The Education in the world of Epilepsy

Alexis Arzimanoglou,Editor-in-Chief.

Dear Colleague and Friend,
As the official journal of the International League Against Epilepsy, alongside Epilepsia, Epileptic Disorders is primarily devoted to the educational aspects of epilepsy and related disorders.
Today, Epileptic Disorders is undergoing a number of changes but remains committed to its initial principle: the sharing of clinical practices and research perspectives within the Epilepsy Community.


Benjamin Cretin

A guide to diagnosing even the rarest types of consciousness loss in routine practice. Healthcare providers are frequently called upon to deal with patients who have experienced fainting with and without consciousness loss. The multitude of possible causes makes this very difficult to diagnose. This book lists all...


Epileptic Disorders

Volume 16
Number 1,
March 2014

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Original articles

Side-to-side axial movements

Sara Franca, Deepak K. Gupta, Shyam Rao, Elia Pestana, Joel Freitas, Jakrin Loplumlert, Samden D. Lhatoo

generalised tonic-clonic seizures (GTCS). Methods. We retrospectively studied 86 GTCS, 13 primary and 73 secondary, in 58 patients who underwent video-EEG (vEEG) evaluation in our epilepsy monitoring unit. Eleven patients had generalised epilepsy and 47 focal epilepsy. Two expert epileptologists, blinded to diagnosis, examined the vEEGs independently for the presence of five semiological signs. Results. Asymmetry of limb movements in clonic phase, side-to-side axial movements, and asymmetric seizure termination occurred more frequently ( p<0.05) in secondary GTCS compared to primary GTCS. Combining asymmetry of limb movements in clonic phase and side-to-side axial movements provided the greatest value in differentiating secondary GTCS from primary GTCS. Conclusion. Careful examination of GTCS seizure semiology can help differentiate primary from secondary GTCS. The semiological sign of side-to-side axial movements, which has not previously been studied in this context, may add to existing literature of semiological signs and be of value for the evaluation of surgical patients in the epilepsy monitoring unit. In the out-patient setting, a clear history of these signs may help guide drug treatment choices. [ Published with video sequences].

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Dyskinesia as a new adverse effect of hormonal treatment in West syndrome

Biayna G. Sukhudyan, Petia S. Dimova, Alessandro Capuano, Federico Vigevano

receiving steroid treatment who presented with unusual, mostly hyperkinetic, movements. Facial grimacing, repetitive mouth opening, adduction and abduction of upper and lower extremities, and periodical strabismus in different combinations were observed in all patients, independent of formulation, dose, duration, and efficacy of treatment. Symptoms disappeared in sleep and reappeared immediately on arousal. Dyskinesias stopped gradually after a month of discontinuation of treatment. Repeated EEGs did not show corresponding epileptiform activity. We conclude that these abnormal movements can be attributed to side effects of hormonal treatment. [ Published with video sequences]

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Confirming an expanded spectrum of SCN2A mutations: a case series

Dena Matalon, Ethan Goldberg, Livija Medne, Eric D. Marsh

1 (Nav1.1), causes Dravet syndrome spectrum disorders. Mutations in SCN2A have been identified in patients with benign familial neonatal-infantile epilepsy (BFNIE), generalised epilepsy with febrile seizures plus (GEFS+), and a small number of reported cases of other infantile-onset severe intractable epilepsy. Here, we report three patients with infantile-onset severe intractable epilepsy found to have de novo mutations in SCN2A. While a causal role for these mutations cannot be directly established, these findings contribute to growing evidence that mutation of SCN2A is associated with a range of epilepsy phenotypes including severe infantile-onset epilepsy.

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Understanding the pathophysiology of reflex epilepsy using simultaneous EEG-fMRI

Manglore Sandhya, Rose Dawn Bharath, Rajanikant Panda, S.R. Chandra, Naveen Kumar, Lija George, A. Thamodharan, Arun Kumar Gupta, P. Satishchandra

understanding brain function. In this study, we investigated three patients with drug-resistant reflex epilepsy using EEG-fMRI. Different types of reflex epilepsy such as eating, startle myoclonus, and hot water epilepsy were included in the study. The analysis of EEG-fMRI data was based on the visual identification of interictal epileptiform discharges on scalp EEG. The convolution of onset time and duration of these epilepsy spikes was estimated, and using these condition-specific effects in a general linear model approach, we evaluated activation of fMRI. Patients with startle myoclonus epilepsy experienced epilepsy in response to sudden sound or touch, in association with increased delta and theta activity with a spike-and-slow-wave pattern of interictal epileptiform discharges on EEG and fronto-parietal network activation pattern on SPECT and EEG-fMRI. Eating epilepsy was triggered by sight or smell of food and fronto-temporal discharges were noted on video-EEG (VEEG). Similarly, fronto-temporo-parietal involvement was noted on SPECT and EEG-fMRI. Hot water epilepsy was triggered by contact with hot water either in the bath or by hand immersion, and VEEG showed fronto-parietal involvement. SPECT and EEG fMRI revealed a similar fronto-parietal-occipital involvement. From these results, we conclude that continuous EEG recording can improve the modelling of BOLD changes related to interictal epileptic activity and this can thus be used to understand the neuro-haemodynamic substrates involved in reflex epilepsy.

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Cortical network dysfunction in musicogenic epilepsy reflecting the role of snowballing emotional processes in seizure generation: an fMRI-EEG study

Volker Diekmann, Anselm Cornelius Hoppner

to play a role in the process triggering the seizure, however, the significance of these emotions and the brain regions involved are unclear. In order to shed some light on this, we conducted fMRI and EEG in a case of musicogenic epilepsy. Methods. In a 32-year-old male patient with seizures induced by a specific piece of Russian music, we performed video-EEG monitoring as well as simultaneous fMRI and EEG registration. Results. Video-EEG monitoring revealed a left temporo-frontal epileptogenic focus. During fMRI-EEG co-registration, BOLD signal alterations were not only found in the epileptogenic focus but also in areas known for their role in the processing of emotions. Prior to a seizure in some of these areas, BOLD contrasts exponentially increased or decreased. Conclusion. These results suggest that in our case, dysfunction of the regulation processes of the musically-induced emotions, and not the musical stimulus itself, led to the seizures.

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Nocturnal variant of benign myoclonic epilepsy of infancy: a case series

Aparna M. Prabhu, Sheel Pathak, Divya Khurana, Agustin Legido, Karen Carvalho, Ignacio Valencia

with excessive myoclonic jerks, only during sleep, and abnormal EEG during the events. Although only one third of the events had EEG epileptiform correlate, the presence of myoclonus without epileptiform EEG correlate has been described in patients with benign myoclonic epilepsy of infancy. We hypothesize that these findings may represent a variant of benign myoclonic epilepsy of infancy.

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A 15-year follow-up of first unprovoked seizures: a prospective study of 200 children

Cristina Pereira, Cristina Resende, Isabel Fineza, Conceição Robalo

with first unprovoked seizures. We studied prospectively 200 children under the age of 11 years who attended hospital emergency with a first unprovoked seizure. Demographic variables, personal and family history, neurological examination, EEG, psychiatric, and cognitive and educational profiles were analysed. Patients who developed epilepsy were characterised with respect to: time to relapse, remission rate, duration of epilepsy, neuroimaging, aetiology, epileptic syndrome, and therapeutic regimen. These results were compared to data of patients who had a single seizure over a follow-up period of 15 years. Thirty percent of children who had a first unprovoked seizure developed epilepsy. Partial seizure type was a statistically significant variable for the development of epilepsy. An EEG with epileptic abnormalities proved to be the main risk factor for recurrence. Fifteen years later, the group with epilepsy exhibited a 2.6 greater risk of psychiatric and academic comorbidities, compared to the group without epilepsy.

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Predicting diurnal and sleep/wake seizure patterns in paediatric patients of different ages

Sriram Ramgopal, Christine Powell, Marcin Zarowski, Andreas V. Alexopoulos, Sanjeev V. Kothare, Tobias Loddenkemper

Medical charts of patients that underwent video-EEG were reviewed. Seizures were evaluated based on their occurrence in three-hour time intervals (bins) and between wakefulness and sleep. Patients were classified according to EEG localisation and age: infants (≤3 years), children (3-12 years), and adolescents (>12-21 years). Analysis utilising generalised estimating equations with a negative binomial distribution was performed. Results. A total of 390 patients (188 girls; mean age: 9.2 years; SD: 6.0) had 1,754 seizures. Generalised seizures (109 patients; 490 seizures) occurred more during wakefulness ( p<0.001) and during the day ( p<0.001). Modelling revealed a greater occurrence of seizures at night with increasing age ( p=0.046). Temporal lobe seizures (62 patients; 271 seizures) occurred overall more frequently during wakefulness ( p=0.03). Frontal lobe seizures (41 patients; 184 seizures) occurred more frequently during wakefulness in infants ( p<0.05) and more frequently during sleep in adolescents ( p<0.0001). Adolescents with frontal lobe seizures were 3.6 times more likely to have seizures during sleep compared to other children (95% CI: 1.8-7.2). Conclusion. These findings are suggestive of changes in circadian rhythmicity that may alter seizure susceptibility in different age groups. The results may assist in prediction of periods of greatest seizure propensity.

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Auditory event-related potentials (P300) and mesial temporal sclerosis in temporal lobe epilepsy patients

Artemios K. Artemiadis, Maria Fili, George Papadopoulos, Fotini Christidi, Stergios Gatzonis, Ioannis Zalonis, George Nikolaou, Nikolaos Triantafyllou

(3 men and 13 women; median age: 32.5 years old) suffering from TLE with MTS and 43 healthy controls (12 men and 31 women; median age: 35 years old) participated in the study. P300 was elicited using an auditory two-stimulus oddball paradigm. In order to address the aim of the study, we adopted two statistical approaches; hierarchical linear regression analyses and ROC curves. Results. After adjusting for age, MTS patients had a mean reduction of P300 amplitude by 6.93 μV and a mean increase of P300 latency by 38.78 ms, compared to controls. Age and MTS-TLE status accounted for 32 and 16% of the variance of latency and amplitude, respectively. Diagnostic analyses to detect MTS-TLE status revealed a sensitivity and specificity of 88 and 65% for amplitude and 81 and 70% for latency, respectively. No association between duration of disease and P300 characteristics were found. Conclusions. This study, along with other studies, contributes to our understanding and clinical significance of centrally recorded P300s in MTS-TLE patients. Future studies should focus on the association of these P300s with cognition in such patients.

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What are the predictors of major depression in adult patients with epilepsy?

Chen Kui, Pan Yingfu, Xu Chenling, Wu Wenqing, Li Xiuhua, Sun Di

in a cohort of adult patients with epilepsy (PWEs) living in north China, and investigate the predictors of major depression in PWEs. A total of 215 consecutive cases were enrolled and divided into two groups: PWEs with major depression and PWEs without major depression. Patients were assessed for demographic characteristics, epilepsy details, and social status. A total of 65 of 215 (30.23%) PWEs exhibited comorbid major depression. A binary logistic regression model revealed the strong predictor variables of major depression to be drug responsiveness (odds ratio [OR]=0.23; p=0<0.01; 95% CI [0.13-0.39]), presence of chronic medical illnesses (OR=0.19; p=0.015<0.05; 95% CI [0.05-0.72]), and employment status (OR=0.42; p=0.015<0.05; 95% CI [0.21-0.84]).

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Clinical commentaries

Generalised electrographic seizures presenting as perioral myoclonia

Jennifer L Dearborn, Peter W Kaplan

seizure seven years before, he had mentioned several episodes of chin twitching over the years. In the clinic, there were intermittent chin movements without apparent confusion, as he was able to provide a complete history and was fully oriented with intact memory. His video-EEG showed paroxysms of polyspike and slow-wave activity, with the longest burst-free interval being 20 seconds. Discharges were maximal over the fronto-central regions, correlating with the chin myoclonus. He was able to tap his hand continuously, and remained alert. The case represents an atypical presentation of idiopathic generalised epilepsy without manifestation of absence or limb myoclonus. Although juvenile myoclonic epilepsy and other idiopathic epilepsies are rarely associated with perioral myoclonia, this sign was the principal clinical feature for this patient. Oral treatment with levetiracetam resolved his seizures. [ Published with video sequences]

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Extreme startle and photomyoclonic response in severe hypocalcaemia

Marcello Moccia, Roberto Erro, Elvira Nicolella, Pasquale Striano, Salvatore Striano

EEG showed a photomyoclonic response, and blood tests indicated severe hypocalcaemia. Additional clinical data, treatment strategies, and long-term follow-up visits were reported. The present report discusses the difficulties in distinguishing between epileptic and non-epileptic startles, and shows, for the first time, exaggerated startle reflex and extreme photomyoclonic response due to severe hypocalcaemia. [ Published with video sequences]

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Sensory stimulus-sensitive drop attacks and basal ganglia calcification: new findings in a patient with FOLR1 deficiency

Sandra P Toelle, David Wille, Bernhard Schmitt, Ianina Scheer, Beat Thöny, Barbara Plecko

disorder. We report on a 5-year-old boy with progressive ataxia, from the age of 2 years and 6 months, with myoclonic jerks, regression, and impressive myoclonic tonic spasms with drop attacks, which were partially provoked by touching his face or washing his hands. Delayed myelination and cerebellar atrophy on cranial MRI were important clues to the diagnosis of cerebral folate transport deficiency, which was confirmed by homozygosity for the known nonsense mutation p.R204X in the FOLR1 gene. Computed tomography taken after head injury revealed bilateral calcifications in the basal ganglia as a novel finding in a patient with FOLR1 mutation. [ Published with video sequences]

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Self-induction seizures in sunflower epilepsy: a video-EEG report

Vincenzo Belcastro, Pasquale Striano

of seizure precipitation employed by either intellectually disabled or healthy photosensitive individuals. Absences and myoclonic jerks are the most common seizure types in self-induction. We report on a girl with normal intelligence who self-induced seizures by waving her outspread fingers in front of a bright light. This situation is called sunflower epilepsy. [ Published with video sequences]

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Hot water epilepsy

Aglaia Vignoli, Miriam Nella Savini, Francesca La Briola, Valentina Chiesa, Elena Zambrelli, Angela Peron, Maria Paola Canevini

taken while hot water was poured onto his chest. Hot water epilepsy is rarely described in European countries, where bathing epilepsy in younger children is more common and often confused with this type of epilepsy. [ Published with video sequences]

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Severe cardioinhibitory vasovagal syncope in sleep and supine posture

Zheyu Xu, Simon Bower, Udaya Seneviratne

occurs in erect posture and is often provoked by emotional or physical triggers. We report two patients who presented with severe cardioinhibitory vasovagal syncope accompanied by cardiac asystole resulting in seizure-like motor manifestations in sleep and supine posture. Both cases were initially diagnosed as epilepsy and treated with antiepileptic drugs. We discuss the putative mechanisms of this rare condition and its potential for misdiagnosis as epilepsy. [ Published with video sequences]

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Alcohol-responsive epilepsia partialis continua

Trevor A Steve, Donald W Gross

partialis continua who failed to respond to conventional anticonvulsant medications but experienced a dramatic transient response to alcohol and a subsequent response to primidone. This pattern of sensitivity, which is similar to that seen in essential tremor, has led to the hypothesis that the two disorders are associated with pathology within the same anatomical network. A new pathophysiological model is thus proposed for the occurrence of epilepsia partialis continua in both cortical and subcortical disease processes. [ Published with video sequences]

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A simple febrile seizure with focal onset

Sophie Hamelin, Laurent Vercueil

We report the case of fortuitous video-EEG recording of a FS, clinically classified as “simple”, which demonstrated a focal, temporal onset. This clinical finding is in agreement with animal model studies demonstrating focal onset. [ Published with video sequences]

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EEG-confirmed epileptic activity in a cat with VGKC-complex/LGI1 antibody-associated limbic encephalitis

Akos Pakozdy, Ursula Glantschnigg, Michael Leschnik, Harald Hechinger, Teresa Moloney, Bethan Lang, Peter Halasz, Angela Vincent

temporal lobe hyperintensities and the EEG was consistent with ictal epileptic seizure activity. After antiepileptic and additional corticosteroid treatment, the cat recovered and by 10 months of follow-up was seizure-free without any problem. Retrospectively, antibodies to LGI1, a component of the voltage-gated potassium channel-complex, were identified. Feline focal seizures with orofacial involvement have been increasingly recognised in client-owned cats, and autoimmune limbic encephalitis was recently suggested as a possible aetiology. This is the first report of EEG, MRI and long-term follow-up of this condition in cats which is similar to human limbic encephalitis.

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Ezogabine treatment of childhood absence epilepsy

David G. Vossler, Ugur Yilmaz

treatment of focal-onset seizures. This is a case report of an adult with childhood absence epilepsy whose daily absence seizures ceased with adjunctive ezogabine. A 59-year-old woman, with a history of typical absence seizures since the age of 6 years, had multiple seizures daily despite trials of over 11 antiepileptic drugs. While taking lamotrigine and zonisamide, ezogabine at 50 mg daily was added. The dose was slowly increased and once a total dose of only 200 mg/day was reached, she became seizure-free for three months. After subsequently discontinuing zonisamide, absence seizures returned. Further increasing the ezogabine to 400 mg/day, in addition to lamotrigine, did not restore seizure freedom, but adding back zonisamide at half dose again reduced their frequency. Ezogabine at low dose, added to lamotrigine and zonisamide, led to sustained absence seizure freedom. The return of seizures after zonisamide discontinuation suggests that the seizure freedom may have been the result of the different mechanisms of action of the antiepileptic drugs.

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Mesial temporal extraventricular neurocytoma: a rare cause of refractory complex partial seizure

Rui Feng, Jie Hu, Jinsong Wu, Liqin Lang, Bei Wang, Haixia Bi, Xin Gu, Jun Guo, Li Pan

in this article. Methods. We report two cases of mtEVN and review the related literature, with particular emphasis on radiological characteristics, clinical features, and operative techniques. Results. After successful surgery, our two cases of mtEVN achieved excellent outcome. Including the cases presented here, a total of three cases of mtEVNs and 11 of neocortical temporal extraventricular neurocytoma (ntEVNs) are reported in the literature. mtEVNs are distinct from ntEVNs with regards to demographics, aetiology, radiological features, and operative techniques . Conclusion. mtEVNs and ntEVNs exhibit distinguishing features. Under electrocorticographic monitoring, tailored resection of the neocortical epileptogenic focus, as well as the entire tumour and mesial temporal structures, can yield excellent outcome and satisfactory seizure control.

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Novel neurofibromatosis type 2 mutation presenting with status epilepticus

Jacopo C. DiFrancesco, Roberta Sestini, Federica Cossu, Martino Bolognesi, Elena Sala, Silvana Mariani, Enrico Saracchi, Laura Papi, Carlo Ferrarese

with a predisposition to development of benign tumours in the central nervous system. Even though cerebral cortical lesions are frequently associated with seizures, epilepsy is rarely described in NF2. Here, we describe an adult case of NF2 in which the onset of symptoms was characterised by status epilepticus. In this patient, we identified the novel c.428_430delCTTdel mutation in NF2, involving the amino-terminal FERM domain, which is fundamental for the correct tumour suppressor function of the protein. Bioinformatic analyses revealed an important structural perturbation of the FERM domain, with a predicted impairment of the anti-tumour activity.

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Cobalamin deficiency triggering de novo status epilepticus

Haralabos Anastogiannis, Panagiotis Karanasios, Alexandra Makridou, Nicolaos Makris, Andreas A. Argyriou

of unusual presentation of cobalamin (vitamin B12) deficiency with de novo SE with the intention to bolster the argument that a de novo manifestation of SE due to cobalamin deficiency might not be that uncommon. We also support the importance of prompt identification and treatment of the underlying causes of SE, particularly those which are uncommon.

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