bluuebunny/crossref_metadata_2025 huggingface dataset:
Dampak Presidential Thresold Terhadap Pencalonan Presiden Dalam Pemilu Tahun 2024
Authors: Muhammad Faishal Rizqullah  , Nurhasanah Putri Aliyya , Muhammad Luthfi Permana  , Nurlaili Rahmawati
Published: March 2024

Pemilihan presiden di Indonesia telah lama menjadi sorotan utama dalam sistem politik demokratis negara ini. Salah satu aspek penting dalam pemilihan presiden adalah Presidential Threshold, yaitu persyaratan jumlah dukungan dari partai politik atau calon legislatif yang diperlukan bagi seorang calon presiden agar dapat mengikuti pemilihan presiden. Pada Pemilu tahun 2019, Presidential Threshold telah mengalami perubahan signifikan dengan peningkatan persentase dukungan yang diperlukan dari sebelumnya. Jurnal ini bertujuan untuk menyelidiki dampak perubahan Presidential Threshold terhadap pencalonan presiden dalam Pemilu tahun 2024 di Indonesia dan implikasinya terhadap dinamika politik dan partisipasi dalam pemilihan presiden. Berdasarkan hasil penelitian menunjukkan bahwa peningkatan Presidential Threshold telah mempengaruhi strategi calon presiden dan partai politik dalam menghadapi pemilu. Calon presiden diharuskan untuk menggandeng lebih banyak partai politik atau mencari dukungan lebih luas dari calon legislatif untuk memenuhi syarat. Implikasi dari perubahan ini adalah munculnya koalisi yang lebih besar dan kompleks dalam perebutan kekuasaan presiden. Jurnal ini akan membahas lebih lanjut hasil penelitian ini dan menyajikan rekomendasi kebijakan yang relevan untuk menjaga keseimbangan antara persyaratan yang ketat dan partisipasi pemilih dalam pemilihan presiden di masa depan.
Wojna w Ukrainie – przyczyny i skutki po ponad roku trwania
Authors:   Emerytowany prof. Uniwersytetu Wrocławskiego, Wiktor Zbigniew
Published: March 2024

The war in Ukraine has been going on for more than a year now and there is no indication that it will end soon. The causes of its outbreak are very complex. One of them was the victory of the counter-revolution and the collapse of socialism, followed by the collapse of the former Soviet Union in December 1991. At that time, fifteen independent republics were established to varying degrees – their disintegration took place not only ideologically and politically, but also economically. In many of them, nationalisms arose or were revived, including those based on religion, which included not only the main former union republics, but also numerous national minorities, particularly the Baltic states, the Caucasus, and the Volga region. Some of them turned into local wars. An important role in the disintegration of the countries of the former Soviet Union and the weakening of the Russian Federation was played by external forces, imperialist states, mainly the US, some European countries, NATO in general, and the rivalling with Russia European Union and other forces of international capital. The war in Ukraine has deeper causes, and in case of Russia’s defeat, it will have further adverse consequences for it as a great power, and may even become the beginning of its disintegration. Hence, the result is the full determination of the Russian ruling forces to achieve victory. At the same time, Ukraine enjoys the support of the entire „global West”, led by the US, NATO, and the European Union. The war was preceded by numerous growing contradictions and accompanying conflicts, which took the form of the ‚Orange Revolution’ in 2004, the so-called Revolution of Dignity and the Maidan coup, the declaration of independence by Crimea and the Donetsk and Luhansk Republics in 2014. It took another eight years for the conflict to escalate into a full-blown war, although Russia officially still defines it as a „special military operation”.
Structural-Linguistic and Cognitive Models of Metonymy in English and Azerbaijani Literary Texts
Authors:   Baku Slavic University, Azerbaijan, Hasanova Medina Murad
Published: March 2024

The article is devoted to lingua-cognitive models of metonymy in English and Azerbaijani literary texts. First, metonymy and metonymic models were reported. Speaking about metonymic models, we turned to the concepts of J. Lakov, R. Jacobson, J. Raden, Z. Kovaks, E. Paducheva, etc., and analyses were carried out on their basis. The works of such prominent writers as T. Dreiser, F. Karimzadeh, V. Scott, and V. Somerset were used as factual material. From the conducted typological studies, it is concluded that metonymic models in English and Azerbaijani are similar and universal. The scientific results of linguistic research have played an important role in studying this problem in a psychological, philosophical and social context. It turned out that the ancient variants of meaning transfers arose based on abstraction and generalisation of meaning that arose in the minds of primitive people. Their formation as a model of consciousness occurred in developing people’s life experiences from simple to complex. The appearance of elements of logical cognition in primitive people was an incentive for the formation of more advanced models of consciousness.
Tendency to Syntagmatic Expansion of the Generating Base in Bilingual Children
Authors:   Azerbaijan University of Languages, Azerbaijan, Sobor Aida Burzu
Published: March 2024

The difference between children’s innovations and the occasionalism of artistic and adult everyday speech lies, first of all, in their “simultaneity”, close connection with the objective situation and the child’s specific activity. While working in German schools, we often observed that children from Azerbaijani migrant families also tend to use regular and ordinary types of word – and form formation instead of irregular and rarer ones. Foreign children’s illiterate use of word forms testifies, first of all, to the meta-communicative and stylistic competence of the younger schoolchild that is being formed on their part. Indeed, the generalisation of grammatical form is not alien to the modern German language. The use of these kinds of words in the speech of foreign children is mainly because, at their young age, they cannot correctly identify individual word-building elements with the help of which one word or another is formed. With all the striving of foreign-speaking children to streamline the language system in some way convenient for them, to make it “correct”, they themselves cannot avoid paradoxes.
„Nie można być cywilizowanym na dwa sposoby…” – o różnorodności cywilizacji w twórczości wielkiej sławy myśliciela Feliksa Konecznego (1862–1949)
Authors:   Uniwersytet Opolski, Banaś Agnieszka
Published: March 2024

This article presents the view on the concept of civilization of the Krakow historian and publicist, Feliks Koneczny. The author believed that all historical facts should be explained in the context of its civilisational background. However, he emphasised that man was created to live in a group, but only in one, because one cannot be “civilised” for these two particular communities; to call them a civilisation, and why did some civilisations fight each other. Feliks Koneczny has been looking for answers to these questions for years. The paper demonstrates and explains his scientific scrutiny.
Focal motor seizure with automatisms in a newborn
Authors: Vecchi Marilena , Suppiej Agnese , Mastrangelo Massimo , Boniver Clementina
Published: March 2024

Continuous synchronized video‐EEG‐polygraphic recordings allow us to better define the electroclinical patterns of epileptic events and to study the semiological features of neonatal seizures. Recently, complex behaviours and motor automatisms in newborns have been reported as being epileptic in nature. However, the debate on physiopathology (cortical or brainstem release phenomena) is ongoing. We present the synchronized video‐EEG documentation of epileptic complex motor behaviours in a newborn male. Our case contributes to the discussion regarding the semiological classification of the neonatal seizures.

[Published with video sequences]
Complex partial status epilepticus is an unrecognised feature in SESA syndrome: new insights into its pathophysiology
Authors: Fernández‐Torre José L. , Hernández‐Hernández José L. , Jiménez‐Bonilla Julio , González‐Mandly Andrés , García‐Regata Oscar
Published: March 2024

We report a patient diagnosed as having subacute encephalopathy with frequent seizures in alcoholics (SESA syndrome), in which recurrent, non‐convulsive seizures of frontal origin contributed significantly to the alteration of the mental state. Our case suggests that the occurrence of episodes of complex partial status epilepticus (CPSE) may contribute greatly to the origin and pathophysiology of the confusional state in this rare, epileptic entity.
A case of congenital bilateral perisylvian syndrome due to bilateral schizencephaly
Authors: Vinayan K. Puthenveettil , Terada Kiyohito
Published: March 2024

A case of congenital bilateral perisylvian syndrome (CBPS) associated with bilateral perisylvian schizencephaly in a 24‐year‐old woman is reported. She presented the classical clinical triad of CBPS, which included congenital facio‐masticatory diplegia, epilepsy and only mild mental retardation, despite the presence of bilateral, open‐lip clefts in the perisylvian region. We hypothesize that the minimal loss of cortical tissue, along with the possible sparing of vital white matter association fibers and neuronal plasticity might have contributed to the better functional outcome in this patient.
Charles Bonnet syndrome in hemianopia, following antero‐mesial temporal lobectomy for drug‐resistant epilepsy
Authors: Contardi Sara , Rubboli Guido , Giulioni Marco , Michelucci Roberto , Pizza Fabio , Gardella Elena , Pinardi Federica , Bartolomei Ilaria , Tassinari Carlo Alberto
Published: March 2024

Charles Bonnet syndrome (CBS) is a disorder characterized by the occurrence of complex visual hallucinations in patients with acquired impairment of vision and without psychiatric disorders. In spite of the high incidence of visual field defects following antero‐mesial temporal lobectomy for refractory temporal lobe epilepsy, reports of CBS in patients who underwent this surgical procedure are surprisingly rare. We describe a patient operated on for drug‐resistant epilepsy. As a result of left antero‐mesial temporal resection, she presented right homonymous hemianopia. A few days after surgery, she started complaining of visual hallucinations, such as static or moving “Lilliputian” human figures, or countryside scenes, restricted to the hemianopic field. The patient was fully aware of their fictitious nature. These disturbances disappeared progressively over a few weeks. The incidence of CBS associated with visual field defects following epilepsy surgery might be underestimated. Patients with post‐surgical CBS should be reassured that it is not an epileptic phenomenon, and that it has a benign, self‐limiting, course which does not usually require treatment.
Ipsilateral blinking seizures during left fronto‐temporal ictal pattern on scalp EEG
Authors: Pestana Elia M. , Gupta Ajay
Published: March 2024

We report an infant with left eye blinking seizures accompanying a left (ipsilateral) fronto‐temporal scalp EEG ictal pattern. The epileptogenic lesion was a left frontal encephalomalacia along the ventriculo‐peritoneal shunt tract. The shunt was inserted for treatment of communicating hydrocephalus. This case illustrates the lateralizing value of the ictal blinking. Review of the literature suggests that seizures with unilateral blinking are likely to be produced by activation of ipsilateral trigeminal fibers innervating subdural intracranial structures and pial vessels in temporal and frontal lobes. Ipsilateral blinking could also be produced by activation of the ipsilateral cerebellar hemisphere.
Granular cell dispersion and bilamination: two distinct histopathological patterns in epileptic hippocampi?
Authors: Silva Alexandre V. , Houzel Jean‐Christophe , Croaro Ingrid , Yacubian Elza Márcia Targas , Stavale João Norberto , Centeno Ricardo Silva , Cavalheiro Esper Abrão
Published: March 2024

Cytoarchitectural modifications of the dentate gyrus are among the most obvious abnormalities observed in the hippocampal sclerosis associated with refractory epilepsy. Here, we examined the morphological changes of granular cells (dispersion, bilamination and cell loss) in sclerotic hippocampi from nine TLE patients, comparing abnormal and preserved areas. A total of 2 577 granular cells were analyzed with respect to four different histopathological patterns: areas with bilamination (n = 936), areas with dispersion (n = 905), areas with neuronal loss (n = 279), and preserved areas (n = 457). Quantitative parameters included somatic perimeter
(P)
, area
(A)
and form factor
(ff)
.Although different patterns were often observed in the same patient, highly significant differences were observed (p < 0.0001) when patterns were compared to one another. Since granular cell dispersion and bilamination have different morphological aspects in sclerotic hippocampi from TLE patients, we suggest that these patterns should be considered separately. Future studies are needed to determine the frequency with which these patterns occur in the general population and whether each one can interfere with seizure susceptibility.
Epilepsia partialis continua: semiology and differential diagnoses
Authors: Bien Christian G. , Elger Christian E.
Published: March 2024

Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. It may have vascular, immune‐mediated, neoplastic or metabolic‐toxic causes. The origin of EPC has been linked with the motor cortex. This has been solidly supported by sophisticated electrophysiological studies. Here, a series of video sequences from patients with EPC (due to Rasmussen encephalitis, early‐stage multiple sclerosis, and steroid responsive encephalopathy with autoimmune thyroiditis), and other cases with repetitive myoclonic jerks or movement disorders (myoclonic epilepsy associated with ragged‐red fibers, Jacksonian march, myoclonic seizures in other types of frontal lobe or idio‐pathic generalized epilepsies, and different types of tremor) is presented.
[Published with video sequences].
Ictal asystole in temporal lobe epilepsy before and after pacemaker implantation
Authors: Strzelczyk Adam , Bauer Sebastian , Knake Susanne , Oertel Wolfgang H. , Hamer Hajo M. , Rosenow Felix
Published: March 2024

Events of ictal bradycardia or asystole may be of relevance in epilepsy patients presenting with ictal falls, and are a potential contributor to SUDEP. The literature on ictal bradycardia or asystole is anecdotal and consists of case reports and small case series. There are no guidelines for the care of patients with ictal arrythmias. Insertion of cardiac pacemakers may prevent life‐threatening cardiac arrest, syncope and trauma.

We report the case of a 41‐year‐old man who presented with refractory partial seizures resulting in syncope leading to severe head trauma, as the only ictal semiology. During presurgical video‐EEG monitoring, two episodes of ictal bradycardia followed by asystole and syncope were recorded. A cardiac pacemaker was implanted. At the nine‐month follow‐up, the patient reported no overt seizures, syncopes or traumatic falls. Our case demonstrates that implantation of a cardiac pacemaker while continuing AEDs may render a patient free from ictal symptoms and prevent ictal syncope and subsequent trauma.[Published with video sequences].
Periodic eye opening and swallowing movements associated with post‐anoxic burst‐suppression EEG pattern
Authors: Fernández‐Torre José L. , Calleja Jesús , Infante Jon
Published: March 2024

We report a video‐electroencephalogram study of a 50‐year‐old woman who developed a clinical picture consisting of stereotyped periodic eye opening followed by eye closing, with or without swallowing movements, after a prolonged cardiopulmonary arrest. These movements were associated with a burst‐suppression pattern on the electroencephalogram.
[Published with video sequences]
Poor presurgical performance on both verbal and visual memory measures is associated with low risk for memory decline following left temporal lobectomy for intractable epilepsy
Authors: Busch Robyn M. , Chapin Jessica S. , Umashankar Gopalan , Diehl Beate , Harvey Daniel , Naugle Richard I. , Nair Dileep , Najm Imad M.
Published: March 2024

Studies have shown a lower risk for verbal memory decline following dominant anterior temporal lobectomy (ATL) among patients with poor, presurgical verbal memory scores. It is unclear however, if the risk of decline is increased in patients who also have reduced visual memory. Objective and subjective memory outcome following left ATL was examined in twelve patients with reduced presurgical visual and verbal memory scores. Only one patient demonstrated a meaningful decline in memory scores, with a decline in visual memory following surgery. Presurgically, this patient demonstrated poor memory bilaterally on Wada testing and small discrepancy in hippocampal volumes. She was also one of two patients who continued to have seizures post‐surgery. This preliminary study suggests that patients with unilateral, left TLE and poor verbal and visual memory are unlikely to show meaningful memory declines following left ATL, particularly if they demonstrate expected patterns on Wada testing, hippocampal volume discrepancy (left < right), and postsurgical seizure‐freedom.
Cough syncope misinterpreted as epileptic seizure
Authors: Gelisse Philippe , Genton Pierre
Published: March 2024

Cough syncopes are rare but may be one of the cause of sudden loss of consciousness. We recorded under video‐EEG monitoring a typical syncope triggered by voluntary coughing (video) and Valsava maneuvre in a patient referred for the diagnosis of generalized tonic‐clonic seizures. There were no signs of active epilepsy. The attacks were associated with overweight, obstructive sleep apnea and heavy consumption of cigarettes.
[Published with video sequences]
Absence status epilepticus in monozygotic twins with Jeavons syndrome
Authors: Yang Tianhua , Liu Yonghong , Liu Ling , Yan Bo , Zhang Qin , Zhou Dong
Published: March 2024

As a generalized form of nonconvulsive status epilepticus (NCSE), absence status epilepticus is the most common form. It manifests as prolonged, confusional states of varying severity, and continuous or repetitive generalized discharges of spikes, multiple spikes, and slow waves on EEG. Jeavons syndrome (JS) is a new type of epilepsy syndrome. Hitherto, only four sets of monozygotic twin with JS have been reported. Absence status epilepticus occurring in monozygotic twins with JS have not been reported. Here we report on monozygotic male twins of Chinese origin with JS. Both of them presented with status epilepticus with eyelid myoclonia and absences.

[Published with video sequences]
Anatomo‐electro‐clinical correlations: the University of Cleveland, USA Case Report Case 03‐2008: Medically intractable seizures originating from the primary somatosensory hand area
Authors: Koubeissi Mohamad Z. , Maciunas Robert J. , Tanner Adriana , Lüders Hans O.
Published: March 2024

[Case records of
Epileptic Disorders.
Anatomo‐electro‐clinical correlations. Case 03‐2008]

A 33‐year‐old woman had begun having intractable somatosensory seizures affecting the left hand since the age of 13 years. Occasionally, her seizures progressed to left arm posturing followed by secondary generalization. Scalp EEG revealed interictal epileptiform discharges in the right posterior quadrant, but with no ictal EEG correlates. Brain MRI showed a right temporal encephalomalacia, sparing mesial temporal structures, suggestive of a perinatal vascular insult. Ictal electrocorticogram, electrical stimulation mapping, and somatosensory evoked potentials localized the ictal onset to the hand area of the postcentral gyrus. Resection of that area resulted in total resolution of seizures with no significant lasting deficits. Potential complications of resecting the primary somatosensory hand area can be severe, as proprioceptive sensory loss may be permanent, resulting in significant disability. Such deficits may be temporary however, and the literature continues to report conflicting results regarding postsurgical outcome. Cortical plasticity may explain recovery of sensory deficits after partial resection of the primary somatosensory hand area. Multiple subpial transections of that area are sometimes performed to minimize functional deficits, but seizure control may be less optimal than with cortical resection.
Anatomo‐electro‐clinical correlations: the Great Ormond Street Hospital, UK Case Report ‐ Case 05‐2008: Early‐onset symptomatic focal epilepsy: a dilemma in the timing of surgery
Authors: Desai Neely , Pressler Ronit M. , Jolleff Nicola , Clark Maria , Neville Brian , Eltze Christin , Harkness William , Cross J. Helen
Published: March 2024

[Case records of
Epileptic Disorders.
Anatomo‐electro‐clinical correlations. Case 05‐2008]

We report the case of a six‐year‐old boy who presented in infancy with infantile spasms and left focal seizures. An MR scan at two months was suggestive of a right parietal cortical dysplasia, although this was less apparent on repeat scan at 11 months. The initial response to anti‐epileptic medications was good; surgery was therefore deferred at that time. Subsequently, seizure control fluctuated and developmental progress was, on the whole, good. However, ultimately seizures increased despite changing the AED, and he began showing developmental problems. Surgery was reconsidered. Again, a repeat MR scan did not define the lesion well. Following full further evaluation, including functional imaging that still implicated the right parietal cortex, subdural grid and depth electrode monitoring were undertaken at 6.5 years, which located the ictal onset zone deep within the lesion. This enabled a right inferior parietal lobe resection to be performed. Four years post‐surgery he remains seizure‐free and has shown progress in development.
Seizure‐related automatic locomotion triggered by intracerebral electrical stimulation
Authors: Gardella Elena , Rubboli Guido , Francione Stefano , Tassi Laura , Russo Giorgio Lo , Grillner Sten , Tassinari Carlo Alberto
Published: March 2024

We describe the case of an eight‐year‐old boy, who underwent a video‐stereo EEG (SEEG) investigation for the presurgical assessment of drug‐resistant epilepsy, related to a right fronto‐lateral cortical dysplasia and who became seizure‐free after epilepsy surgery. The unexpected finding of the investigations was that intracerebral, high frequency (50 Hz) electrical stimulation (HFS) triggered the emergence of automatic and involuntary forward‐backward locomotion during a focal seizure while the boy was standing. This clinical manifestation was different from the chaotic motor activity described during epileptic wanderings. The stimulation of the same fronto‐lateral region, while the patient was lying, produced only the subjective sensation that the legs were moving, although there was no physical manifestation of this. Human locomotion is an innate motor behavior that is normally due to the activation of the spinal network for locomotion (central pattern generator). The emergence of different stereotyped motor behaviors during focal epileptic seizures or sleep disorders has recently been interpreted as a release of subcortical central pattern generators (Tassinari
et al.
2005). In view of this, we hypothesize that the involuntary and robot‐like locomotion of our patient could be the ictal expression of the release of subcortical locomotor CPGs.
[Published with video sequences]
Catatonic psychosis related to forced normalization in a girl with Dravet's syndrome
Authors: Gobbi Giuseppe , Giovannini Simona , Boni Antonella , Visconti Paola , Beghi Massimiliano , Cornaggia Cesare Maria
Published: March 2024

It has been reported that the clinical presentation of forced normalization can vary from paranoid hallucinatory states to anxiety and conversion phenomena, and that it may occur in both generalised and focal epilepsies. On the basis of the evaluation of a video recording, we found that forced normalization was concomitant with catatonic psychosis in a patient with epilepsy, intellectual disability and pervasive developmental disorder. Catatonic psychosis accompanying forced normalization has not been previously reported. As the psychotic symptoms and quality of life worsen seizure control improves, we believe it may be better for the patient to tolerate some seizures, thus preserving their capacity to interact socially.
[Published with video sequences]
Pure photosensitive ictus emeticus
Authors: Thomas Pierre , Zifkin Benjamin G.
Published: March 2024

Content available: Video
Benign myoclonus of early infancy
Authors: Pachatz Christa , Fusco Lucia , Vigevano Federico
Published: March 2024

Benign myoclonus of early infancy, first described by Fejerman and Lombroso, is a paroxysmal phenomenon of the first 2 years of life which occurs in neurologically healthy infants during wakefulness, and is usually triggered by excitement or frustration. We studied the neurophysiological features of the phenomenon in five children, aged 7 to 11 months, who were monitored by video‐EEG recording, and by polygraphic recording in 3 of the 5 cases. The phenomenon is characterized by a shudder‐type, paroxysmal motor manifestation involving mainly the trunk and sometimes the head, associated with tonic limb contractions of variable intensity, from hardly noticeable to more sustained. The EEG counterpart never showed modifications, the polygraphic study demonstrated a brief tonic limb contraction. The clinical manifestation should not be confused with the spasms of epileptic infantile spasms syndrome, or with tonic reflex seizures of early infancy. Although the phenomenon is already widely known, its polygraphic recording is rarely reported in literature. The polygraphic data of our patients contributes to the diagnosis and understanding of the pathophysiology of this paroxysmal manifestation.
Tactile evoked spikes in children
Authors: De Marco Pasquale , Calzolari Stefano
Published: March 2024

Tactile evoked spikes (TES) are a well known EEG feature in children. We reviewed our previous studies concerning this phenomenon, with special reference to the influence of sleep and stimulation rate, the relation with middle‐long latency somatosensory evoked potentials (MLSEP) and the relation between TES and spontaneous spikes in the same individual. New data were obtained by re‐evaluating 566 children with TES, by assessing MLSEP from each finger, in 15 subjects with TES, and by studying MLSEP following posterior tibial nerve stimulation in 12 children with TES.

TES appear to be enlarged components of MLSEP which are present in some children aged 4 to 14 years; they tend to increase during NREM sleep and to decrease during REM sleep; Their amplitude decreases at stimulation rates above 3 Hz. TES are associated with an increased risk of epileptic seizures, which usually have a benign prognosis. The possible neurophysiological mechanisms underlying this phenomenon are discussed.
Benign epileptic seizures in infancy followed by paroxysmal choreoathetosis during adolescence
Authors: Koch C. , Bednarek N. , Motte J.
Published: March 2024

Content available: Video
Somatosensory evoked potentials and EEG findings in siblings of juvenile myoclonic epilepsy patients
Authors: Atakli D. , Soysal A. , Atay T. , Altintas H. , Arpaci B. , Baybas S.
Published: March 2024

Purpose

In order to demonstrate cortical hyperexitability and to determine the efficacy of 'SEP of high amplitude (HASEP) in detecting asymptomatic relatives of JME patients, we evaluated the EEG findings and SEP in asymptomatic siblings of JME patients.

Methods

We analysed the EEG and SEP findings of 37 JME patients and 48 asymptomatic siblings of 27 patients and compared the results with healthy volunteers.

Results

Of 13 asymptomatic siblings with EEG abnormalities (27.1%), 5 had 4‐6 Hz spike/polyspike and wave paroxysms (10.4%), 5 had slow wave paroxysms (10.4%), and the remaining 3 patients exhibited focal spike and wave activity during hyperventilation (6.25%). No significant difference was found between JME patients, asymptomatic siblings and control groups with respect to N20 latencies. N20/P25 amplitudes were significantly higher in both JME group and asymptomatic siblings than those of control cases. HASEP were observed in 8 out of 37 JME patients (21.6%), 10 out of 48 healthy siblings (20.8%) and none of the control cases. The consanguinity rate was higher in parents of patients who had at least one asymptomatic sibling with EEG and/or SEP abnormalities (68.7%) than in those of patients who had siblings with no EEG and SEP abnormalities (9.9%). We suggest that the high rate of SEP and EEG changes among offspring of consanguineous marriages might reflect the genetic heterogeneity of the disease.
Paroxysmal non‐kinesigenic dyskinesia: pathophysiological investigations
Authors: Lombroso Cesare T. , Fischman Alan
Published: March 2024

The paroxysmal dyskinesias constitute a heterogeneous group of disorders whose pathophysiologies and nosologies are still generally unknown. In a boy with severe, paroxysmal non‐kinesigenic dyskinesia (PNKD), an invasive video‐electrographic study demonstrated that his PNKD did not originate from the cortex, while a discharge was registered from the caudate nuclei. An
18
FDG PET scan failed to show metabolic anomalies. A
18
FDOPA and a
11
C raclopride PET scans revealed a marked reduction in the density of presynaptic dopa decarboxylase activity in the striatum, together with an increased density of postsynaptic dopamine D
2
receptors. These findings may suggest a chronic upregulation of postsynaptic dopa receptors, either because of an increase in their numbers or changes in their affinity. It remains unknown if these are secondary to reduced dopamine synthesis or altered release, or if the changes in striatal dopamine receptor binding are a primary or a compensatory mechanism.
Gelastic seizures: video‐EEG and scintigraphic analysis of a case with a frontal focus; review of the literature and pathophysiological hypotheses
Authors: Biraben A. , Sartori E. , Taussig D. , Bernard A.M. , Scarabin J.M.
Published: March 2024

We report scalp EEG and SPECT findings in a young patient who experienced gelastic seizures; clinical, EEG and scintigraphic data strongly suggested a frontal focus in a context of cryptogenic epilepsy. Few cases of gelastic seizures originating in the frontal lobe have been reported in the literature, most of them involving a diencephalic hamartoma or a temporal focus although, no clinical pattern has been found to be specific for each of these three anatomical regions. The ictal laughter is of variable nature, unmotivated or associated with feelings of mirth, forced or natural, except in the case of a frontal focus where the laughter seems consistently described as forced and unmotivated. However, mirth and laughter are two dissociable clinical elements; their genesis probably involves distinct mechanisms. Anatomical considerations lead to several hypotheses concerning laughter generation: it could be a simple reactional behavior in response to a modified cognitive process, an automatic behavior or a forced action. In a few cases with a temporal focus, laughter seems directly related to a disorganization of the associative temporal cortex and may be considered as a reactional behavior. In cases with frontal focus, anterior cingulate and orbital structures would be particularly implicated in laugher genesis, although with possible different pathophysiological routes: in the first case by disconnection within the premotor mesial system or by an imbalance between premotor mesial and premotor lateral systems, and in the second case by activation of a previously conditioned orbital region.
Rotatory seizures in a patient with tuberous sclerosis
Authors: Ramelli Gian Paolo , Donati Filippo , Kollar Mihaly , Remonda Luca , Vassella Franco
Published: March 2024

We present an 11‐year‐old girl with tuberous sclerosis who developed seizures characterized by circling behavior.

Rotatory seizures are uncommon and occur mainly secondary to a focal pathology. Our patient had a right temporal epileptic focus, confirmed by magnetic resonance imaging (MRI) to be a subcortical lesion in the right temporal region.

This case is probably the first reported case of tuberous sclerosis associated with rotatory seizures with an ictal EEG.
Letter from the editor
Authors: Aicardi Jean
Published: March 2024

Content available: Video
Piracetam and levetiracetam: close structural similarities but different pharmacological and clinical profiles
Authors: Genton Pierre , Van Vleymen Betty
Published: March 2024

Piracetam (PIR) and levetiracetam (LEV), an S‐enantiomer, are pyrrolidone derivatives that share similar chemical structures but have distinct pharmacological profiles and consequently different clinical uses. Although the mode of action of neither drug has been fully elucidated, they do not interact with inhibitory or excitatory neurotransmission or alter membrane excitability. A brain‐specific stereoselective binding site has been identified for which LEV and other S‐enantiomers, but not PIR, have high affinity. In preclinical studies, PIR significantly improves learning and memory; in contrast, LEV has less effect but is much more active in preventing seizures. Both drugs have a high therapeutic index and are well tolerated. PIR, a nootropic drug, is used in the therapy of age‐related cognitive disturbances and poststroke aphasia. Clinical experience has also shown that at high doses it is effective against cortical myoclonus. LEV is an antiepileptic drug. Clinical trials have confirmed its efficacy in partial seizures and preliminary findings suggest that it is also effective in generalized seizures and myoclonus.
The role of gamma knife surgery in the treatment of severe epilepsies
Authors: Régis Jean , Bartolomei Fabrice , Hayashi Motohiro , Roberts David , Chauvel Patrick , Peragut Jean‐Claude
Published: March 2024

There is a strong rationale for investigation of the role of gamma knife radiosurgery in the treatment of medically intractable epilepsy. To explore this potential application, the current outcome and morbidity associated with established microsurgical treatment, as well as the associated advantages and disadvantages of open surgery, are reviewed. The preliminary evidence in support of radiosurgical treatment and the recent experience with gamma knife treatment for epilepsy associated with mesial temporal sclerosis, cavernous angioma, and hypothalamic hamartoma or other lesions are presented. The strengths and limitations of this application are discussed, and the challenges facing both microsurgical and radiosurgical approaches are considered. Gamma knife surgery can be a main approach among others in the armamentarium of epilepsy surgery. Although the benefits of comfort and reduced invasivity can be clearly perceived, larger series and long‐term follow up are still required in order to evaluate the future of this particular surgical approach.
Infantile spasms and Menkes disease
Authors: Sfaello Ignacio , Castelnau Pierre , Blanc Nathalie , Ogier Hélène , Evrard Philippe , Arzimanoglou Alexis
Published: March 2024

Epileptic seizures are a common feature in Menkes disease, an X‐linked genetic disorder of copper metabolism. Details of type of seizures are rarely reported. We report the evolution of infantile spasms in two patients with Menkes disease and the relation with subcutaneous administration of copperhistidine.
Pathogenesis of Lennox‐Gastaut syndrome: Considerations and hypotheses
Authors: Blume Warren T.
Published: March 2024

No other epilepsy more substantially combines cryptogenicity and intractability than does Lennox‐Gastaut Syndrome. Although antecedent neurological conditions have preceeded LGS in some patients, others with similar ills fail to develop this syndrome, and precise charting of the pathway between any presumed aetiology and the epilepsy has eluded researchers. Despite these frustrations, LGS has elicited a host of publications over the years, possibly because it combines features of many other epileptic disorders and thereby occupies a crossroads position among them. Lacking a comprehensive experimental model of LGS, this article combines relevant data from several clinical and basic sources in order to formulate a concept of pathogenesis. Data are presented to suggest that the occurrence of factors enhancing excitability during a vulnerable period of cortical and thalamic development may permanently imprint a bilateral, diffuse epileptogenic system upon the mammalian brain.
Benign paroxysmal tonic upgaze of childhood with ataxia
Authors: Lispi Maria Luisa , Vigevano Federico
Published: March 2024

Paroxysmal tonic upgaze deviation (PTU) is a rare neuro‐ophthalmological disorder with onset in infancy or early childhood. It consists of episodes of sustained, conjugate, upward deviation of the eyes, down beating saccades in attempted downgaze, apparently preserved horizontal eye movements, frequent association with mild ataxia or clumsiness at time of illness, normal metabolic, electroencephalographic and neuroradiological findings. Symptoms are frequently relieved by sleep and can be exacerbated by fatigue or illness. Although PTU generally tends to disappear spontaneously within a few months or years, subsequent case reports have demonstrated the heterogeneous nature of the syndrome with respect to outcome. To date, the pathogenesis of the condition is still unknown. We present a new case of PTU with ataxia occurring in an otherwise healthy child, as documented by video recording of the phenomenon.
Asymmetric tonic seizures with bilateral parietal lesions resembling frontal lobe epilepsy
Authors: Ikeda Akio , Matsumoto Riki , Ohara Shinji , Kunieda Takeharu , Shirakashi Yoshitomo , Kaji Ryuji , Fukuyama Hidenao , Shibasaki Hiroshi
Published: March 2024

We performed long‐term video/ EEG monitoring and a single photon emission computed tomographic (SPECT) study to clarify generating mechanism of bilateral tonic motor seizures resembling seizures generated by the supplementary motor area (SMA), in patients with bilateral parietal lesions. We describe 2 patients (age 24 and 32 years), with bilateral parietal lesions. Clinically, seizures were preceded by lightning sensation in the body, followed by asymmetric tonic posturing of both hands and thrashing movements of the feet, lasting for less than 1 min.

Ictal rhythmic (7‐15 Hz) activity at the vertex was observed on the EEG in 1 patient. Interictal SPECT in 2 patients showed decreased blood flow in both parietal areas, consistent with bilateral parietal abnormalities on T2‐ and T1‐weighted MRIs. Ictal SPECT in 1 patient showed increased blood flow in the right parietal and frontopolar areas.

The present 2 patients had clinically asymmetric tonic seizures, most likely resulting from spreading of the ictal activity from the parietal lesions via the superior longitudinal fasciculus to the SMA. Bilateral, homologous lesions in the parietal area might cause disinhibition on the unilateral epileptogenic side.
Perioral myoclonia with absence seizures: a rare epileptic syndrome
Authors: Bilgiç Başar , Baykan Betül , Gürses Candan , Gökyiğit Ayşen
Published: March 2024

We present the clinical and video‐EEG data on an epileptic boy whose absence seizures with marked perioral movements had started at the age of 1.5 years. From age 12 years, he experienced frequent episodes of typical absence status epilepticus (ASE) lasting 1‐2 hours with marked perioral myoclonia and moderate confusion. Initial therapy with carbamazepine was substituted by valproate because of worsening of the absence seizures. At the age of 17, the patient was admitted to our clinic with his usual, but long lasting ASE attack, accompanied by 2 generalized tonic‐clonic convulsions. ASE was confirmed with the EEG which showed continuous 3 Hz spike and wave paroxysms with occasional normal intervals of 1‐5 seconds. IV injection of clonazepam improved the clinical and EEG findings immediately. Video‐ EEG examination performed after a few weeks demonstrated typical absence seizures with perioral myoclonia. Based on the characteristics of seizure semiology, other clinical data and EEG findings, the patient was diagnosed as having the syndrome of “perioral myoclonia with absence seizures” described by Panayiotopoulos.
A clinical spectrum of the myoclonic manifestations associated with typical absences in childhood absence epilepsy. A video‐polygraphic study
Authors: Capovilla Giuseppe , Rubboli Guido , Beccaria Francesca , Lorenzetti Maria Elena , Montagnini Alessandra , Resi Cristina , Gardella Elena , Gambardella Antonio , Romeo Antonino , Tassinari Carlo Alberto
Published: March 2024

We investigated the electroclinical features of 12 patients with childhood absence epilepsy (CAE), presenting with typical absence seizures associated with myoclonic manifestations of the face or neck. All patients underwent repeated and prolonged split‐screen video‐polygraphic EEG recordings. The polygraphic recordings and clinical correlations of the absence seizures were analysed. All patients presented with multi‐quotidian, typical absence seizures. During the absences, the patients could show mild, rhythmic, myoclonic jerks involving facial areas (eyebrows, nostrils, perioral region, chin) or neck muscles (sternocleidomastoideus), with the same frequency as the spike‐wave complexes. Polygraphic tracings demonstrated that the myoclonias were correlated to the spike component. Clinically, all patients showed a benign course, with complete seizure control under antiepileptic treatment. In the follow‐up, 7 patients withdrew from treatment without relapse.

We conclude that all our patients showed an electroclinical picture consistent with CAE. The occurrence of myoclonic manifestations of the face or neck associated with the absences did not influence the benign course of their disease. The electroclinical features observed in our group of patients differentiates our cases both from epilepsy with myoclonic absences and from absences with perioral myoclonia (
with Video
).
Accessibility of spoken, written, and sign language in Landau‐Kleffner syndrome: a linguistic and functional MRI study
Authors: Sieratzki Jechil S. , Calvert Gemma A. , Brammer Michael , David Anthony , Woll Bencie
Published: March 2024

Landau‐Kleffner syndrome (LKS) is an acquired aphasia which begins in childhood and is thought to arise from an epileptic disorder within the auditory speech cortex. Although the epilepsy usually subsides at puberty, a severe communication impairment often persists. Here we report on a detailed study of a 26‐year old, left‐handed male, with onset of LKS at age 5 years, who is aphasic for English but who learned British Sign Language (BSL) at age 13. We have investigated his skills in different language modalities, recorded EEGs during wakefulness, sleep, and under conditions of auditory stimulation, measured brain stem auditory‐evoked potentials (BAEP), and performed functional MRI (fMRI) during a range of linguistic tasks. Our investigation demonstrated severe restrictions in comprehension and production of spoken English as well as lip‐reading, while reading was comparatively less impaired. BSL was by far the most efficient mode of communication. All EEG recordings were normal, while BAEP showed minor abnormalities. fMRI revealed: 1) powerful and extensive bilateral (R > L) activation of auditory cortices in response to heard speech, much stronger than when listening to music; 2) very little response to silent lip‐reading; 3) strong activation in the temporo‐parieto‐occipital association cortex, exclusively in the right hemisphere (RH), when viewing BSL signs. Analysis of these findings provides novel insights into the disturbance of the auditory speech cortex which underlies LKS and its diagnostic evaluation by fMRI, and underpins a strategy of restoring communication abilities in LKS through a natural sign language of the deaf (with Video).
Atypical evolution of Panayiotopoulos syndrome: A case report
Authors: Ferrie Colin D. , Koutroumanidis Michael , Rowlinson Shaun , Sanders Sue , Panayiotopoulos C.P.
Published: March 2024

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

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

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.