bluuebunny/crossref_metadata_2025_split huggingface dataset:
Magnetoencephalographic studies of focal epileptic activity in three patients with epilepsy suggestive of Lennox‐Gastaut syndrome
Authors: Sakurai Kotaro , Tanaka Naoaki , Kamada Kyousuke , Takeuchi Fumiya , Takeda Youji , Koyama Tsukasa 
Published: March 2024

ABSTRACT

Purpose
To determine the electromagnetic sources of localized epileptic activities using magnetoencephalography (MEG) in three adult patients with epilepsy suggestive of Lennox‐Gastaut syndrome (LGS).

Methods
MEG and simultaneous electroencephalography (EEG) were recorded from three adult patients using a 204‐channel, whole‐head MEG system. Equivalent current dipoles (ECDs) were calculated for epileptic spikes on MEG according to the single dipole model.

Results
In two patients, MEG showed epileptiform discharges restricted to the unilateral temporal area, corresponding to the EEG spikes. The ECDs calculated from these MEG spikes were clustered in the unilateral temporal lobe. In our third patient, MEG spikes appeared in the right centroparietal area; ECDs were located to the right parietal lobe.

Conclusions
The sources of epileptiform discharges that were detected in a restricted area were localized to specific parts of the brain cortex. Despite certain limitations (small number of patients; atypical late‐onset epilepsy in one) our study suggests that MEG may prove to be a useful tool for investigating electromagnetic features of localized epileptic discharges in patients with LGS. Based on these preliminary results, further studies performed in patients with typical LGS features are justified.
Adult epileptic spasms: a clinical and video‐polygraphic study
Authors: d'Orsi Giuseppe , Demaio Vincenzo , Minervini Mauro G. 
Published: March 2024

ABSTRACTPurposeTo analyse the video‐polygraphic features of two patients with adult epileptic spasms (ES).MethodsTwo patients with epileptic encephalopathy related to different cortical malformation; epileptic spasms had been present since the age of 18 and 25 years respectively. The patients underwent a clinical and neurophysiological examination (awake and sleep video‐polygraphic monitoring).ResultsIn patient 1, epileptic spasms were characterized by loss of contact and a drooping forward of the head and upper extremities. ES were in clusters, of brief duration, lasting for 20“'‐1”; all events occurred during eating. Awake and sleep interictal EEG disclosed asyncronous paroxysmal abnormalities in the temporo‐parieto‐occipital regions. The ictal EEG showed a diffuse slow wave complex with intermingled fast activity, prevalent on both anterior regions; on polygraphic recordings, the prevalence of the muscle activation, increscendo‐decrescendo, was in the neck and the right side. In patient 2, ES were characterized by loss of awareness, head deviation to the left, jerks of the head and left limbs. ES occurred in cluster, and were of brief duration (< 1”), usually on awakening. Interictal EEG showed epileptiform abnormalities in the right fronto‐centro‐temporal regions; abnormalities present in the awake EEG were enhanced during NREM sleep. The ictal EEG showed a diffuse, slow wave complex with intermingled fast activity, prevalent in the right regions, with concomitant muscle activation, increscendo‐decrescendo, on the left deltoid and tibialis anterior.ConclusionsOur two cases of adult ES were characterized by distinct clinical and polygraphic features, which were of brief duration, mild intensity, rare frequency per minute, and with preservation of cluster organization. In one patient, ES were induced by eating (reflex periodic spasms triggered by eating). A firm diagnosis of adult ES can be reached using video‐ polygraphic monitoring.
Near‐infrared spectroscopy as an alternative to the Wada test for language mapping in children, adults and special populations
Authors: Gallagher Anne , Thériault Martin , Maclin Ed , Low Kathy , Gratton Gabriele , Fabiani Monica , Gagnon Louise , Valois Katja , Rouleau Isabelle , Sauerwein Hannelore C. , Carmant Lionel , Nguyen Dang K. , Lortie Anne , Lepore Franco , Béland Renée , Lassonde Maryse 
Published: March 2024

ABSTRACT
The intracarotid amobarbital test (IAT) is the most widely used procedure for pre‐surgical evaluation of language lateralization in epileptic patients. However, apart from being invasive, this technique is not applicable in young children or patients who present mental retardation and/or language deficits. Functional magnetic resonance imaging (fMRI) is increasingly employed as a non‐invasive alternative. Again, this method is more difficult to use with young children, especially hyperactive ones, since they have to remain motionless during data acquisition. The aim of this study was to determine whether near‐infrared spectroscopy (NIRS) can be used as an alternative technique to investigate language lateralization in children and special populations. Unlike Wada test, NIRS is non‐invasive, and it is more tolerant to movement artefacts than fMRI. In the present study, NIRS data were acquired in four epileptic children, a 12‐year‐old boy with pervasive developmental disorder and a 3‐year‐old, healthy child, as well as three healthy and two epileptic adults, while they performed a verbal fluency task and a control task. When applicable, the results were compared to the subjects' fMRI and/or IAT findings. Clear laterality of speech was obtained in all participants, including the two non‐epileptic children, and NIRS results matched fMRI and IAT findings. These results, if replicable in larger samples, are encouraging and suggest that NIRS has the potential to become a viable, non‐invasive alternative to IAT and fMRI in the determination of speech lateralization in children and clinical populations that cannot be submitted to more invasive techniques.
Clinical evaluation of attentional processes in children with benign childhood epilepsy with centrotemporal spikes (BCECTS)
Authors: Deltour Laëtitia , Quaglino Véronique , Barathon Marion , De Broca Alain , Berquin Patrick 
Published: March 2024

ABSTRACTThe aim of this study was to identify the attentional processes specifically affected in children with benign childhood epilepsy with centrotemporal spikes (BCECTS). The impact of several factors ‐ laterality of discharges, age‐at‐onset and duration of epilepsy, and medication ‐ on these cognitive functions was also studied. A group of 29 children with BCECTS was evaluated using standardized tests performed in routine clinical practice and specifically designed to assess executive and attentional functions. This group obtained mean scores significantly lower than normative means specifically for tasks involving attention control processes, i.e. cognitive flexibility and inhibitory processes. Neither the epilepsy‐related factors that we studied nor the medication appeared to influence performance of these tasks. These data suggest an impact of BCECTS on attentional processes, the most affected being attention control processes that develop late.
Gelastic seizures: not always hypothalamic hamartoma
Authors: Cheung Christina S. , Parrent Andrew G. , Burneo Jorge G. 
Published: March 2024

ABSTRACT

Gelastic seizures are often associated with hypothalamic hamartomas. However, focal cortical dysplasias can also cause “laughing seizures”, and such cases can be difficult to localize with EEG. This case report presents a 29‐year‐old woman who was successfully rendered free of gelastic seizures after resection of a frontal cortical dysplasia, localized through MRI and SPECT imaging.
[Published with video sequences]
Ictal hiccup during absence seizure in a child
Authors: Ponnusamy Athi , Rao Ganesh , Baxter Peter , Field Patricia 
Published: March 2024

ABSTRACT

Absence seizures are one of the features of idiopathic generalised epilepsy (IGE) and occur as component of many different syndromes. They are commonly associated with various clinical features such as mild clonic components, change in postural tone, automatisms and autonomic phenomena. Childhood absence epilepsy (CAE) is the prototype IGE with typical absence seizures. We report a child who had prominent ictal hiccups during an absence seizure, and discuss the possible mechanisms.
[Published with video sequences]
Unpleasant auditory illusions and related avoidance behaviour in a child
Authors: Barba Carmen , Guerrini Renzo 
Published: March 2024

ABSTRACT

Auditory aura is a rare symptom in focal epilepsy. It has been described in autosomal dominant partial epilepsy with auditory features, but is, in general, poorly documented. We report on a 7‐year‐old, right‐handed boy, who suffered seizures characterized by positive auditory illusions with verbal and gestural automatisms and noticeable attempts at covering his ears. Clinical evaluation and video‐recording of the seizures, confirmed that most of the ictal behavior was deliberately directed at trying to prevent the unpleasant sensations reaching his ears.
[Published with video sequences]
Zonisamide for the treatment of myoclonic seizures in progressive myoclonic epilepsy: an open‐label study
Authors: Vossler David G. , Conry Joan A. , Murphy Jerome V. 
Published: March 2024

ABSTRACT

Purpose
To examine the safety and efficacy of zonisamide in treating myoclonic seizures associated with progressive myoclonic epilepsy (PME), in an open‐label setting.

Methods
Thirty patients with refractory PME (aged ≥ 5 years), who were taking up to three antiepileptic drugs, received adjunctive zonisamide (≤ 6 mg/kg/day) therapy for 16 weeks. Myoclonic seizures were recorded daily over a 24‐hour period or in 10‐minute epochs in the morning, afternoon, and evening. Safety was assessed via adverse events (AEs); efficacy was measured by the percentage of patients experiencing a ≥ 50% decrease in myoclonic seizure frequency from baseline.

Results
Treatment‐related AEs, experienced by 53% (n = 16/30) of patients, led to five patients discontinuing zonisamide. The most common AEs were decreased appetite, somnolence, and asthenia. Overall, 36% of patients (n = 10/28) had a ≥ 50% reduction in myoclonic seizure frequency.

Conclusions
These results suggest that zonisamide may be useful in the treatment of patients with PME. However, due to the size and open‐label character of this study, further research is required.
Long‐term cognitive and behavioural follow‐up in three patients with eye closure‐triggered paroxysmal activity
Authors: Termine Cristiano , Teutonico Federica , Balottin Umberto , Fasce Marco , Ferri Matteo , Perna Silvia , Piccinelli Paolo , Rubboli Guido , Veggiotti Pierangelo 
Published: March 2024

ABSTRACTPurposeTo study clinical, EEG, neuropsychological and behavioural evolution of three patients presenting with epileptic activity triggered by eye closure (EC) over a mean 10‐year follow‐up period.MethodsAll patients were studied at the time of the first observation (T0) and after a long follow‐up period (T1). At both T0 and T1, each patient underwent: 1) traditional and specific activation techniques during prolonged video‐EEG monitoring to detect possible inducing factors; 2) neuropsychological evaluations during video‐EEG monitoring either with eyes closed or eyes open to detect any transient cognitive impairment (TCI); 3) detailed neuropsychological assessment without simultaneous EEG recording, to detect any stable cognitive impairment (SCI).ResultsEEG recordings showed transient, generalized paroxysms in one case and a continuous epileptic activity triggered by eye closure in the other two cases, at both T0 and T1. In all patients, no particular epileptiform discharge‐induced factors were identified except for eye blinking (spontaneous, voluntary or induced by corneal reflex). The results of neuropsychological assessment while eyes were closed as compared to performances with eyes open, showed no significant differences at T0 or at T1 in two cases, thus possibly indicating the absence of TCI. Wechsler Intelligence Scales showed a decrease in performance at T1 in the two patients with eye closure‐induced, continuous epileptiform activity. Detailed neuropsychological assessment without EEG recordings demonstrated an impairment of facial recognition ability in all three patients at T1.ConclusionsThe lack of any differences between the results of neuropsychological tests performed with eyes open and eyes closed in two patients might suggest that not all eye‐closure‐triggered paroxysms are associated with TCI. On the other hand, our data highlight that EC‐triggered, EEG epileptic discharges can produce long‐lasting neuropsychological and behavioural effects, and also indicate that EEG discharges recurring over time might exert a disruptive effect on cognitive functions. Our three patients showed extreme variability across the neuropsychological tasks except for a facial recognition deficit that was evident in all cases, thus suggesting a possible dysfunction of temporo‐occipital brain structures and/or of the fusiform face area as recently demonstrated by combined fMRI/EEG studies in patients with fixation‐off sensitivity.
Ictal fear depends on the cerebral laterality of the epileptic activity
Authors: Guimond Anik , Braun Claude M.J. , Bélanger Émilie , Rouleau Isabelle 
Published: March 2024

ABSTRACTGlascher and Adolph (2003) proposed that both amydalae are specialized for fear, but that the right one is a fast, short, and relatively automatic fear processor, whereas the left one is more detail‐oriented and perceptual‐cognitive. According to this model, early ictal fear should occur more often in cases with a right temporal lobe epileptic focus. Several authors have tried to find a hemispheric specialization for ictal fear, but have not reached the power to attain a statistically significant effect of focus side. In this study, using previously published cases of unilateral epileptic focus causing early ictal symptoms of fear, we found 144 cases, of which 98 had a right hemisphere focus (68%) and 46 having left hemisphere focus (32%, p < 0.0005). Several control variables were assembled to verify possible alternative explanations of the main effect.
Ictal singing: case report and reappraisal of the literature
Authors: Bentes Carla , Pimentel José , Costa João , Santos Rosa , Rolo Vasco 
Published: March 2024

ABSTRACT
Singing is a rare ictal symptom of focal epilepsy. We report a case of a left‐handed woman, with no musical education, experiencing refractory seizures consisting of the singing of a popular Portuguese folksong, with simultaneous, rhythmic hand clapping. A left mesial temporal sclerosis was successfully treated by selective amygdalohippocampectomy. As in most previously reported cases, the epileptogenic zone was located in the left hemisphere, although the ictal onset zone could not be lateralized on scalp EEG. This clinical case supports the evidence that cerebral music production seems to require both hemispheres, in accordance to the concept that brain specializations for music is associated with a dynamic, well‐orchestrated interaction of music‐specific neural networks.

[Published with video sequences]
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Late‐life absence status epilepticus: a female disorder?
Authors: Szűcs Anna , Barcs Gábor , Jakus Rita , Rásonyi György , Lalit Narula , Holló András , Kelemen Anna , Janszky József , Halász Péter 
Published: March 2024

ABSTRACT
We report on three women and a review of the literature on absence status epilepticus over the age of 50 years. Our aim was to characterize the male‐female ratio in this condition. Out of 16 studies on absence status epilepticus over the age of 50, including our cases, a female dominance was found in 15. We found altogether, 104 (71%) females and 42 (29%) males. This gender difference is highly significant (p < 0.00001). We conclude that absence status epilepticus over the age of 50 is predominantly a female disorder.
Pediatric extratemporal epilepsy presenting with a complex auditory aura
Authors: Clarke Dave F. , Boop Frederick A. , McGregor Amy L. , Perkins F. Frederick , Brewer Vickie R. , Wheless James W. 
Published: March 2024

ABSTRACT

Introduction
Ear plugging (placing fingers in or covering the ears) is a clinical seizure semiology that has been described as a response to an unformed, auditory hallucination localized to the superior temporal neocortex. The localizing value of ear plugging in more complex auditory hallucinations may have more involved circuitry. We report on one child, whose aura was a more complex auditory phenomenon, consisting of a door opening and closing, getting louder as the ictus persisted.

Methods
This child presented, at four years of age, with brief episodes of ear plugging followed by an acute emotional change that persisted until surgical resection of a left mesial frontal lesion at 11 years of age. Scalp video‐EEG, magnetic resource imaging, magnetoencephalography, and invasive video‐EEG monitoring were carried out.

Results
The scalp EEG changes always started after clinical onset. These were not localizing, and encompassed a wide field over the bi‐frontal head regions, the left side predominant over the right. Intracranial video‐EEG monitoring with subdural electrodes over both frontal and temporal regions localized the seizure‐onset to the left mesial frontal lesion. The patient has remained seizure‐free since the resection on June 28, 2006, approximately one and a half years ago.

Conclusion
Ear plugging in response to simple auditory auras localize to the superior temporal gyrus. If the patient has more complex, formed auditory auras, not only may the secondary auditory areas in the temporal lobe be involved, but one has to entertain the possibility of ictal‐onset from the frontal cortex.

[Published with video sequences]
Pre‐surgical epilepsy evaluation using 3T MRI. Do surface coils provide additional information?
Authors: Strandberg Maria , Larsson Elna‐Marie , Backman Sofia , Källén Kristina 
Published: March 2024

ABSTRACT

Purpose
To assess if 3T MRI can be further improved by adding surface coil imaging, in the context of detection and characterization of cerebral lesions in patients with drug‐resistant epilepsy.

Methods
Twenty five patients with drug‐resistant epilepsy undergoing evaluation for epilepsy surgery were examined with high resolution 3T MRI. The patients were MRI‐negative (n = 15), or had unclear findings (n = 10), on previous MRI at 1.0‐1.5T. Surface coils were applied over the suspected epileptogenic zone after imaging in the head coil. In MRI‐negative patients, placement of the coils was defined by semiological analysis, extracranial video‐EEG, and, in selected cases, subtraction ictal SPECT co‐registered with MRI and PET. Coil placement was reanalyzed and graded, based on the degree of convergence between different investigational modalities.

Results
Surface coil MRI allowed visualization of the cortical lesions with somewhat better demarcation and detail, but did not contribute to detection of previously undiagnosed lesions and did not provide additional information regarding type of lesion. Possible epileptogenic lesions were detected on 3T MRI in 12 patients. No abnormalities were found in the remaining 13 patients. 3T MRI provided new or additional information about the cortex, compared with reports from previous 1.0‐1.5T MRI in 5 patients (20%).

Conclusion
3T MRI with high resolution is valuable for lesion detection, especially MCD, in patients with drug‐resistant epilepsy. We question the additional contribution from supplementary surface coil imaging at 3T MRI.
Late‐onset epilepsy in a surgically‐treated Sturge‐Weber patient
Authors: Jacobs Julia , LeVan Pierre , Olivier André , Andermann Frederick , Dubeau François 
Published: March 2024

ABSTRACT

Sturge‐Weber syndrome is a rare, congenital, neurocutaneous disorder. It can be associated with a variety of symptoms including severe epilepsy. Patients often become symptomatic during childhood and the severity of the epilepsy correlates with the patient's neurological outcome. The patient reported here remained asymptomatic until age 24, when he started to experience migraine accompanied by visual scotoma and hemiparesis. Ten years later, he developed severe, refractory epilepsy, with prolonged postictal neurological deficits and ictal as well as post‐ictal headaches. It became increasingly difficult to distinguish between migraines and seizures as both could last for several hours, as demonstrated in the two video EEGs. Both the epilepsy and the migraine may therefore have contributed to the patient's severe, neurological deterioration, probably by accelerating the progressive neuronal damage. Surgery improved the situation, but lesional areas were too extensive for complete removal. Late‐onset symptoms in Sturge‐Weber syndrome may thus result in a severe course for the disease. Early intervention and surgical treatment options are discussed.
[Published with video sequences]
Frequency of attacks in patients with psychogenic non‐epileptic seizures
Authors: Asadi‐Pooya Ali A. , Bahrami Zahra 
Published: March 2024

ABSTRACT

Aims
. The aim of this study was to investigate the frequency of attacks (psychogenic seizures) in patients with psychogenic non‐epileptic seizures (PNES) and to characterize factors potentially associated with attack frequency.

Methods
. In this retrospective study, all patients with PNES, who were studied at Shiraz Comprehensive Epilepsy Center at Shiraz University of Medical Sciences, Iran, from 2008 until 2018, were reviewed. We categorized the attack frequency in the patients as (1) daily; (2) weekly; and (3) frequency of less than one per week.

Results
. Three hundred and ten patients were studied. Attack frequency in patients was 34 ± 67 per month. One hundred and eleven patients (36%) had daily attacks, 93 (30%) had weekly attacks, and 106 (34%) had less than weekly attacks. Sixty‐five patients (21%) reported having more than one attack per day. Demographic variables, attack‐related variables, PNES associated factors, and use of AEDs were not significantly associated with attack frequency in the patients.

Conclusion
. We observed that two thirds of the patients with PNES had frequent daily or weekly attacks. The findings of our study could be helpful in designing future clinical trials. First, attack frequency is an unbiased outcome measure in the design of such studies. Second, it is easily measurable using attack calendars; we suggest that attack frequency be assessed daily using daily attack calendars. Finally, it is very easy to recruit patients with PNES for clinical trials (with regards to their attack frequency) since many of them have frequent attacks.
The role of cannabinoids in epilepsy treatment: a critical review of efficacy results from clinical trials
Authors: Nabbout Rima , Thiele Elizabeth A. 
Published: March 2024

ABSTRACT

CBD was shown to have anti‐seizure activity based on
in vitro
and
in vivo
models. However, several reports of small series or case reports of the use of cannabis extracts in epilepsy yielded contradictory results and the efficacy of cannabis use in patients with epilepsy have also been inconclusive. In 2013, the first Phase 1 trial for a purified form of CBD (Epidiolex/Epidyolex; >99% CBD), developed by GW Pharma, showed some efficacy signals and subsequently, a comprehensive program on the efficacy and tolerability of this compound for the treatment of drug‐resistant epilepsies was initiated. Results of these trials led to the FDA and EMA approval respectively in 2018 and 2019 for the treatment of seizures associated with two rare epilepsies: Lennox‐Gastaut syndrome (LGS) or Dravet syndrome (DS) in patients two years of age and older. Thus, CBD became the first FDA‐approved purified drug substance derived from cannabis and also the first FDA‐approved drug for the treatment of seizures in DS. We detail the clinical studies using purified CBD (Epidiolex/Epidyolex), including the first open interventional exploratory study and Randomized Control Ttrials for DS and LGS.