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Epileptic Disorders: International Epilepsy Journal with Videotape

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https://www.readbyqxmd.com/read/29313492/rufinamide-for-the-treatment-of-lennox-gastaut-syndrome-evidence-from-clinical-trials-and-clinical-practice
#1
Pasquale Striano, Rob McMurray, Estevo Santamarina, Mercè Falip
Rufinamide was granted orphan drug status in 2004 for the adjunctive treatment of seizures associated with Lennox-Gastaut syndrome in patients aged ≥4 years, and was subsequently approved for this indication in several countries, including Europe and the United States. Structurally unrelated to other antiepileptic drugs, rufinamide is thought to act primarily by prolonging the inactivation phase of voltage-gated sodium channels. Rufinamide was approved on the basis of an international, randomised, placebo-controlled Phase III trial, conducted in 138 patients with Lennox-Gastaut syndrome, which demonstrated its favourable tolerability profile and efficacy in significantly reducing the frequency of drop attacks and total seizures, compared with placebo...
January 9, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29265005/facing-the-hidden-wall-in-mesial-extratemporal-lobe-epilepsy
#2
Tom Theys, Thomas Decramer, Wim Van Paesschen, Xavier De Tiège, Lorella Minotti, Marec von Lehe, Stephan Chabardès, Philippe Kahane, Anne-Sophie Job
Refractory extratemporal lobe epilepsy (ETLE) tends to have a less favourable surgical outcome in comparison to temporal lobe epilepsy. ETLE poses specific diagnostic and therapeutic challenges, particularly in cases where seizures develop from the midline. This review focuses on the diagnostic challenges and therapeutic strategies in mesial ETLE. The great diversity of interhemispheric functional areas and extensive connectivity to extramesial structures results in very heterogeneous seizure semiology. Specific signs, such as ictal body turning, can suggest a mesial onset...
December 21, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29258971/adult-onset-rasmussen-encephalitis-associated-with-focal-cortical-dysplasia
#3
Katharina Hohenbichler, Julie Lelotte, Renaud Lhommel, Riëm El Tahry, Pascal Vrielynck, Susana Ferrao Santos
Rasmussen encephalitis is a rare, devastating condition, typically presenting in childhood. Cases of adult-onset Rasmussen have also been described, but the clinical picture is less defined, rendering final diagnosis difficult. We present a case of adult-onset Rasmussen encephalitis with dual pathology, associated with focal cortical dysplasia and encephalitis. We interpreted the Rasmussen encephalitis to be caused by severe and continuous epileptic activity due to focal cortical dysplasia. The best therapeutic approach for such cases remains unclear...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29258970/mowat-wilson-syndrome-presenting-with-fever-associated-seizures
#4
Se Eun Seo, Se Hee Kim, Seung Tae Lee, Jong Rak Choi, Joon Soo Lee, Heung Dong Kim, Hoon-Chul Kang
Mowat-Wilson syndrome (MWS) is a disorder caused by mutations or deletions of the zinc finger E-box-binding homeobox 2 (ZEB2) gene. Diagnosis of MWS can be challenging to neurologists, because its manifestations are diverse and the spectrum of genetic mutations are broad. Here, we describe two patients with MWS who initially showed atypical forms of fever-triggered seizures during childhood. Both had characteristic facial features, cognitive impairment, and genito-urinary anomalies consistent with MWS. By performing targeted next-generation sequencing (NGS) using a gene panel for epilepsy, we were able to identify a nonsense mutation (c...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29258969/a-case-of-anti-nmda-receptor-encephalitis-revealed-by-insular-epilepsy
#5
Charlotte De Maeseneire, Riem El Tahry, Susana Ferrao Santos
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system that typically manifests predominantly as a psychiatric disorder. However, other manifestations such as epileptic seizures, abnormal movements, and memory or language complications are not unusual. Here, we report the case of a young man who presented with a new-onset epilepsy, with ictal semiology suggestive of insular involvement; this hypothesis was supported by a PET-CT study. Anti-NMDAR antibodies were found in the CSF, confirming the diagnosis of anti-NMDAR encephalitis...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29258968/refractory-chronic-epilepsy-associated-with-neuronal-auto-antibodies-could-perisylvian-semiology-be-a-clue
#6
Lisa Gillinder, Linda Tjoa, Basil Mantzioris, Stefan Blum, Sasha Dionisio
We report a case series of 10 patients with chronic medically refractory antibody-positive autoimmune epilepsy and assess their common clinical features. Immune-mediated seizures are most commonly reported in the context of encephalitis or encephalopathy, with few reports focusing on lone, chronic epilepsy in the outpatient setting. Our aim was to define the potential diagnostic clues that might be present in these cases, leading to consideration of an autoimmune cause of the epilepsy. We performed a retrospective review of all patients presenting to the outpatient department of our unit who underwent autoimmune screening...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29258967/occipital-ulegyria-causing-epilepsy-and-visual-impairment-an-easily-overlooked-epilepsy-syndrome
#7
Antoinette O'Connor, Daniel J Costello
Ulegyria refers to scarring of the cerebral cortex usually arising from perinatal ischaemia. The scarring has a specific configuration in which small atrophic circumvolutions at the bottom of a sulcus underlie an intact spared gyral apex. This disconnection of overlying cortex may allow an "epileptogenic" island of cortex to generate seizures. Ulegyria is often associated with epilepsy and developmental delay, however, the syndromic association of visual impairment with epilepsy due to occipital ulegyria may not be recognised as a specific entity...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29258966/sensitive-quantitative-detection-of-somatic-mosaic-mutation-in-double-cortex-syndrome
#8
John A Damiano, Hongdo Do, Ezgi Ozturk, Rosemary Burgess, Renate Kalnins, Nigel C Jones, Alexander Dobrovic, Samuel F Berkovic, Michael S Hildebrand
Somatic mutation of the lissencephaly-1 gene is a cause of subcortical band heterotopia ("double cortex"). The severity of the phenotype depends on the level of mutation in brain tissue. Detecting and quantifying low-level somatic mosaic mutations is challenging. Here, we utilized droplet digital PCR, a sensitive method to detect low-level mutation. Droplet digital PCR was used in concert with classic genotyping techniques (SNaPshot assays and pyrosequencing) to detect and characterize the tissue mosaicism of a somatic mutation (LIS1 c...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29171405/seizure-semiology-of-anti-lgi1-antibody-encephalitis
#9
Nicholas J Beimer, Linda M Selwa
Limbic encephalitis associated with anti-LGI1 antibody (LGI1 encephalitis) presents with a variety of features, the most prominent of which include seizures and progressive disturbance of memory and behaviour. Although varied in semiology, recognition of the pattern of seizures in LGI1 encephalitis is important, as early diagnosis and definitive treatment may prevent subsequent development of cognitive impairment. We present a patient with LGI1 encephalitis and "faciobrachial dystonic seizures-plus", which began as classic faciobrachial dystonic seizures and progressed to focal seizures with impaired awareness, dacrystic/gelastic-like outbursts, ictal speech, manual automatisms, and autonomic signs (tachycardia)...
November 24, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29171397/crossing-the-lines-between-epilepsy-syndromes-a-myoclonic-epilepsy-variant-with-prominent-eyelid-myoclonia-and-atonic-components
#10
Pinelopi Dragoumi, Jacqueline Emery, Fiona Chivers, Megan Brady, Archana Desurkar, J Helen Cross, Krishna B Das
Accurate diagnosis of a distinct epilepsy syndrome is based on well-defined electroclinical features that differentiate separate nosological entities. In clinical practice, however, syndromes may overlap and cases may present with unusual manifestations posing a diagnostic challenge. This heterogeneity has been documented in several cases presenting with eyelid myoclonia with or without absences (EMA) diagnosed either as Jeavons syndrome (JS) variants or as genetic generalised epilepsies defined by the presence of this unique clinical entity...
November 24, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29171396/sleep-related-hypermotor-epilepsy-activated-by-rapid-eye-movement-sleep
#11
Xiaoli Wang, Lara V Marcuse, Lang Jin, Ying Zhang, Wenjuan Zhang, Beibei Chen, Na Yuan, Bi Wang, Yonghong Liu
Most sleep-related seizures occur during non-rapid eye movement (NREM) sleep, particularly during stage changes. Sleep-related hypermotor epilepsy (SHE) is a rare epileptic syndrome characterized by paroxysmal motor seizures, mainly arising from NREM sleep. Here, we report a patient with SHE who had seven seizures captured on video-EEG-polysomnography during REM sleep. Ictal semiology of this patient ranged from brief paroxysmal arousals to hypermotor seizures. On EEG-polysomnography, the spontaneous arousals were more frequent during REM than NREM sleep, with a considerably higher arousal index in REM sleep (20/hour)...
November 24, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29165302/prominent-eye-muscle-artefact-on-the-eeg-of-a-patient-with-hashimoto-thyroiditis-with-primary-hypothyroidism
#12
Irma Liezl Reyes, Douglas R Nordli
No abstract text is available yet for this article.
November 22, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29160210/exceptional-response-to-brivaracetam-in-a-patient-with-refractory-idiopathic-generalized-epilepsy-and-absence-seizures
#13
Alberto Grande-Martín, David Sopelana-Garay, José Manuel Pardal-Fernández, Rosa María Sánchez-Honrubia, Álvaro Abelardo Sánchez-Larsen
Brivaracetam is currently indicated as adjunctive therapy for patients with focal-onset seizures with or without secondary generalization. However, it has been suggested that it could provide broad-spectrum efficacy given its similarity to levetiracetam and based on the results from preclinical studies and others of patients with generalized epilepsy. We present the case of a woman with refractory idiopathic generalized epilepsy and absence seizures with dramatic response to brivaracetam. Our report supports a consideration of treatment with this new antiepileptic drug on a case-by-case basis in patients with refractory generalized epilepsy, while we await further studies on this topic...
November 21, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29160209/de-novo-absence-status-epilepticus-of-late-onset-dnaslo-precipitated-by-oral-treatment-with-cefuroxime-description-of-an-ambulatory-case
#14
José L Fernández-Torre, Alicia Paramio-Paz, Anjana López-Delgado, María Martín-García, Isabel González-Aramburu, Miguel A Hernández-Hernández
We describe the case of an elderly woman with an episode of ambulatory de novo absence status epilepticus of late onset (DNASLO) after oral treatment with cefuroxime. A high level of suspicion of DNASLO in cases of unexplained confusion in adults or elderly subjects taking cephalosporins is essential to prompt an emergency EEG and, in turn, rapidly achieve an appropriate diagnosis and enable optimal treatment.
November 21, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29160208/a-case-of-post-leptospirosis-autoimmune-epilepsy-presenting-with-sleep-related-hypermotor-seizures
#15
Prashant Makhija, Siby Gopinath, Sudheeran Kannoth, Kurupath Radhakrishnan
This video-illustrated case report concerns a 49-year-old woman who presented with sleep-related hypermotor seizures. The antecedent history of leptospirosis, high frequency of new-onset seizures, presence of an unclassified anti-neuronal antibody, and dramatic response to steroids strongly supported post-infectious immune-mediated pathogenesis in our patient. To the best of our knowledge, post-leptospirosis autoimmune epilepsy presenting as sleep-related hypermotor seizures has not hitherto been reported. [Published with video sequences on www...
November 21, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29160207/serial-eeg-findings-in-anti-nmda-receptor-encephalitis-correlation-between-clinical-course-and-eeg
#16
Jun Ueda, Michi Kawamoto, Ryota Hikiami, Junko Ishii, Hajime Yoshimura, Riki Matsumoto, Nobuo Kohara
Anti-NMDA receptor encephalitis is a paraneoplastic encephalitis characterised by psychiatric features, involuntary movement, and autonomic instability. Various EEG findings in patients with anti-NMDA receptor encephalitis have been reported, however, the correlation between the EEG findings and clinical course of anti-NMDA receptor encephalitis remains unclear. We describe a patient with anti-NMDA receptor encephalitis with a focus on EEG findings, which included: status epilepticus, generalised rhythmic delta activity, excess beta activity, extreme delta brush, and paroxysmal alpha activity upon arousal from sleep, which we term"arousal alpha pattern"...
November 21, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28984246/the-role-of-eeg-in-the-diagnosis-and-classification-of-the-epilepsy-syndromes-a-tool-for-clinical-practice-by-the-ilae-neurophysiology-task-force-part-1
#17
Michalis Koutroumanidis, Alexis Arzimanoglou, Roberto Caraballo, Sushma Goyal, Anna Kaminska, Pramote Laoprasert, Hirokazu Oguni, Guido Rubboli, William Tatum, Pierre Thomas, Eugen Trinka, Luca Vignatelli, Solomon L Moshé
The concept of epilepsy syndromes, introduced in 1989, was defined as "clusters of signs and symptoms customarily occurring together". Definition of epilepsy syndromes based on electro-clinical features facilitated clinical practice and, whenever possible, clinical research in homogeneous groups of patients with epilepsies. Progress in the fields of neuroimaging and genetics made it rapidly clear that, although crucial, the electro-clinical description of epilepsy syndromes was not sufficient to allow much needed development of targeted therapies and a better understanding of the underlying pathophysiological mechanisms of seizures...
October 6, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28872032/atlas-of-electroencephalography-3rd-edition
#18
Ali Akbar Asadi-Pooya, Dennis Dlugos, Christopher Skidmore, Michael R Sperling
Electroencephalography (EEG) is the recording of the electrical activity of the brain. EEG is a reliable test to assess cerebral function. It aids in diagnosis, classification, and management of patients with epilepsy. It also has practical uses in conditions other than epilepsy. Moreover, it is harmless and inexpensive. EEG is an important tool for evaluating patients with epilepsy. It may help classify the syndrome, identify the likely source of focal seizures, and confirm a diagnosis of status epilepticus...
September 5, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28869205/ictal-asystole-in-a-patient-with-posterior-reversible-encephalopathy-syndrome-pres-and-seizures
#19
Joanna Suski, Reginald Ho, Maromi Nei
We describe a case of ictal asystole in a patient with posterior reversible encephalopathy syndrome (PRES), which has never been described previously. Ictal asystole is rare and has possible serious medical consequences, including syncope, and may be a potential mechanism for sudden unexpected death in epilepsy (SUDEP). Awareness that PRES may be observed with recurrent ictal asystole may aid in the recognition and treatment of seizures in this condition and the prevention of asystole-associated complications...
September 1, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28832005/the-use-of-single-bipolar-scalp-derivation-for-the-detection-of-ictal-events-during-long-term-eeg-monitoring
#20
Frank C Bennis, Evelien E Geertsema, Demetrios N Velis, Elise Em Reus, Gerhard H Visser
Epilepsy is difficult to diagnose using routine EEG recordings of short duration in patients who have low seizure frequency. Long-term EEG may be useful but is impractical in an out-of-hospital setting. We investigated whether single-channel scalp EEG placed behind the earlobe is suitable for seizure identification during prolonged EEG monitoring. Scalp EEG samples were selected from subjects over 15 years of age, and comprised two segments of either background followed by seizure or background followed by background...
September 1, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
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