bluuebunny/crossref_metadata_2025_split huggingface dataset:
Atypical evolution of Panayiotopoulos syndrome: A case report
Authors: Ferrie Colin D. , Koutroumanidis Michael , Rowlinson Shaun , Sanders Sue , Panayiotopoulos C.P. 
Published: March 2024

ABSTRACT
Panayiotopoulos syndrome is a relatively common condition with susceptibility to early onset benign childhood seizures, which manifests primarily with autonomic and mainly emetic symptoms. It predominantly affects children of 3‐6 years of age (13% of those with one or more non‐febrile seizures). EEG shows great variability, with occipital, extra‐occipital spikes or brief generalised discharges alone or in combination; it may also be consistently normal. Occipital spikes do not occur in one third of children. Despite the high prevalence of autonomic status epilepticus, the prognosis of Panayiotopoulos syndrome is usually excellent. Remission usually occurs within 1‐2 years from onset, one third have a single seizure but 5‐10% may have more than 10 seizures or a more prolonged course. Atypical evolutions with absences, atonic seizures and intellectual deterioration are exceptional; only two cases have been previously reported. We present a girl who initially had a prolonged autonomic status epilepticus typical of Panayiotopoulos syndrome, followed by seizures, with concurrent symptoms of Rolandic epilepsy. She then had an atypical evolution with atypical absences, absence status epilepticus, atonic seizures and mild impairment of scholastic performance. The case emphasises the close links between Panayiotopoulos syndrome and Rolandic epilepsy, both of which probably represent different clinical phenotypes of a maturational‐related benign childhood seizure susceptibility syndrome [published with videosequences].
Postictal signs of lateralizing and localizing significance
Authors: Leutmezer Fritz , Baumgartner Christoph 
Published: March 2024

ABSTRACT
Epileptic seizures are followed by dynamic alterations in neurological function in the postictal period. Although the first observation of such phenomena dates back to Bravais who described postictal hemiparesis in 1827, this critical period of time after seizure offset has received little attention in the literature.
In this article, we report some of the most important postictal signs, such as postictal hemiparesis, postictal nose wiping, postictal language dysfunction, postictal headache and postictal cognitive impairment. Summarizing our own experience of the postictal state in more than 160 patients with focal epilepsy, we conclude that postictal signs can provide reliable information for the localization of the seizure onset zone in patients with focal epilepsy. Since the lateralization and localization of the seizure onset zone has become a major issue for the presurgical evaluation of those patients, we argue that more attention should be paid to the postictal state during video EEG monitoring. Moreover, in an ambulatory setting, the postictal period and its related signs is usually the only part of an epileptic seizure that is accessible to the physician's examination and might therefore add some information to the differential diagnosis of paroxysmal events, such as epileptic or psychogenic seizures [published with videosequences].
Ulcerative colitis, status epilepticus and intractable temporal seizures
Authors: Akhan Galip , Andermann Frederick , Gotman Marilyn Jones 
Published: March 2024

ABSTRACT

Three patients with ulcerative colitis (UC) and without any other risk factors, developed intractable status epilepticus
de novo.
Intractable temporal lobe seizures followed and were investigated 7‐19 years later. Two had bi‐temporal abnormalities and catastrophic memory loss and the third, severe temporoparietal damage. Status occurred in the context of steroid taper, fasting and abdominal surgery respectively, and it is unlikely that it was merely due to coincidence. Encephalitis or vasculitis were suspected in all cases, but without evidence; metabolic factors may have been present. Status epilepticus in UC may lead to catastrophic sequelae.
Epilepsy and ionic channels
Authors: Roll Patrice , Szepetowski Pierre 
Published: March 2024

ABSTRACT
Ion channels play critical roles in a broad range of physiological processes. In particular, they represent a common basis for the excitable properties of different tissues. Consequently, ion channels of many different types have been implicated in several human genetic disorders of the heart, brain and skelctal muscle. An increasing number of genes encoding neurotransmitter‐ or voltage‐gated ion channel subunits have proved to be mutated in various idiopathic human epilepsies as well as in different animal models. The epilepsies could thus be considered as one of many paroxysmal disorders that are due to mutations in ion channel genes, the so‐called channelopathies. However, recent data supported the hypothesis that other genes with different properties could also be responsible for human idiopathic epilepsies, thus opening new and exciting areas of research. Effort is still needed to identify the genes responsible for the large variety of other epileptic disorders inherited as Mendelian traits and evaluate the role of these genes in the more common and polygenic forms. Defining the genetic bases of the latter will also require that exhaustive association studies are performed. These studies may help understand the pathophysiology of human epilepsies and represent the first step towards the discovery of new therapeutic targets, as exemplified in the case of the KCNQ potassium channels.
Long‐term neuropsychological follow‐up and nosological considerations in five patients with Continuous Spikes and Waves during Slow Sleep
Authors: Veggiotti Pierangelo , Termine* Cristiano , Granocchio Elisa , Bova Stefania , Papalia Grazia , Lanzi Giovanni 
Published: March 2024

ABSTRACT
Continuous spikes and waves during slow sleep (CSWSS) is a well‐known EEG pattern that can be associated with cognitive and behavioural deterioration. We present the long‐term neuropsychological follow‐up and nosological considerations of five patients who developed CSWSS during childhood. All five of our patients presented CSWSS, although the duration and severity of this pattern varied. The outcome was of three basic types: acquired frontal dementia, language deficits and normal. Four of our patients were initially diagnosed with Landau‐Kleffner syndrome but have had markedly diverse outcomes in terms of the severity and type of compromise. Our data suggest that the initial diagnosis, according to current nosological categories, has almost no prognostic significance, while the length and the age of onset of CSWSS, the site of epileptiform activity and the individual neuropsychological profile are more useful for identifying the long‐term outcome of patients with CSWSS.
The role of the temporal pole in auditory processing
Authors: Bougeard Renaud , Fischer Catherine 
Published: March 2024

ABSTRACT
Experiments in animals have shown that the temporal pole and the amygdala are involved in auditory processing. Comparable data are scarce in humans. It is only known that after temporal lobectomy, hearing may be impaired and dichotic listening scores reduced on the ear contralateral to the operated temporal lobe. Middle‐latency auditory evoked potentials (MLAEPs) and late auditory evoked potentials (LAEPs) have been recorded in 14 patients before and after temporal lobectomy for the treatment of drug‐resistant temporal lobe epilepsy. The Na and Pa components of MLAEPs were significantly delayed and the Pa component amplitude reduced before and after surgery as compared to a group of 21 healthy subjects. Data in pre‐ and postoperative period were not statistically different. N100 and P200 component of LAEPs were similarly altered. It is concluded that in humans the amygdala and probably the temporal pole may be involved in the process of auditory activation‐inhibition. The possible role of auditory processing impairment in cognitive deficits in TLE patients can also be questioned.
Video‐EEG evidence of lateralized clinical features in primary generalized epilepsy with tonic‐clonic seizures
Authors: Casaubon Leanne , Pohlmann‐Eden Bernd , Khosravani Houman , Carlen Peter L. , Wennberg Richard 
Published: March 2024

Background
Whether cortical or subcortical structures, specifically the thalamus, play the dominant role in generating primary generalized seizures has been the subject of long debate. Most experimental data implicate a hyperexcitable cortical generator of spike‐and‐wave activity, with the thalamus quickly recruited to sustain the generalized oscillations through a reverberating thalamocortical network. However, there is little clinical evidence to support the cortical generator hypothesis. We present video‐EEG recordings of generalized tonic‐clonic seizures in three patients with proven primary generalized epilepsy (PGE), all of whom showed a consistent pattern of lateralized seizure onset compatible with a focal frontal lobe generator.

Methods
Among 300 patients referred for video‐EEG monitoring for intractable epilepsy, three were found to have PGE with tonic‐clonic convulsions. All had a positive family history for epilepsy and no other epilepsy risk factors. Epilepsy onset was during adolescence (2/3) or childhood (1/3). Patients were taking 1‐4 antiepileptic drugs (AEDs) at admission, none of which was valproic acid.

Results
Interictal EEG showed very active, bilaterally synchronous generalized spike‐and‐wave or polyspike‐and‐wave discharges between 2.5‐4.5 Hz, maximal over the midfrontal structures symetrically in all patients. Ictal EEG showed generalized rhythmic activity without lateralization at seizure onset. Surprisingly, in all 6 recorded tonic‐clonic seizures there was a sustained (10‐15 seconds), stereotyped, clinical lateralization at onset, which took the form of a tonic “fencing posture” in one patient (two seizures) and forced head/eye/torso version in two patients (four seizures). Two patients became seizure‐free shortly after switching to valproate monotherapy. One patient refused valproate but has improved more than 90 % with a change in AEDs to lamotrigine and phenobarbital (follow‐up in all patients > 18 months).

Conclusions
Tonic‐clonic seizures are presumed to be generalized from onset in patients with PGE. However, video‐EEG monitoring in these patients is rarely performed and the actual clinical features of the seizures may be underappreciated. The demonstration of sustained lateralization at onset in our patients, with features clinically indistinguishable from focal onset frontal lobe seizures, is compatible with the hypothesis of a focal region of cortical hyperexcitability situated in the frontal lobes of some patients with PGE. Whether this cortical generator is autonomous or “triggered” by ascending, possibly normal, thalamocortical volleys is unresolved. [Published with video sequences].
Positron emission tomography in epileptogenic hypothalamic hamartomas
Authors: Ryvlin Philippe , Ravier C. , Bouvard S. , Mauguière François , Le Bars D. , Arzimanoglou Alexis , Petit Jérôme , Kahane Philippe 
Published: March 2024

ABSTRACT
Whether the intrinsic epileptogenicity of hypothalamic hamartomas (HH) is responsible for the entire clinical spectrum of epileptic, neuropsychological and behavioural disorders associated with HH, remains an open issue, in as much as morphologically similar HH can be associated with dramatically different seizure types and cognitive outcomes.
The aim of this study was to investigate brain glucose metabolism in patients with epileptogenic HH, in an attempt to identify signs of focal cortical and subcortical dysfunction which might correlate with other clinical data.

We have studied five patients with epileptogenic HH using [
18
F]‐fluorodesoxyglucose and positron emission tomography (FDG‐PET). All our patients also underwent an optimal MRI and a video‐EEG monitoring, as well as an intra‐cranial EEG recording in one of them. The anatomical distribution of FDG‐PET abnormalities was compared to that of interictal and ictal electroclinical findings.

All five patients demonstrated focal hypometabolism, ipsilateral to the predominant EEG abnormalities and side of HH. Hypometabolic areas greatly varied between patients, but were grossly concordant with the cortical regions suspected to participate in the ictal discharges in each individual.
Epileptogenic hypothalamic hamartomas are usually associated with focal cortical hypometabolism in regions which might participate in the overall HH‐driven epileptic network. Whether these cortical abnormalities only reflect the propagation of ictal discharges, or a potentially independent seizure onset zone remains unknown.
The ideal pharmacokinetic properties of an antiepileptic drug: how close does levetiracetam come?
Authors: Perucca Emilio , Johannessen Svein I. 
Published: March 2024

ABSTRACT
The pharmacokinetic properties of a drug are the primary determinant of the extent and duration of drug action, and influence susceptibility to clinically important drug interactions. Most of the older‐generation antiepileptic drugs (AEDs) are far from ideal in terms of pharmacokinetics and interaction potential. For example, phenytoin, carbamazepine, and valproic acid exhibit non‐linear kinetics; carbamazepine and valproic acid have relatively short half‐lives; and most of these drugs cause either enzyme induction (phenytoin, phenobarbital, primidone, carbamazepine) or enzyme inhibition (valproic acid). Compared with older agents, certain new‐generation AEDs offer a number of pharmacokinetic advantages, particularly in terms of reduced inter‐patient variability in drug clearance and a lower interaction potential. One of the most recently developed of these drugs, levetiracetam, comes especially close to fulfilling the desirable pharmacokinetic characteristics for an AED: (1) it has a high oral bioavailability, which is unaffected by food; (2) it is not significantly bound to plasma proteins; (3) it is eliminated partly in unchanged form by the kidneys and partly by hydrolysis to an inactive metabolite, without involvement of oxidative and conjugative enzymes; (4) it has linear kinetics; and (5) it is not vulnerable to important drug interactions, nor does it cause clinically significant alterations in the kinetics of concomitantly administered drugs. Although its half‐life is relatively short (6 to 8 hours), its duration of action is longer than anticipated from its pharmacokinetics in plasma, and a twice‐daily dosing regimen is adequate to produce the desired response.
Safety profile of levetiracetam
Authors: Arroyo Santiago , Crawford Pamela 
Published: March 2024

ABSTRACT
A good balance between safety and tolerability is necessary for an antiepileptic drug (AED) to be successful in the management of patients with epilepsy. Levetiracetam is one of the new generation of AEDs licensed as an add‐on therapy for the treatment of patients with partial‐onset seizures. Levetiracetam's mechanisms of action are not fully understood. Controlled clinical trials, open‐label studies, and postmarketing surveillance indicate that levetiracetam has a favorable safety profile characterized by little effect on vital signs or clinical laboratory values, reported adverse events that are mild to moderate, and no known drug‐drug interactions. The tolerability of levetiracetam may extend to both pediatric and elderly patients based on analyses of small numbers of patients. Tolerability is maintained over the long term. Levetiracetam does not appear to have a different safety profile in learning‐disabled patients. Levetiracetam appears to have a good balance between tolerability and efficacy in the treatment of a wide variety of patients with partial epilepsy.
Intra‐lesional stereo‐EEG activity in Taylor's focal cortical dysplasia
Authors: Francione Stefano , Nobili Lino , Cardinale Francesco , Citterio Alberto , Galli Carlo , Tassi Laura 
Published: March 2024

ABSTRACT
Focal cortical dysplasia are a frequent histological finding in epilepsy surgery series. Among the different types of focal cortical dysplasia, distinctive anatomical, electrical and clinical details have been identified for Taylor's focal cortical dysplasia, and in a recent article we reported a better post‐surgical outcome in Taylor's focal cortical dysplasia than in other histological subtypes of cortical dysplasias. In the present study, we analysed the intra‐lesional electrical activity directly recorded inside Taylor's focal cortical dysplasia during a stereo‐EEG diagnostic procedure in 21 patients selected from among the 27 cases in which post‐operative neuropathological examination demonstrated this kind of lesion.
Our data show the existence of a peculiar interictal pattern characterised by the presence of repetitive and rhythmic spike and poly‐spike and wave, frequently associated with short bursts of fusiform micro poly‐spikes. Moreover, an almost pathognomonic ictal pattern (mid‐amplitude 14‐18 Hz rhythmic activity followed by a low voltage recruiting fast activity) is present in 12 of these 21 patients. These electrical peculiarities suggest a high level of epileptogenicity of Taylor's focal cortical dysplasia and could possibly explain the high percentage of post‐surgical success among patients with this kind of lesion.
Focal cortical resection in malformations of cortical development
Authors: Russo Giorgio Lo , Tassi Laura , Cossu Massimo , Cardinale Francesco , Mai Roberto , Castana Laura , Colombo Nadia , Bramerio Manuela 
Published: March 2024

ABSTRACT
Malformations of cortical development may be associated with drug‐resistant partial epilepsy suitable for surgical therapy. From the anatomopathological point of view, this categorisation has been used in reference to a wide range of alterations of the cortical mantle. Focal cortical dysplasias represent the main group of malformations of cortical development, but there are also other types of alterations, such as heterotopias, double cortex or polymicrogyria. Defining candidacy for surgical therapy and tailored resection requires thorough pre‐surgical evaluation so that the approach will be individualised for each patient. We present our series of 126 patients with malformation of cortical development selected from 321 consecutively operated patients. Within this group encompassing different types of malformation of cortical development, including periventricular heterotopia (nine patients), polymicrogyria (three patients), hemimegalencephaly (one patient) and subcortical band heterotopia (one patient), the largest group was 81 individuals with focal cortical dysplasia. For this last group, we propose a simplified classification defining 42 architectural dysplasias, 12 cytoarchitectural dysplasias and 27 Taylor's focal cortical dysplasias. In addition, at routine neuropathological investigation, the only morphological alteration shown by 31 patients was diffuse neuronal heterotopia. All patients underwent scalp EEG and video‐EEG, and 75 patients (59.5%) also underwent stereo‐EEG. Magnetic resonance imaging and stereotactic stereoscopic angiography represented the indispensable premises for further studies, in particular stereo‐EEG, and for planning surgery and tailoring resection. Magnetic resonance imaging was unhelpful in 17 out of 81 patients with focal cortical dysplasia and in seven out of 31 with neuronal heterotopia, while signal alterations were present in all other cases. Common characteristics corresponding to clinical‐histopathological homogeneous subgroups were found within the focal cortical dysplasia group. In patients with architectural dysplasia, the epileptogenic zone was mainly in the temporal lobe and there was a lower seizure frequency than in patients with Taylor's focal cortical dysplasia. Patients with Taylor's type had an epileptogenic zone that was mainly extra‐temporal, and a distinctive interictal stereo‐EEG. The best outcome was observed in patients with Taylor's type dysplasia: 69% seizure‐free (Engel class Ia) after at least 1 year of follow‐up, compared with 45% of cytoarchitectural dysplasia and 49% of architectural dysplasia patients.
Alternating hemiplegia of childhood: presentation of two cases regarding the extent of variability
Authors: Saltik Sema , Çokar Ozlem , Uslu Turkan , Uludüz Derya , Dervent Aysin 
Published: March 2024

ABSTRACT
Alternating hemiplegia of childhood is an episodic neurological disorder, the diagnosis of which is solely clinical. In this report, two patients with alternating hemiplegia, one as a representative of the classical picture and the other with unusual features, are presented by video display. Some clinical manifestations and the variability of symptoms are discussed with regard to their place in the diagnosis of the disease. (Published with videosequences)
Panayiotopoulos syndrome: video‐EEG illustration of a typical seizure
Authors: Demirbilek Veysi , Dervent Aysin 
Published: March 2024

ABSTRACT
Panayiotopoulos syndrome (PS) is a form of idiopathic, partial epilepsy of childhood with a high prevalence rate, but with poor clinical recognition, possibly due to the characteristics of the seizure, which not infrequently lack common epileptic motor and/or sensory phenomena. Instead, autonomic symptoms such as retching and vomiting, predominate. Semiological knowledge of the seizures in PS depends mainly on parental observations, but not enough ictal‐EEG data are available, possibly due to the rarity of seizures. In addition to previous knowledge regarding the occipital onset of seizures in PS, it has been recognized that other areas, mainly the frontal regions, may be involved. The present report demonstrates the video‐EEG findings of a seizure in a patient with PS. Subclinical ictal EEG discharges with occipital onset precede the clinical autonomic symptoms; eye deviation which is typical of occipital involvement, appears in later sequences. These findings suggest that at least for certain seizures an occipital origin may be considered, even in seizures without the early manifestations typical of this region. (Published with videosequences).
Dissociation and childhood abuse history in epileptic and pseudoseizure patients
Authors: Akyuz Gamze , Kugu Nesim , Akyuz Aytekin , Dogan Orhan 
Published: March 2024

ABSTRACT
The aim of this study was to examine dissociative experiences, childhood abuse and anxiety in epileptic and pseudoseizure female patients. Thirty‐three patients with pseudoseizures and thirty patients with epilepsy were recruited from Cumhuriyet University Hospital Psychiatry and Neurology Units. We assessed each participant using the Dissociative Experiences Scale, the Clinician‐Administered Dissociative States Scale and the Childhood Abuse and Neglect Questionnaire. The female patients with pseudoseizures showed significantly higher levels of dissociative experiences and childhood trauma. Epileptic female patients showed higher levels of anxiety. The significantly higher incidence of dissociative experiences and childhood trauma in the patients with pseudoseizures makes a case for dissociation in the pathogenesis of these seizures.