bluuebunny/crossref_metadata_2025 huggingface dataset:
Implementasi Penataan Toko Modern dan Pemberdayaan Pasar Tradisional
Authors: Ayunda Citra Lestari
Published: March 2024

Fenomena yang terjadi yakni banyak toko modern berdekatan dengan Pasar Bronggalan, Kelurahan Pacar Kembang, Kecamatan Tambaksari, Kota Surabaya. Tujuan penelitian adalah untuk menjelaskan secara aktual dan faktual mengenai Implementasi Pemberdayaan Pasar Modern dan Pasar Tradisional serta mengetahui faktor-faktor yang mendukung dan menghambat Implementasi Penataan Toko Modern dan Pemberdayaan Pasar Tradisional. Metode penelitian ini menggunakan penelitian kualitatif-studi kasus dengan satu variabel yaitu, Implementasi Pemberdayaan Pasar Modern dan Pasar Tradisional (Studi Kasus Alfamart, Alfamidi, Indomaret, dan Pasar Bronggalan, Keluahan Pacar Kembang, Kota Surabaya). 

            Dalam Implementasi Penataan Toko Modern dan Pemberdayaan Pasr Tradisional Studi Kasus di Alfamart, Alfamidi, Indomaret, dan Pasar Bronggalan, Kelurahan Pacar Kembang, Kecamatan Tambaksari, Kota Surabaya), dipengaruhi oleh enam faktor diantaranya, standar/ukuran kebijakan, komunikasi, sumber daya, disposisi, dan struktur birokrasi, dan kondisi sosial, ekonomi, dan politik. Faktor pendukung kebijakan implementasi pemberdayaan Alfamart, Alfamidi, Indomaret, dan Pasar Bronggalan yakni Koordinasi Dinas Perdagangan Kota Surabaya dan PD Pasar Surya Surabaya dan Pemerataan. Sedangkan faktor penghambatnya ialah kesadaran yang rendah dan pengawasan belum optimal
Analisis Konflik antara Pemerintah Provinsi Jawa Timur dan Pemerintah Kota Surabaya dalam Penggunaan Mobil PCR di Masa Pandemi Covid-19
Authors: Ahmed Indra Nur Ramadhan
Published: March 2024

The purpose of this thesis is to find out the causes behind the conflict between the East Java Provincial Government and the Surabaya City Government in the use of PCR cars, to determine the form of conflict that occurred between the East Java Provincial Government and the Surabaya City Government in the use of PCR cars, and also to know the conflict resolution taken by the East Java Provincial Government with the Surabaya City Government. This study uses a qualitative research method with a descriptive qualitative approach. The results of this study explain about 1.) The background of the cause of the conflict is the difference in the interests of the Surabaya City Government which wants its territory to immediately accelerate the detection of Covid-19 for the citizens of Surabaya City, while the interests of the East Java Provincial Government are to balance the needs in other areas that need assistance, and there was a miscoordination on the scheduling of this PCR car assistance. 2.) The forms of conflict patterns that occur are included in the type of interpersonal conflict which is a conflict between a group within the same organization, on the other hand the form of this conflict pattern includes latent and manifest conflicts because seen from the triggers of the conflict already visible or open, and has been known to many parties . 3.) Conflict resolution taken by the two parties uses a negotiation method with rescheduling for the purposes of the City of Surabaya
Pengaruh Kebijakan Perzela Terhadap Tingkat Kualitas Pelayanan Perizinan Online Di Dinas Penanaman Modal Dan Pelayanan Terpadu Satu Pintu Kabupaten Lamongan
Authors: Ahmad Hizbullah Mawardi Ubaidillah
Published: March 2024

Penelitian ini pada dasarnya memiliki tujuan diantaranya untuk mengetahui tingkat pelaksanaan kebijakan perizinan elektronik (PERZELA) DPMPTSP Kabupaten Lamongan menurut masyarakat, kemudian mengetahui tingkat kualitas pelayanan perizinan online di DPMPTSP Kabupaten Lamongan, dan untuk menganalisis pengaruh kebijakan PERZELA terhadap tingkat kualitas pelayanan perizinan online di DPMPTSP Kabupaten Lamongan. Metode penelitian yang digunakan dalam penelitian ini adalah mix method antara metode kualitatif dan kuantitatif, dengan menggunakan teknik analisis statistik deskriptif, statistik inferensial, dan deskriptif kualitatif. Hasil penelitian ini adalah: Pertama, masyarakat menilai skor rata-rata untuk variabel X yaitu pelaksanaan Kebijakan Perzela yaitu 4,1 dari total skor tertinggi 5,0 yang artinya masyarakat cukup percaya bahwa kebijakan Perzela telah berjalan dengan baik. Kedua, menurut masyarakat, skor rata-rata untuk variabel Y yaitu tingkat kualitas pelayanan perizinan online di DPMPTSP Lamongan yakni sebesar 4,0 dari total skor tertinggi sebesar 5,0 yang berarti bahwa masyarakat menilai tingkat kualitas pelayanan perijinan online dari DPMPTSP Kabupaten Lamongan berada pada level kualitas tinggi. Ketiga, terdapat pengaruh positif yang signifikan antara Kebijakan Perzela terhadap Kualitas Layanan Perizinan Online, namun dengan tingkat hubungan atau pengaruh yang rendah, dimana pengaruh Kebijakan Perzela (X) terhadap Kualitas Layanan Perizinan Online (Y) sebesar 6,5% sedangkan layanan 93,5% kualitas dipengaruhi oleh variabel lain yang tidak diteliti.
Transdisciplinary Study of Hedging Strategy in Evaluative Academic Discourse
Authors:   Saratov State University, Viktorova Elena
Published: March 2024

The article presents the research into hedges and hedging strategy realization in evaluative academic genres viewed from a transdisciplinary perspective. To this end, 64 dissertation reviews written by Russian male and female scholars in humanities (linguistics, literature studies, history) and sciences (physics, chemistry, medicine) were analyzed. Hedging is understood as a system of linguistic means, which perform a mitigating function associated with expressing vagueness, uncertainty, skepticism and doubt. Perlocutionary effects of hedging are connected with reducing the illocutionary strength of the writer's claims or risks of opposition, face saving, positive and negative politeness. The following classification of hedges is proposed: self-mentions, mitigation hedges, hedged performatives, evaluations of propositions and vagueness hedges. The results of the comparative quantitative and qualitative analyses reveal that hedging strategy is determined by a number of discursive and personal factors such as sphere of communication, speech genre, gender and professional experience of the writer. In dissertation reviews hedges show high frequency of occurrence, which can be explained by the public nature of reviews, their potential traumatic effect on the reader, the existing requirements for scientific data presentation in a tentative and indeterminate way, as well as high academic competence of reviewers. The research proves that in humanities hedges demonstrate a higher frequency rate than in sciences. At the same time, male reviewers reveal higher hedging frequency than female reviewers.
Oriental Text and the Subtext of Russian Paroemiology
Authors:   Saint Petersburg State University, Mokienko Valery , Nikitina Tatiana ,   Pskov State University
Published: March 2024

The article examines the geocultural image of the East, which has developed in Russian linguaculture. The purpose of the work is to systematize the conceptual and substantive characteristics of the Eastern world displayed by proverbs, to identify the axiological load of the Eastern-marked paroemias. The involvement in the study of the material of canonical proverbs, which accumulate centuries-old folk experience and fix the priorities of everyday consciousness, allows the authors to see the specifics of folk ideas about the Eastern world against the background of philosophical and aesthetic reconstructions of the image of the East, performed to date on the material of fiction of the 17 th – 20 th centuries. Transformations of proverbs and neoparoemias with an oriental cultural component, which function in the Internet space, make it possible to trace the dynamics of the geocultural image of the East, the specifics of its interpretation from the positions of the modern native speaker. This seems relevant in the context of the activation of the eastern vector of Russian state interests. As a result of the conducted research, conclusions are drawn about the deactualization of most of the paroemias based on phantom representations and real historical facts of interaction between Russians and the peoples of the East, trends in the use of oriental images in modern formation and transformation of phraseological units and paroemias are revealed. Special attention is paid to the paroemia The East is a delicate matter, which originates from cinematography. An unlimited range of its transformations is shown, thus forming a single paroemiological nest, which in the future can be described in an innovative paroemiological dictionary.
The Structure and Linguistic Representation of Sanctio in Old English and Old Russian Charters
Authors:   Tyumen State University, Belonogova Tatiana
Published: March 2024

The article identifies universal and specific structural and linguistic peculiarities of the sanctio (prohibitive formula / curse), an element of the main part of the Old English (Anglo-Saxon) and Old Russian charters. The analysis has shown that the prohibition of violating a contract or changing its terms and the punishment awaiting those who violate the contract are universal and integral components of the internal form of the sanctio of Anglo-Saxon and Old Russian charters. The motive for breach of a contract also belongs to the universal components of the sanctio but is optional and not presented in all documents. In both Anglo-Saxon and Old Russian charters, violation of the contract was identified with the commission of a sin and was prosecuted by religious sanctions. The distinctive feature of the Anglo-Saxon charters in Latin was the usage of Christian images alongside the images of ancient mythology resulting from the borrowing of ready-made formulas from Latin documents and the increasing influence of the Christian Church. Old Russian charters, apart from spiritual punishment, contained mentioning the secular punishment, either monetary or corporal. Syntactically, the sanctio were conditional constructions, characterized by prescriptive modality and reflecting some elements of colloquial speech (in Old Russian charters). The use of negatively coloured vocabulary (mainly in Anglo-Saxon charters) had to increase the emotional impact on the participants of the legal act. Appealing to the feeling of fear, which served as a means of maintaining law and order, the compilers of the charters threatened the potential transgressor with God's punishment. They called on the Lord, the Most Holy Theotokos (in Old Russian charters) and various saints to be heavenly judges. The linguistic peculiarities of sanctions are presented in their functional syncretism, blending of the spheres of legal, everyday, and religious communication.
Social Inclusion and Social Exclusion as a Tool for Reflecting Social Processes in the Lexical Neoformations of Youth English Slang
Authors:   Volgograd State University, Shamne Nikolay , Titarenko Sergey ,   Volgograd State University
Published: March 2024

The article discusses various approaches to defining the terminological base, the ambiguity of definitions, the lack of clear boundaries and signs of the concepts of social exclusion and social inclusion. The notions under study are characterized by way of their reference to communication process circumstances and participants, who are contrasted in their social status, cultural experience, ideological ideas and worldview to other social groups. The article analyzes slang neoformations with the concern for stylistically reduced register of communication as objectifying categories of social exclusion and social inclusion within the framework of youth discourse, which determine the developing vector of both society structure on the whole and social subsystems of the language. The novelty and relevance of the study lie in the use of linguocognitive analysis of authentic material, on the basis of which the mechanisms of exclusion and inclusion of slang lexical units are differentiated. The regularity in actualization of social exclusion or inclusion is understood in connection with the mechanisms and possibilities of using communicative practices in order to include social groups in social processes. These phenomena are reflected at the lexical level of the language and might contribute to various transformations of social consciousness, increasing language efficiency and its ability to respond to current challenges. It is concluded that modern youth slang is a specific language model that acts as an instrument of social exclusion or inclusion; in communicative practices, it is embodied through pejorative slang nominations thus strategically and tactically forming a negative image and antisocial behavior. The results of the research contribute to further study of the communicative nature of sociolinguistic phenomena, to comparative studies of the discursive construction of socially significant fragments of social reality, and the communicative manifestation of multidirectional social processes that determine the social structure and stratification of society.
Utilitarian Evaluation of Benefit in Bisubstantive Sentences
Authors:   State University of Education, Vatina Anastasia
Published: March 2024

The article describes the ways and means of expressing the evaluation of benefit in sentences that have two nouns in combination with a link in the predicative core. The place of utilitarian evaluation in the system of evaluative knowledge is determined. A scale of such evaluation "useful – harmless – indifferent – useless – harmful" is presented, and the identification of a neutral zone on it, formed by the value 'indifferent', is justified. The elements of the sentence with the utilitarian evaluation are identified, and the predominant representation of this evaluation in the link-substantive predicate is noted. The structure of the utilitarian evaluation of benefit in bisubstantive sentences is analyzed and described: the subject of evaluation, the subject of benefit or the subject of harm, the object of evaluation and the basis for evaluation, which coincides with the goal. The identification of the subject of harm is motivated. Productive grammatical means of expressing the utilitarian evaluation of benefit in bisubstantive sentences have been established. Cases of combining different subject types in this kind of sentences are characterized. Three groups of possible subjects of benefit were studied: a person (specific and abstract), a group of people (specific and abstract), animals as a special group in which the subjects of evaluation are always combined. The potential of utilitarian benefit evaluation compatibility with other types of evaluation is determined.
Electro‐clinical evolution of refractory non‐convulsive status epilepticus caused by West Nile virus encephalitis
Authors: Bagic Anto , Boudreau Eilis A. , Greenfield Jacquelyn , Sato Susumu
Published: March 2024

West Nile virus (WNV) has re‐emerged with a much wider geographic distribution and a higher incidence than ever. In spite of some recent reports on the neurological manifestations and EEG changes caused by WNV encephalitis, there are few data on the incidence of seizures, status epilepticus or post‐encephalitic epilepsy. There is also no systematic review of EEG changes caused by WNV encephalitis that is based on a large series of patients. Here, we review the pertinent literature, and report the electroclinical evolution and therapeutic complexity of a patient with WNV encephalitis who developed refractory, non‐convulsive status epilepticus.
Dissociation between
in vitro
and
in vivo
epileptogenicity in a rat model of cortical dysplasia
Authors: Kellinghaus Christoph , Möddel Gabriel , Shigeto Hiroshi , Ying Zhong , Jacobsson Berit , Gonzalez‐Martinez Jorge , Burrier Candice , Janigro Damir , Najm Imad M.
Published: March 2024

Objective.

Malformations of cortical development are frequent causes of human refractory epilepsy. The freeze‐lesion model in rats shows histopathological features similar to those found in human polymicrogyria. Previous studies reported
in vitro
hyperexcitability in this model, but
in vivo
epileptogenicity has not been confirmed.

Methods

Neocortical freeze lesions were induced in Sprague‐Dawley rat pups (n = 10) on postnatal day 0 or 1 (P0/P1). Sham‐operated animals served as controls (n = 10). On P60, animals were implanted with epidural electrodes for long‐term video‐EEG monitoring (4 weeks). The threshold for pentylenetetrazolinduced seizures was determined. Animals were sacrificed and brain sections processed for histological staining and
in vitro
electrophysiological recordings. Epileptiform field potential repetition rate, amplitude and integral were compared between slices containing a cortical freeze lesion, and slices from sham‐operated rats.

Results

No interictal spikes and no electrographic or clinical seizures occurred in either group. The median threshold for pentylenetetrazol‐induced seizures was 60 mg/kg for lesioned, and 45 mg/kg for control animals (difference not significant). No spontaneous epileptiform field potentials were recorded from either freeze‐lesion or control slices bathed in normal, artificial cerebrospinal fluid (ACSF). Upon omission of Mg
2+
from the bath, epileptiform field potentials were elicited that showed a significantly higher burst integral in the freeze lesion slices compared to control slices.

Conclusion

Neocortical freeze lesions induced in newborn rat pups show histological characteristics reminiscent of human cortical dysplasia. Brain slices containing neocortical freeze lesions display hyperexcitability
in vitro
, but the same lesion does not appear to show spontaneous epileptogenicity
in vivo
.
Depression in epilepsy: phenomenology, diagnosis and management
Authors: Seethalakshmi R. , Krishnamoorthy Ennapadam S.
Published: March 2024

1) Depression is a common and important accompaniment of epilepsy. 2) Depression in epilepsy is phenomenologically different from the usual forms of depression and it is essential that treating physicians assess for these varied forms as well. 3) Depression in epilepsy may be managed more effectively if the relationship to the ictus is better understood. 4) Other factors such as stressful life events, related or unrelated to epilepsy, may contribute to the depressive symptoms. 5) Antiepileptic drugs, particularly GABAergic agents such as vigabatrin, tiagabine, topiramate and phenobarbitone are depressogenic in nature. 6) The newer antidepressants, SSRIs such as sertraline, citalopram and paroxetine do not lower seizure threshold and can be safely used to treat depression in epileptic individuals. Fluoxetine may be avoided because of its longer half‐life.
Giant hypothalamic hamartoma and dacrystic seizures
Authors: López‐Laso Eduardo , González M. Elena Mateos , León Rafael Camino , González M. Dolores Jiménez , Rodríguez Javier Esparza
Published: March 2024

Ictal crying is a rare type of epileptic seizure associated with hypothalamic hamartoma and with other lesions such as tumours, vascular malformations, hippocampal sclerosis, or cerebral infarction. We describe the case of an infant with gelastic, dacrystic and other types of seizures associated with a giant hypothalamic hamartoma, and present a video sequence of dacrystic seizures. Dacrystic episodes presented in clusters at sleep onset, initially in the form of moaning followed by face‐flushing that rapidly evolved to crying, associated with a lateral and upper deviation of both eyeballs, along with clonic aspects of the eyelids. After a few seconds, the crying became less intense, she stared, and oro‐alimentary automatisms became prominent along with some slow horizontal movements of the eyes and the head. Following surgery, at the age of nine months, the gelastic seizures stopped, but dacrystic seizures persisted.

[
Published with video sequences
]
Treatment adherence and outcomes in the management of convulsive status epilepticus in the emergency room
Authors: Muayqil Taim , Rowe Brian H. , Ahmed S. Nizam
Published: March 2024

Purpose

According to published literature status epilepticus (SE) is associated with 7‐39% mortality. Timely management is one variable that potentially influences the outcome. We sought to review the process of acute management of SE at the University of Alberta Hospital and correlate outcome with adherence to a recommended treatment protocol.

Methods

We identified 86 patients 18 years of age or older who presented with convulsive SE to our emergency room between 2000 and 2004. We defined SE as continuous convulsive activity for 30 or more minutes or ≥ 2 convulsions with incomplete recovery in the interim. Information was collected pertaining to etiology, epidemiology, and management. We then reviewed the relationship of the treatment protocol in terms of mortality and morbidity.

Results

Forty five patients were included. There were 18 males and 27 females with a mean age of 45 years; 80% were known to have epilepsy. Subtherapeutic drug levels were found in the majority 60%; benzodiazepines (diazepam 81% and lorazepam 19%) were the first line agent in 93.3% mostly initiated by paramedics (EMS); 48.9% of patients required intubation and 26.7% required admission to intensive care. Four patients died. Control of convulsive SE was obtained sooner for patients in whom therapy was administered according to the recommended time frame (p ≤ 0.02).

Conclusion

The presence of strict treatment protocols for SE made readily available for the treating staff could potentially improve the outcome of patients. Despite the lack of standardized treatment protocols among various physicians, most patients are treated according to generally recommended sequence and time frames. Analysis of this data will help devise prospective treatment protocols.
Multimodality imaging for focus localization in pediatric pharmacoresistant epilepsy
Authors: Kurian M. , Spinelli L. , Delavelle J. , Willi J.P. , Velazquez M. , Chaves V. , Habre W. , Meagher‐Villemure K. , Roulet E. , Villemure J.G. , Seeck M.
Published: March 2024

Multiple structural and functional imaging modalities are available to localize the epileptogenic focus. In pre‐surgical evaluation of children with pharmacoresistant epilepsy, investigations with the maximum yield should be considered in order to reduce the complexity of the workup.

Objective

To determine the extent to which PET, ictal/interictal SPECT and its co‐registration with the patient's MRI contributes to correct localization of the epileptogenic focus, surgical intervention and to the post surgical outcome in paediatric patients.

Methods

The study population included children and adolescents with pharmacoresistant epilepsy (n = 50) who underwent preoperative evaluation, surgery and had postoperative follow‐up for at least 12 months. Outcome was measured by postoperative seizure frequency using Engel's classification.

Results

Thirty‐nine patients (78%) became completely seizure free after surgical intervention. The likelihood to benefit from surgical treatment was significantly higher if localization with more imaging modalities (MRI, PET, SPECT) were concordant with respect to the resected brain area (p < 0.01). Preoperative PET examination provided better localizing information in patients with extratemporal epilepsy and/or dysplastic lesions, whereas SPECT was found to be superior to PET in patients with temporal lobe epilepsy and/or tumors (p < 0.05). No significant difference was noted in the surgical outcome in younger or older age group, in children with or without special education needs.

Conclusion

In paediatric epilepsy pre‐surgical evaluation, the combined use of multiple functional imaging modalities for a precise localisation of the epileptogenic focus is worthwhile for both extratemporal and temporal lobe epilepsy, also when EEG and MRI alone are non‐contributive, given the potential benefit of complete postoperative seizure control.
“Tonic‐absence seizures”: an unusual seizure phenotype, but not necessarily in that order
Authors: Das Krishna B. , Boyd Stewart G.
Published: March 2024

Video telemetry in a 15‐year‐old boy with moderate learning difficulties revealed episodes of staring and cessation of activity, followed by sudden stiffening of the body for several seconds, abduction of the arms and a brief vocal utterance (“ugh”). Each episode lasts around 30 seconds, 3‐4 times/day despite treatment. The EEG showed generalized 3‐4Hz spike‐wave discharges during the “absence” period followed immediately by a run of fast polyspikes typical of a tonic seizure, terminating in a run of 1‐2 Hz sharp‐slow wave complexes. Although tonic‐absence seizures have been reported rarely, the clinical sequence appears to be reversed in this patient, with the occurrence of “absence‐tonic” attacks. We present video documentation of such attacks and discuss their nosology.

[Published with video sequences]
Status gelasticus associated with levetiracetam as add‐on treatment
Authors: Pustorino Giuseppina , Spano Maria , Sgro Domenica Lucia , Di Rosa Gabriella , Tricomi Giovanni , Bellantone Dorotea , Tortorella Gaetano
Published: March 2024

We report the case of a five‐year‐old girl, presenting with difficult‐to‐treat, symptomatic focal epilepsy, who developed status gelasticus following the introduction of levetiracetam as add‐on treatment to oxcarbazepine and diazepam. Gelastic seizures were documented by video‐EEG and were responsive to i.v. administration of diazepam. A possible causative role of levetiracetam is suggested. Specific susceptibility to some AEDs is also discussed, as this patient, at the age of four years, had presented an episode of non‐convulsive status epilepticus, following introduction of tiagabine, in association with vigabatrin and nitrazepam.

[Published with video sequences]
Temporal lobe dual pathology in malignant migrating partial seizures in infancy
Authors: Coppola Giangennaro , Operto Francesca Felicia , Auricchio Gianfranca , D'Amico Alessandra , Fortunato Delia , Pascotto Antonio
Published: March 2024

A child had the characteristic clinical and EEG pattern of migrating partial seizures in infancy with left temporal lobe atrophy, hippocampal sclerosis and cortical‐subcortical blurring. Seizures were drug‐resistant, with recurring episodes of status epilepticus. The child developed microcephaly with arrest of psychomotor development. Focal brain lesions, in the context of migrating partial seizures, have not been previously reported.

[Published with video sequences]
Ring chromosome 17 epilepsy may resemble that of ring chromosome 20 syndrome
Authors: Ricard‐Mousnier Brigitte , N'Guyen Sylvie , Dubas Frédéric , Pouplard François , Guichet Agnès
Published: March 2024

A four‐year‐old boy with ring chromosome 17 presenting with early‐onset, pharmacoresistant epilepsy underwent repeated 24‐hour video‐EEG monitoring and cytogenetic analyses, including fluorescentin situhybridization with telomeric and locus‐specific probes of chromosome 17. Epilepsy was characterized by nocturnal motor seizures and by prolonged diurnal electrical status epilepticus. The 46, XY, r (17) karyotype was observed in the majority of cell lines. Fluorescentin situhybridization revealed a deletion at the 17p telomere on the ring chromosome, whereas the 17q telomere and the Miller‐Dieker lissencephaly locus were undeleted. The epileptic syndrome observed in this case of ring chromosome 17 resembles the one described in the ring chromosome 20 syndrome, raising the question of the specificity and the pathogenesis of ring chromosome epileptic syndromes.

[Published with videosequences]
Familial generalized epilepsy in Bulgarian Roma
Authors: Tournev Ivailo , Royer Barbara , Szepetowski Pierre , Guergueltcheva Velina , Radionova Melania , Velizarova Reana , Yonova Mila , Lathrop Mark , Jamali Sarah , Petkov Radoslav , Raycheva Margarita , Genton Pierre
Published: March 2024

Aims

Gypsy communities constitute cultural and frequently inbred genetic isolates. Several genetic neurological disorders have been identified in these communities. Epilepsy appears as a fairly frequent medical condition among Bulgarian Gypsies, and many patients can be related to large pedigrees that may then be studied by conventional genetic linkage analyses.

Patients and methods

We identified two large Wallachian Gypsy families from the Plovdiv and Varna regions of Bulgaria, with detailed clinical questionnaires and examination, and EEG recordings for many. Genetic linkage analysis was performed using microsatellite markers spaced across the human genome.

Results

Although phenotypes were not always easy to identify, epilepsy appears in both families as a dominant, or pseudo‐dominant trait, with the characteristics of idiopathic generalized epilepsy with onset at various ages, with infrequent, generalized tonic‐clonic seizures, some associated with fever in childhood, but without sensitivity to fever in later life. While few markers yielded LOD scores > 2, no locus showed significant linkage, assuming autosomal dominant or recessive modes of inheritance.

Conclusion

Idiopathic generalized epilepsy, with a marked familial character, has not been reported to date in Bulgarian Gypsies. Both pedigrees studied here present with an identifiable epilepsy type inherited as a Mendelian trait. Despite the current lack of significant linkage, these families may constitute interesting ground for further genetic studies, on condition that more patients and families can be recruited.

[
Published with supplemental data on DVD
].
Self‐induced stretch syncope of adolescence: a video‐EEG documentation
Authors: Mazzuca Michel , Thomas Pierre
Published: March 2024

We present the first video‐EEG documentation, with ECG and EMG features, of stretch syncope of adolescence in a young, healthy 16‐year‐old boy. Stretch syncope of adolescence is a rarely reported, benign cause of fainting in young patients, which can be confused with epileptic seizures. In our patient, syncopes were self‐induced to avoid school. Dynamic transcranial Doppler showed evidence of blood flow decrease in both posterior cerebral arteries mimicking effects of a Valsalva manoeuvre. Dynamic angiogram of the vertebral arteries was normal. Hypotheses concerning the physiopathology are discussed.
[Published with video sequences]
.
Are dyslexia and dyscalculia associated with Rolandic epilepsy? A short report on ten Italian patients
Authors: Canavese Carlotta , Rigardetto Roberto , Viano Vilma , Vittorini Roberta , Bassi Bianca , Pieri Ilaria , Capizzi Giorgio
Published: March 2024

Rolandic epilepsy (RE) is the most common childhood epilepsy syndrome with a good, long‐term outcome. Nevertheless, some studies indicate that children with RE have more scholastic and neuropsychological problems than controls. The purpose of this study was to describe neuropsychological findings in a small group of Italian children with RE, focusing on dyslexia and dyscalculia. Possible correlations between these findings and the age‐at‐onset of seizures, duration of active epilepsy, frequency, type and localization of epileptic discharges were examined. Children affected by RE, aged nine to eleven years were selected from patients admitted to the outpatient service of our Clinic. They underwent cognitive evaluation, specific evaluation for dyslexia and dyscalculia, and awake and sleep EEG recordings. We found two patients out of the ten with dyscalculia, one of whom also had characteristics of dyslexia. This small study suggests that dyscalculia and dyslexia might be more frequent than expected in children with RE. No significant correlations between this finding and EEG, seizure‐frequency or age‐at‐onset of epilepsy were found in our patients.
Evaluation of susceptibility loci in an extended pedigree with idiopathic generalized epilepsy
Authors: Klein Karl Martin , Preisig‐Mμller Regina , Knake Susanne , Hamer Hajo M. , Oertel Wolfgang H. , Neubauer Bernd A. , Daut Jμrgen , Rosenow Felix
Published: March 2024

Purpose

Evaluation of the loci 2q36, 3q26, 5q34 and 14q23 in a German family with autosomal dominant idiopathic generalized epilepsy (IGE).

Methods

A linkage analysis was performed including 10 family members (six affected, three unaffected, one probably affected), for the loci 2q36, 3q26, 5q34 and 14q23. Subsequently, a sequence analysis of the inward rectifier potassium channel gene KCNJ13 at 2q37 was carried out.

Results

Suggestive linkage for IGE was found on 2q36‐37 at D2S2308 and D2S2193. No mutations were identified in the coding or 5'‐non‐coding regions of the exons of candidate gene
KCNJ13.

Conclusions

Our results corroborate former linkage findings on 2q36‐2q37 and warrant further investigation of additional candidate genes within this chromosomal area in IGE.
Benign neonatal sleep myoclonus
Authors: Marx Catherine , Masruha Marcelo Rodrigues , Garzon Eliana , Vilanova Luiz Celso Pereira
Published: March 2024

Benign neonatal sleep myoclonus is a non‐epileptic disorder. This phenomenon of the first weeks of life is characterized by erratic myoclonic jerks occurring only during sleep and with no electroencephalographic changes. It is not associated with perinatal complications, disappears spontaneously within two to four months, and it does not compromise future development. We illustrate with a video this relatively frequent condition, which is often misdiagnosed as epileptic in nature, and discuss the clinical characteristics and differential diagnosis.

[Published with video sequences]
.
Refractory and lethal status epilepticus in a patient with ring chromosome 20 syndrome
Authors: Jacobs Julia , Bernard Geneviève , Andermann Eva , Dubeau François , Andermann Frederick
Published: March 2024

Purpose

The only consistent symptom of ring chromosome 20 syndrome (r(20)) is severe, refractory epilepsy often associated with a characteristic, although not pathognomonic, EEG pattern. Patients suffer from severe seizures with accompanying cognitive decline and frequent episodes of nonconvulsive status epilepticus (SE). Other features of this rare disorder, such as dysmorphic changes, mental retardation and behavioral disturbances are variable. Because of the variability of the clinical presentation, some patients with r(20) undergo invasive investigations before being diagnosed.

Case study

We present the case of a young boy with no dysmorphic traits, who was only diagnosed with r (20) syndrome at the age of 13. His first seizure occurred at the age of four. Later seizures were of various types including non‐convulsive SE, with deterioration of the background EEG and severe cognitive decline. Despite multiple trials of anti‐epileptic medications, his seizures remained highly refractory, and he died as the result of an uncontrollable, prolonged SE, shortly after the diagnosis was made.

Discussion

Non‐convulsive SE is common in patients with r(20) syndrome and may be caused by a dysfunction in dopaminergic neurotransmission. However, until now, no case of lethal status epilepticus has been reported. This case report suggests that patients with unexplained refractory seizures and episodes of non‐convulsive SE should undergo genetic testing early in their disease, even in the absence of any morphologic features or dysmorphic traits suggestive of a chromosomal disease.
[Published with video sequences]
Pathways of seizure propagation from the temporal to the occipital lobe
Authors: Jacobs Julia , Dubeau François , Olivier André , Andermann Frederick
Published: March 2024

Propagation of ictal epileptic discharges influences the clinical appearance of seizures. Fast propagation from the occipital to temporal lobe has been well described, but until now the reverse direction of spread has not been emphasized. We describe two patients who experienced ictal propagation from temporal to occipital regions. One case presented with amaurosis during a seizure with temporal onset and temporal‐occipital spread. In the second, temporal‐occipital spread was documented during a seizure, which continued in the occipital lobe for six minutes. Depth electrode studies suggested the temporal ictal onset of seizures in both patients. Propagation from temporal to occipital lobe structures must be considered in the assessment of patients who have seizures with both temporal and occipital features. The propagation may have predictive value for their surgical outcome. The underlying anatomical structure might be the inferior longitudinal fasciculus.
Anatomo‐electro‐clinical correlations: the Marseille, France Case Report Case 02‐2008: MRI‐negative prefrontal epilepsy due to cortical dysplasia explored by stereoelectroencephalography (SEEG)
Authors: McGonigal Aileen , Gavaret Martine , Da Fonseca Agnès Trébuchon , Guye Maxime , Scavarda Didier , Villeneuve Nathalie , Régis Jean , Bartolomei Fabrice , Chauvel Patrick
Published: March 2024

[Case records of
Epileptic Disorders.
Anatomo‐electro‐clinical correlations. Case 02‐2008]

We report the case of a young boy presenting with pre‐frontal seizures including singing automatisms. There was no visible lesion on MRI, but following localisation using stereoelectroencephalography (SEEG), surgery revealed an underlying dysplastic lesion.
[Published with video sequences]
Source of cannabinoids: what is available, what is used, and where does it come from?
Authors: Specchio Nicola , Pietrafusa Nicola , Cross Helen J.
Published: March 2024

Cannabis sativa L
. is an ancient medicinal plant wherefrom over 120 cannabinoids are extracted. In the past two decades, there has been increasing interest in the therapeutic potential of cannabis‐based treatments for neurological disorders such as epilepsy, and there is now evidence for the medical use of cannabis and its effectiveness for a wide range of diseases. Cannabinoid treatments for pain and spasticity in patients with multiple sclerosis (Nabiximols) have been approved in several countries. Cannabidiol (CBD), in contrast to tetra‐hydro‐cannabidiol (THC), is not a controlled substance in the European Union, and over the years there has been increasing use of CBD‐enriched extracts and pure CBD for seizure disorders, particularly in children. No analytical controls are mandatory for CBD‐based products and a pronounced variability in CBD concentrations in commercialized CBD oil preparations has been identified. Randomized controlled trials of plant‐derived CBD for treatment of Lennox‐Gastaut syndrome (LGS) and Dravet syndrome (DS) have provided evidence of anti‐seizure effects, and in June 2018, CBD was approved by the Food and Drug Administration as an add‐on antiepileptic drug for patients two years of age and older with LGS or DS. Medical cannabis, with various ratios of CBD and THC and in different galenic preparations, is licensed in many European countries for several indications, and in July 2019, the European Medicines Agency also granted marketing authorisation for CBD in association with clobazam, for the treatment of seizures associated with LGS or DS. The purpose of this article is to review the availability of cannabis‐based products and cannabinoid‐based medicines, together with current regulations regarding indications in Europe (as of July 2019). The lack of approval by the central agencies, as well as social and political influences, have led to significant variation in usage between countries.
Cardioembolic acute cerebral micro‐infarcts in the context of atrial fibrillation after low‐dose intravenous infusion of lacosamide
Authors: Corbellini Álvaro Beltrán , Torre Paula Pérez , Hristova Velina Nedkova , Sanz Beatriz Zarza , García Adriana Celdrán de Castro , Jorge Fernando Rodriguez , García Juan Luís Chico , Díaz Paloma Parra , Catevilla Francisco Javier Buisan
Published: March 2024

Aims
: Lacosamide (LCM) is a well‐tolerated and increasingly used second‐generation AED, and side effects such as atrial fibrillation are rare and poorly characterized. Supported by a literature review, we share our experience of the management of the first reported case of cardioembolic cerebral infarcts in the context of
de novo
atrial fibrillation, which appeared following a 200‐mg intravenous infusion of LCM for the treatment of non‐convulsive status epilepticus.

Methods
: Case report and literature review using search items including “atrial fibrillation OR atrial flutter AND LCM” in the thesaurus of Medline.

Results
: We found three cases of atrial fibrillation/atrial flutter secondary to LCM, one following a 200‐mg intravenous infusion. In one patient, previous risk factors for atrial fibrillation were reported and another was started on warfarin; all required suspension of LCM for cessation of atrial fibrillation. Previous risk factors for atrial fibrillation in our patient were older age, male gender, obesity, hypertension, valvular disease, first‐degree atrioventricular block and left anterior fascicle block. Atrial fibrillation appeared at the end of the infusion and ceased after a loading dose of amiodarone and suspension of LCM. Apixaban was initiated indefinitely five days later, and MRI showed four acute silent infarctions.

Conclusions
: The appearance of atrial fibrillation has severe therapeutic and clinical implications and the use of LCM might be reconsidered within a context of increased predisposition to developing atrial fibrillation. If atrial fibrillation appears, the drug should be discontinued and anticoagulation should be considered according to embolic risk. Further investigation is needed in order to better categorize the risk profile of lacosamide regarding atrial fibrillation.
Video‐EEG and ictal SPECT in three patients with both epileptic and non‐epileptic seizures
Authors: Biraben Arnaud , Taussig Delphine , Bernard Anne‐Marie , Vignal Jean‐Pierre , Scarabin Jean‐Marie , Chauvel Patrick , Duncan Roderick
Published: March 2024

We report scalp EEG and ictal SPECT findings in epileptic (complex partial) and non‐epileptic seizures in three patients who experienced both types of event during presurgical investigation of medically intractable epilepsies. In all three patients, ictal SPECT showed localizing changes in cerebral blood flow during epileptic seizures, but showed no change during pseudoseizures. In two patients, the physical manifestations of the pseudoseizures were similar to those of the epileptic seizures, supporting the contention that physiological activation is unlikely to mimic ictal perfusion changes. In one patient, the EEG recording was rendered difficult to interpret by muscle artefact, while SPECT was clear and showed no change. SPECT is not a primary tool for diagnosis of pseudoseizures, but when patients undergoing presurgical investigation are injected during pseudoseizures, then SPECT is unlikely to show misleading perfusion changes due to activation effects, and may aid diagnosis where there is muscle artefact on EEG.
Epilepsy and perisylvian lipoma/cortical dysplasia complex
Authors: Guye Maxime , Gastaut Jean‐Louis , Bartolomei Fabrice
Published: March 2024

Intracranial lipomas are congenital malformations composed of mature adipocytes. They are usually located in the midline, particularly in the pericallosal region, a hemispheric location accounting for only 3 to 7% of cases. Review of the literature found 21 previous cases of hemispheric lipoma. Although hemispheric cerebral lipomas are rare, association with epilepsy appears to be frequent.

We have recently studied two patients in whom epilepsy was the first clinical manifestation of hemispheric cerebral lipoma in the sylvian region. The patients presented with simple motor partial seizures as the first manifestation of the lesion. Neurological examination was normal. MRI disclosed in both cases a lesion involving the sylvian fissure with characteristics of the lipid signal. MRI also demonstrated abnormalities involving the cerebral cortex in the vicinity of the lesion (pachygyria‐like aspect). Partial excision of the lesion was achieved in one patient but was followed by a worsening of seizures and neurological condition (hemiparesis). According to the literature, the prognosis for epilepsy in patients with hemispheric lipoma appears good. Several other arguments support non‐surgical management: the lesion is benign and can be identified with a high degree of certainty by imaging; surgery is technically difficult due to adherence to adjacent vascular and cerebral structures and hypervascularity; location near functional brain tissue increases the risk of postoperative sequelae. In addition, mechanisms of epilepsy probably involve vascular and cortical dysplasic abnormalities. In consideration of the complexity of the lesion, hemispheric lipomas are more appropriately classified with localized cortical malformations rather than as simple extracerebral malformations.
Focal seizures
versus
focal epilepsy in infancy: a challenging distinction
Authors: Gérard F. , Kaminska A. , Plouin P. , Echenne B. , Dulac O.
Published: March 2024

Content available: Video
Reading epilepsy in a patient with previous idiopathic focal epilepsy with centrotemporal spikes
Authors: Valenti Maria Paola , Tinuper Paolo , Cerullo Angelina , Carcangiu Roberta , Marini Carla
Published: March 2024

Rationale

Reading epilepsy (RE) is a form of reflex epilepsy currently classified as an idiopathic localization‐related epilepsy (ILAE, 1989).

Methods

We describe a 30 year‐old right‐handed male who suffered since the age of 8 from nocturnal partial motor seizures. Clinical features were typical of BECT. We reviewed the EEG recorded at that time which showed centrotemporal spike and waves. He was seizure‐free from the age of 12 to the age of 17 when seizures evoked only by reading appeared. No other stimuli provoked seizures. Neurological and neuroradiological (CT and MR) investigations were normal. Baseline video‐polygraphic EEG recordings were normal while reading aloud provoked myoclonic jerks in the facial muscles related to bilateral spike and wave discharges. Therapy with carbamazepine and valproic acid strongly reduced seizure frequency.

Conclusion

Recent papers have debated the difficulties in classifying RE among the generalized or focal syndromes. Literature reports describe an association with RE and juvenile myoclonic epilepsy, supporting the hypothesis of an idiopathic generalized form. We report the first documented case with a clear‐cut idiopathic localization‐related epilepsy evolving to a primary reading epilepsy.
Psychosocial functioning in chronic epilepsy: relation to hippocampal volume and histopathological findings
Authors: Seeck M. , Lazeyras F. , Murphy K. , Naimi A. , Pizzolatto G.P. , de Tribolet N. , Delavelle J. , Villemure J.‐G. , Landis T.
Published: March 2024

MRI volumetric measurements of the ipsi‐ and contralateral hippocampal volume (HV) were performed in a group of 37 patients with lesional and non‐lesionai temporal (TLE), and extratemporal lobe (ETLE) lobe epilepsy. Twenty‐six patients underwent surgery and the resected tissues subjected to histopathological examination. Psychosocial scores were calculated on the basis of employment, partnership/family status and presence of a positive psychiatric history. Poorest values were obtained for the group with right lesional and non‐lesional TLE (RTLE, p < 0.001). In this group, significant positive correlations between psychosocial functioning and the left hippocampal volume or age of onset were observed. We found that the left hippocampal volume in RTLE was smaller with increasing epilepsy duration. We conclude that patients with RTLE represent a particular group with regard to the structural and psychosocial consequences of chronic epilepsy. With regard to histopathological analysis, no particular type of lesion was identified as the major determinant of the results described above.
Can protrusion of the tongue stop seizures in Rolandic Epilepsy?
Authors: Veggiotti P. , Beccaria F. , Gatti A. , Papalia G. , Resi C. , Lanzi G.
Published: March 2024

We report the case of a child with benign partial epilepsy with centro‐temporal spikes (BECT) in whom protrusion of the tongue stopped the interictal abnormalities, and describe the polygraphic EEG recording of a seizure which terminated upon voluntary protrusion of the tongue.

We mention the close link between the post‐central (somatosensory) cortex and pre‐central (motor) cortex, and how the primary sensory area has direct access to the motor cortex. We also examine how a tactile stimulus may provoke the inhibition of an epileptic discharge.