bluuebunny/crossref_metadata_2025 huggingface dataset:
Adversive seizures associated with periodic lateralised epileptiform discharges (PLEDs) after left orbital contusion
Authors: Takeda Takahiro , Osawa Mikio , Toi Sono , Mizuno Satoko , Shimizu Yuko , Uchiyama Shinichiro
Published: August 2019

We report a patient who presented with adversive seizures associated with periodic lateralised epileptiform discharges (PLEDs), a month after head trauma. The PLEDs predominantly involving the left frontal contacts became more frequent at the onset of adversive seizures during EEG. Brain MRI demonstrated a contusion scar in the left orbital cortex with reduced diffusion, not only around this orbital lesion but also in the ipsilateral anteromedial thalamus. Single photon emission computed tomography revealed focal cerebral hyperperfusion in the left medial orbitofrontal region, basal ganglia, and thalamus. The abnormal metabolism involving the thalamus and striatum could be associated with the ipsilateral orbital contusion and might have been caused by cortical‐subcortical, trans‐synaptic hyperactivity. Further studies are warranted to determine the role of subcortical structures in the generation of PLEDs and adversive seizures. [Published with video sequences]
Heterotopic reelin in human nodular heterotopia: a neuropathological study
Authors: Rossini Laura , Tassi Laura , Spreafico Roberto , Garbelli Rita
Published: August 2019

Aim

The extracellular matrix glycoprotein reelin plays a crucial role in the control of neuronal migration and during development is expressed by Cajal‐Retzius cells in the marginal zone. The purpose of this study was to investigate the possible involvement of reelin in the pathogenesis of human nodular heterotopia, a malformation of cortical development frequently associated with focal drug‐resistant epilepsy.

Methods

Five patients presenting with subcortical nodular heterotopia and referred for epilepsy surgery, after a comprehensive presurgical investigation, were considered. The surgical specimens were studied by combining immunohistochemistry, double immunofluorescence, and in situ hybridisation procedures.

Results

The selected cases were characterised by the presence of multiple nodules presenting in the core cell‐free zones, reminiscent of the cortical molecular layer. In all cases, small reelin‐positive cells, without typical Cajal‐Retzius cell features, were distributed inside the nodules and localised in these cell body‐sparse regions.

Conclusion

The presented data corroborate the hypothesis that reelin might be involved in human heterotopic nodular formation.
Electroconvulsive therapy for psychosis in a patient with epilepsy related to hypothalamic hamartoma
Authors: Ruppert Elisabeth , Lalanne Laurence , Foucher Jack , Zimmermann Marie‐Agathe , Hirsch Edouard , Vidailhet Pierre
Published: August 2019

Psychosis is more common in people with temporal lobe epilepsy than it is in the general population. Treatment can be difficult in these patients because of the complex interactions between antipsychotic and antiepileptic drugs. Some antipsychotic drugs also decrease the seizure threshold. We report the case of a 49‐year‐old man with a hypothalamic hamartoma, with a history of both gelastic and temporal lobe seizures. The patient was rendered seizure‐free after three neurosurgical procedures but developed a drug‐resistant paranoid psychosis. He was treated with electro‐convulsive therapy (ECT). After two weeks with six stimulations that resulted in seizures, the psychiatric phenomena disappeared completely. There was no relapse of either the psychiatric symptoms or the seizures during the 42 months of follow‐up. This case report suggests that ECT might be safe for psychosis in patients with a history of seizures that have previously been successfully treated with neurosurgery, although caution should be exercised in drawing general conclusions from a single case report.
Successful treatment of early myoclonic encephalopathy using lidocaine and carbamazepine
Authors: Nakano Kousuke , Kobayashi Katsuhiro , Maniwa Satoshi , Kodani Nobuyuki , Ohtsuka Yoko
Published: August 2019

We report two female infants with early myoclonic encephalopathy (EME) whose intractable focal seizures were suppressed with lidocaine and carbamazepine (CBZ). Although EME is a form of early‐onset epileptic encephalopathy characterised by myoclonus and focal seizures that are highly resistant to treatment, lidocaine and CBZ may prove effective in treating this disorder. Future studies should be performed in order to determine whether there are common specific mechanisms of seizure generation related to the sodium channel in these patients.
Acute amnesia and seizures in a young female
Authors: García María Eugenia García , Castrillo Sergio Muñiz , Morales Irene Garcia , Sacoto Daniela Di Capua , Dolado Alberto Marcos
Published: August 2019

Limbic encephalitis is a condition characterised by an acute or sub‐acute onset of memory disorder, associated with seizures and psychiatric manifestations. Investigations such as brain MRI usually reveal a high intensity signal in the medial temporal lobe and cerebrospinal fluid analysis shows mild pleocytosis and oligoclonal bands. It may occur in association with cancer, infection, or as an isolated clinical condition, often accompanying autoimmune disorders. Immune‐mediated limbic encephalitis is now subclassified according to the presence and type of autoantibodies, which has significant consequences regarding the effectiveness of treatment and prognosis. Glutamic acid decarboxylase (GAD) is an enzyme that catalyses glutamic acid into gamma aminobutyric acid. Anti‐GAD antibodies are associated with different neurological and non‐neurological disorders, but only a few cases of limbic encephalitis associated with anti‐GAD antibodies have been reported in the literature, most of them non‐paraneoplastic. Here, we report the case of a young female patient with a medical history of psoriasis who developed an acute onset and chronic evolution of anterograde amnesia, associated with drug‐resistant epilepsy. Brain MRI showed hyperintensity in the medial temporal lobes and the biochemical studies revealed intrathecal synthesis of anti‐GAD antibodies. Screening tests for tumours were negative. Despite antiepileptic drugs, intravenous immunoglobulins and immunosuppressive treatment, the patient did not show clinical improvement and one year later, she continues to present refractory temporal epilepsy and cognitive deficits.
Early‐onset absence epilepsy aggravated by valproic acid: a video‐EEG report
Authors: Belcastro Vincenzo , Caraballo Roberto Horacio , Romeo Antonino , Striano Pasquale
Published: August 2019

Early‐onset absence epilepsy refers to patients with absence seizures beginning before age 4 and comprises a heterogeneous group of epilepsies. Onset of absence seizures in the first year of life is very rare. We report a boy with absence seizures with onset at age 11 months, whose seizures increased in frequency after the introduction of valproic acid (VPA) treatment and substantially improved upon cessation of treatment. The mechanism of seizure worsening did not involve VPA toxicity, encephalopathy, Glut‐1 deficiency or overdosage, and the reason for absence seizure aggravation remained unclear. The patient showed complete control of absence seizures with levetiracetam treatment and the course was benign, both in terms of seizure control and neuropsychological aspects. The similar overall electroclinical picture and outcome between children with early‐onset absences and those with CAE support the view that these conditions are a continuum within the wide spectrum of IGE. [Published with video sequences]
Auditory hallucinations as ictal phenomena in a patient with voltage‐gated potassium channel antibody‐associated limbic encephalitis
Authors: Boyd Michael , Attarian Hrayr , Raizer Jeffrey , Kumthekar Priya , Macken Micheal P , Schuele Stephan U , Gerard Elizabeth
Published: August 2019

Limbic encephalitis involving anti‐voltage‐gated potassium channel antibodies (VGKC‐LE) has become increasingly recognised, with seizures and psychotic features, such as hallucinations, being typical clinical manifestations. Though the literature supports auditory hallucinations as ictal phenomena, there are no reported cases of these hallucinations correlating with electrographic seizure for this disease entity. Early recognition of auditory hallucinations as seizures could alter treatment and subsequently affect short‐term outcomes in these patients. We report the case of a patient with auditory hallucinations and progressive cognitive decline, as well as serological evidence of VGKC antibodies, in whom ictal hallucinations were identified by continuous video‐EEG monitoring. This case highlights the subtlety of this entity, in both clinical and electrographic detection. [Published with video sequences]
Alcohol‐responsive epilepsia partialis continua
Authors: Steve Trevor A , Gross Donald W
Published: August 2019

Epilepsia partialis continua is typically associated with lesions of the cerebral cortex. However, subcortical lesions can also cause this condition. We present a patient with epilepsia 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]
Extreme startle and photomyoclonic response in severe hypocalcaemia
Authors: Moccia Marcello , Erro Roberto , Nicolella Elvira , Striano Pasquale , Striano Salvatore
Published: August 2019

We report the case of 62‐year‐old woman referred to our department because of a clinical suspicion of tonic‐clonic seizures. Clinical examination revealed an exaggerated startle reflex, 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]
Cobalamin deficiency triggering de novo status epilepticus
Authors: Anastogiannis Haralabos , Karanasios Panagiotis , Makridou Alexandra , Makris Nicolaos , Argyriou Andreas A.
Published: August 2019

Cobalamin deficiency is included in the spectrum of very uncommon underlying causes of status epilepticus (SE) and the literature contains very few such cases. We herein report a case 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.
Chronic periodic lateralised epileptic discharges and anti‐N‐methyl‐D‐aspartate receptor antibodies
Authors: Sakakibara Eisuke , Takahashi Yukitoshi , Murata Yoshiko , Taniguchi Go , Sone Daichi , Watanabe Masako
Published: August 2019

Periodic lateralised epileptiform discharges (PLEDs) are uncommon transient electroencephalographic findings accompanied by acute brain lesions. A small proportion of PLEDs persist for more than three months and are called “chronic” PLEDs, the pathophysiology of which is still debated. Herein, we report a man with right hemispheric PLEDs which lasted for more than 14 months and mild left hemispatial neglect after he experienced status epilepticus. Although MRI was normal, positron emission tomography revealed right temporo‐parieto‐occipital hypometabolism, which coincided with the source area of PLEDs estimated by magnetoencephalography. In addition, levels of anti‐N‐methyl‐d‐aspartate (NMDA) receptor antibodies and granzyme B were found to be high in the cerebrospinal fluid. Following two courses of steroid pulse therapy, the patient's left spatial neglect improved and the PLEDs were partially resolved. These findings suggest that the chronic PLEDs present in this case were an interictal phenomenon and that their pathophysiology involved autoimmune processes.
Spatial memory alterations in children with epilepsy of genetic origin or unknown cause
Authors: Cimadevilla José Manuel , Lizana Julio Ramos , Roldán Maria Dolores , Cánovas Rosa , Rodríguez Eva
Published: August 2019

Genetic generalised epilepsy or epilepsy of unknown cause can remit before adolescence. In many children, the disease does not interfere with their academic achievement. Although there are neuropsychological studies characterising the cognitive profile, there are no studies in this population focused on spatial orientation abilities. In this study, we compared children with genetic generalised epilepsy or epilepsy of unknown cause with a control group using a virtual spatial learning task. Children with epilepsy showed worse performance on the spatial orientation task, although their visuo‐spatial memory, attention, and working memory were normal. These results confirm that genetic generalised epilepsy or epilepsy of unknown cause is associated with more cognitive deficits. Virtual reality technologies can complement clinical assessment.
Quantitative MR and cognitive impairment in cryptogenic localisation‐related epilepsy
Authors: Jansen Jacobus FA , van der Kruijs Sylvie JM , Vlooswijk Mariëlle CG , Majoie HJ Marian , Hofman Paul AM , Aldenkamp Albert P , Backes Walter H
Published: August 2019

For patients with chronic cryptogenic localisation‐related epilepsy (CLRE), conventional MRI does not provide measures to discern between patients with or without cognitive complaints. We investigated, in a preliminary study, whether it is possible to detect cerebral biomarkers of cognitive impairment in patients with CLRE using sensitive quantitative MRI techniques. Neuropsychological assessment and quantitative 3.0 T MRI, comprising T2 relaxometry, diffusion tensor imaging, and spectroscopic imaging, were applied to 35 patients with CLRE and 21 healthy controls. Analysis included the left and right hippocampi, and frontal and temporal lobes. Differences between the groups and correlations with cognitive and clinical characteristics were assessed. Patients with epilepsy scored significantly worse on cognitive tasks compared to healthy controls. Significantly larger CSF fractions in the hippocampi and left temporal lobe, a longer T2 relaxation time in the left hippocampus, and a significantly higher concentration of glutamate/glutamine in the left frontal lobe were observed in patients with epilepsy. Moreover, poor memory performance was significantly correlated with larger CSF fractions in the right hippocampus and left temporal lobe in patients. In the temporal lobe, an association between subtle changes in morphology (indicative of atrophy) and memory performance was found, consistent with previous literature. These results may help to explain the alterations in brain functioning in patients with epilepsy.
Limbic encephalitis with anti‐GAD antibodies and Thomsen myotonia: a casual or causal association?
Authors: Licchetta Laura , Bisulli Francesca , Naldi Ilaria , Mainieri Greta , Tinuper Paolo
Published: August 2019

The association between hereditary myotonic disorders and epilepsy is seldom described in the literature. To date, few reports have dealt with dystrophic myotonias, whereas a single case demonstrating an association between sporadic congenital myotonia and epilepsy was recently reported in a patient carrying a de novo mutation of the CLCN1 gene. Additional evidence for a role of CLCN1 in the pathogenesis of epilepsy is derived from large‐scale exome analysis of ion channel variants and expression studies. Here, we describe the first case of association between familial Thomsen myotonia and epilepsy. All the affected members of a two‐generation family presented myotonia and disclosed a pathogenic mutation in CLCN1. In addition, one individual experienced epileptic seizures due to limbic encephalitis (LE) with anti‐GAD antibodies. The occurrence of the two diseases in this patient could be a chance association, however, CLCN1 mutation, as a susceptibility factor for epilepsy through dysfunction of GABAa inhibitory signalling, cannot be ruled out as a possible influence.
Outcome of status epilepticus. What do we learn from animal data?
Authors: Auvin Stéphane , Dupuis Nina
Published: August 2019

Status Epilepticus (SE) is a life‐threatening neurologic disorder defined as 5 minutes or more of a continuous seizure. SE can represent an exacerbation of a preexisting seizure disorder, the initial manifestation of a seizure disorder, or an insult other than a seizure disorder. In humans, there are several differences between SE that occurs in adults and children. In adult patients, the mortality is high but the incidence is lower than in childhood. Experimental studies have been essential in helping clinicians describe SE, and since these early initial studies, further experimental studies have helped us to better understand the consequences of SE. Animal models of SE support the notion that SE induces brain damage and contribute to epileptogenesis. Laboratory models of SE in developing animals demonstrate age‐ and model‐dependent propensity for brain injury and for epileptogenesis. The use of models with a double hit including a clinical relevant component to seizures provides data that allows us to further understand the contribution of early‐life events in the future development of epilepsy. Using this approach, it has been shown that inflammation or a preexisting brain lesion enhance epileptogenesis in the developing brain. The use of models of SE also permits to establish that treatment to stop the seizure and/or the duration of the SE results in a decrease of SE induced cell injury. Preventing epileptogenesis remains an important goal to modify the development of comorbidities, and it still represents an area of research in need of much progress.
Focal epilepsy recruiting a generalised network of juvenile myoclonic epilepsy: a case report
Authors: Khaing Myo , Lim Kheng‐Seang , Tan Chong‐Tin
Published: August 2019

We report a patient with juvenile myoclonic epilepsy who subsequently developed temporal lobe epilepsy, which gradually became clinically dominant. Video telemetry revealed both myoclonic seizures and temporal lobe seizures. The temporal lobe seizures were accompanied by a focal recruiting rhythm with rapid generalisation on EEG, in which the ictal EEG pattern during the secondary generalised phase was morphologically similar to the ictal pattern during myoclonic seizures. The secondary generalised seizures of the focal epilepsy responded to sodium valproate, similar to the myoclonic epilepsy. In this rare case of coexistent Juvenile Myoclonic Epilepsy and Temporal lobe epilepsy, the possibility of focal epilepsy recruiting a generalised epileptic network was proposed and discussed.
Multifocal dysembryoplastic neuroepithelial tumours associated with refractory epilepsy
Authors: Yang Andrew I. , Khawaja Ayaz M. , Ballester‐Fuentes Leo , Pack Svetlana D. , Abdullaev Ziedulla , Patronas Nicholas J. , Inati Sara K. , Theodore William H. , Quezado Martha M. , Zaghloul Kareem A.
Published: August 2019

Dysembryoplastic neuroepithelial tumours (DNET) are a common cause of tumour‐associated epilepsy, and are usually located in the temporal lobes. We present a case of multifocal DNETs in both infra‐ and supra‐tentorial locations, in a 23‐year‐old man with a coincident Type I Chiari malformation, presenting with medically refractory focal seizures. The extensive anatomical distribution of the lesions suggests a genetic component in their tumourigenesis.
Time to relapse after epilepsy surgery in children: AED withdrawal policies are a contributing factor
Authors: Boshuisen Kim , Schmidt Dieter , Uiterwaal Cuno S.P.M. , Arzimanoglou Alexis , Braun Kees P.J. , Study Group TimeToStop
Published: August 2019

Aim. It was recently suggested that early postoperative seizure relapse implicates a failure to define and resect the epileptogenic zone, that late recurrences reflect the persistence or re‐emergence of epileptogenic pathology, and that early recurrences are associated with poor treatment response. Timing of antiepileptic drugs withdrawal policies, however, have never been taken into account when investigating time to relapse following epilepsy surgery. Methods. Of the European paediatric epilepsy surgery cohort from the “TimeToStop” study, all 95 children with postoperative seizure recurrence following antiepileptic drug (AED) withdrawal were selected. We investigated how time intervals from surgery to AED withdrawal, as well as other previously suggested determinants of (timing of) seizure recurrence, related to time to relapse and to relapse treatability. Uni‐ and multivariable linear and logistic regression models were used. Results. Based on multivariable analysis, a shorter interval to AED reduction was the only independent predictor of a shorter time to relapse. Based on univariable analysis, incomplete resection of the epileptogenic zone related to a shorter time to recurrence. Timing of recurrence was not related to the chance of regaining seizure freedom after reinstallation of medical treatment. Conclusion. For children in whom AED reduction is initiated following epilepsy surgery, the time to relapse is largely influenced by the timing of AED withdrawal, rather than by disease or surgery‐specific factors. We could not confirm a relationship between time to recurrence and treatment response. Timing of AED withdrawal should be taken into account when studying time to relapse following epilepsy surgery, as early withdrawal reveals more rapidly whether surgery had the intended curative effect, independently of the other factors involved.
Developmental pharmacology of benzodiazepines under normal and pathological conditions
Authors: McGoldrick Meaghan K. , Galanopoulou Aristea S.
Published: August 2019

Benzodiazepines are allosteric agonists of GABAA receptors (GABAAR), pentameric ligand‐gated Cl− channels, which serve both an important neurodevelopmental role but are also the principal inhibitory system in the brain. However, their subunit composition, channel properties, and function, as well as their region‐specific expression patterns, change through development. These processes have been extensively studied in rodents and to some extent confirmed in higher species. Specifically, GABAARs acquire faster kinetics with age and their pharmacology changes rendering them more sensitive to drugs that have higher affinity for α1 subunit‐containing GABAARs, such as benzodiazepines, but also, their inhibitory function becomes more potent as they shift from having depolarising to hyperpolarising responses due to a shift in Cl− gradient and cation chloride cotransporter expression. Concerns have been raised about possible pro‐apoptotic and paradoxical effects of benzodiazepines in the neonatal normal rat brain, although it is unclear, as yet, whether this extends to brains exposed to seizures. Growing evidence indicates that the pharmacology and physiology of GABAARs may be altered in the brain of rats or humans with seizures or epilepsy, or different aetiologies that predispose to epilepsy. These changes follow different paths, depending on sex, age, region, cell type, aetiology, or time‐point specific factors. Identification of dynamic biomarkers that could enable these changes in vivo to be monitored would greatly facilitate the selection of more effective agonists with fewer side effects.
Musicogenic and spontaneous seizures: EEG analyses with hippocampal depth electrodes
Authors: Tezer F. Irsel , Bilginer Burçak , Oguz Kader K. , Saygi Serap
Published: August 2019

Musicogenic seizure is classified as a rare form of complex reflex seizures. We present a patient with musicogenic seizures from whom invasive recordings were obtained using subdural arrays, as well as hippocampal depth electrodes. Interestingly, this patient had both spontaneous seizures and musicogenic seizures, and they originated from different hippocampi. Due to bilateral independent musicogenic seizures and spontaneous seizures, our patient was not eligible for surgery, but vagal nerve stimulation treatment was almost successful. [Published with video sequence]
Management of epilepsy in resource‐limited settings
Authors: Caraballo Roberto , Fejerman Natalio
Published: August 2019

Epilepsy is one of the most common and widespread neurological disorders affecting over 65 million people worldwide. Although estimates vary considerably, the annual incidence is considered to be almost 50 per 100,000 and prevalence around 700 per 100,000. It is thought, however, that more people are affected in low‐ and middle‐income countries where resources to improve the care for people with epilepsy are limited. Of all people with epilepsy, around 80% live in resource‐limited countries and up to 90% of these patients receive no treatment at all. National epilepsy programs to organize comprehensive care and cover educational, economic, and research aspects are necessary. A referral network will enable local healthcare workers to consult patients with more complex diseases and may ensure routine availability of inexpensive AEDs. Adequately identifying people with epilepsy and delivering cost‐effective care in resource‐limited countries is an important challenge for epileptologists and healthcare policy makers alike. Here we give an overview of the present situation and review the needs and the efforts currently being made in the field.
Drug‐resistant parietal epilepsy: polymorphic ictal semiology does not preclude good post‐surgical outcome
Authors: Francione Stefano , Liava Alexandra , Mai Roberto , Nobili Lino , Sartori Ivana , Tassi Laura , Scarpa Pina , Cardinale Francesco , Castana Laura , Cossu Massimo , Lo Russo Giorgio
Published: August 2019

We investigated the anatomo‐electro‐clinical features and clinical outcome of surgical resections strictly confined to the parietal lobe in 40 consecutive patients who received surgery for pharmacoresistant seizures. The population was subcategorized into a paediatric (11 subjects; mean age at surgery: 7.2+/−3.7 years) and an adult group (29 patients; mean age at surgery: 30+/−10.8 years). The paediatric group more frequently exhibited personal antecedents, neurological impairment, high seizure frequency, and dysplastic lesions. Nonetheless, compared with adults, children had better outcome and more frequently reached definitive drug discontinuation after surgery.

After a mean follow‐up of 9.4 years (range: 3.1–16.7), 30 subjects (75%) were classified as Engel Class I. The presence of multiple types of aura in the same patient, as well as a high incidence of secondary generalization, represented a characteristic feature of parietal seizures and did not correlate negatively with surgical outcome. A total resection of the epileptogenic zone and a localizing/regional interictal EEG were statistically significant predictive factors of outcome. Intracerebral investigation, performed in 55% of cases, contributed to complete tailored resections of the epileptogenic area and determination of prognosis. Frequent subjective manifestations of parietal lobe seizures, such as vertiginous, cephalic and visual‐moving sensations, underscore their potential misdiagnosis as non‐epileptic events.
Anomia produced by direct cortical stimulation of the pre‐supplementary motor area in a patient undergoing preoperative language mapping
Authors: Rozanski Verena Eveline , Peraud Aurelia , Noachtar Soheyl
Published: August 2019

There is sparse data on the analysis of supplementary motor area in language function using direct cortical stimulation of the supplementary motor area. Here, we report a patient who experienced isolated anomia during stimulation of the anterior supplementary motor area and discuss the role of the supplementary motor area in speech production. The role of the pre‐supplementary motor area in word selection, observed in fMRI studies, can be confirmed by direct cortical stimulation.
Short‐term outcomes and major barriers in the management of convulsive status epilepticus in children: a study in Georgia
Authors: Shatirishvili Teona , Kipiani Tamar , Lomidze Giorgi , Gabunia Maia , Tatishvili Nino
Published: August 2019

Aim. Convulsive status epilepticus is the most common childhood neurological emergency in developing countries, where poor healthcare organisation could play a negative role in the management of the condition. Unavailability of second‐line injectable anticonvulsants is an additional hindering factor in Georgia. This report reflects the results of the first study aimed at evaluating the epidemiological features of convulsive status epilepticus, as well as identifying obstacles influencing the management of patients with convulsive status epilepticus in Georgia.

Methods. A prospective, hospital‐based study was performed. Paediatric patients with convulsive status epilepticus, admitted to the emergency department of a referral academic hospital from 2007 to 2012, were included in the study.

Results. Forty‐eight paediatric patients admitted to hospital met the criteria for convulsive status epilepticus. Seizure duration was significantly shorter among the group with adequate and timely pre‐hospital intervention. Moreover, patients with appropriate pre‐hospital treatment less frequently required mechanical ventilation (p=0.039). Four deaths were detected during the follow‐up period, thus the case fatality rate was 8%. Only 31% of patients received treatment with intravenous phenytoin.

Conclusion. The study results show that adequate and timely intervention could improve outcome of convulsive status epilepticus and decrease the need for mechanical ventilation. Mortality parameters were comparable to the results from other resource‐limited countries. More than one third of patients did not receive appropriate treatment due to unavailability of phenytoin.
Treatable newborn and infant seizures due to inborn errors of metabolism
Authors: Campistol Jaume , Plecko Barbara
Published: August 2019

About 25% of seizures in the neonatal period have causes other than asphyxia, ischaemia or intracranial bleeding. Among these are primary genetic epileptic encephalopathies with sometimes poor prognosis and high mortality. In addition, some forms of neonatal infant seizures are due to inborn errors of metabolism that do not respond to common AEDs, but are amenable to specific treatment. In this situation, early recognition can allow seizure control and will prevent neurological deterioration and long‐term sequelae. We review the group of inborn errors of metabolism that lead to newborn/infant seizures and epilepsy, of which the treatment with cofactors is very different to that used in typical epilepsy management.
Resection of focal cortical dysplasia located in the upper pre‐central gyrus
Authors: Eldin Samer Serag , Iwasaki Masaki , Nishio Yoshiyuki , Jin Kazutaka , Nakasato Nobukazu , Tominaga Teiji
Published: August 2019

The primary motor cortex of the oro‐facial level can be removed without permanent deficits, because of the bilateral representation of the innate functions. In contrast, resective surgery of the hand motor cortex or higher levels presents more challenges. We treated two adult patients with intractable epilepsy caused by small focal cortical dysplasia in the pre‐central gyrus located between the foot and hand primary motor cortices. Focal cortical resection was guided by cortical EEG and intra‐operative motor evoked potential, resulting in seizure freedom without neurological deficits in both cases. These cases illustrate that resective surgery can be safely performed in the primary motor cortex even dorsal to the oro‐facial level, as long as the critical regions of the hand and foot motor cortices remain intact. Accurate delineation of the anatomical lesion and functional areas using intra‐operative neurophysiological monitoring is crucial for successful outcome of the surgery.
Peri‐ictal water drinking: a rare automatic behaviour in temporal lobe epilepsy
Authors: Pietrafusa Nicola , Trivisano Marina , de Palma Luca , Serino Domenico , Moavero Romina , Benvenga Antonella , Cappelletti Simona , Boero Giovanni , Vigevano Federico , La Neve Angela , Specchio Nicola
Published: August 2019

Peri‐ictal water drinking (PIWD) has been reported as the action of drinking during or within two minutes of an electroclinical seizure. It is considered a peri‐ictal vegetative symptom, evident both during childhood and adulthood epilepsy. The aim of this paper was to describe the clinical and electroencephalographic features of two new adult subjects suffering from symptomatic temporal lobe epilepsy with episodes of PIWD recorded by VIDEO‐EEG and to review literature data in order to better define this peculiar event during seizures, a rare and probably underestimated semiological sign. To date, 51 cases with focal epilepsy and seizures associated with PIWD have been reported. All patients presented with temporal lobe epilepsy. All cases but one had symptomatic epilepsy. Most of the patients had an involvement of the right hemisphere. Water drinking was reported as an ictal sign in the majority of patients, and less frequently was reported as postictal. We believe that PIWD might be considered a rare automatic behaviour, like other automatisms. Automatisms are more frequently described in patients with temporal lobe epilepsy. PIWD was reported also to have lateralizing significance in the non‐dominant temporal lobe, however, because of its rarity, this finding remains unclear.
Mega‐dose phenobarbital therapy for super‐refractory status epilepticus
Authors: Byun Jung‐Ick , Chu Kon , Sunwoo Jun‐Sang , Moon Jangsup , Kim Tae‐Joon , Lim Jung‐Ah , Jun Jin‐Sun , Lee Han Sang , Lee Woo‐Jin , Lee Doo Young , Jeon Daejong , Lee Soon‐Tae , Jung Keun‐Hwa , Jung Ki‐Young , Lee Sang Kun
Published: August 2019

Aims. To evaluate the efficacy and safety of mega‐dose phenobarbital (MDPB; enteral or parenteral phenobarbital >10 mg/kg/day) for treating super‐refractory status epilepticus (SRSE; continuous or recurrent status epilepticus for ≥24 hours after the onset of continuous anaesthetic treatment) in adult patients.

Methods. Adult patients with SRSE who were treated with MDPB in our institution from March 2005 to September 2014 were reviewed. We collected data on basic demographics, clinical features, functional status, anticonvulsant treatment, and possible adverse events. SRSE outcome was divided into six categories: successful therapy, initial failure, breakthrough seizures, withdrawal seizures, intolerable side effects, and death during treatment.

Results. Ten adult patients with SRSE received MDPB. Median age at seizure onset was 38 years (range: 18‐59), and half were male. All patients had no history of seizures and had symptoms suggestive of viral encephalitis. Median duration of status epilepticus was 17.5 days (range: 6–60) and anaesthetics were used for a median of 14.0 days (range: 2–54) before MDPB. Successful control of SRSE was achieved in half of the patients, however, only one of ten patients was able to fully recover at discharge. Median duration of the MDPB was 45.5 days and the maximum serum phenobarbital level reached a median of 151.5 μg/ml. Patients with successful MDPB therapy had normal brain imaging (80% vs. 0%; p=0.048) and better functional outcome at discharge and after three months of follow‐up. Infection was the most critical complication, along with cardiorespiratory depression.

Conclusion. MDPB is a therapeutic option for control of SRSE when other choices are exhausted.
Characterization of ictal slow waves in epileptic spasmsCharacterization of ictal slow waves in epileptic spasms
Authors: Honda Ryoko , Saito Yoshiaki , Okumura Akihisa , Abe Shinpei , Saito Takashi , Nakagawa Eiji , Sugai Kenji , Sasaki Masayuki
Published: August 2019

We characterized the clinico‐neurophysiological features of epileptic spasms, particularly focusing on high‐voltage slow waves during ictal EEG. We studied 22 patients with epileptic spasms recorded during digital video‐scalp EEG, including five individuals who still had persistent spasms after callosotomy. We analysed the duration, amplitude, latency to onset of electromyographic bursts, and distribution of the highest positive and negative peaks of slow waves in 352 spasms. High‐voltage positive slow waves preceded the identifiable muscle contractions of spasms. The mean duration of these positive waves was 569±228 m, and the mean latency to electromyographic onset was 182±127 m. These parameters varied markedly even within a patient. The highest peak of the positive component was distributed in variable regions, which was not consistent with the location of lesions on MRI. The peak of the negative component following the positivity was distributed in the neighbouring or opposite areas of the positive peak distribution. No changes were evident in the pre‐ or post‐surgical distributions of the positive peak, or in the interhemispheric delay between both hemispheres, in individuals with callosotomy. Our data imply that ictal positive slow waves are the most common EEG changes during spasms associated with a massive motor component. Plausible explanations for these widespread positive slow waves include the notion that EEG changes possibly reflect involvement of both cortical and subcortical structures.
Diaphragm myoclonus followed by generalised atonia in a patient with trisomy 4p: unusual semiology in an unusual condition
Authors: Varley James , Wehner Tim , Sisodiya Sanjay
Published: August 2019

In this report, we describe a female patient with trisomy 4p, a rare genetic condition, with unusual seizure semiology. The patient is one of the oldest reported survivors with this condition. This semiology was noted while she was being monitored by inpatient video telemetry. We observed a series of myoclonic shoulder jerks, followed by hiccup‐like episodes, and finally an atonic head drop. Corresponding ictal EEG showed semi‐rhythmic high‐amplitude slow waves with spikes superimposed over the frontotemporal areas. This semiology was confirmed as habitual by her parents. Subsequent hiccup‐like episodes had no EEG correlate, and the head drop was again associated with semi‐rhythmic high‐amplitude slow waves and superimposed spikes, more prominent over the right hemisphere. In addition, we review the several cases in which hiccups have been associated with seizures and how this may relate to the neural pathways involved in the pathophysiology of hiccups. We believe the ictal hiccup‐like episodes followed by atonia to be a seizure semiology that has not previously been documented. [Published with video sequence]
Successful epilepsy surgery in frontal lobe epilepsy with startle seizures: a SEEG studyFrontal epilepsy with startle seizures
Authors: Ciurea Ana , Popa Irina , Maliia Mihai Dragos , Csilla‐Johanna Nagy , Barborica Andrei , Donos Cristian , Ciurea Jean , Opris Ioan , Mindruta Ioana
Published: August 2019

Pre‐surgical assessment and surgical management of frontal epilepsy with normal MRI is often challenging. We present a case of a 33‐year‐old, right‐handed, educated male. During childhood, his seizures presented with mandibular myoclonus and no particular trigger. As a young adult, he developed seizures with a startle component, triggered by unexpected noises. During his ictal episodes, he felt fear and grimaced with sudden head flexion and tonic axial posturing. Similar seizures also occurred without startle. Neuropsychological assessment showed executive dysfunction and verbal memory deficit. The cerebral MRI was normal. Electro‐clinical reasoning, investigations performed, the results obtained and follow‐up are discussed in detail. [Published with video sequence]
Nocturnal interictal epileptic discharges in adult Lennox‐Gastaut syndrome: the effect of sleep stage and time of night
Authors: Sforza Emilia , Mahdi Rima , Roche Frederic , Maeder Malin , Foletti Giovanni
Published: August 2019

Aim. Lennox‐Gastaut syndrome (LGS) is characterized by interictal epileptiform discharges (IEDs) occurring during sleep. The aim of this study was to determine whether sleep influences not only the frequency of seizures and IEDs, but also the time‐dependent evolution that may support the hypothesis of homeostatic influences on epileptic threshold.

Methods. Video polysomnography data from our database were reviewed to identify adult LGS patients with at least seven hours of nocturnal recording. Thirteen patients were identified and a second polysomnography was available for nine. The number, duration and index of IEDs, relative to total sleep, sleep stages, and time during the night, were calculated.

Results. The majority of IEDs occurred during non‐rapid eye movement sleep, mainly in stage 2 and slow‐wave sleep. Adjusting for time spent in each sleep stage, we found 45 IEDs/hour in stage 1, 123/hour in stage 2, 106/hour in slow‐wave sleep, and 26/hour in rapid eye movement sleep. The temporal distribution of IEDs showed a significant rise in the first three hours of sleep, followed by a progressive decrease at the end of the night (F=85.6; p<0.0001).

Conclusion. Interictal epileptiform discharges occurrence in adult LGS is facilitated by non‐rapid eye movement sleep with an evident effect of stage 2 and slow‐wave sleep. The significant IED occurrence in the first part of the night and the subsequent decline suggests a link between epileptic threshold and homeostatic sleep mechanisms. The latter should be considered regarding choice of therapy.
Early experiences with tachycardia‐triggered vagus nerve stimulation using the AspireSR stimulator
Authors: El Tahry Riëm , Hirsch Martin , Van Rijckevorsel Kenou , Santos Susana Ferrao , de Tourtchaninoff Marianne , Rooijakkers Herbert , Coenen Volker , Schulze‐Bonhage Andreas
Published: August 2019

Many epilepsy patients treated with vagus nerve stimulation additionally use an “on‐demand” function, triggering an extra stimulation to terminate a seizure or diminish its severity. Nevertheless, a substantial number of patients are not able to actively trigger stimulations by use of a magnet, due to the absence of an aura or inability for voluntary actions in the early phase of a seizure. To address this need, a novel implantable pulse generator, the AspireSR VNS system, was developed to provide automated ictal stimulation triggered by a seizure‐detecting algorithm. We report our experience with three patients in assessing the functionality of ictal stimulation, illustrating the detection system in practice. Detection of ictal tachycardia and variable additional detections of physiological tachycardia depended on the individual seizure‐detecting algorithm settings.
Epilepsia partialis continua: aetiology, semiology and prognosis in a Spanish adult cohort
Authors: Bejr‐kasem Helena , Sala‐Padró Jacint , Toledo Manuel , Santamarina Estevo , Sarria Silvana , Gonzalez‐Cuevas Montserrat , Sueiras‐Gil María , Quintana Manolo , Salas‐Puig Xavier
Published: August 2019

Aim. To describe the semiological features in patients suffering with Epilepsia Partialis Continua (EPC), also referred as Kozhevnikov syndrome and their relationship with aetiology, duration, and prognosis, as well as recurrence during follow‐up.

Methods. We analysed consecutive EPC patients diagnosed and followed in our centre over a seven‐and‐a half year period. We collected demographic and clinical data, along with neuroimaging and EEG recordings. All patients were followed for more than six months. Patients were categorised with single body area or multiple body area involvement according to the body parts affected. Recurrence was defined as a second EPC episode after one week.

Results. We collected data from 27 adult patients; 70.4% were men, the mean age was 65.2 years old (range: 17–89 years), and 40.7% had previous epilepsy. EPC causes were structural in 85.1% (stroke being the most frequent; 44.4%), metabolic in 11.1%, and of unknown origin in 7.4%. A cortical lesion on neuroimaging was shown in 70.4%. Involvement of multiple body areas was reported in 55.6% of patients. The optimal cut‐off period to predict death was nine days (with a sensitivity of 62.5% and specificity of 75%; p=0.039), and this group of patients exhibited more multiple body area involvement (88.9% vs 38.9%; p=0.04). During follow‐up, patients with cortical lesions had more EPC relapses (p=0.037).

Conclusions. The most frequent aetiology of EPC in our patients was stroke. Multiple body area involvement and duration were associated with mortality. Patients with cortical lesions had more EPC relapses during follow‐up.
Drug‐resistant parietal lobe epilepsy: clinical manifestations and surgery outcome
Authors: Asadollahi Marjan , Sperling Michael R. , Rabiei Amin H. , Asadi‐Pooya Ali A.
Published: August 2019

Aim. We reviewed a large surgical cohort to investigate the clinical manifestations, EEG and neuroimaging findings, and postoperative seizure outcome in patients with drug‐resistant parietal lobe epilepsy (PLE).

Methods. All drug‐resistant PLE patients, who were investigated for epilepsy surgery at Jefferson Comprehensive Epilepsy Center between 1986 and 2015, were identified. Demographic data, seizure data, EEG recordings, brain MRI, pathological findings, and postsurgical seizure outcome were reviewed.

Results. In total, 18 patients (11 males and seven females) were identified. Sixteen patients (88%) had tonic‐clonic seizures, 12 (66%) had focal seizures with impaired awareness, and 13 (72%) described auras. Among 15 patients who had brain MRI, 14 patients (93%) had parietal lobe lesions. Only three of 15 patients (20%) who had interictal scalp EEG recordings showed parietal interictal spikes. Of 12 patients with available ictal surface EEG recordings, only three patients (25%) had parietal ictal EEG onset. After a mean follow‐up duration of 8.6 years, 14 patients (77.7%) showed a favourable postoperative seizure outcome.

Conclusion. In patients with PLE, semiology and EEG may be misleading and brain MRI is the most valuable tool to localize the epileptogenic zone. Postsurgical seizure outcome was favourable in our patients with drug‐resistant parietal lobe epilepsy.