bluuebunny/crossref_metadata_2025 huggingface dataset:
Frontal lobe seizures
Authors: Canevini Maria Paola , Tassi Laura , Fusco Lucia , Giallonardo Anna Teresa , Guerrini Renzo , Mai Roberto , Rubboli Guido , Tinuper Paolo , Vigevano Federico
Published: March 2024

This video teaching course presents several patterns of frontal lobe seizures recorded in a number of Italian Epilepsy Centers. The general features of frontal lobe seizures are outlined in the introduction. Sixteen vldeo‐EEG sequences involving 10 different patients are shown. A short presentation of the clinical history precedes each case report.

The electroclinical characteristics of seizures implicating the frontal lobe are extremely variable. According to the literature, general characteristics allowing differentiation between seizures of different topography include: appearance of seizures in clusters; seizures predominantly nocturnal or while the patient is falling asleep; short duration of each episode (approximately 30 s); abrupt onset and end; relatively short post‐ictal phase, when present; frequent secondary generalization and falls; frequent subjective sensations, usually difficult to describe; impairment of consciousness is not a constant feature; episodes of status are frequent; differentiation from pseudo‐seizures may prove to be difficult.

When confirmation of the epileptic nature of paroxysmal phenomena is needed, video‐EEG recordings may be of some help.

Possible subjective manifestations are: cephalic and neuro‐vegetative (even epigastric) manifestations, as well as
“pensée forcée”
, fear, olfactory hallucinations, non‐lateralized visual disturbances. Motor manifestations include tonic, dystonic, postural, oculocephalic deviations and falls.

Verbal and more often gestural automatisms can be complex, very brief and often violent, but elementary automatisms, such as automatic hand and mouth movements, or bimanual and pedaling movements, are also frequent.

In this video teaching course, we first present seizures featuring minimal motor signs, followed by seizures with a more complex semiology. The most significant clinical and EEG features are discussed. (Published with videosequences.)
Influence du pattern EEG critique sur les variations du débit sanguin cérébral, mesurées par tomographie d'émission monophotonique
Authors: Biraben Arnaud , Bernard A. , Seigneret Éric , Scarabin Jean‐Marie
Published: March 2024

Les corrélations anatomo‐électro‐cliniques établies par stéréo‐électro‐encéphalographie (SEEG) ou Electro‐cortico‐graphie (EcoG) sont les techniques de référence pour déterminer la zone épileptogène (ZE) chez un patient épileptique, ces techniques prennent en compte la dimension temporelle de la crise. D'introduction plus récente, la tomographie d'émission monophotonique (TEMP) ictale permet elle aussi d'établir des corrélations anatomo‐cliniques lors des crises d'épilepsie. La TEMP est un reflet du débit sanguin cérébral pendant la crise, ces variations importantes de débit pendant les crises sont liées à l'activité électrique locale. Peu d'études sont consacrées à l'étude des liens unissant activité électrique et débit sanguin chez l'homme dans ces conditions. Globalement il est possible d'en retirer que si l'injection est faite pendant la décharge électrique ictale, une hyperperfusion est observée localement, cette hyperperfusion intéresse les régions où la décharge a débuté ainsi que les régions où elle s'est secondairement propagée. Si l'injection est plus tardive, une hypoperfusion apparaît dans ces régions, ayant aussi une bonne valeur localisatrice. Le
timing
de l'injection est donc fondamental à l'interprétation.
Retentissement cognitif de l'épilepsie à pointes centro‐temporales
Authors: de Saint‐Martin Anne , Seegmuller Caroline , Carcangiu Roberta , Kleitz Catherine , Hirsch Édouard , Marescaux Christian , Metz‐Lutz Marie‐Noëlle
Published: March 2024

Les épilepsies focales liées à l'âge et notamment l'épilepsie à paroxysmes rolandiques (EPR) ou à pointes centro‐temporales (EPCT) sont considérées comme étant d'excellent pronostic avec une disparition spontanée des crises et des anomalies électroencéphalographiques (EEG) à l'adolescence. L'individualisation de ce syndrome par Beaussart et Nayrac en 1959 a été une avancée considérable en épileptologie infantile: il existait des épilepsies focales non lésionnelles, «bénignes», d'évolution spontanément favorable. Depuis, ont été conduites plusieurs études portant sur le développement cognitif et les apprentissages de ces enfants au cours de la maladie, apportant des résultats hétérogènes. Toutes les études s'accordent à dire cependant que les enfants atteints d'épilepsie à paroxysmes rolandiques gardent une efficience intellectuelle normale au cours de leur évolution et un excellent pronostic à long terme. Néanmoins, chez certains d'entre eux, peuvent apparaître un déficit oromoteur transitoire, des troubles neuropsychologiques spécifiques, ou des troubles attentionnels retentissant sur les apprentissages scolaires, au cours de la phase active de la maladie. L'analyse des corrélations neuropsychologiques et neurophysiologiques met en évidence un effet significatif de la latéralité du foyer pour de rares épreuves spécifiques. La présence prolongée d'un foyer lent intercritique et de pointe‐ondes amples et très activées au sommeil sont associées de façon significative à une baisse des performances cognitives et à des troubles attentionnels. Ces anomalies sont observées essentiellement au cours des formes dites sévères ou atypiques. Les premières études longitudinales confirment le caractère transitoire de ces déficits. Ainsi l'existence d'un foyer épileptique actif, en l'absence de toute lésion, ou même de traitement, interfèrerait avec la maturation de fonctions cognitives en cours de développement.
Idiopathic generalised epilepsy with phantom absences and absence status in a child
Authors: Panayiotopoulos Chrysostomos P. , Ferrie Colin D. , Koutroumanidis Michael , Rowlinson Shaun , Sanders Sue
Published: March 2024

A syndrome of idiopathic generalised epilepsy with phantom absences of undetermined onset has been recently described. This syndrome clinically becomes apparent in adulthood with generalised tonic clonic seizures and frequently absence status epilepticus. We report an 11 year‐old normal girl with frequent episodes of absence status and no other overt clinical manifestations. However, appropriate video‐EEG recordings documented that she had frequent absence seizures that were so mild as to escape recognition by her and the parents. These consisted of mild impairment of cognition and eyelid fluttering during brief generalised discharges of spike/multiple spike and slow waves. No further seizures occurred and the EEG normalised after appropriate drug treatment. Thus, it appears that this syndrome of phantom absences and absence status may start much earlier, in late childhood. Appropriate video‐EEG documentation is needed for the recognition of these patients that may be more common than it appears from the few published cases (
with Video
).
Intraoperative methods for confirmation of correct placement of the vagus nerve stimulator
Authors: Vaughn Bradley V. , Bernard Estrada , Lannon Susan , Mann Billie , D'Cruz O'Neill F. , Shockley William , Passanante Anthony
Published: March 2024

Vagus nerve stimulation is a progressive therapy for intractable epilepsy. Variations in cervical anatomy can complicate localization of the vagus nerve and may lead to inappropriate placement of the stimulator leads. We have developed two intraoperative techniques that improve correct identification of the vagus nerve. Both of these techniques utilize the co‐localization of the recurrent laryngeal nerve with the vagus nerve. For patients undergoing stimulator placement with regional and local anesthesia, the stimulator current intensity is increased until alteration of voice can be confirmed with a voice test. Patients undergoing general anesthesia can also be tested by direct stimulation of the isolated vagus nerve. Utilizing visualization of the larynx and vocal cords via fiberoptic endoscopy, direct stimulation of the vagus nerve will produce a contraction of the left lateral wall of the larynx and tightening of the left vocal cord. Neither of these procedures produce any untoward effects for the patients. We have found these methods improve our ability to confirm correct placement of the stimulator with minimal increase in operative time (
with Video
).
Comparison of twice‐ and three times daily tiagabine for the adjunctive treatment of partial seizures in refractory patients with epilepsy: an open label, randomised, parallel‐group study
Authors: Biraben A. , Beaussart M. , Josien E. , Pestre M. , Savet J.‐F. , Schaff J.‐L. , Tourniaire D. , Sevestre M. , Renault‐Djouadi J.
Published: March 2024

A multicentre, open label, randomised, parallel group study compared the efficacy, tolerability and safety of two dosing regimens, t.i.d. and b.i.d., of tiagabine as an adjunctive therapy for the treatment of refractory patients with partial seizures. A total of 347 patients were randomised and treated (175 t.i.d. and 172 b.i.d.). Each group was administered the same daily dose of tiagabine incremented stepwise during a 12‐week fixed‐schedule titration period to a target dose of 40 mg/day. The patients were followed for a further 12‐week flexible continuation phase. A significantly greater number of patients in the t.i.d. group completed the fixed schedule titration period (81.4%
versus
73.1%; 95% CI of
odds ratio
= 0.331, 0.970; p = 0.038). The proportion of responders (patients showing at least a 50% decrease in all‐seizure frequency from baseline) was similar for both groups (42.3% for b.i.d. and 47.1% for t.i.d.) during the last 8 weeks of the flexible phase and seven (4.0%) patients in the b.i.d. group were seizure‐free compared to 14 (8.1%) patients in the t.i.d. group. Adverse events were of similar incidence in both groups, and mainly occurred during the fixed schedule titration period; they were mainly mild and CNS‐related with somnolence being the most frequently reported.

Conclusion: Tiagabine was effective as add‐on treatment of refractory partial seizures. Although both regimens appear to offer a similar efficacy and safety profile, significantly more patients completed the study in the t.i.d. group compared to b.i.d., probably as a consequence of a lesser tolerability when high doses are given undivided. These results confirm that a slow titration and appropriate adjustment of dosing are essential conditions to ensure optimal use of tiagabine.
Sleep‐related, low voltage Rolandic and vertex spikes: an EEG marker of benignity in infancy‐onset focal epilepsies
Authors: Bureau Michelle , Cokar Ozlem , Maton Bruno , Genton Pierre , Dravet Charlotte
Published: March 2024

Although benign forms of epilepsies with onset in infancy have recently been recognized, the occurrence of seizures in an infant or very young child is very often an event of great significance and the prognosis concerning both epilepsy and neuropsychological development must be guarded. No reliable clinical or electroencephalographic (EEG) markers that can predict the outcome have been described. In a retrospective series of 10 patients, we found a peculiar EEG pattern seen across sleep stages, but not in the waking state in infants whose first seizures appeared before the age of 1 year, and which were mostly complex focal. In all cases, follow‐up showed a favourable outcome with complete seizure remission and no cognitive impairment. The specificity of these EEG changes was 100%, but the sensitivity was lower, since they were not seen in some of the infants with the same favourable outcome. We discuss the clinical similarities between our patients and those cases reported earlier by other authors as benign, non‐familial or familial focal epilepsies, in whom, however, no interictal abnormalities had been seen on the EEG. Such EEG changes are probably specific to benign, self‐limited, early onset focal epilepsies.
Epileptic nystagmus: A case study video‐EEG correlation
Authors: García‐Pastor Andrés , López‐Esteban Pilar , Peraita‐Adrados Rosa
Published: March 2024

Epileptic nystagmus (EN) is an uncommon phenomenon characterized by repetitive and rapid saccades, in association with epileptic discharges. We present a critical video‐EEG recording in a patient with occipital seizures that appeared clinically as EN.

The subject, male, 70 years‐old, was examined because of generalized tonic‐clonic seizures, preceded by left cephalic version. These were controlled using i.v. PHT, but partial seizures persisted, which we recorded using video‐EEG. Clinically, we observed episodes of left conjugate deviation of the eyes, accompanied by horizontal nystagmoid movements, with a rapid leftward component and visual hallucinations. The patient did not lose consciousness. Ictal EEG: spike rhythm in the posterior right occipito‐temporal region extending to adjacent and contralateral regions, followed by post‐discharge of slow waves. The video‐EEG was interpreted as partial oculo‐clonic status epilepticus of right temporo‐occipital origin. Cranial MRI: old, bilateral hemorrhaging occipital contusions associated with previous cranial injury. The picture persisted for two days, and disappeared with administration of CBZ 600 mg/d.

Our patient's nystagmus seemed to be related to the critical activity recorded in the right occipito‐temporal region. The co‐existence of visual hallucinations and the video‐EEG correlation support this possibility. This phenomenon is probably due to epileptic activation of the cortical center of saccadic movements, with a rapid phase of nystagmus, contralateral to the focus, and a slow ipsilateral phase in association with a defect in the gaze‐fixing system (“leaky neural integrator”) [published with videosequences].
Misleading lateralization by ictal SPECT in temporal lobe epilepsy – a case report
Authors: Janszky J. , Holló A. , Barsi P. , Rásonyi G. , Er⊚Tss L. , Kalóczkai A. , Halász P.
Published: March 2024

A 28‐year‐old woman with temporal lobe epilepsy underwent presurgical evaluation. Scalp‐EEG showed non‐localizing seizure patterns. MRI revealed a right hippocampal sclerosis. Ictal HMPAO‐SPECT showed a marked left temporal hyperperfusion. Video‐EEG monitoring with foramen ovale electrodes (FOE) showed an initial seizure pattern which appeared in the right FOE and which shifted to the left 8 s after clinical onset. Three years after a right temporal lobectomy, the patient is seizure‐free. In conclusion, although the ictal SPECT suggested a left temporal seizure focus, the intracranial EEG and the postoperative seizure‐freedom confirmed the right‐sidelocation of the epileptogenic region. A rapid right‐left seizure spread explains the mechanism of falsely lateralizing ictal SPECT.
Anatomy of the temporal pole region
Authors: Chabardès Stephan , Kahane Philippe , Minotti Lorella , Hoffmann Dominique , Benabid Alim‐Louis
Published: March 2024

The temporopolar region is not clearly defined from an anatomical point of view. A line going through the rostral area of the inferior temporal, occipito temporal and superior temporal sulci is considered to represent its posterior limit on the lateral and inferior sides. On the internal side, this posterior limit corresponds to the rhinal sulcus, an anterior and internal extention of the collateral sulcus. From a cyto‐architectonic point of view, the temporopolar region is caracterized by a dysgranular paralimbic cortex which ensures the transition between allo‐ and isocortical areas. The temporal pole is mainly connected with the amygadala, the hippocampus, the superior temporal gyrus, and the occipitobasal cortex, but also with the orbitary gyrus and the insula with which it forms the insulo‐orbito‐polartemporo‐complex.
Direct electrical stimulations of the temporal pole in human
Authors: Ostrowsky Karine , Desestret Virginie , Ryvlin Philippe , Coste Sébastien , Mauguière François
Published: March 2024

We report the results of 105 intracortical electrical stimulations of the temporo‐polar cortex performed in 30 patients during stereo‐electroencephalography (SEEG) investigation of drug‐resistant temporal lobe epilepsy. Clinical responses were elicited in 50% of the patients during 26 of the 105 stimulations. Responses are coherent with data found in the literature with mostly psychic, viscero‐sensitive, autonomic and viscero‐motor phenomena being elicited. Nearly all responses were evoked by stimulation of the antero‐medial part of the temporo‐polar cortex whereas only once was a clinical response elicited during stimulation of the lateral part of the temporo‐polar cortex. Induced auras were more frequently reported when there was an afterdischarge associated to the clinical response (60 and 75% of the stimulation followed by a localized or a diffusing after‐discharge, respectively) then when there wasn't any after‐discharge following stimulation. The clinical responses evoked during stimulation of the temporo‐polar cortex only have a limited topographic specificity since they closely resemble symptoms elicited by stimulation of other limbic or paralimbic areas. Nonetheless these results do suggest that the antero‐medial part of the temporo‐polar cortex is included in the symptomatogenic zone in so‐called “temporo‐limbic” epilepsies.
Developmental aspects of the basal ganglia and therapeutic perspectives
Authors: Velíšková Jana , Claudio Olga I. , Galanopoulou Aristea S. , Kyrozis Andreas , Lado Fred A. , Ravizza Teresa , Velíšek Libor , Moshé Solomon L.
Published: March 2024

Development and sex hormones play an important role in the expression of seizures. Sex‐specific differences in the development of seizure‐suppressing neuronal networks may account, at least in part, for age‐ and sex‐related susceptibility to seizures. The substantia nigra pars reticulata is a site involved in the control of seizures. In adult male rats, there are two distinct GABA
A
sensitive regions within the substantia nigra pars reticulata, which mediate opposite effects in flurothyl seizures. Muscimol infused into the anterior region is anticonvulsant while similar infusions into the posterior region are proconvulsant. These two regions differ morphologically, and utilize different efferent networks. In contrast, in postnatal day 15 male rats, there is no such differentiation and muscimol infusions have only proconvulsant effects.

The hallmark of the female substantia nigra pars reticulata is the fact that muscimol‐mediated proconvulsant effects cannot be demonstrated in any region at any age. The sex‐related difference in nigral seizure control may be related to the lack of testosterone in females. Accordingly, neonatal castration of males results in the loss of the “proconvulsant” region. The
“male”
type of the substantia nigra pars reticulata effects can be induced by exogenous testosterone administration in neonatally castrated male or in female rats. The phenotype of nigral GABAergic neurons, as characterized by GABA
A
receptor subunit composition, muscimol‐induced electrophysiological responses, and connectivity of output networks may each be altered by the presence of testosterone. Better understanding of the influence of the endocrine system on brain development and neuronal activity may provide new insight into the treatment of age‐ and sex‐dependent seizure disorders.
Deep brain stimulation in epilepsy with particular reference to the subthalamic nucleus
Authors: Chabardès Stéphan , Kahane Philippe , Minotti Lorella , Koudsie Adnan , Hirsch Edouard , Benabid Alim‐Louis
Published: March 2024

Alternative methods, for the treatment of medically refractory epileptic patients, who cannot be treated by resective surgery, such as chronic deep brain neurostimulation, are under development. Such methods have been used in the cerebellum, various thalamic nuclei, and in the caudate nucleus. In Grenoble, encouraged by the suppressive effects of pharmacological or electrical inhibition of the STN on different types of seizure in animal models of epilepsy, and by our experience with STN high frequency stimulation (HFS) in patients with movement disorders, we have evaluaded the high frequency stimulation of the subthalamic nucleus (STN HFS). STN HFS was performed in five patients suffering from medically intractable seizures and considered unsuitable for resective surgery. A 67% to 80% reduction in seizure frequency was observed in three patients, with a partial symptomatic epilepsy of the central region. An additional patient suffering from severe myoclonic epilepsy (Dravet syndrome) also responded to STN HFS, with a weaker reduction of seizure frequency. The fifth patient who suffered from an autosomal dominant frontal lobe epilepsy with insulo‐frontal seizures did not show any improvement. These results suggest that stimulation of STN could be a promising treatment for patients with drug‐resistant epilepsy who would not benefit from conventional surgery.
MRI evidence for the involvement of basal ganglia in epileptic seizures: an hypothesis
Authors: Dematteis Maurice , Kahane Philippe , Vercueil Laurent , Depaulis Antoine
Published: March 2024

Recent clinical and experimental studies have suggested that the basal ganglia are involved in epileptic seizures as a propagation pathway or as a remote inhibitory control circuit. The present case report may provide additional evidence from post‐ictal magnetic resonance imaging (MRI) supporting this hypothesis. A healthy 13 year‐old boy was admitted for a complex partial status epilepticus. MRI, performed one week later, revealed bilateral T2 hyperintense signals in the striata and a left temporal arachnoid cyst. Left temporal slow waves were noted on EEG recording. No obvious metabolic alterations were identified. During the next six years of follow‐up, no seizure occurred and striatal alterations progressively disappeared. The clinical characteristics of the seizures, EEG slow waves, and probably the presence of an arachnoid cyst suggest that seizures originated from the left temporal lobe. The long‐lasting MRI changes suggest that bilateral striatal alterations may have been secondary to an inflammatory process, which in turn could have disrupted a striatal inhibitory control over seizures. On the basis of these arguments, we speculate involvement of basal ganglia in epileptic seizures, as a part of a modulatory control system over seizures rather than a propagation pathway. Future reports will support or invalidate our hypothesis.
The anatomy and embryology of the hypothalamus in relation to hypothalamic hamartomas
Authors: Freeman Jeremy L.
Published: March 2024

The hypothalamus is involved in a variety of autonomic, endocrine, neurological and behavioural functions including temperature, osmostatic and autonomic nervous system regulation, pituitary, thyroid, adrenal and gonadal control, thirst, appetite and weight control, memory and emotional behaviour including aggression and laughter, and biological (circadian) rhythms. The functional anatomy of the hypothalamus and its major afferent and efferent neurological connections are described, with particular reference to hypothalamic hamartomas (HH), gelastic seizures, MRI of the hypothalamus, and potential effects of surgery for HH. Normal development of the hypothalamus is reviewed in relation to models of forebrain development, descriptive hypothalamic embryology and the importance of known transcription factors. Potential environmental antecedents to HH development are discussed, and the significance for sporadic, isolated HH of several syndromes associated with HH is explored.
Cortical development and focal cortical dysplasia
Authors: Bentivoglio Marina , Tassi Laura , Pech Emanuel , Costa Catarina , Fabene Paolo F , Spreafico Roberto
Published: March 2024

A brief survey of cortical development is presented, focusing on neuronal migration and its alterations. Corticogenesis is achieved through ordered temporospatial steps, via the formation of transient structures, and successive waves of cell proliferation and migration (followed by cell differentiation and maturation), and apoptotic cell death. The appearance of the proliferative ventricular zone and marginal zone, and of the superficial primordial plexiform layer, is followed by the formation of the prospective layer I, of the subplate, whose neurons are destined to die, and of the cortical plate that will give rise to layers II‐VI. Cells arising in the ventricular zone migrate radially using radial glia as a scaffold, and are destined to form pyramidal cells. Cortical interneurons are mainly generated in the ganglionic eminence and migrate along axonal substrates following tangential routes. Disorders of this complex process lead to a wide range of alterations, and focal derangements of cortical organization have been grouped under the term focal cortical dysplasia (FCD). As the result of a neuropathological revision of FCD cases with intractable epilepsy, a novel classification comprising three subgroups of FCD has been introduced, and is supported by electroclinical and neuroimaging data, as well as by the postsurgical outcome of patients: i) architectural dysplasia, characterized by altered cortical lamination; ii) cytoarchitectural dysplasia, with the occurrence of giant neurons besides cortical dyslamination; iii) Taylor‐type cortical dysplasia, in which altered cortical lamination is consistently associated with the occurrence of giant, dysmorphic and ectopic neurons, and frequently with the so‐called balloon cells.
Epilepsy and hypothalamic hamartoma: look at the hand Pallister‐Hall syndrome
Authors: Kremer Stéphane , Minotti Lorella , Thiriaux Anne , Grand Sylvie , Satre Véronique , Bas Jean‐Francois Le , Kahane Philippe
Published: March 2024

We report the case of a 29‐year‐old patient, who suffered from drug resistant laughing seizures since childhood. The clinical examination was normal, except for sequelae of hand and feet surgery during infancy for post‐axial polydactyly. Cerebral MRI showed a hypothalamic hamartoma. The association of complex limb abnormalities with hypothalamic hamartoma lead to the diagnosis of Pallister‐Hall syndrome.

This syndrome is related to a mutation of gene GLI3, located on chromosome 7p13, and its inheritance is autosomal dominant.

In the case of laughing seizures, a cerebral MRI should be performed to look for a hypothalamic hamartoma. The observation of such lesions indicates the necessity of standard radiographies of the hands and feet, to search for associated abnormalities. These findings might help to recognize a Pallister‐Hall syndrome, thus allowing genetic counseling.
Functional neuroimaging of malformations of cortical development
Authors: Cross Judith Helen
Published: March 2024

Functional neuroimaging over the past 10 years has led to greater insights into the pathophysiology underlying symptomatic epilepsy. Such imaging has been used to localize cerebral dysfunction, predominantly through disturbances in metabolism or blood flow. Techniques available include single photon emission computed tomography (SPECT), positron emission tomography (PET) and functional magnetic resonance imaging (fMRI). Although the use of these diagnostic techniques is widely reported for presurgical evaluation, there has been little work with specific reference to malformations of cortical development.
Anoxic‐epileptic seizures: home video recordings of epileptic seizures induced by syncopes
Authors: Stephenson John , Breningstall Galen , Steer Chris , Kirkpatrick Martin , Horrocks Iain , Nechay Alla , Zuberi Sameer
Published: March 2024

Occasionally, but more often than has been reported, true epileptic seizures are triggered by non‐epileptic syncopes. This combination of syncope and epileptic seizure has been called an anoxic‐epileptic seizure. A few examples of such anoxic‐epileptic seizures, including the induction of status epilepticus, have been reported in books and medical journals, but no video‐recordings have been published. We show here home video recordings of the first three known examples of the transition from the triggering syncope and anoxic seizure, to the subsequent epileptic seizure. In the first two children, a neurally‐mediated syncope, probably mediated by prolonged expiratory apnoea (so‐called breath‐holding spells), induces a long, clonic epileptic seizure with some features of myoclonic absence. In the third example, a compulsive Valsalva in an older autistic child provokes a vibratory tonic epileptic seizure. In addition, we show two further video clips of the most usual type of epileptic seizure induced by syncopes in very young children. In one, the video recording begins after the end of the triggering syncope and shows a rhythmic clonic seizure that includes repetitive vocalizations. The final recoding is of a spontaneous epileptic seizure with features of myoclonic absence: this child had both epilepsy and identical episodes induced by syncopes, that is, anoxicepileptic seizures. Not only paediatricians and paediatric neurologists, but also adult neurologists and epileptologists in general, should be aware of the important clinical scenario of true epileptic seizures induced by syncopes. This phenomenon is not considered in any international classification. (Published with videosequences)
Antiepileptic drugs: indications other than epilepsy
Authors: Spina Edoardo , Perugi Giulio
Published: March 2024

Antiepileptic drugs (AEDs) are increasingly used for the treatment of several non‐epileptic neurological conditions and psychiatric disorders. Most of the information available on the use of these agents in clinical disorders outside epilepsy is from case series, uncontrolled studies or small randomised clinical trials, and their apparent efficacy requires confirmation through well designed, large, phase III trials.

With regard to neurological conditions other than epilepsy, experimental evidence for the efficacy of AEDs is only available for the treatment of patients with trigeminal neuralgia, neuropathic pain syndromes, migraine and essential tremor. Carbamazepine is commonly prescribed as first‐line therapy for patients with trigeminal neuralgia. Gabapentin has been recently marketed for the management of neuropathic pain syndromes, particularly diabetic neuropathy and postherpetic neuralgia. Valproic acid (sodium valproate), in the form of divalproex sodium, is approved for migraine prophylaxis. Primidone can be considered a valuable option for the treatment of essential tremor.

AEDs are also used to treat psychiatric conditions, in particular bipolar disorder. So far, the most commonly utilized AEDs in the treatment of this disorder have been carbamazepine and valproic acid, which have showed an antimanic efficacy and a probable long‐term, mood‐stabilizing effect in many bipolar patients, including those refractory or intolerant to lithium. The availability of a new generation of AEDs has broadened the therapeutic options in bipolar disorder. Lamotrigine, oxcarbazepine, gabapentin and topiramate appear to be promising in the treatment of refractory bipolar disorder, as a monotherapy as well as in combination with traditional mood stabilizers. In addition, newer AEDs appear to have a more favourable tolerability and drug interaction profile as compared to older compounds, so thus improving compliance to treatment.
Épilepsie et retard psychomoteur
Authors: Bednarek Nathalie , Motte Jacques
Published: March 2024

Le retard mental peut être associé à une épilepsie. Cette dernière peut constituer la cause du retard des acquisitions (encéphalopathie épileptogène) ou bien n'être qu'une traduction supplémentaire conséquence d'une pathologie neurologique sous jacente commune (encéphalopathie avec épilepsie). Elle n'est alors pas seule responsable du retard. Dans le cadre des encéphalopathies avec épilepsie une démarche diagnostique rigoureuse est nécessaire avec notamment un examen cutané (syndromes neuro‐cutanés), un examen moteur (séquelles anoxo‐ischémiques de la période périnatale), un EEG, une imagerie par résonance magnétique (IRM) cérébrale ainsi que la recherche d'anomalies associées (cardiaque, rénale…). D'autre part il existe des encéphalopathies épileptogènes telles que les syndromes liés à l'âge : syndrome hémiconvulsions, hémiplégie, épilepsie (HHE) ; syndrome de Rasmussen, épilepsie myoclonique sévère du nourrisson (syndrome de Dravet), épilepsie myoclono‐astatique (Doose) que nous développerons comme exemples. L'âge, le type de crises et l'EEG sont utiles pour le diagnostic dans ce cas, les autres examens étant généralement peu contributifs. Retard et épilepsie sont souvent associés et la première étape du diagnostic est d'essayer de comprendre si l'épilepsie est une cause active du désordre cognitif ou non, afin d'affiner la démarche étiologique mais aussi d'adapter la prise en charge thérapeutique.
Les épilepsies myocloniques progressives : mythe ou réalité ?
Authors: Genton Pierre , Bureau Michelle
Published: March 2024

Les épilepsies myocloniques progressives (EMP) sont des maladies rares, et leur individualisation comme catégorie diagnostique et pronostique, bien qu'elle ait été reconnue par la dernière proposition de classification de la Ligue internationale contre l'épilepsie, peut sembler bien théorique à des cliniciens qui ont peu de chances d'en rencontrer dans leur pratique. Les EMP se définissent par l'association : 1) d'une épilepsie de type généralisé (bien que des crises focales soient possibles dans certaines étiologies), 2) d'un syndrome myoclonique souvent invalidant et 3) d'autres atteintes neurologiques (ataxie, démence, atteintes sensorielles), qui varient énormément en fonction de l'étiologie. Il s'agit en effet d'un groupe hétérogène de maladies répondant à des étiologies diverses. Leur prévalence varie en fonction du contexte ethnique et elles peuvent être relativement fréquentes sous certains climats, en raison de facteurs génétiques (isolat et/ou consanguinité, par exemple, pour les formes liées à une transmission récessive). Les anomalies génétiques et biochimiques responsables des EMP sont de mieux en mieux connues. Il faut, de nos jours, aborder le diagnostic des EMP de manière rationnelle, en tenant compte du contexte ethnique et génétique, de l'âge de survenue et du poids respectif des différents symptômes. Des outils thérapeutiques originaux sont en cours de développement et les traitements purement symptomatiques disponibles actuellement sont efficaces s'ils sont bien utilisés. Il est donc possible de reconnaître les EMP comme un groupe de maladies s'exprimant préférentiellement soit par une épilepsie, soit par un mouvement anormal, soit encore par des symptômes neurologiques ou cognitifs, et de prendre en charge ces affections rares de manière logique et globale.
Startle‐induced seizures associated with infantile hemiplegia: implication of the supplementary motor area
Authors: Nolan Melinda A. , Otsubo Hiroshi , Iida Koji , Minassian Berge A.
Published: March 2024

This case illustrates an uncommon form of symptomatic startle‐induced epilepsy associated with infantile hemiplegia. Seizure semiology, neuroimaging and neurophysiological findings support involvement of the supplementary motor area in the generation of this seizure type. We present the case of an 11‐year‐old girl with an uncommon form of startle‐induced seizures, illustrated on video‐EEG, against the background of left infantile hemiplegia associated with extensive right hemispheric porencephaly but preserved cognitive functioning. The epileptic focus appears to be in the dorsolateral frontal lobe, with seizure semiology involving the supplementary motor cortex. [Published with videosequences]
Epilepsy in middle‐aged and elderly people: a three‐year observation
Authors: Paradowski Boguslaw , Zagrajek Mieszko M.
Published: March 2024

An analysis of the medical documentation and investigation of 130 cases of epilepsy diagnosed in a group of people over 50 years of age (average: 65.4 years) revealed that the most common type of seizure in the group studied was partial (66.2%), followed by seizures with secondary generalization (33.8%). Epilepsy was caused by cerebrovascular disease (50.8%) considerably more often in patients over 74 years of age, craniocerebral trauma in patients addicted to alcohol (13.1%), especially those under 65 years of age, primary or metastatic neoplastic disease (10.7%), and others. The authors wish to draw attention to the leukoaraiosis factor, which might be the proepileptogenic cause of epilepsy recognized in the group of patients over 74 years of age (56.5%) and is much more frequent in this group than in the group of patients under 65 years of age (1.6%). Moreover, some drugs, such as L‐dopa and Baclofen, might have been related to the epileptic seizures. In 29 patients (22.3%) the definite cause of late‐onset epilepsy was unknown. The authors suggest in such cases, both follow – up tomographic examination and careful clinical examinations. In the study group of patients with initially unknown seizure etiology, some diseases, such as cerebral tumor or colon and pancreatic neoplasm, were diagnosed during follow‐up examination. These processes were revealed several months after the first epileptic seizure.
A semiological classification of status epilepticus
Authors: Lüders H.O. , Rona S. , Rosenow F. , Arnold S. , Carreño M. , Diehl B. , Ebner A. , Fritsch B. , Hamer H. , Holthausen H. , Knake S. , Noachtar S. , Pieper S. , Tuxhorn I.
Published: March 2024

To the Editor:We read with great interest the thoughtful review of Prof Simon Shorvon of our paper titled «A semiological classification of status epilepticus» (Epileptic Disord2005; 7: 1‐3). Prof Shorvon is one of the world experts in status epilepticus, and this is certainly reflected in this insightful review.
Ictal dysprosody and the role of the non‐dominant frontal operculum
Authors: Montavont Alexandra , Demarquay Geneviève , Guenot Marc , Isnard Jean , Mauguière François , Ryvlin Philippe
Published: March 2024

Prosody is an important feature of language and refers to variations in the acoustic properties of timing, intensity and fundamental frequency that are used to convey affective or linguistic information. The prosodic component of ictal speech has not yet been specifically studied in epileptic patients. We report the case of a patient with right mesial temporal lobe epilepsy who developed ictal, recurrent speech utterances associated with an altered prosody during both spontaneous seizures and a very localized stimulation‐induced discharge of the non‐dominant precentral operculum, a finding consistent with results of functional magnetic resonance imaging studies in healthy subjects. This should prompt future studies, with the aim of better evaluating the localizing value of ictal dysprosody in patients with drug resistant, partial epilepsy.
[Published with videosequences]
A case of systemic lupus erythematosus with complex partial status epilepticus
Authors: Tsuji Masayasu , Tanaka Hisashi , Yamakawa Masahito , Sagawa Ryuichi , Azuma Hideki
Published: March 2024

We describe a woman with systemic lupus erythematosus who had complex partial status epilepticus as the sole epileptic manifestation. The patient presented with a confusional state, initially considered to be psychotic in nature, due to SLE. However, the EEG demonstrated the epileptic etiology of her symptoms. In patients with lupus presenting with prolonged episodes of confusion and behavioural disturbance, an EEG should be performed, whenever possible during the episode, even in the absence of a previous history of epilepsy.
Can drug resistance in epilepsy be minimized? Challenging commonly held beliefs
Authors: Perucca Emilio
Published: March 2024

Until more efficacious antiepileptic drugs (AEDs) that can tackle the challenge of drug‐refractory epilepsy are developed, the best way to minimize inadequate seizure control is to exploit at best available treatments. There are however, discrepancies between commonly held opinions on several aspects of drug therapy, and the body of scientific evidence which exists to support them. This article highlights a few examples, discussing evidence that, contrary to common belief, (i) a significant proportion of patients with newly diagnosed epilepsy respond to concentrations of AEDs below the “therapeutic range” quoted in the literature; (ii) only a small group of patients unresponsive to low to moderate AED dosages become seizure‐free after increasing dosage up to the limit of tolerability; (iii) knowledge of mechanisms of AED action can aid in the rational use of AEDs in the clinic; (iv) monitoring serum levels of new generation AEDs can be usefully exploited to improve management, and (v) at least in a subgroup of patients, successful epilepsy surgery cannot be regarded as curative, because seizure control may be dependent upon continuation of AED therapy. It is hoped that increased awareness of these issues could eventually contribute not only to improved clinical outcome, but also to high quality studies in many areas where gaps in knowledge prevent application of truly evidence‐based management.
Epilepsy and common comorbidities: improving the outpatient epilepsy encounter
Authors: Gilliam Frank G. , Mendiratta Anil , Pack Alison M. , Bazil Carl W.
Published: March 2024

Epilepsy is a chronic disorder that has been associated with other specific health problems. Evidence from recent clinical and basic investigations indicates that aspects of cerebral dysfunction associated with a lowered seizure threshold may also predispose toward other disorders such as depression, cognitive impairment, sleep disorders, and migraine. Similarly, certain types of brain injury may also increase the risk of adverse antiepileptic drug (AED) effects. For example, a history of febrile seizures is associated with a three fold increase in the occurrence of negative psychiatric effects of two newer AEDs. Poor fitness and obesity are also reported at higher rates in epilepsy. Some comorbid conditions in epilepsy, such a depression and anxiety, may have a greater influence on subjective health status than does seizure rate. Management strategies employed in the outpatient clinic to maximize overall health outcomes should include screening and treatment for the commonly coexistent conditions in persons with epilepsy.
EEG and seizure exacerbation induced by carbamazepine in Panayiotopoulos syndrome
Authors: Kikumoto Kenichi , Yoshinaga Harumi , Oka Makio , Ito Minako , Endoh Fumika , Akiyama Tomoyuki , Ohtsuka Yoko
Published: March 2024

We report on a 4‐year 8‐month‐old boy with Panayiotopoulos syndrome who showed atypical evolution with newly developed absence seizures and EEG exacerbation induced by carbamazepine. 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.
Sleep‐related laryngospasm: a video‐polysomnographic recording
Authors: Iriarte Jorge , Urrestarazu Elena , Alegre Manuel , Goñi Concepción , Viteri César , Artieda Julio
Published: March 2024

We present a case of laryngospasm in a 12‐year‐old male who experienced sudden, nocturnal episodes of breathing difficulties and agitation. Apart from laryngospasm, the main differential 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]
Non‐epileptic clinical diagnoses in children referred for an outpatient EEG using video monitoring
Authors: Apakama Okwuchi , Appleton Richard
Published: March 2024

Simultaneous video (closed circuit television [CCTV]) and EEG recordings are important in the differentiation of epileptic and non‐epileptic paroxysmal episodes and in the classification of epilepsy syndromes. An additional benefit from the observation of the child on CCTV is the possible identification of specific clinical, including genetic, conditions. This three‐year prospective study of 2780 consecutive children undergoing routine EEG investigations identified 17 conditions that had not previously been diagnosed by the clinicians who had requested the EEG.
Status epilepticus in a patient with fragile X syndrome: electro‐clinical features and peri‐ictal neuroimaging
Authors: Di Bonaventura Carlo , Mari Francesco , Pierallini Alberto , Mecarelli Oriano , Randi Franco , Manfredi Mario , Prencipe Massimiliano , Giallonardo Anna Teresa
Published: March 2024

Fragile X syndrome (Fra‐X) is a common cause of mental retardation that can be associated with partial epilepsy characterized by a variety of electro‐clinical features. A wide spectrum of interictal activities are reported, although no data regarding ictal EEG activity have as yet been published. Drug‐resistant seizures are uncommon, and the occurrence of clustering seizures or status epilepticus has only been reported anecdotally. We describe a Fra‐X patient with refractory partial epilepsy related to a malformation of cortical development who experienced a partial status epilepticus that was well documented by video‐EEG and MRI. We report the electro‐clinical features and peri‐ictal neuroimaging data.
Gelastic seizures involving the right parietal lobe
Authors: Shin Hee‐Young , Hong Seung Bong , Joo Eun Yeon , Tae Woo Suk , Han Sun Jung , Cho Jae Wook , Seo Dae Won , Kim Sun Hyung , Lee Jong‐Min , Kim Sun I
Published: March 2024

Gelastic seizures have been described in various epilepsies arising from the temporal or frontal lobes, although the most commonly encountered form is related to the presence of an hypothalamic hamartoma. We report a patient with gelastic seizures involving the right parietal lobe. Our patient, a 32‐year‐old man, underwent video‐EEG monitoring, interictal and ictal brain SPECTs during gelastic seizures. Subtraction ictal SPECT co‐registered to MRI (SISCOM), was performed to localize any ictal hyperperfusion during these gelastic seizures. The seizures consisted of brief staring followed by smiling and laughing. Electroencephalography during the gelastic seizures showed rhythmic sharp waves in the right parietal lobe. SISCOM showed ictal hyperperfusion in the right parietal lobe and medial portions of right cerebellum. Our findings suggest that the right parietal lobe may actively participate in the particular epileptogenic network generating gelastic seizures.
Levetiracetam‐induced myoclonic status epilepticus in myoclonic‐astatic epilepsy: a case report
Authors: Kröll‐Seger Judith , Mothersill Ian William , Novak Simon , Sälke‐Kellermann Ritva Anneli , Krämer Günter
Published: March 2024

We report on a 3‐year‐old boy with myoclonic‐astatic epilepsy who developed myoclonic status epilepticus with continuous twitching of the face and unresponsiveness under monotherapy with levetiracetam. Recently, a nonconvulsive status epilepticus in an adult epilepsy patient has also been described. Our observation points to the possibility of a causal relationship between the induction of myoclonic status by levetiracetam in certain patients with Doose's syndrome. However, a spontaneous evolution cannot be excluded. Levetiracetam is a well‐known drug for the control of myoclonic seizures. A controlled study would provide a better understanding of any possible aggravating role in certain forms of myoclonic‐astatic epilepsy.