Article
Behavioral Sciences
Dong Ah Lee, Junghae Ko, Ho-Joon Lee, Hyung Chan Kim, Bong Soo Park, Sihyung Park, Il Hwan Kim, Jin Han Park, Yoo Jin Lee, Kang Min Park
Summary: The study found no significant volume differences in the nuclei of the amygdala and hippocampal subfields between patients with JME and healthy controls. However, there were significant differences in the global network, with decreased mean clustering coefficient in JME patients. Specific regions in the hippocampal subfields also showed increased betweenness centrality in patients with JME compared to healthy controls.
BRAIN AND BEHAVIOR
(2021)
Article
Clinical Neurology
Magnus Spangsberg Boesen, Malene Landbo Borresen, Soren Kirchhoff Christensen, Amalie Wandel Klein-Petersen, Sahla El Mahdaoui, Malini Vendela Sagar, Emilie Schou, Anna Korsgaard Eltvedt, Maria Jose Miranda, Alfred Peter Born, Peter Vilhelm Uldall, Lau Caspar Thygesen, Melita Cacic Hribljan
Summary: The study found that juvenile absence epilepsy and juvenile myoclonic epilepsy had slightly poorer school performance compared to the control group, with absence epilepsy having an increased risk for special education needs. Both types of epilepsy patients had higher use of sleep medication.
JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Edouard Hirsch, Jacqueline French, Ingrid E. Scheffer, Alicia Bogacz, Taoufik Alsaadi, Michael R. Sperling, Fatema Abdulla, Sameer M. Zuberi, Eugen Trinka, Nicola Specchio, Ernest Somerville, Pauline Samia, Kate Riney, Rima Nabbout, Satish Jain, Jo M. Wilmshurst, Stephane Auvin, Samuel Wiebe, Emilio Perucca, Solomon L. Moshe, Paolo Tinuper, Elaine C. Wirrell
Summary: This paper aims to define the four syndromes comprising the idiopathic generalized epilepsies (IGEs) and provides updated diagnostic criteria. For patients who do not meet the criteria for these syndromes but have generalized seizure types, a classification is also provided. Recognizing these syndromes as a special grouping helps determine prognosis and treatment implications.
Article
Neurosciences
Anatolie Vataman, Dumitru Ciolac, Vitalie Chiosa, Daniela Aftene, Pavel Leahu, Yaroslav Winter, Stanislav A. Groppa, Gabriel Gonzalez-Escamilla, Muthuraman Muthuraman, Sergiu Groppa
Summary: In this study, the temporal and spatial organization of functional networks and their dynamic properties in juvenile myoclonic epilepsy (JME) patients were characterized using high-density EEG (hdEEG) recordings and MRI data. It was found that the flexibility and controllability of network modules exhibit antagonistic dynamics during SWD generation in JME. The flexibility and controllability of the fronto-temporal module were found to be related to seizure frequency and cognitive performance in JME patients. These findings have implications for the diagnosis and treatment of JME.
NEUROBIOLOGY OF DISEASE
(2023)
Article
Clinical Neurology
Eugen Trinka, Taoufik Alsaadi, Hiroko Goji, Taketoshi Maehara, Satoru Takahashi, Julia Jacobs, Rosaria Renna, Francisco Jose Gil-Lopez, Rob McMurray, Ricardo Sainz-Fuertes, Vicente Villanueva
Summary: This study aimed to evaluate the effectiveness and tolerability of PER in the treatment of idiopathic generalized epilepsy under real-world conditions. The results showed that PER had good effectiveness and tolerability in people with idiopathic generalized epilepsy in clinical practice, consistent with clinical trial evidence.
Article
Clinical Neurology
Katherine Nickels, Eric H. Kossoff, Krista Eschbach, Charuta Joshi
Summary: This study presented the largest cohort of patients with EMAS to date, revealing that global developmental delay was present in nearly half of the children during the course of the disease. Most children experienced seizures after the age of 2, with EEG showing abnormal findings in the majority. Diet therapy was found to be significantly more effective than the first-line antiseizure drugs in achieving seizure reduction.
Article
Health Care Sciences & Services
Siew-Na Lim, Tony Wu, Wei-En Johnny Tseng, Chun-Wei Chang, Hsiang-Yao Hsieh, Mei-Yun Cheng, Hsing- Chiang, Chih-Hong Lee, Wey-Ran Lin, Chun-Jing Liu
Summary: This study aimed to investigate the clinical and social features of patients with juvenile myoclonic epilepsy (JME) and determine the factors associated with outcomes. The findings showed that despite optimal treatment with antiseizure medications, a considerable proportion of JME patients did not achieve seizure freedom. The presence of epileptiform discharges and seizures during sleep were significantly associated with worse seizure outcomes, and seizure-free patients had a higher employment rate compared to those with ongoing seizures.
Article
Clinical Neurology
Lorenzo Caciagli, Corey Ratcliffe, Fenglai Xiao, Louis A. A. van Graan, Karin Trimmel, Christian Vollmar, Maria Centeno, John S. S. Duncan, Pamela J. J. Thompson, Sallie Baxendale, Matthias J. J. Koepp, Britta Wandschneider
Summary: The objective of this study was to characterize the cognitive profile of juvenile absence epilepsy (JAE), identify familial cognitive traits, determine the clinical significance of JAE-associated cognitive traits, compare JAE with juvenile myoclonic epilepsy (JME) to identify shared or syndrome-specific cognitive traits, and identify relationships between cognitive abilities and clinical characteristics.
Review
Neurosciences
Sara Bernardi, Federica Gemignani, Maria Marchese
Summary: Progressive myoclonic epilepsies (PMEs) are rare neurodegenerative diseases characterized by myoclonus, seizures, and neurological deterioration. The involvement of the cerebellar cortex and the loss of Purkinje cells (PCs) in PMEs are associated with motor impairments and epilepsy. This review focuses on the role of PCs in epilepsy and particularly highlights their involvement in seizure phenotype in neuronal ceroid lipofuscinosis (NCL).
NEUROBIOLOGY OF DISEASE
(2023)
Article
Behavioral Sciences
Laura Canafoglia, Ilaria Vigano, Edoardo Ferlazzo, Elisa Visani, Tiziana Granata, Aglaia Vignoli, Francesca Ragona, Sara Gasparini, Umberto Aguglia, Maria Paola Canevini, Giulia Varotto, Ferruccio Panzica, Silvana Franceschetti
Summary: The study compared EEG signals of drug-naive patients with juvenile myoclonic epilepsy (JME) and juvenile absence epilepsy (JAE) and found different network dysfunctions, potentially leading to distinct presentations of the two syndromes. JME showed regional network changes in alpha and beta bands, while JAE exhibited reduced Global/Local Efficiency and Clustering Coefficient in the gamma band.
EPILEPSY & BEHAVIOR
(2021)
Article
Clinical Neurology
Arife Cimen Atalar, Bengi Gul Turk, Esme Ekizoglu, Duygu Kurt Gok, Betul Baykan, Aynur Ozge, Semih Ayta, Fusun Ferda Erdogan, Seher Naz Yeni, Bahar Tasdelen, Sibel K. Velioglu, Zuhal Yapici, Ipek Midi, Serap Saygi, Ulufer Celebi, Elif Sarica Darol, Kadriye Agan, Senem Ayca, Sibel Gazioglu, Zeynep Vildan Okudan, Nermin Gorkem Sirin, Nerses Bebek, Nese Dericioglu, Ilknur Guclu Altun, Ayse Destina Yalcin, Reyhan Surmeli, Oguz Osman Erdinc, Abidin Erdal, Demet Ilhan Algin, Gulnihal Kutlu, Semai Bek, Yuksel Erdal, Akcay Ovunc Ozon, Aylin Reyhani, Baburhan Guldiken, Baris Baklan, Bulent Oguz Genc, Ebru Aykutlu Altindag, Gokcen Karahan, Guray Koc, Handan Misirli, Ibrahim Oztura, Kezban Aslan-Kara, Merve Melodi Cakar, Nur Turkmen, Onur Bulut, Omer Karadas, Ozlem Kesim Sahin, Sevgi Ferik, Mehmet Taylan Pekoz, Pinar Topaloglu, Sibel Ustun Ozek, Ulkuhan Duzgun, Vildan Yayla, Yasemin Gomceli, Zeynep Unlusoy Acar
Summary: The study found a prominent link between headache and epilepsy in patients with idiopathic/genetic epilepsy, and patients were clustered based on their headache and epilepsy features, revealing two distinct groups.
Article
Clinical Neurology
Ali A. Asadi-Pooya, Mahtab Rostamihosseinkhani, Mohsen Farazdaghi
Summary: This study investigated the seizure and social outcomes of patients with juvenile myoclonic epilepsy (JME) and found that JME at tertiary referral centers is not as benign as previously suggested. The employment status of JME patients is significantly worse compared to the general population. However, most JME patients have marital status and educational levels comparable to the general public.
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY
(2022)
Article
Clinical Neurology
Marinho A. Lopes, Dominik Krzeminski, Khalid Hamandi, Krish D. Singh, Naoki Masuda, John R. Terry, Jiaxiang Zhang
Summary: The study investigated the applicability of the BNI framework to resting-state MEG in patients with JME, finding that patients with JME had a higher propensity to generate seizures compared to healthy controls. The BNI framework was effective in differentiating between epilepsy patients and healthy controls.
CLINICAL NEUROPHYSIOLOGY
(2021)
Article
Clinical Neurology
Michelle E. Ernst, Evan H. Baugh, Amanda Thomas, Louise Bier, Natalie Lippa, Nicholas Stong, Maureen S. Mulhern, Sulagna Kushary, Cigdem I. Akman, Erin L. Heinzen, Raymond Yeh, Weimin Bi, Neil A. Hanchard, Lindsay C. Burrage, Magalie S. Leduc, Josephine S. C. Chong, Renee Bend, Michael J. Lyons, Jennifer A. Lee, Pim Suwannarat, Eva Brilstra, Marleen Simon, Marije Koopmans, Ellen van Binsbergen, Daniel Groepper, Julie Fleischer, Caroline Nava, Boris Keren, Cyril Mignot, Sophie Mathieu, Grazia M. S. Mancini, Suneeta Madan-Khetarpal, Elena M. Infante, Judith Bluvstein, Andrea Seeley, Kristine Bachman, Eric W. Klee, Laura E. Schultz-Rogers, Linda Hasadsri, Sarah Barnett, Marissa S. Ellingson, Matthew J. Ferber, Vinodh Narayanan, Keri Ramsey, Anita Rauch, Pascal Joset, Katharina Steindl, Theodore Sheehan, Annapurna Poduri, Alejandra Vasquez, Claudia Ruivenkamp, Susan M. White, Lynn Pais, Kristin G. Monaghan, David B. Goldstein, Tristan T. Sands, Vimla Aggarwal
Summary: CSNK2B has been identified as a disease gene for neurodevelopmental disability (NDD) and epilepsy. This study presented 25 new patients with CSNK2B variants, showing a wide spectrum of phenotypic severity, ranging from early normal development with pharmacoresponsive seizures to profound intellectual disability with intractable epilepsy and recurrent refractory status epilepticus. The findings suggest that CSNK2B should be considered in the diagnostic evaluation of patients with a broad range of NDD with treatable or intractable seizures.
Article
Neurosciences
Laiyang Ma, Guangyao Liu, Pengfei Zhang, Jun Wang, Wenjing Huang, Yanli Jiang, Yu Zheng, Na Han, Zhe Zhang, Jing Zhang
Summary: This study found significant differences in connectivity between the cerebrum and cerebellum in JME patients compared to healthy controls, with disruptions primarily in the direction of EC from the cerebellum to the cerebrum. The negative correlation between EC and disease severity may provide insights into the underlying neural circuit mechanisms in JME.
Article
Clinical Neurology
Esme Ekizoglu, Hasim Gezegen, Pinar Yalinay Dikmen, Elif Kocasoy Orhan, Mustafa Ertas, Betul Baykan
Summary: Headache is a common adverse event following COVID-19 vaccination, with 30.6% of healthcare workers affected, predominantly females with pre-existing primary headaches, thyroid disorders, headache during COVID-19, or headache related to the influenza vaccine.
Article
Clinical Neurology
Ayse Nur Ozdag Acarli, Ayse Deniz Elmali, Nermin Gorkem Sirin, Betul Baykan, Nerses Bebek
Summary: Ictal blinking is a useful lateralizing sign in focal seizures, especially when it is unilateral or asymmetrical. It often appears early in the seizures and is associated with fronto-temporal seizure onset. However, symmetrical blinking does not seem to be valuable in lateralization and localization of focal seizures. Further studies using invasive recordings and periocular electrodes are needed to evaluate the value of blinking in lateralization and localization.
CLINICAL EEG AND NEUROSCIENCE
(2023)
Article
Clinical Neurology
Arife Cimen Atalar, Bengi Gul Turk, Esme Ekizoglu, Duygu Kurt Gok, Betul Baykan, Aynur Ozge, Semih Ayta, Fusun Ferda Erdogan, Seher Naz Yeni, Bahar Tasdelen, Sibel K. Velioglu, Zuhal Yapici, Ipek Midi, Serap Saygi, Ulufer Celebi, Elif Sarica Darol, Kadriye Agan, Senem Ayca, Sibel Gazioglu, Zeynep Vildan Okudan, Nermin Gorkem Sirin, Nerses Bebek, Nese Dericioglu, Ilknur Guclu Altun, Ayse Destina Yalcin, Reyhan Surmeli, Oguz Osman Erdinc, Abidin Erdal, Demet Ilhan Algin, Gulnihal Kutlu, Semai Bek, Yuksel Erdal, Akcay Ovunc Ozon, Aylin Reyhani, Baburhan Guldiken, Baris Baklan, Bulent Oguz Genc, Ebru Aykutlu Altindag, Gokcen Karahan, Guray Koc, Handan Misirli, Ibrahim Oztura, Kezban Aslan-Kara, Merve Melodi Cakar, Nur Turkmen, Onur Bulut, Omer Karadas, Ozlem Kesim Sahin, Sevgi Ferik, Mehmet Taylan Pekoz, Pinar Topaloglu, Sibel Ustun Ozek, Ulkuhan Duzgun, Vildan Yayla, Yasemin Gomceli, Zeynep Unlusoy Acar
Summary: The study found a prominent link between headache and epilepsy in patients with idiopathic/genetic epilepsy, and patients were clustered based on their headache and epilepsy features, revealing two distinct groups.
Article
Clinical Neurology
Jennifer A. Frontera, Arina A. Tamborska, Mohamed F. Doheim, David Garcia-Azorin, Hasim Gezegen, Alla Guekht, Abdul Hanif Khan Yusof Khan, Michele Santacatterina, James Sejvar, Kiran T. Thakur, Erica Westenberg, Andrea S. Winkler, Ettore Beghi
Summary: This study analyzed data collected from the US Vaccine Adverse Event Reporting System from January 1, 2021 to June 14, 2021. The study found that the rates of neurological events following COVID vaccination were rare, while the rates of neurological complications following acute COVID infection were significantly higher.
ANNALS OF NEUROLOGY
(2022)
Review
Clinical Neurology
Burcu Atasu, Ayse Nur Ozdag Acarli, Basar Bilgic, Betul Baykan, Erol Demir, Yasemin Ozluk, Aydin Turkmen, Ann-Kathrin Hauser, Gamze Guven, Hasmet Hanagasi, Hakan Gurvit, Murat Emre, Thomas Gasser, Ebba Lohmann
Summary: This study shows that biallelic pathogenic variants in the SCARB2 gene can cause a spectrum of features associated with AMRF. Common features include myoclonus, ataxia, seizures, dysarthria, tremor, and renal impairment, while uncommon features involve peripheral neuropathy, hearing loss, and cognitive impairment. The C terminal localization of the pathogenic variant significantly affects the age at onset of the disease.
Article
Clinical Neurology
David Garcia-Azorin, Betul Baykan, Ettore Beghi, Mohamed F. Doheim, Cesar Fernandez-de-las-Penas, Hasim Gezegen, Alla Guekht, Fan Kee Hoo, Michele Santacatterina, James Sejvar, Arina A. Tamborska, Kiran T. Thakur, Erica Westenberg, Andrea S. Winkler, Jennifer A. Frontera
Summary: Delayed-onset headache following COVID-19 vaccination may be a specific feature of cerebrovascular events. Analysis showed that headache occurring 3 days after vaccination may serve as an accurate diagnostic biomarker for the occurrence of concomitant cerebrovascular events.
Article
Clinical Neurology
Cem Ismail Kucukali, Busra Sengul, Duygu Gezen-Ak, Erdinc Dursun, Ece Erdag, Gurler Akpinar, Murat Kasap, Zerrin Karaaslan, Nermin Gorkem Sirin, Pinar Tekturk, Betul Baykan, Erdem Tuzun
Summary: A novel neuronal surface antibody to Kv5.1 was identified in patients with treatment-resistant epilepsy of unknown cause, suggesting a potential autoimmune etiology that requires further investigation.
Article
Clinical Neurology
Ebru Nur Vanli Yavuz, Ebru Altindag, Erdem Tuzun, Betul Baykan
Summary: This survey study on the attitudes of neurologists towards autoimmune epilepsy (AE) suggests that there is a relatively high level of awareness and understanding of AE, but there are still some deficiencies. Participants reported a lack of satisfactory guidelines for diagnosis and treatment, as well as widespread laboratory support. In addition, neurologists with less experience and those working outside of training hospitals tend to seek more consultation from experienced clinicians and conduct more detailed investigations.
NEUROLOGICAL SCIENCES
(2022)
Article
Clinical Neurology
Emanuele Cerulli Irelli, Enrico Cocchi, Georgia Ramantani, Roberto H. Caraballo, Loretta Giuliano, Tulay Yilmaz, Alessandra Morano, Eleni Panagiotakaki, Francesca F. Operto, Beatriz Gonzalez Giraldez, Katri Silvennoinen, Sara Casciato, Marion Comajuan, Simona Balestrini, Francesco Fortunato, Antonietta Coppola, Giancarlo Di Gennaro, Angelo Labate, Vito Sofia, Gerhard J. Kluger, Dorothee G. A. Kasteleijn-Nolst Trenite, Antonio Gambardella, Betul Baykan, Sanjay M. Sisodiya, Alexis Arzimanoglou, Pasquale Striano, Carlo Di Bonaventura
Summary: This study investigated electrodinical endophenotypes and long-term seizure outcome in patients with eyelid myoclonia with absences (EMA). The results showed that early epilepsy onset was the most relevant prognostic factor for poor treatment response. Additionally, two distinct endophenotypes were identified, EMA-plus and EMA-only, which differed in terms of remission rates and cognitive outcomes.
Article
Clinical Neurology
Kubra Yeni, Zeliha Tulek, Arif Ozer, Aysel Cavusoglu, Gorkem Sirin Inan, Betul Baykan, Nerses Bebek
Summary: This study examined the impact of COVID-19 fear on the quality of life in epilepsy patients, and found that fear increased anxiety and depression, ultimately leading to a decrease in quality of life.
Article
Medicine, Research & Experimental
Serkan Aksu, Tuba Cerrahoglu Sirin, Buse Rahime Hasirci Bayir, Cagri Ulukan, Ahmet Zihni Soyata, Adnan Kurt, Sacit Karamursel, Betul Baykan
Summary: This study found that long-term extended transcranial direct current stimulation (tDCS) can effectively alleviate allodynia symptoms in migraine patients and meet the goals of prophylactic treatment.
Article
Clinical Neurology
Emel Oguz-Akarsu, Baris Salman, Sibel Ugur-Iseri, Betul Baykan
Summary: This article reports a case of a 20-year-old woman with long-lasting bilateral paroxysmal high-voltage slow waves on EEG, which were interrupted by sudden verbal stimuli. Whole exome sequencing revealed potential genetic etiology. This rare manifestation of subclinical periodic discharges terminated by verbal stimuli deserves particular attention.
CLINICAL EEG AND NEUROSCIENCE
(2023)
Article
Clinical Neurology
Emanuele Cerulli Irelli, Enrico Cocchi, Georgia Ramantani, Antonella Riva, Roberto Horacio Caraballo, Alessandra Morano, Loretta Giuliano, Tulay Yilmaz, Eleni Panagiotakaki, Francesca F. Operto, Beatriz Gonzalez Giraldez, Simona Balestrini, Katri Silvennoinen, Sara Casciato, Marion Comajuan, Francesco Fortunato, Anna Teresa Giallonardo, Rimma Gamirova, Antonietta Coppola, Giancarlo Di Gennaro, Angelo Labate, Vito Sofia, Gerhard Josef Kluger, Antonio Gambardella, Dorothee G. A. Kasteleijn-NolstTrenite, Betul Baykan, Sanjay M. Sisodiya, Alexis Arzimanoglou, Pasquale Striano, Carlo Di Bonaventura
Summary: Based on age at epilepsy onset (AEO), this study identified three subtypes of objective epilepsy with eyelid myoclonia (EEM) and analyzed their distinct clinical features. Early onset EEM was associated with higher rates of intellectual disability, antiseizure medication refractoriness, and psychiatric comorbidities, while late onset EEM had the highest proportion of myoclonia involving body regions other than eyelids and generalized tonic-clonic seizures. Intermediate onset EEM had the lowest observed rate of additional findings. Family history of EEM was more frequent in the subtypes with intermediate and late onset. Patients with body-MYO showed higher rates of migraine and generalized tonic-clonic seizures.
Article
Clinical Neurology
Betul Baykan, Silvana Franceschetti, Laura Canafoglia, Gianpiero L. Cavalleri, Roberto Michelucci, Ingrid E. Scheffer
Summary: Familial adult myoclonic epilepsy (FAME) is a under-recognized disorder characterized by cortical myoclonus, generalized tonic-clonic seizures, and additional clinical symptoms, which vary depending on the FAME subtype. FAME is caused by pentanucleotide repeat expansions of intronic TTTCA/TTTTA in different genes.
Article
Clinical Neurology
Irem Ilgezdi Kaya, Betuel Baykan
Summary: The ILAE Task Force on Classification released a report in 2015 to clarify the classification of status epilepticus. Non-convulsive status epilepticus (NCSE) was defined as SE without prominent motor symptoms, with or without coma. The increasing evidence in patients, who do not meet the Salzburg Consensus Criteria for NCSE diagnosis of 'NCSE' or 'possible NCSE', but whose clinical and electrophysiological features are still suspicious for NCSE, may pave the way for developing more comprehensive criteria. Therefore, we present here the 'generalized non-reactive alpha activity' in the electroencephalogram (EEG) of an elderly patient with no known epilepsy before, who presented with acute confusional state of unexplained cause, which we suspected as NCSE and managed accordingly with success. Considering that 'time is brain', early and correct NCSE diagnosis is critical and the NCSE EEG criteria should be more inclusive for the patients in the 'grey zone like the one presented here with 'generalized non-reactive alpha activity'.
ARCHIVES OF EPILEPSY
(2023)
