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Editorial
Epileptic Disorders anniversary: time for new developments
Alexis Arzimanoglou
Review article
Descriptions of clinical semiology of seizures in literature
Peter Wolf
with varying detail. This review looks at these, and considers the authors’ background knowledge of epilepsy.The first group of writers suffered seizures themselves. Some of them provide remarkable and novel insights into the subjective symptoms and experiences in and around seizures.A second group draws from their own observations of seizures in others who may have been close relatives or fortuitous strangers. Here, the outside view prevails, and seizures may be described with clinical objectiveness. Commonly, the reactions of onlookers become part of the narrative beyond the seizure description.The third group writes from second-hand knowledge, which may be other observer reports, interviews with people with epilepsy or with experts, information being available in dictionaries, medical textbooks and the like. The professional standard of most writers means that the resulting descriptions are quite accurate, but there are also examples of poor use of such information.Many authors’ sources are not known, but can be assumed to belong to one of the above categories, and we can sometimes guess. It appears that even authors with no first-hand knowledge are often aware of seizure types other than the most widely known, generalised tonic-clonic, and there is a widespread interest in warnings and how the afflicted respond to them.The quality of an author’s fictional account of epilepsy should not be judged from a translation, because specific aspects and the language involved in the description may have been misunderstood by the translator.
Original article
Cognitive functions in children with benign childhood epilepsy with centrotemporal spikes (BECTS)
Florence Pinton, Béatrice Ducot, Jacques Motte, Anne-Sophie Arbuès, Catherine Barondiot, Marie-Anne Barthez, Yves Chaix, Renée Cheminal, Marie-Odile Livet, Marie-José Penniello, Sylviane Peudenier, Anne de Saint-Martin, Catherine Billard
with BECTS were studied in terms of neuropsychological and learning abilities: intellectual quotient, oral language (phonological production, naming skills, verbal fluency and syntactic comprehension), drawing and visuo-spatial skills, visual and selective attention, verbal and visuo-spatial memory, reading, numeracy and spelling. The mean IQ of the population was within the normal range, but individual results were heterogeneous. Verbal functions and memory were normal. In contrast, drawing and visuo-spatial skills, attention and visuo-spatial memory were significantly weak compared to the normal range for age. Reading, numeracy and/or spelling ability were significantly delayed by one academic year or more in ten of the children. In conclusion, despite its benign outcome in terms of epilepsy, BECTS can be accompanied by specific cognitive disorders and low academic achievement.
The consequences of idiopathic partial epilepsies in relation to neuropsychological functioning: a closer look at the associate
Z Hande Sart, Veysi Demirbilek, Barış Korkmaz, Peter D Slade, Ayşın Dervent, Brenda D Townes
risk of academic underachievement, are not rare. We investigated neuropsychological functioning including attention, memory, visuomotor ability, and executive functioning with a closer look at the associated mathematical ability in patients with idiopathic partial epilepsies. A battery of age-appropriate, neuropsychological and mathematics achievement tests was administered to 30 participants with idiopathic partial epilepsy [13 children with benign epilepsy with centrotemporal spikes (BECTS), 17 children with idiopathic childhood occipital epilepsies (ICOE)], and to 30 healthy participants matched for age, sex, handedness, and socioeconomic status. Results did not support any impairment in overall neuropsychological functioning in participants with idiopathic partial epilepsies, whereas, isolated deficits did exist. The mean performance of the IPE group was significantly lower than the control group in six out of 12, neuropsychological measures: drawing (p < 0.01), digit span (p < 0.05), verbal learning (p < 0.01), object assembly (p < 0.01), similarities (p < 0.05), and vocabulary (p < 0.001). Results suggested that one should be cautious regarding neuropsychological and academic prognosis in the so-called benign idiopathic partial epilepsies of childhood.
Oligogenic inheritance in photosensitive juvenile myoclonic epilepsy?
Gerrit-Jan de Haan, Dalila Pinto, Ed Bertram, Dorothee GA Kasteleijn-Nolst Trenité, Bobby PC Koeleman, Dick Lindhout
inheritance model, which although rather simplified, may explain at least some of the clinical observations. Here we describe a family in which the clinical phenotype in the offspring can be explained by a combination of photosensitivity and epilepsy traits that segregated independently of each other. This case history demonstrates the need to evaluate family histories in more detail in order to uncover potential clinical markers for genetic factors in complex epilepsies.
Identifying baseline characteristics of placebo responders versus nonresponders in randomized double-blind trials of refractory
Ida Niklson, Pascal Edrich, Peter Verdru
between placebo groups in different trials are not given in the literature. This exploratory analysis was undertaken to compare the baseline characteristics of placebo responders and nonresponders, in an attempt to identify common features. The pooled statistical analysis was performed on the database for three pivotal studies of levetiracetam (n = 904). Using the 50% response definition, we found that 45.6% of placebo nonresponders were on one antiepileptic drug at baseline, compared with 69% of placebo responders. The difference in number of baseline antiepileptic drugs was almost statistically significant (p = 0.056). Placebo nonresponders also tended to have epilepsy for longer than responders. The mean age at onset of epilepsy was consistently different between placebo nonresponders and responders (15.2 versus 20.8 years, respectively; p = 0.019). These findings suggest that the placebo response is higher in patients with partial-onset seizures who are taking only one antiepileptic drug at baseline and have later onset and shorter duration of epilepsy than in patients on more than one antiepileptic drug at baseline with earlier onset and longer duration of epilepsy.
Isolated paroxysmal arousals as focal epilepsy
Maria Paola Valenti, Sébastien Froelich, Gabrielle Rudolf, Anne Thibault, Serge Chassagnon, Alexis Arzimanoglou, Edouard Hirsch
a common clinical problem. Video-EEG monitoring during sleep, recording several episodes in the same patient, is essential in order to characterize these phenomena. It offers the possibility to compare electro-clinical data, to demonstrate the eventual stereotyped pattern of motor phenomena and their progression in time, and to study EEG-polygraphic correlates. The recently described double split-screen synchronized display (DSSSD) technique represents a useful tool for comparing particular clinical patterns of epileptic seizures when dealing with complex, hypermotor phenomena observed in frontal lobe epilepsy. We reviewed the data of 24 patients admitted during a two-year period (2002-2003) to our epilepsy sleep unit for isolated paroxysmal sleep motor events. Four patients presented with very brief paroxysmal arousals without daytime fits. Three of our patients presented isolated paroxysmal arousals, whereas in one, the events were associated with hypermotor seizures. We present a simplified variant of the DSSSD method (modified DSSSD) that can be used to study episodes of paroxysmal arousals in order to confirm their stereotyped motor pattern. The clinical aspects and the EEG-polygraphy patterns were informative, with the absence of asymmetrical tonic or dystonic posturing of the limbs. Scalp EEG alone does not usually provide much information in patients with isolated paroxysmal arousals. Coupled to the modified DSSSD technique, it may allow confirmation of the diagnosis of frontal epilepsy, as was the case in our four patients.[Published with video sequences]
Case report
EEG and seizure exacerbation induced by carbamazepine in Panayiotopoulos syndrome
Kenichi Kikumoto, Harumi Yoshinaga, Makio Oka, Minako Ito, Fumika Endoh, Tomoyuki Akiyama, Yoko Ohtsuka
Soon after the introduction of carbamazepine, EEGs began to worsen, and finally absence seizures and myoclonic seizures appeared. Immediately after we discontinued carbamazepine, the seizures disappeared and the EEG improved. Carbamazepine may induce unusual electroclinical features, electrophysiologically explained by bilateral synchrony. This case provides more evidence of the close links between Panayiotopoulos syndrome and benign childhood epilepsy with centrotemporal spikes.
Herpes encephalitis as a cause of nonconvulsive status epilepticus
Aysegul Gunduz, Ali Fuat Beskardes, Ayse Kutlu, Cigdem Ozkara, Naci Karaagac, Seher Naz Yeni
phenomena. Recognition of the consequences of nonconvulsive status epilepticus has attracted greater attention to this condition. We present a 24-year-old woman with epilepsy who developed nonconvulsive status epilepticus during pregnancy. Despite treatment with antiepileptic drugs, the seizures persisted and confusion deepened. Further workup to explain the etiology revealed the diagnosis as herpes encephalitis. She recovered completely by the third day of parenteral acyclovir administration. Herpes simplex encephalitis causing nonconvulsive status epilepticus in a pregnant, epileptic woman is an unfortunate and unusual condition, which was simultaneously complicated by the presence of multiple etiological factors.
“Burst and slow complexes” in nonconvulsive epileptic status
Akio Ikeda, Masaru Matsui, Yoshiki Hase, Takehumi Hitomi, Yukitoshi Takahashi, Hiroshi Shibasaki, Shun Shimohama
convulsions and fragmented myoclonus in the whole body. Two types of short-latency SEPs were obtained separately during the burst and slow phase of the EEG (SEP-burst and SEP-slow, respectively), which showed a two fold greater amplitude of N20 in the former than in the latter. This suggests enhanced responsiveness to the peripheral stimuli during the burst phase as compared with the slow phase. CSF and serum were positive for autoantibodies to NMDA receptors. The “burst and slow complexes” reported here are considered to be an atypical EEG pattern of a generalized epileptic phenomenon.
Video case report
Post-ictal forceful yawning in a patient with nondominant hemisphere epilepsy
Alexei E Yankovsky, Frederick Andermann, François Dubeau
hemisphere seizures and post-ictal yawning. Yawning was irresistible, forceful and often repetitive. We reviewed the few similar epileptic cases described in the literature and discussed the possible mechanisms. [Published with video sequences]
Sleep-related laryngospasm: a video-polysomnographic recording
Jorge Iriarte, Elena Urrestarazu, Manuel Alegre, Concepción Goñi, César Viteri, Julio Artieda
diagnoses included frontal seizures, sleep-related choking syndrome, sleep asthma, sleep apnoea and REM sleep behaviour disorder. The video and the EEG recordings supported the diagnosis of laryngospasm. The pH-metry confirmed the existence of reflux. Its treatment successfully controlled the episodes. This case illustrates, with a typical video recording, this infrequent type of paroxysmal event with an important differential diagnosis.[Published with video sequences]
Uncommon, exceptional and atypical: a case of frontal lobe epilepsy due to cortical dysplasia
Arthur C Grant, Richard B Kim
seizure at age 29, well into adulthood. Seizures had been easily controlled with medication for 15 years, then without provocation they became medically intractable during a single, identifiable day. Resection of the dysplastic tissue in the posterior dorsolateral right frontal cortex rendered the patient seizure-free, but produced a significant, non-fluent, Broca type, crossed aphasia. In addition, the seizure semiology was striking and remarkable, a common finding in frontal lobe epilepsy. [Published with video sequences]
Myoclonus relieved by carbamazepine in subacute sclerosing panencephalitis
Aytaç Yiğit, Sevda Sarikaya
The favorable effect of the carbamazepine on myoclonus was demonstrated by discontinuing and reintroducing carbamazepine, and videoing the consequent reappearance and disappearance of the myoclonus.[Published with video sequences]
Debate
Are epilepsy classifications based on epileptic syndromes and seizure types outdated?
HO Lüders, J Acharya, A Alexopoulos, C Baumgartner, J Bautista, R Burgess, M Carreño, B Diehl, D Dinner, A Ebner, N Foldvary, J Godoy, H Hamer, A Ikeda, K Källén, C Kellinghaus, P Kotagal, D Lachhwani, T Loddenkemper, J Mani, R Matsumoto, G Möddel, D Nair, S Noachtar, CA O’Donovan, S Rona, F Rosenow, S Schuele, CA Szabo, N Tandon, A Tanner, P Widdess-Walsh
