutiam no seu contexto de vida.