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Editorial
Epileptic Disorders: 10th anniversary!
Alexis Arzimanoglou
Review article
Epilepsia partialis continua: semiology and differential diagnoses
Christian G Bien, Christian E Elger
linked with the motor cortex. This has been solidly supported by sophisticated electrophysiological studies. Here, a series of video sequences from patients with EPC (due to Rasmussen encephalitis, early-stage multiple sclerosis, and steroid responsive encephalopathy with autoimmune thyroiditis), and other cases with repetitive myoclonic jerks or movement disorders (myoclonic epilepsy associated with ragged-red fibers, Jacksonian march, myoclonic seizures in other types of frontal lobe or idiopathic generalized epilepsies, and different types of tremor) is presented. [Published with video sequences].
Original article
Blockade of androgen receptors is sufficient to alter the sexual differentiation of the substantia nigra pars reticulata seizur
James G Heida, Jana Velíšková, Solomon L Moshé
proconvulsant effects in males but not in females. In males, administration of an androgen receptor antagonist flutamide between P0-P2 led to the disappearance of the proconvulsant muscimol effects at P15. Thus, activation of androgen receptors is important for the presence of proconvulsant SNR muscimol responses.
Evaluation of susceptibility loci in an extended pedigree with idiopathic generalized epilepsy
Karl Martin Klein, Regina Preisig-Müller, Susanne Knake, Hajo M Hamer, Wolfgang H Oertel, Bernd A Neubauer, Jürgen Daut, Felix Rosenow
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.ResultsSuggestive 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.ConclusionsOur results corroborate former linkage findings on 2q36-2q37 and warrant further investigation of additional candidate genes within this chromosomal area in IGE.
Periodic eye opening and swallowing movements associated with post-anoxic burst-suppression EEG pattern
José L Fernández-Torre, Jesús Calleja, Jon Infante
without swallowing movements, after a prolonged cardiopulmonary arrest. These movements were associated with a burst-suppression pattern on the electroencephalogram. [Published with video sequences]
Long-term cognitive and behavioural follow-up in three patients with eye closure-triggered paroxysmal activity
Cristiano Termine, Federica Teutonico, Umberto Balottin, Marco Fasce, Matteo Ferri, Silvia Perna, Paolo Piccinelli, Guido Rubboli, Pierangelo Veggiotti
follow-up period. Methods. All patients were studied at the time of the first observation (T0) and after a long follow-up period (T1). At both T0 and T1, each patient underwent: 1) traditional and specific activation techniques during prolonged video-EEG monitoring to detect possible inducing factors; 2) neuropsychological evaluations during video-EEG monitoring either with eyes closed or eyes open to detect any transient cognitive impairment (TCI); 3) detailed neuropsychological assessment without simultaneous EEG recording, to detect any stable cognitive impairment (SCI). Results. EEG recordings showed transient, generalized paroxysms in one case and a continuous epileptic activity triggered by eye closure in the other two cases, at both T0 and T1. In all patients, no particular epileptiform discharge-induced factors were identified except for eye blinking (spontaneous, voluntary or induced by corneal reflex). The results of neuropsychological assessment while eyes were closed as compared to performances with eyes open, showed no significant differences at T0 or at T1 in two cases, thus possibly indicating the absence of TCI. Wechsler Intelligence Scales showed a decrease in performance at T1 in the two patients with eye closure-induced, continuous epileptiform activity. Detailed neuropsychological assessment without EEG recordings demonstrated an impairment of facial recognition ability in all three patients at T1. Conclusions. The lack of any differences between the results of neuropsychological tests performed with eyes open and eyes closed in two patients might suggest that not all eye-closure-triggered paroxysms are associated with TCI. On the other hand, our data highlight that EC-triggered, EEG epileptic discharges can produce long-lasting neuropsychological and behavioural effects, and also indicate that EEG discharges recurring over time might exert a disruptive effect on cognitive functions.Our three patients showed extreme variability across the neuropsychological tasks except for a facial recognition deficit that was evident in all cases, thus suggesting a possible dysfunction of temporo-occipital brain structures and/or of the fusiform face area as recently demonstrated by combined fMRI/EEG studies in patients with fixation-off sensitivity.
Zonisamide for the treatment of myoclonic seizures in progressive myoclonic epilepsy: an open-label study
David G Vossler, Joan A Conry, Jerome V Murphy
with refractory PME (aged ≥ 5 years), who were taking up to three antiepileptic drugs, received adjunctive zonisamide (≤ 6 mg/kg/day) therapy for 16 weeks. Myoclonic seizures were recorded daily over a 24-hour period or in 10-minute epochs in the morning, afternoon, and evening. Safety was assessed via adverse events (AEs); efficacy was measured by the percentage of patients experiencing a ≥ 50% decrease in myoclonic seizure frequency from baseline.ResultsTreatment-related AEs, experienced by 53% (n = 16/30) of patients, led to five patients discontinuing zonisamide. The most common AEs were decreased appetite, somnolence, and asthenia. Overall, 36% of patients (n = 10/28) had a ≥ 50% reduction in myoclonic seizure frequency.ConclusionsThese results suggest that zonisamide may be useful in the treatment of patients with PME. However, due to the size and open-label character of this study, further research is required.
Clinical commentary
Unpleasant auditory illusions and related avoidance behaviour in a child
Carmen Barba, Renzo Guerrini
on a 7-year-old, right-handed boy, who suffered seizures characterized by positive auditory illusions with verbal and gestural automatisms and noticeable attempts at covering his ears. Clinical evaluation and video-recording of the seizures, confirmed that most of the ictal behavior was deliberately directed at trying to prevent the unpleasant sensations reaching his ears. [Published with video sequences]
Ictal asystole in temporal lobe epilepsy before and after pacemaker implantation
Adam Strzelczyk, Sebastian Bauer, Susanne Knake, Wolfgang H Oertel, Hajo M Hamer, Felix Rosenow
or asystole is anecdotal and consists of case reports and small case series. There are no guidelines for the care of patients with ictal arrythmias. Insertion of cardiac pacemakers may prevent life-threatening cardiac arrest, syncope and trauma.We report the case of a 41-year-old man who presented with refractory partial seizures resulting in syncope leading to severe head trauma, as the only ictal semiology. During presurgical video-EEG monitoring, two episodes of ictal bradycardia followed by asystole and syncope were recorded. A cardiac pacemaker was implanted. At the nine-month follow-up, the patient reported no overt seizures, syncopes or traumatic falls. Our case demonstrates that implantation of a cardiac pacemaker while continuing AEDs may render a patient free from ictal symptoms and prevent ictal syncope and subsequent trauma. [Published with video sequences].
Hot water epilepsy: a video case report of a Caucasian toddler
Berten Ceulemans, Muriel Koninckx, Kristien Garmyn, Marek Wojciechowski
warm bath. The diagnosis of hot water epilepsy was supported by an ictal EEG. Hot water epilepsy, also known as bathing epilepsy or water-immersion epilepsy is, in the Caucasian population, a rare form of benign epilepsy, where seizures are provoked by immersion in a hot or even just a warm bath. This is the first comprehensive video publication of a seizure provoked by water-immersion in a Caucasian child. [Published with video sequences].
Seizures induced by the sight of moving water
Sebastian Bauer, Susanne Knake, Anja Haag, Anke Hermsen, Adam Strzelczyk, Hajo M Hamer, Felix Rosenow
has also been reported. We report a patient with seizures induced by the sight of moving water in the absence of bright sunlight. [Published with video sequences]
Ictal hiccup during absence seizure in a child
Athi Ponnusamy, Ganesh Rao, Peter Baxter, Patricia Field
features such as mild clonic components, change in postural tone, automatisms and autonomic phenomena. Childhood absence epilepsy (CAE) is the prototype IGE with typical absence seizures. We report a child who had prominent ictal hiccups during an absence seizure, and discuss the possible mechanisms. [Published with video sequences]
Temporal lobe neoplasm and seizures: how deep does the story go?
Lara E Jehi, Hans Ö Lüders, Richard Naugle, Paul Ruggieri, Harold Morris , Nancy Foldvary, Elaine Wyllie, Prakash Kotagal, Bill Bingaman, Dudley Dinner, Richard Prayson, Beate Diehl, Andreas Alexopoulos, Jocelyn Bautista, Robyn Busch
neighboring brain tissue. The main surgical approaches in the treatment of medically refractory epilepsy related to such developmental tumors include a lesionectomy versus a tailored cortical resection, often guided by an invasive evaluation. This case report describes the surgical management of a 26-year-old female with olfactory auras evolving into automotor seizures and convulsions, occurring in the context of a right temporo-parietal developmental lesion. It illustrates the pros and cons of various surgical approaches, and discusses some pathophysiological aspects of developmental tumors, dysplasia and epilepsy. [Published with video sequences]
Letter to the Editor
Sturge-Weber syndrome, without a facial port-wine stain, with epilepsy onset in the fifth decade
Woojun Kim, Joong-Seok Kim, Jae-Young An, Seung-Jae Lee, So-Ryeong Chung, Yeong-In Kim, Kwang-Soo Lee
