bluuebunny/crossref_metadata_2025_split huggingface dataset:
Predictive factors for a good prognosis following surgery for temporal lobe epilepsy: a cohort study in Spain
Authors: Forcadas‐Berdusán María Isabel , Bustos‐Sánchez José Luis , Valle‐Quevedo Elena , Obieta Jon Aurrecoechea , Goñi Beatriz Mateos , Martinez‐Indart Lorea , Salazar Ana Molano , Gomez‐Esteban Juan Carlos , Garamendi‐Ruíz Iñigo 
Published: August 2019

ABSTRACTObjectiveTo investigate the outcome of temporal lobe epilepsy surgery and identify the variables which predict a good prognosis with respect to seizures in postoperative follow‐up after two and four years.MethodsThis retrospective study included 115 selected patients who underwent surgery for temporal lobe epilepsy between 1996 and 2007.ResultsIn the second year after surgery 86.1% of patients had a good prognosis for seizure control (73.9% Engel class I and 12.2% Engel class II) and 89.2% (76.3% Engel class I and 12.9% Engel class II) in the fourth year. Sixty‐four of 93 (68.8%) patients were free of disabling seizures (Engel class I) during the entire period and 78 (83.8%) had good prognosis (Engel class I and II). For the second year, logistic regression analysis revealed the following variables to be independently predictive of good seizure control: absence of two or more seizure episodes in the first year after surgery, normal postoperative video‐EEG, and age at surgery of less than 35 years. In the fourth year, mesial temporal sclerosis, female sex and normal postoperative video‐EEG were the predictive factors. For the group with a good prognosis in both the second and the fourth year, the predictive variables were: absence of two or more seizure episodes in the first year after surgery (OR: 13.762, CI 95%: 2.566‐73.808, p<0.002) and normal postoperative video‐EEG (OR: 16.301, CI 95%: 3.704‐71.740, p<0.001).DiscussionThis study illustrates the sustained benefit of temporal lobe epilepsy surgery. The multivariate logistic regression analysis failed to identify a good predictive model composed of preoperative variables alone, although it was possible to build such a model with either pre‐ and postoperative variables or only postoperative variables.
Transcallosal endoscopic resection of hypothalamic hamartoma in a case with Pallister‐Hall syndrome
Authors: Dericioglu Nese , Saygi Serap , Akalan Nejat 
Published: August 2019

ABSTRACTPallister‐Hall syndrome (PHS) is a very rare syndrome characterized by hypothalamic hamartoma (HH), polydactyly, panhypopituitarism, imperforate anus and other visceral anomalies. Contrary to patients with isolated HH, neurological dysfunction and precocious puberty are uncommon and seizures are usually well controlled with anticonvulsant medication. Therefore, conservative management of HH is advised. To the best of our knowledge, seven cases of PHS with surgical resection of the HH have so far been reported. Five patients were either seizure‐free or had >90% seizure reduction postoperatively. Here, we present a case of PHS of a patient who also underwent transcallosal endoscopic resection of the HH with a subsequent 70% reduction in seizure frequency.
Association analysis of intractable epilepsy with C3435T and G2677T/A ABCB1 gene polymorphisms in Iranian patients
Authors: Sayyah Mohammad , Kamgarpour Fateme , Maleki Mehri , Karimipoor Morteza , Gharagozli Kourosh , Shamshiri Ahmad Reza 
Published: August 2019

ABSTRACTObjective. The results from studies investigating a possible association between ABCB1 polymorphism and drug‐resistant epilepsy are so far inconsistent. Moreover, recent meta‐analyses studies do not confirm any link between ABCB1 C3435T polymorphism and drug resistance. Yet, if patients with comparable clinical status (same type of epilepsy, antiepileptic drugs, epilepsy onset and gender) are evaluated, the link between ABCB1 polymorphisms and drug resistance may be unmasked. We studied the association between C3435T and G2677T/A ABCB1 gene polymorphisms and drug resistance in Iranian epilepsy patients. Methods. Two hundred healthy subjects and 332 epilepsy patients (200 drug‐responsive and 132 drug‐resistant) were selected. Genotypes were determined by polymerase chain reaction followed by restriction fragment length polymorphism or the amplification refractory mutation system. Results. The risk of drug resistance was higher in patients with a C/T genotype than in those with C/C or T/T genotypes at position 3435 in patients with cryptogenic epilepsy (p=0.01). A higher risk of drug resistance was observed in adult patients with a C/C genotype than in those with a T/T genotype at position 3435 (25.8% vs 15.8%, p=0.01). The risk of drug resistance was also higher in female patients with a C/C genotype than in those with a T/T genotype at position 3435 (26.8% vs 16.3%, p=0.04). No significant association was found between G2677T/A polymorphism and epilepsy drug resistance in the different subgroups of patients. Conclusion. Iranian adult female patients with a C/C genotype at position 3435 of the ABCB1 gene have a higher risk of resistance to antiepileptic drugs. Replication studies with large sample sizes are needed to confirm the results.
Recurrence of childhood absence epilepsy as pyknolepsy in adolescence
Authors: Yozawitz Elissa G. , Scantlebury Morris H. , Robinson Julie , Jehle Rana , Moshé Solomon L. 
Published: August 2019

ABSTRACTA developmentally normal adolescent boy with a history of childhood absence epilepsy presented with recurrence of pyknolepsy after a seven‐year period of remission. The characteristics of his EEG showed the same 3‐Hz generalised spike‐wave discharge as in his previous EEG in childhood. To our knowledge, this is the first case report describing recurrence of childhood absence epilepsy as pyknolepsy in an adolescent. [Published with video sequences]
Quality of life in patients with partial‐onset seizures under adjunctive therapy with zonisamide: results from a prospective non‐interventional surveillance study
Authors: Helmstaedter Christoph , Stefan Hermann , Witt Juri‐Alexander 
Published: August 2019

ABSTRACTThis prospective, multicenter, non‐interventional surveillance study (ZADE study) explored seizure outcome and tolerability of adjunctive treatment with zonisamide (ZNS) in a non‐selected sample of patients with partial‐onset seizures in everyday clinical practice. Changes in quality of life (QOL) and health status were also recorded. Clinical status was assessed before and 4 months after introduction of ZNS. The herein reported evaluation of QOL and health status was based on a representative subsample of 207 patients. In this subgroup, a reduced QOL had been apparept in 68% of patients at baseline. After introduction of ZNS, all measures improved, with ameliorations in QOL in up to 35% of patients. Major determinants for a better QOL outcome were (1) a better score at baseline, (2) a higher degree of seizure reduction, and (3) a lower number of concomitant AEDs. Tolerability was subjectively rated as good by 89% of patients. With a ZNS dose of 244.8±108 mg/d at study end, seizure frequency had dropped from 8.8±19.2 within 8 weeks before baseline to 3.6±9.1 seizures within the period of 8 weeks before study end. A total of 79% of patients responded to ZNS treatment with a ≥50% reduction in seizure frequency; 34% became seizure free. In conclusion, adjunctive treatment with ZNS seems to be efficacious and well tolerated. QOL improvement was predicted by baseline score, seizure outcome, and overall drug load, and is thus more likely a result of enhanced seizure control, rather than an intrinsic psychotropic effect of zonisamide.
Early‐onset absence epilepsy at eight months of age
Authors: Kobayashi Yu , Akasaka Noriyuki , Ohashi Tsukasa , Saitoh Shinji , Tomonoh Yuko , Hirose Shinichi , Tohyama Jun 
Published: August 2019

ABSTRACTEarly‐onset absence epilepsy refers to patients with absence seizures beginning before age four and comprises a heterogeneous group of epilepsies. Onset of absence seizures in the first year of life is very rare. We report a girl with intractable absence seizures with onset at age eight months. Her seizures were characterised by loss of responsiveness, with eyes drifting upwards and some myoclonic jerks of the upper and lower limbs. These symptoms were accompanied by bilaterally symmetric high‐amplitude 2‐2.5Hz generalised spike‐and‐wave discharges on the electroencephalogram. Her seizures were refractory to conventional antiepileptic drugs; treatment with adrenocorticotropic hormone was transiently effective. Comprehensive metabolic screening, cytogenetic, and genetic analysis did not determine an underlying cause of her condition. Patients with intractable, very early‐onset absence epilepsy with a myoclonic component have an unfavourable outcome and may be classified under a new epileptic syndrome, such as “early infantile absence epilepsy”.
Clinical patterns and pathophysiology of hypermotor seizures: an ictal SPECT study
Authors: Masuda Hiroshi , Shariff Erum , Tohyama John , Murakami Hiroatsu , Kameyama Shigeki 
Published: August 2019

ABSTRACTHypermotor seizures (HMS) can include different forms of hypermotor behaviour due to various mechanisms associated with generation of ictal automatisms. Despite the varied location of seizure onset, similar semiologic features during seizures may exist. Ictal single‐photon emission tomography (SPECT) apparently reflects not only the origin of epileptic discharge but also the spread to adjacent cortical areas. Taking this benefit of SPECT studies into account, preoperative SPECT results from 13 patients with HMS who underwent epilepsy surgery were analysed. The radioisotope 99mTc‐ECD was injected in all patients within five seconds after seizure onset. Group analysis was performed with statistical parametric mapping (SPM) of paired ictal‐interictal SPECTs in order to identify regions of significant ictal hyperperfusion. Hyperperfused regions with a corrected cluster‐level significance p‐value of < 0.002 were considered significant. Seizure onset at implanted subdural electrodes was defined as the epileptic focus in 12 of 13 patients. Two patterns were recognized: HMS‐1 with marked agitation and HMS‐2 with mild agitation. Ictal hyperperfusion images revealed significant hyperperfusion in the anterior cingulate cortex, orbito‐frontal gyrus, lentiform nucleus, midbrain and pons. These hyper‐perfused areas represent the symptomatogenic zone which was different from the epileptogenic zone, as confirmed by the favourable outcomes after surgical resection. The present findings suggest that a network, including frontal and possibly extrafrontal brainstem and limbic structures, is involved in the genesis of the complex epileptic manifestations of HMS. Moreover, ictal SPECT analysed by SPM is a useful method for studying the neural networks of different types of seizures.
Upward gaze and head deviation with frontal eye field stimulation
Authors: Kaiboriboon Kitti , Lüders Hans O , Miller Jonathan P , Leigh R John 
Published: August 2019

ABSTRACTUsing electrical stimulation to the deep, most caudal part of the right frontal eye field (FEF), we demonstrate a novel pattern of vertical (upward) eye movement that was previously only thought possible by stimulating both frontal eye fields simultaneously. If stimulation was started when the subject looked laterally, the initial eye movement was back to the midline, followed by upward deviation. Our finding challenges current view of topological organisation in the human FEF and may have general implications for concepts of topological organisation of the motor cortex, since sustained stimulation also induced upward head movements as a component of the vertical gaze shift. [Published with video sequences]
Late‐onset temporal lobe epilepsy in a patient with juvenile myoclonic epilepsy
Authors: Lie Octavian V , Holmes Mark D 
Published: August 2019

ABSTRACTWe report a patient with longstanding, severe juvenile myoclonic epilepsy who subsequently developed features of temporal lobe epilepsy, which gradually became clinically dominant. Over the years, there was an electrographic evolution from the typical generalised epileptiform patterns, characteristic of juvenile myoclonic epilepsy, to the novel appearance of interictal temporal spikes immediately preceding bisynchronous discharges, and subsequently to temporal intermittent rhythmic delta activity and temporal lobe‐onset seizures. In this rare case of coexistent primary generalised epilepsy and focal epilepsy, the epileptic networks of the two forms of epilepsy appear to overlap.
Antiepileptic drug trials: the view from the clinic*
Authors: Faught Edward 
Published: August 2019

ABSTRACTA golden age of antiepileptic drug development has yielded over a dozen useful new compounds, but the nature of clinical trials has made translation to practical use in the clinic difficult. Most clinical trials are designed for regulatory purposes and fail to answer critical clinical questions. These questions include: which drug is best as initial therapy, which drugs work as monotherapy, what are good drug combinations, what is the best starting dose and titration schedule, what is a reasonable target dose, what is the shape of the dose‐response curve and does it vary significantly between patients, what is the true incidence of side effects, and what is the long‐term efficacy of the drug? Most of these questions could be answered by changing trial designs, but many changes would entail additional time and money. There are encouraging signs that trials with procedures more directly applicable to the clinic are becoming common. These include direct comparative trials, longer trials with emphasis on seizure freedom, and trials with more flexible dosing schedules. In the past, funding of longer and more naturalistic trials has fallen to government agencies, but commercial funding has been obtained for several recent studies. Better quality control, innovative endpoints, structured searching for side effects, and standardisation of data collection are also promising topics for development.
Is antiepileptogenesis a realistic goal in clinical trials? Concerns and new horizons*
Authors: Schmidt Dieter 
Published: August 2019

ABSTRACTAny attempt to make antiepileptogenesis a realistic goal in clinical trials should be based on the experience of failures of the past. A wide variety of experimental studies and clinical trials using chronic antiseizure drug therapy during the extended post‐injury period have had minimal success. The disappointing results of these studies may be due to several factors including the possibility that antiseizure drugs, despite the fact they suppress seizure activity, do not interfere in any substantial way with the “epileptogenic” process of focal epilepsies. Although the reasons for the failure are not entirely clear, it may be that the antiseizure drugs may have been tested at the wrong doses, for the wrong duration, or at the wrong time after brain injury. Surprisingly, the anti‐absence drug ethosuximide has also been shown to be antiepileptogenic in several experimental models of absence epilepsy. In addition, clinical trials aimed at preventing focal post‐injury epilepsy have suffered from poor enrolment and other issues related to the comorbidity of severe epilepsies that follow overt brain injury. Testing specific anti‐inflammatory and immunological antiepileptogenic agents to prevent focal epilepsies, as well as prevention trials for genetic epilepsies, possibly with anti‐absence drugs, may be a way to resolve the dilemma. Although more evidence is needed, there is hope on the horizon for antiepileptogenic therapy that works.
Seizure heralding tuberculous meningitis
Authors: Brigo Francesco , Ausserer Harald , Zuccoli Giulio , Tezzon Frediano , Nardone Raffaele 
Published: August 2019

ABSTRACTSeizures may frequently occur during tuberculous meningitis. We describe a patient with an apparent generalised tonic‐clonic seizure, initially not associated with any magnetic resonance imaging (MRI) abnormality, which was the presenting symptom of tuberculous meningitis. Follow‐up MRI, performed after gadolinium administration, showed signs of meningeal involvement. Seizures may be the presenting symptoms of tuberculous meningitis even in the absence of evident intracerebral lesions on MRI. Therefore, contrast‐enhanced brain MRI should be performed in the diagnostic workup for each first seizure, especially in patients with a clinical suspicion of CNS infectious disease. The term “heraldic seizure”, indicating a subset of acute symptomatic seizures presenting at the onset of a brain/systemic injury or preceding the full clinical manifestation of a cerebral insult, may be helpful to classify these seizures retrospectively, based initially on unknown aetiology.
White matter abnormalities revealed by DTI correlate with interictal grey matter FDG‐PET metabolism in focal childhood epilepsies
Authors: Lippé Sarah , Poupon Cyril , Cachia Arnaud , Archambaud Frédérique , Rodrigo Sébastian , Dorfmuller Georg , Chiron Catherine , Hertz‐Pannier Lucie 
Published: August 2019

ABSTRACTFor patients with focal epilepsy scheduled for surgery, including MRI‐negative cases, 18FDG‐PET was shown to disclose hypometabolism in the seizure onset zone. However, it is not clear whether grey matter hypometabolism is informative of the integrity of the surrounding white matter cerebral tissue. In order to study the relationship between metabolism of the seizure onset zone grey matter and the integrity of the surrounding white matter measured by diffusion tensor imaging (DTI), we performed a monocentric prospective study (from 2006 to 2009) in 15 children with pharmacoresistant focal epilepsy, suitable for interictal 18FDG‐PET, T1‐, T2‐, FLAIR sequence MRI and DTI. Children had either positive or negative MRI (eight with symptomatic and seven with cryptogenic epilepsies, respectively). Seven children subsequently underwent surgery. Standardised uptake values of grey matter PET metabolism were compared with DTI indices (fractional anisotropy [FA], apparent diffusion coefficient [ADC], parallel diffusion coefficient [PDC], and transverse diffusion coefficient [TDC]) in grey matter within the seizure onset zone and adjacent white matter, using regions of interest automatically drawn from individual sulcal and gyral parcellation. Hypometabolism correlated positively with white matter ADC, PDC, and TDC, and negatively with white matter FA.In the cryptogenic group of children, hypometabolism correlated positively with white matter ADC. Our results demonstrate a relationship between abnormalities of grey matter metabolism in the seizure onset zone and adjacent white matter structural alterations in childhood focal epilepsies, even in cryptogenic epilepsy. This relationship supports the hypothesis that microstructural alterations of the white matter are related to epileptic networks and has potential implications for the evaluation of children with MRI‐negative epilepsy.
Post‐traumatic epilepsy with isolated memory flashbacks
Authors: Limotai Chusak , Mirsattari Seyed M. 
Published: August 2019

ABSTRACTSome traumatic events can cause both post‐traumatic stress disorder and epileptic seizures. We report the case of a woman who experienced a severe head injury which subsequently led to the development of paroxysmal episodes of isolated memory flashbacks related to the injury. Detailed analysis of her symptoms along with video‐EEG telemetry recordings was helpful to distinguish between these two conditions. [Published with video sequences]
Neonatal suppression‐burst without epileptic seizures: expanding the electroclinical phenotype of STXBP1‐related, early‐onset encephalopathy
Authors: Mastrangelo Massimo , Peron Angela , Spaccini Luigina , Novara Francesca , Scelsa Barbara , Introvini Paola , Raviglione Federico , Faiola Stefano , Zuffardi Orsetta 
Published: August 2019

ABSTRACTEarly‐onset epileptic encephalopathies (EOEEs) are characterised by epileptic seizures beginning in the first months of life, abnormal background EEG activity, and are associated with severe developmental delay and poor prognosis. Mutations and deletions in the STXBP1 gene are associated with Ohtahara syndrome, also known as “early infantile epileptic encephalopathy”. We report an infant affected by EOEE with a 9q34.11 deletion that encompassed the genes STXBP1 and SPTAN1. The infant presented with neonatal encephalopathy without epileptic seizures and an EEG pattern varying from highly discontinuous to suppression‐burst. This was followed by West syndrome at 2 months with atypical hypsarrhythmia and spasms, easily controlled by therapy. Our findings suggest that molecular analysis of STXBP1 should be considered for newborns affected by neonatal encephalopathy associated with a peculiar EEG pattern, even in the absence of neonatal epileptic seizures.
Prolonged ictal monoparesis with parietal Periodic Lateralised Epileptiform Discharges (PLEDs)
Authors: Murahara Takashi , Kinoshita Masako , Usami Kiyohide , Matsui Masashi , Yamashita Kouhei , Takahashi Ryosuke , Ikeda Akio 
Published: August 2019

ABSTRACTWe report a patient with prolonged monoparesis and parietal periodic lateralised epileptiform discharges (PLEDs). The patient was a 73‐year‐old man with chronic myelomonocytic leukaemia who developed persisting monoparesis of the right arm, sensory aphasia, and finger agnosia, initially associated with focal clonic seizures. These neurological deficits remained for seven days without subsequent focal clonic seizures. The EEG showed left‐sided PLEDs, maximal in the left occipito‐parietal area. Ten days later, following phenytoin treatment, these symptoms suddenly improved and parietal PLEDs disappeared. Sustained PLEDs in the left parietal region may have been causally associated with ictal paresis in this patient.
Outcome after epilepsy surgery in children with MRI‐negative non‐idiopathic focal epilepsies
Authors: Bast Thomas 
Published: August 2019

ABSTRACTMRI is one of the most important diagnostic tools in the presurgical evaluation of patients suffering from pharmaco‐refractory focal epilepsies. Presence of a lesion on MRI influences both diagnostic classification as well as selection for surgery; however, the implications for MRI‐negative cases are far from well defined for such patients. Detection of potentially epileptogenic lesions depends on the techniques applied (high‐field MRI, post‐processing, etc.) and the experience of the neuroradiologist. The proportion of MRI‐negative patients in reported epilepsy surgery cohorts ranges from 16 to 47%. Most MRI‐negative patients undergo invasive long‐term EEG recordings before a final decision regarding resection is possible. Post‐operative seizure freedom rates, with few exceptions, range from 40 to 50%. Selection of surgical candidates and post‐operative outcomes may be improved by recent developments in structural and functional imaging techniques and multimodal approaches. This report gives an overview of outcomes after epilepsy surgery in MR‐negative patients with a focus on children. Issues regarding definitions, the role of established and recently introduced diagnostic tools, and the question of how outcome might be improved in the future are discussed.
Mapping of cingulate motor function by cortical stimulation
Authors: Basha Maysaa M , Vaca Guadalupe Fernández‐Baca , Lüders Hans O 
Published: August 2019

ABSTRACTAn 8‐year‐old boy with intractable left mesiofrontal lobe epilepsy underwent placement of stereotactic intracerebral depth electrodes to better localise the epileptogenic zone. Co‐registration of preoperative MRI and post‐electrode implantation CAT allowed for anatomical localisation of electrode contacts. Electrical stimulation of electrodes over the dorsal and ventral banks of the cingulate cortex on the left produced right foot dorsiflexion and right wrist and elbow flexion, respectively, demonstrating detailed representation of cingulate motor function in humans, somatotopically distributed along the banks of the cingulate sulcus, as seen in the non‐human primate. [Published with video sequences]