bluuebunny/crossref_metadata_2025 huggingface dataset:
Epilepsy and anxiety: epidemiology, classification, aetiology, and treatment
Authors: Kimiskidis Vasilios K. , Valeta Thalia
Published: August 2019

Anxiety in epilepsy has recently become a focus of interest for a number of reasons. Epidemiological studies have established that anxiety disorders are twice as common in patients with epilepsy compared to the general population, while in referral centres their prevalence is even higher. In addition, it has been recently appreciated that anxiety exerts a significant negative impact on the quality of life of patients with epilepsy of any age. With regard to the pathogenesis of anxiety in epilepsy, a number of theories have been put forward including those based on psychodynamics, learningcognition, and neurobiology. From a clinical point of view, anxiety may occur as a comorbid disorder with epilepsy or be directly linked with epilepsy as a preictal, ictal, postictal or interictal phenomenon. The treatment of anxiety in patients with epilepsy requires a comprehensive, multidisciplinary, clinical assessment. Regarding pharmacological therapies, it should be recognised that some drugs prescribed for anxiety disorders are associated with a high risk of seizures, whereas some antiepileptic drugs possess anxiolytic properties that could be of use in the management of epileptic patients with anxiety. The correct diagnosis and successful treatment of anxiety is expected to have significant benefits for the quality of life of epileptic patients.
Animal models of drug‐resistant epilepsy*
Authors: Potschka Heidrun
Published: August 2019

Several animal models are discussed in order to outline features of difficult‐to‐treat or drug‐resistant epilepsy. These models can be categorised as those which show a poor response to different antiepileptic drugs and those in which subgroups of drug‐resistant animals are selected, based on interindividual differences. Non‐responders to antiepileptic drugs have been described in the amygdala kindling model, as well as the chronic phase of post‐status epilepticus models. Epileptic dogs which do not respond to standard antiepileptic drugs may serve as a translational model to provide a more clinical environment for drug testing. Drug resistance or a poor response to several antiepileptic drugs has been reported for the 6‐Hz model, lamotrigine‐pretreated kindled rats, pentylentetrazole‐induced seizures in rats pre‐exposed to pilocarpine, as well as following intrauterine exposure of rats to methylazoxymethanol. Using models to select non‐responders is highly time‐consuming and elaborate, limiting their use in routine drug‐screening procedures. Current efforts to identify biomarkers of drug resistance may simplify the selection process, e.g. replacing several weeks of seizure monitoring by a single imaging scan. Moreover, further elucidation of mechanisms of resistance may help to design a series of ex vivo or in vitro screening procedures in order to evaluate whether a test compound is affected.
Photosensitivity: epidemiology, genetics, clinical manifestations, assessment, and management
Authors: Verrotti Alberto , Beccaria Francesca , Fiori Federica , Montagnini Alessandra , Capovilla Giuseppe
Published: August 2019

Photosensitivity is a genetically determined trait that may be asymptomatic throughout life or manifest with epileptic seizures. Photosensitivity usually begins before the age of 20 years with a peak age at onset at around 12. Both natural and artificial light may trigger seizures. Precise investigation must be carried out by intermittent photic stimulation that can elicit a clearly defined EEG response; video‐EEG samples are reported to illustrate the various determinants of response and the main factors altering the effectiveness of intermittent photic stimulation. Management of photosensitive epilepsy includes non‐pharmacological (e.g.avoidance of the provocative stimuli and wearing appropriate tinted glass) and pharmacological treatment. This review focuses on the emerging aspects of photosensitivity, in particular, the new guidelines for intermittent photic stimulation and briefly addresses epidemiological (in non‐epileptic and epileptic subjects), genetic, diagnostic, and therapeutic issues. [Published with video sequences]
Effect of pregabalin add‐on treatment on seizure control, quality of life, and anxiety in patients with brain tumour‐related epilepsy: a pilot study
Authors: Maschio Marta , Dinapoli Loredana , Sperati Francesca , Pace Andrea , Fabi Alessandra , Vidiri Antonello , Pompili Alfredo , Carapella Carmine Maria
Published: August 2019

Objective

An open pilot study to evaluate the effect of pregabalin (PGB) as add‐on therapy on seizure control, quality of life, and anxiety in patients with brain tumour‐related epilepsy (BTRE).

Materials and methods

We recruited 25 consecutive patients with BTRE and uncontrolled seizures. At baseline and during follow‐up, patients underwent a complete physical and neurological examination and were evaluated using the QOLIE 31P (V2), EORTC QLQ C30, Adverse Events Profile, and Hamilton Anxiety Rating Scale (HAM‐A). At baseline, a seizure diary was given.

Results

During follow‐up, 17 patients underwent chemotherapy, none underwent radiotherapy, 9 had disease progression, and 3 died. Mean duration of follow‐up was 4.1 months. Mean PGB dosage was 279 mg/day. At baseline, mean weekly seizure frequency was 5.3 (±10) and at last available follow‐up visit was 2.8±5. This difference was statistically significant (p=0.016). The responder rate was 76%. Ten patients dropped out; 4 as a result of seizure worsening, 1 as a result of unchanged seizure frequency, 3 as a result of a lack of compliance, and 2 as a result of side effects. Based on the QOLIE‐31‐P, a significant improvement of the subscale “seizure worry” (p=0.004) and a significant decrease in distress scores related to AEDs and social life (p=0.009 and p=0.008, respectively) were observed. A significant decrease in HAMA score (p=0.002) was documented.

Conclusions

These data indicate that PGB may represent a valid alternative as add‐on treatment in this patient population, based on its efficacy on seizure control and anxiety.
Seizure control with antiepileptic drug therapy in 517 consecutive adult outpatients at the Kork Epilepsy Centre
Authors: Steinhoff Bernhard J , Staack Anke Maren , Wisniewski Ilona
Published: August 2019

In order to assess the efficacy of modern antiepileptic drug (AED) therapy, we collected data from 517 consecutive adult outpatients referred to our centre between March and August 2011. In total, 211 patients (40.8%) were treated with monotherapy, 208 patients (40.2%) with a combination of two AEDs, and for the remaining patients (n=98; 19%) more than two AEDs were combined. The most common AEDs were valproate, lamotrigine, carbamazepine, and levetiracetam. Of the recent AEDs, leve‐tiracetam was the leading drug with regards to drug combinations. Freedom of seizures for more than one year was achieved in 291 patients (56.3%). Under monotherapy, 168 patients (32.5% of all patients; 79.6% of patients with monotherapy) became seizure‐free. Seizure‐freedom with two AEDs was achieved in 103 patients (19.9% of all patients; 49.5% of patients with two AEDs) and in 20 patients with three AEDs (3.9% of all patients; 25.3% of patients with three AEDs). We conclude from this cross‐sectional survey in a large patient group that combinations may still lead to treatment success in a considerable proportion of patients.
Crying with sorrow evoked by electrocortical stimulation
Authors: Burghardt Tyson , Basha Maysaa M , Fuerst Darren , Mittal Sandeep
Published: August 2019

Dacrystic seizures are rare and have been reported in patients with hypothalamic hamartoma as well as fronto‐temporal epilepsy, involving the non‐dominant hemisphere. We describe the first reported case of cortical stimulation of the left posterior orbito‐frontal gyrus, generating consistent and reproducible crying with affective content in a 41‐year‐old woman with medically intractable left temporal lobe epilepsy, who underwent extraoperative intracranial video‐EEG monitoring for resective non‐lesional epilepsy surgery.
Ictal unilateral hyperkinetic proximal lower limb movements: an independent lateralising sign suggesting ipsilateral seizure onset
Authors: Teotónio Rute , Rodionov Roman , McEvoy Andrew W , Duncan John , Diehl Beate , Wehner Tim
Published: August 2019

Our aim was to determine the lateralising value of ictal repetitive unilateral hyperkinetic proximal lower limb movements. Among 16 patients with ictal hyperkinetic features who underwent intracranial EEG between 2008 and 2011 in our centre, three had repetitive unilateral hyperkinetic proximal lower limb movements, sometimes associated with “hemiballic‐like” movements of the ipsilateral upper limb. There were no other consistent lateralising signs. In all patients, these movements were ipsilateral to the ictal onset zone and started a few seconds after electro‐encephalographic seizure onset. In two patients, intracranial EEG showed seizure onset within the parietal lobe and mesial temporal ictal onset was recorded in a third. Two patients underwent resection and became seizure‐free. Our results suggest that unilateral ictal repetitive hyperkinetic proximal lower limb movements may be an independent lateralising sign to the ipsilateral hemisphere.
Video/EEG findings in a KCNQ2 epileptic encephalopathy: a case report and revision of literature data
Authors: Serino Domenico , Specchio Nicola , Pontrelli Giuseppe , Vigevano Federico , Fusco Lucia
Published: August 2019

We describe the EEG findings of an infant with early‐onset epileptic encephalopathy with mutation of the KCNQ2 gene and a family history of neonatal seizures. The infant presented with multifocal drug‐resistant seizures with onset during the third day of life. Family history was positive for early‐onset neonatal seizures. Metabolic screening and neuroimaging were negative. Direct sequencing of KCQN2 from both the mother and child revealed a heterozygous cytosine‐to‐guanine mutation (Dedek et al., 2003). Interictal EEG showed a very discontinuous pattern which evolved towards a defined burst‐suppression pattern during sleep and a multifocal, random, attenuation pattern during wakefulness. Focal, tonic seizures with head deviation, sometimes followed by asynchronous and asymmetrical clonic jerks, eyelid myoclonias, and polypnoea, were recorded. Ictal EEG was characterised by focal, low‐voltage, fast activity, followed by recruiting theta rhythms and bilateral, focal, spike‐wave complexes, alternatively localised to one hemisphere and subsequently diffusing to the other. ACTH therapy was introduced, resulting in a significant improvement in EEG activity and gradual reduction in seizure frequency, with cessation at age 13 weeks. [Published with video sequences]
Stand Up For Epilepsy San Diego photo‐shoot: a personal odyssey*
Authors: Kaufman Kenneth R. , Kaufman Nathaniel D.
Published: August 2019

Stigma towards epilepsy results in barriers to social integration, education, employment, and marriage, with increased divorce rates. There is a societal need to realise that many persons with epilepsy (PWE) lead normal lives and can be highly educated with effective employment. Integration of PWE into sports is therapeutic both for PWE (decreased seizure frequency, decreased comorbid conditions, and increased psychosocial skills) and society. Stand Up For Epilepsy (SUFE), overseen by the ILAE Task Force on Sports and Epilepsy, is an international project in which action photographs with PWE were taken as a means to destigmatise epilepsy. This autobiographical narrative describes how SUFE initiated effective athlete‐PWE interactions with positive PWE, athlete, and parent responses. Expansion of SUFE is recommended to further destigmatise epilepsy. Participation in SUFE can serve as a personal odyssey for PWE.
Opercular myoclonic‐anarthric status epilepticus due to glutamic acid decarboxylase antibody‐associated encephalitis
Authors: Monnerat Bruno Zanotelli , Velasco Tonicarlo Rodrigues , Nakano Frederico Nakane , Júnior Alexandre Veriano , Martins Ana Paula Pinheiro , Sakamoto Américo Ceiki
Published: August 2019

We report a patient who was diagnosed with opercular myoclonic‐anarthric status epilepticus and found to have glutamic acid decarboxylase antibody (GADA)‐associated encephalitis, a previously unrecognised aetiology of this condition. The patient was a 23‐year‐old female admitted for investigation of focal myoclonic status epilepticus in the right side of the face and glossopharyngeal area. Intravenous corticosteroid was administered and improvement was observed in seizure activity and overall general health. A video sequence of opercular myoclonia is included. Due to the presence of inflammatory elements based on brain MRI and CSF studies, a decision to investigate autoimmune encephalitis was undertaken. Anti‐GAD65 radioimmunoassay was markedly positive. This case study highlights the need for awareness of the clinical presentation of GADA‐associated encephalitis. [Published with video sequences]
Seizure semiology reflects spread from frontal to temporal lobe: evolution of hyperkinetic to automotor seizures as documented by invasive EEG video recordings
Authors: Tezer Fadime Irsel , Agan Kadriye , Borggraefe Ingo , Noachtar Soheyl
Published: August 2019

This patient report demonstrates the importance of seizure evolution in the localising value of seizure semiology. Spread of epileptic activity from frontal to temporal lobe, as demonstrated by invasive recordings, was reflected by change from hyperkinetic movements to arrest of activity with mild oral and manual automatisms. [Published with video sequences]
A common reference‐based indirect comparison meta‐analysis of intravenous valproate versus intravenous phenobarbitone for convulsive status epilepticus
Authors: Brigo Francesco , Igwe Stanley C , Nardone Raffaele , Tezzon Frediano , Bongiovanni Luigi Giuseppe , Trinka Eugen
Published: August 2019

Objective

To compare intravenous valproate (IV‐VPA) with intravenous phenobarbitone (IV‐PB) in the treatment of established generalised convulsive status epilepticus (GCSE). Efficacy and safety were estimated using a common‐reference based indirect comparison meta‐analysis (CRBMA) methodology.

Methods

Randomised controlled trials (RCTs) investigating the use of IV‐VPA or IV‐PB versus intravenous phenytoin (IV‐PHT) for GCSE were identified by a systematic search of the literature. A random effects model was used to estimate Mantel‐Haenszel odds ratios (ORs) for efficacy and safety of IV‐VPA or IV‐PB versus IV‐PHT in a standard meta‐analysis. Adjusted indirect comparisons were then made between VPA and PB using the obtained results.

Results

CRBMA showed that VPA does not lead to significantly higher seizure cessation (OR 1.00; 95% CI: 0.36‐2.76) compared to PB, although it exhibits fewer adverse effects (OR 0.17; 95% CI: 0.04‐0.71). Results of this CRBMA are consistent with results of a recently published head‐to‐head comparison of IV‐VPA and IV‐PB.

Conclusion

There is insufficient evidence to demonstrate superiority of IV‐VPA over IV‐PB for the treatment of GCSE in terms of efficacy. Some direct and indirect comparisons suggest that VPA has a better safety profile than PB. However, the limited numbers of underpowered RCTs included in this meta‐analysis are not sufficient to justify a change in clinical practice. More rigorous and appropriately powered RCTs are therefore required to definitively determine the efficacy and tolerability of VPA for the treatment of GCSE.
End of the barbexaclone era: an experience of treatment withdrawal
Authors: Bolukbasi Feray , Delil Sakir , Bulus Eser , Senturk Asli , Yeni Naz , Karaagac Naci
Published: August 2019

Barbexaclone is a salt compound of phenobarbital and propylhexedrine (a drug with indirect sympathomimetic properties). Due to the presence of the psychostimulating agent, propylhexedrine, this drug has less of a sedative effect and is well tolerated, compared to phenobarbital. Barbexaclone was widely used in Turkey until 2009 when its production ended, however, it gave rise to an epidemic for which we were not prepared. Since then, no standardised management protocol has been developed and each patient has been evaluated individually, thereby creating tailor‐made solutions based on the extent of each patient's supply of remaining drug (from a few tablets to a stock which might last for six months). The rate of seizure freedom was 37.7% under barbexaclone treatment and dropped to 32.2% in the follow‐up period after discontinuation of the drug. In the majority of cases, a new antiepileptic drug was added and this was commonly levetiracetam, a more expensive drug. In this article, we share our experiences of a general problem: the withdrawal of an antiepileptic drug from the market. Although there was prior notification regarding barbexaclone withdrawal, it was not possible to contact all patients since such a database is not available in Turkey. Although no conclusions regarding the efficacy of the drug or comparison of efficacy with other antiepileptic drugs is provided, it is nonetheless noteworthy to share these experiences since some patients had lost seizure control for reasons that could not be explained.
Congenital hemiparesis, unilateral polymicrogyria and epilepsy with or without status epilepticus during sleep: a study of 66 patients with long‐term follow‐up
Authors: Caraballo Roberto Horacio , Cersósimo Ricardo Oscar , Fortini Pablo Sebastián , Ornella Lorena , Buompadre María Celeste , Vilte Carolina , Princich Juan Pablo , Fejerman Natalio
Published: August 2019

Aim. We retrospectively analysed the electroclinical features, treatment, and outcome in patients with unilateral polymicrogyria (PMG), focussing on epileptic syndrome with or without encephalopathy, with status epilepticus during sleep (ESES) or continuous spikes and waves during slow sleep (CSWS) syndrome. Methods. From June 1990 to December 2012, 39 males and 27 females, aged 5‐26 years, were studied. We did not include patients with bilateral PMG or cases with unilateral PMG associated with other cerebral lesions. The mean follow‐up period was 12 years (range: 3‐22 years). Results. Mean age at epilepsy onset was 6.5 years. Focal motor seizures occurred in all cases and 25 had secondary generalised seizures. Six patients also had complex focal seizures. Interictal EEG recordings showed focal spikes in all cases. For 43 of 53 patients with epilepsy, aged 2‐9.5 years, the electroclinical features changed. An increase in frequency of focal motor seizures was reported in 20 patients, negative myoclonus occurred in 32 patients, atypical absences in 25 patients, and positive myoclonus in 19 patients. All patients had a continuous symmetric or asymmetric pattern of spike‐wave activity during slow‐wave sleep. Conclusion. For patients presenting with congenital hemiparesis, negative or positive myoclonus, and absences and focal motor seizures with ESES/CSWS, unilateral PMG should be considered. Brain MRI is mandatory to confirm this cortical malformation. The most commonly used treatments were clobazam, ethosuximide, and sulthiame, alone or in combination. For refractory cases, high‐dose steroids were administered and surgery was performed in two patients. Outcome was relatively benign.
Ictal EEG modifications in temporal lobe epilepsy
Authors: Pelliccia Veronica , Mai Roberto , Francione Stefano , Gozzo Francesca , Sartori Ivana , Nobili Lino , Russo Giorgio Lo , Pizzanelli Chiara , Tassi Laura
Published: August 2019

Temporal lobe epilepsy is the most common type of epilepsy in adults with medically intractable, localisation‐related epilepsy, amenable to surgery. Together with clinical and neuroimaging data, presurgical ictal scalp‐EEG findings are often sufficient to define the epileptogenic zone. It is widely believed that ictal scalp‐EEG findings in temporal lobe epilepsy are represented by 5‐9‐Hz lateralised rhythmic theta activity or 2‐5‐Hz lateralised rhythmic delta activity. On the basis of experimental models and experience with intra‐cerebral EEG recordings, the pattern of low‐voltage fast activity is considered to be the electrophysiological hallmark of the epileptogenic zone. We reviewed the ictal scalp‐EEG data relating to 111 seizures in 47 patients with temporal lobe epilepsy who underwent video‐EEG recordings during presurgical work‐up. We found that 35 patients (74.4%) showed flattening, low‐voltage fast activity or fast activity as the initial EEG pattern. When visible, the rhythmic delta or theta activity followed the fast activity. Low‐voltage fast activity, flattening or fast activity occurs in the majority of patients with temporal lobe epilepsy and represents the main ictal EEG pattern. Low‐voltage fast activity (or similar) is also identifiable as the initial ictal EEG pattern in scalp‐EEG recordings.
Neonatal epilepsy and underlying aetiology: to what extent do seizures and EEG abnormalities influence outcome?
Authors: Ramantani Georgia
Published: August 2019

Neonatal seizures constitute the most common and distinctive sign of neurological dysfunction in the first weeks of life and reflect a wide variety of underlying central nervous system disorders. Acute symptomatic seizures occur more often during the neonatal period than at any period of life and are associated with adverse long‐term neurodevelopmental sequelae and an increased risk of post‐neonatal epilepsy. The improvements of neonatal care in the last decades have changed the spectrum of insults to which the immature brain is exposed and facilitated a decrease in mortality of newborns with seizures. However, the prevalence of long‐term morbidity in survivors remains unchanged. Whereas aetiology is presumed to be the main predictor of long‐term outcome in neonates with seizures, there is converging evidence that specific electroencephalographic (EEG) abnormalities are related to unfavourable outcomes. Interictal EEG abnormalities, especially concerning background activity patterns, thus constitute a major indicator of disease severity and predictor of outcome, while the added value of sequential EEG assessments is so far controversial. Moreover, experimental as well as clinical studies of hypoxic‐ischaemic encephalopathy support the notion that recurrent seizures may amplify injury to the developing brain beyond that associated with the underlying aetiology, thus justifying antiepileptic drug treatment. To date, unresolved issues in seizure detection and classification, in addition to the significant variation in gestational ages and brain insults of neonates, still impede clinical research of neonatal seizures. The wider use of long‐term EEG or amplitude integrated EEG monitoring may prove crucial for timely neonatal seizure identification and treatment initiation, and thus ultimately improve outcome.
Extreme delta brush in a patient with anti‐NMDAR encephalitis
Authors: Di Capua Daniela , García‐Ptacek Sara , García‐García Maria Eugenia , Abarrategui Belen , Porta‐Etessam Jesús , García‐Morales Irene
Published: August 2019

Anti‐N‐methyl‐D‐aspartate receptor encephalitis is a severe, potentially treatable, disorder and prognosis depends on early recognition and prompt immunotherapy. We report a case of anti‐N‐methyl‐D‐aspartate receptor encephalitis with atypical age and gender, and a characteristic electroencephalographic pattern that supported the diagnosis. A 66‐year‐old male presented with psychiatric disturbances and focal seizures with alteration of consciousness, and progressed to a state of akinetic mutism. Auxiliary tests were negative or non‐specific for anti‐NMDAR encephalitis. Electroencephalographic monitoring revealed a unique pattern; the extreme delta brush. The patient improved with immunotherapy and was asymptomatic at six months of follow‐up. Ancillary testing was positive for anti‐N‐methyl‐D‐aspartate receptor antibodies. Extreme delta brush is a recently described electroencephalographic pattern presenting in only one third of patients with anti‐N‐methyl‐D‐aspartate receptor encephalitis. The identification of this pattern, as in our case, may guide early diagnosis and treatment of anti‐N‐methyl‐D‐aspartate receptor encephalitis.
Generalised electrographic seizures presenting as perioral myoclonia
Authors: Dearborn Jennifer L , Kaplan Peter W
Published: August 2019

A 41‐year‐old man, during a neurological consultation, reported “chin twitching” over a period of a week, which was diagnosed as intermittent perioral myoclonia. With only one tonic‐clonic seizure seven years before, he had mentioned several episodes of chin twitching over the years. In the clinic, there were intermittent chin movements without apparent confusion, as he was able to provide a complete history and was fully oriented with intact memory. His video‐EEG showed paroxysms of polyspike and slow‐wave activity, with the longest burst‐free interval being 20 seconds. Discharges were maximal over the fronto‐central regions, correlating with the chin myoclonus. He was able to tap his hand continuously, and remained alert. The case represents an atypical presentation of idiopathic generalised epilepsy without manifestation of absence or limb myoclonus. Although juvenile myoclonic epilepsy and other idiopathic epilepsies are rarely associated with perioral myoclonia, this sign was the principal clinical feature for this patient. Oral treatment with levetiracetam resolved his seizures. [Published with video sequences]
Early add‐on treatmentvsalternative monotherapy in patients with partial epilepsy
Authors: Semah Franck , Thomas Pierre , Coulbaut Safia , Derambure Philippe
Published: August 2019

Aim. The aim of this study was to evaluate the impact of two different therapeutic strategies in patients with partial seizures who were intractable to the first prescribed antiepileptic drug (AED); alternative monotherapy vs early add‐on treatment.

Methods. We conducted an open, cluster‐randomised, prospective, controlled trial in patients with persistent partial seizures, despite treatment with one AED, who were never administered any other AEDs. Neurologists were randomised to two strategies: in group A, an alternative monotherapy with a second AED was employed; in group B, add‐on treatment with a second AED was employed. The primary outcome was the percentage of seizure‐free patients during a two‐month period after six months of treatment. The secondary outcomes were: (i) the percentage of patients achieving a 50% reduction in the number of seizures at six months; (ii) the quality of life based on the Quality Of Life In Epilepsy scale; and (iii) tolerability.

Results. A total of 143 neurologists were included and randomised, and 264 patients were evaluated. At six months, the primary outcome was 51% in group A and 45% in group B (p=0.34). The percentage of patients achieving a 50% reduction in the number of seizures at six months was 76% in group A and 84% in group B (p=0.53). The quality of life and the tolerability did not significantly differ between the two groups.

Conclusions. Alternative monotherapy or early treatment initiation with another AED drug resulted in similar efficacy, and the side effects associated with monotherapy and combined therapies were similar, which suggests that individual susceptibility is more important than the number and burden of AEDs used.
Panayiotopoulos syndrome and symptomatic occipital lobe epilepsy of childhood: a clinical and EEG study
Authors: Tata Gulten , Guveli Betul Tekin , Dortcan Nimet , Cokar Ozlem , Kurucu Hatice , Demirbilek Veysi , Dervent Aysin
Published: August 2019

Aim. Panayiotopoulos syndrome (PS) is an age‐related seizure susceptibility syndrome that affects the central autonomic system. Although the majority of the few ictal recordings obtained so far suggest an occipital origin, semiological and interictal EEG data appear to favour more extensive involvement. In this study, the characteristics (including those based on semiology and EEG) of children with Panayiotopoulos syndrome (n=24) and those with lesion‐related, symptomatic occipital lobe epilepsy (SOLE) (n=23) were compared.

Methods. Detailed semiological information and EEG parameters including the localisation, distribution, density (n/sec), reactivity, and morphological characteristics of spike‐wave foci and their relationship with different states of vigilance were compared between the two groups.

Results. The age at seizure onset was significantly younger in patients with symptomatic occipital lobe epilepsy than in those with PS (mean age at onset: 3.4 versus 5.6 years, respectively; p=0.044). Autonomic seizures (p=0.001) and ictal syncope (p=0.055) were more frequent in PS than in symptomatic occipital lobe epilepsy (87.5% and 37.5% versus 43.5% and 13%, respectively). The interictal spike‐wave activity increased significantly during non‐rapid eye movement (non‐REM) sleep in both groups. The spike waves in non‐REM seen in PS tended to spread mainly to central and centro‐temporal regions.

Conclusions. The results indicate that although common features do exist, Panayiotopoulos syndrome differs from symptomatic occipital lobe epilepsy and has a unique low epileptogenic threshold related to particular brain circuits.
Seizure control in Unverricht‐Lundborg disease: a single‐centre study
Authors: Roivainen Reina , Karvonen Matti K. , Puumala Tarja
Published: August 2019

New antiepileptic drug (AED) options for generalised seizure types have been adopted for use as treatment for Unverricht‐Lundborg disease. Whether this has led to improved seizure control or functional outcome in ULD patients remains obscure. We retrospectively identified all patients seen at Helsinki University Hospital due to Unverricht‐Lundborg disease during 2003–2008 in order to determine which AED treatments had been retained for long‐term use. The majority of the patients had severe functional disabilities. In the year preceding the last hospital visit, all patients (n=20) were receiving polytherapy and 14 patients had been free of tonic‐clonic seizures. During follow‐up, improvement in myoclonia had been recorded for the majority of patients with either add‐on piracetam, topiramate, or levetiracetam, but valproate was still in use by all patients. Treatment with lamotrigine had been started and retained less often relative to other AEDs. Add‐on AED treatment was often associated with significant adverse effects. Unverricht‐Lundborg disease patients may benefit from add‐on treatment with levetiracetam or topiramate for seizure control. Treatment of eventual comorbidities with other than AEDs is also discussed.
Intraoperative ElectroCorticoGraphy (ECog): indications, techniques, and utility in epilepsy surgery
Authors: Yang Tong , Hakimian Shahin , Schwartz Theodore H.
Published: August 2019

Aims. To describe the technique, indications, and utility of intraoperative ECoG monitoring during various surgeries for medically intractable epilepsy.Methods. Literature was reviewed to obtain published results on using intraoperative ECoG techniques for the surgical treatment of medically intractable epilepsy of various underlying aetiologies.Results. General intraoperative ECoG techniques were described, including effects of anaesthetic agents on intraoperative ECoG signals. Use of intraoperative ECoG in temporal lobe epilepsy with mesial temporal sclerosis (MTS) is generally considered not necessary, whereas intraoperative ECoG in temporal lobe epilepsy without mesial temporal sclerosis may provide useful information. Intraoperative ECoG in extra‐temporal epilepsy with structural lesions may facilitate resection, whereas the use of intraoperative ECoG in extra‐temporal epilepsy without a structural lesion is more controversial.Conclusions. Intraoperative ECoG is a useful technique to be employed in surgical treatment of medically intractable epilepsy. However, its effectiveness may vary depending on the underlying pathological causes of the seizures.
Stereo‐electroencephalography (SEEG) in 65 children: an effective and safe diagnostic method for pre‐surgical diagnosis, independent of age
Authors: Taussig Delphine , Chipaux Mathilde , Lebas Axel , Fohlen Martine , Bulteau Christine , Ternier Jessica , Ferrand‐Sorbets Sarah , Delalande Olivier , Dorfmüller Georg
Published: August 2019

Aim. We report our experience of stereoelectroencephalography (SEEG) in 65 children with drug‐resistant seizures, with a particular emphasis on young children.Methods. We retrospectively studied all SEEG performed between 2009 and 2011 in our centre. As SEEG can have several indications, the patients were classified into three categories, according to the probability of surgery. The contribution of SEEG to the final decision regarding surgery was evaluated for each category separately. We also compared the main demographic and surgical data of children younger than 5 years of age (Group 1; 21 children) with those older than five years of age at the time of investigation (Group 2; 44 patients).Results. MRI was not contributory in 20% of patients (9.5% in group 1; 25% in group 2). Electrical stimulations localised the motor area in all patients when performed (49% of patients), even in group 1 (62% of patients). SEEG led to surgery in 78% of patients (90.5% in group 1; 73% in group 2), after a second invasive investigation in 9.2 % of patients. The resection involved more than one lobe in 25% of patients (37% in group 1; 19% in group 2). Ultimately, 78% of patients with a low probability of having surgery before SEEG received surgery (88% in group 1). The surgical outcome of Engel class 1 was reported for 67% of patients (79% of patients in group 1 and 59% in group 2). No complications occurred.Conclusion. SEEG in children is safe and useful, and the surgical outcome in younger children is as good as, or sometimes even better than, that in older children. As a result of lower rates of complication and morbidity, SEEG appears to be more appropriate, in comparison to subdural grids, in situations where it is unclear if patients will have surgery after an invasive investigation.
What are the best ways to deliver benzodiazepines in children/patients with prolonged convulsive seizures?
Authors: Chin Richard FM
Published: August 2019

Aetiology is the main determinant of morbidity and mortality in convulsive status epilepticus (CSE) but longer seizure durations may also increase risk of worse outcome. Thirty minutes of seizure activity is usually the time period used in longstanding definitions of CSE but it is not acceptable to wait for 30 minutes before treatment. Whilst intravenous therapy is best, pre‐hospital treatment by a non‐intravenous route is most practical in treating children. Benzodiazepines are the main class of first‐line emergency antiepileptic drugs. This review will examine the available data on benzodiazepines according to: stability in the conditions of the emergency room services, drug absorption via non‐intravenous route, clinical efficacy and safety, and ease of delivery and social acceptability.
What are the effects of prolonged seizures in the brain?
Authors: Scott Rod C
Published: August 2019

Convulsive status epilepticus is the most common neurological emergency in children and is associated with significant morbidity and mortality. The morbidities include later development of epilepsy, cognitive impairment, and psychiatric impairments. There has been a long‐standing hypothesis that these outcomes are, at least in part, a function of brain injury induced by the status epilepticus. There is evidence from animal models and prospective human studies that the hippocampus may be injured during febrile status epilepticus although this pathophysiological sequence remains uncommon. Potential mechanisms include excitotoxicity, ischaemia, and inflammation. Neuroprotective drugs reduce brain injury but have little impact on epileptogenesis or cognitive impairments. Anti‐inflammatory treatments have given mixed results to date. Broad‐spectrum anti‐inflammatory agents, such as steroids, are potentially harmful, whereas prevention of leucocyte diapedesis across the blood brain barrier appears to have a positive outcome. Therefore, more studies dissecting the inflammatory process are required to establish the most effective strategies for translation into clinical practice. In addition to neuronal loss, cognitive impairments are related to neuronal re‐organisation and disruption of neural networks underpinning cognition. Further understanding of these mechanisms may lead to novel therapies that prevent brain injury, but also therapies that may improve outcomes even if injury has occurred.
Subclinical focal seizures as a sign of progression in gliomas
Authors: Fernández‐Torre José L. , Hernández‐Hernández Miguel , Martino Juan , Hinojo Carmen
Published: August 2019

Background. Subclinical seizures are ictal electrographic discharges lacking signs of clinical seizures, behavioural alteration or subjective symptoms. The diagnosis and detection of this type of non‐convulsive seizures remain challenging, and information is scarce regarding this electroclinical picture in subjects with gliomas. The aim of this report is to describe two patients with gliomas who, after treatment with surgery and radiotherapy, exhibited subclinical seizures on video‐EEG monitoring, as a manifestation of recurrence or progression of their brain tumour.

Methods. Case report and video‐EEG monitoring analysis.

Results. Two patients with gliomas were admitted to our neurosurgical unit after a generalized tonic‐clonic seizure. Brain MRI revealed a recurrence of their tumour. The use of video‐EEG monitoring allowed the detection and characterization of subclinical seizures in both patients that otherwise would have gone undetected. In both cases, subclinical seizures arose from the frontal lobe and were not associated with motor manifestations or subjective symptoms.

Conclusions. We emphasize that the existence of subclinical seizures in patients with gliomas is likely to be underestimated, and can occur in advanced progressive tumours. It is important to carry out continuous video‐EEG monitoring in brain tumour patients who have had recent clinical seizures in order to be able to detect subclinical seizures and make appropriate diagnosis.
Treatment responsive GABA(B)‐receptor limbic encephalitis presenting as new‐onset super‐refractory status epilepticus (NORSE) in a deployed U.S. soldier
Authors: Hainsworth Jeffrey Brian , Shishido Akira , Theeler Brett James , Carroll Craig Grason , Fasano Rebecca Ellen
Published: August 2019

A 23‐year‐old, previously healthy, deployed U.S. soldier presented with bilateral temporal lobe seizures recalcitrant to multiple antiepileptic drugs and anti‐seizure anaesthetic agents. He received methylprednisolone, intravenous immunoglobulins, plasma exchange, and rituximab for presumed autoimmune encephalitis before achieving seizure freedom. Six weeks after presentation, the aetiology of his refractory seizures was found to be due to autoantibodies targeting the anti‐GABA(B)‐receptor. This case is noteworthy for being the first reported case of anti‐GABA(B)‐receptor limbic encephalitis presenting with new‐onset refractory status epilepticus (NORSE), a clinical syndrome that often carries a grave prognosis and in which a treatable aetiology is often never discovered. Our case also supports testing for GABA‐receptor autoantibodies and the upfront use of multi‐modal immunotherapy in patients presenting with limbic encephalitis and new refractory seizures.
The clinical pharmacology of traditional antiepileptic drugs
Authors: Vajda Frank J.E. , Eadie Mervyn J.
Published: August 2019

Despite the availability of newer agents, a number of antiepileptic drugs have continued to be employed reasonably widely, many years after their introduction to human therapeutics. These drugs comprise phenobarbitone and some of its congeners, phenytoin, ethosuximide, carbamazepine, valproate, and certain benzodiazepines. Details of their pharmacological profiles are outlined in the following account.
Myoclonic status epilepticus as a presentation of caspr2 antibody‐associated autoimmune encephalitis
Authors: Ramanathan Sudarshini , Wong Chong H , Rahman Zebunnessa , Dale Russell C , Fulcher David , Bleasel Andrew F
Published: August 2019

We present a case of autoimmune encephalitis associated with antibodies targeting contactin‐associated protein‐like 2. This case is notable because of the presentation with myoclonic status epilepticus and the prolonged clinical course of refractory seizures, which are demonstrated in the accompanying videos, and not previously associated with this condition. Treatment with prednisone, intravenous immunoglobulin, plasma exchange, rituximab, cyclophosphamide, and mycophenolate mofetil resulted in significant functional improvement. Historically, myoclonic status epilepticus is associated with a grave prognosis and minimal chance of meaningful recovery. This case demonstrates that autoimmune encephalitis remains an important differential diagnosis in patients with such a presentation, and that early recognition and the appropriate institution of immunotherapy can result in seizure control and functional recovery. [Published with video sequences]
A comparison of continuous video‐EEG monitoring and 30‐minute EEG in an ICU
Authors: Khan Omar I. , Azevedo Christina J. , Hartshorn Alendia L. , Montanye Justin T. , Gonzalez Juan C. , Natola Mark A. , Surgenor Stephen D. , Morse Richard P. , Nordgren Richard E. , Bujarski Krzysztof A. , Holmes Gregory L. , Jobst Barbara C. , Scott Rod C. , Thadani Vijay M.
Published: August 2019

Aim. To determine whether there is added benefit in detecting electrographic abnormalities from 16–24 hours of continuous video‐EEG in adult medical/surgical ICU patients, compared to a 30‐minute EEG.

Methods. This was a prospectively enroled non‐randomized study of 130 consecutive ICU patients for whom EEG was requested. For 117 patients, a 30‐minute EEG was requested for altered mental state and/or suspected seizures; 83 patients continued with continuous video‐EEG for 16–24 hours and 34 patients had only the 30‐minute EEG. For 13 patients with prior seizures, continuous video‐EEG was requested and was carried out for 16–24 hours. We gathered EEG data prospectively, and reviewed the medical records retrospectively to assess the impact of continuous video‐EEG.

Results. A total of 83 continuous video‐EEG recordings were performed for 16–24 hours beyond 30 minutes of routine EEG. All were slow, and 34% showed epileptiform findings in the first 30 minutes, including 2% with seizures. Over 16–24 hours, 14% developed new or additional epileptiform abnormalities, including 6% with seizures. In 8%, treatment was changed based on continuous video‐EEG. Among the 34 EEGs limited to 30 minutes, almost all were slow and 18% showed epileptiform activity, including 3% with seizures. Among the 13 patients with known seizures, continuous video‐EEG was slow in all and 69% had epileptiform abnormalities in the first 30 minutes, including 31% with seizures. An additional 8% developed epileptiform abnormalities over 16–24 hours. In 46%, treatment was changed based on continuous video‐EEG.

Conclusion. This study indicates that if continuous video‐EEG is not available, a 30‐minute EEG in the ICU has a substantial diagnostic yield and will lead to the detection of the majority of epileptiform abnormalities. In a small percentage of patients, continuous video‐EEG will lead to the detection of additional epileptiform abnormalities. In a sub‐population, with a history of seizures prior to the initiation of EEG recording, the benefits of continuous video‐EEG in monitoring seizure activity and influencing treatment may be greater.
Mixed myoclonic‐absence status epilepticus in juvenile myoclonic epilepsy
Authors: Gélisse Philippe , Crespel Arielle
Published: August 2019

Myoclonic status epilepticus or mixed absence‐myoclonic status is uncommon in juvenile myoclonic epilepsy (JME), often precipitated by sleep deprivation, withdrawal of medication, or inadequate antiepileptic drugs (Thomas et al., ; Crespel et al., ). Such episodes respond well to benzodiazepines or valproate (Crespel et al., ). We present the video‐EEG of a 24‐year‐old woman with JME and bipolar disorder. She had a confusional state five days after withdrawal of clonazepam (14 mg/d) and introduction of oxazepam (200 mg/d), followed by catatonic stupor with subtle myoclonus of the face and the arms. The EEG showed absence status (figures 1, 2), which stopped after IV injection of clonazepam (1 mg) (figure 3). Consciousness returned to normal [Published with video sequence and figures].
Factors influencing medication adherence after epilepsy surgery
Authors: Liu Jianming , Xu Ruxiang , Liu Zhiliang , You Yu , Meng Fanxin
Published: August 2019

Aim. In drug‐responsive epilepsy patients, treatment non‐compliance is a major factor in seizure recurrence, but adherence to prescribed regimens following epilepsy surgery has not been examined. We measured adherence to prescribed antiepileptic drugs (AEDs) after epilepsy surgery and investigated factors influencing treatment non‐compliance.

Methods. Postsurgical epilepsy patients (n=214) were monitored for 18.1±8.1 months. Adherence was measured using the Medication Possession Ratio (MPR) self‐report questionnaire, with MPR<0.8 defined as non‐adherence.

Results. According to the MPR, 58 patients (27.1%) were non‐adherent after surgery. There were no differences in demographic and clinical variables, such as age (p=0.057, t =−1.925), duration of illness (p=0.597, t=0.530), gender ratio (p=0.714, χ2=0.134), and place of residence (urban vs. rural; p=0.874, χ2=0.025), between adherent and non‐adherent patients. Moreover, adherence was not related to surgical outcome as evaluated by the Engel classification (p=0.635, χ2=1.628) or to the types of AEDs after surgery (p=0.165, χ2=6.530). The most common reasons for non‐adherence were seizure‐free status for an extended period (26.5%), forgetfulness (19.1%), and an inability to buy the drugs locally (18.6%).

Conclusion. Adherence to AEDs is improved after epilepsy surgery compared to presurgical estimates, but is still a common and serious problem. Targeted postsurgical management programs and communication strategies are necessary to improve adherence to AEDs after epilepsy surgery.
Ring 17 syndrome: first clinical report without intellectual disability
Authors: de Palma Luca , De Carlo Debora , Lenzini Elisabetta , Boniver Clementina , Tarantino Vincenza , Bacci Barbara , Vecchi Marilena
Published: August 2019

Ring chromosomes are rare abnormalities caused by the fusion of the telomeric regions. Three‐ring chromosome syndromes (Cr 20, Cr 17 and Cr 14) cause epilepsy with variable phenotypes. In ring 17 patients with mild phenotype, some authors have shown an epilepsy syndrome similar to that of ring 20. We report the first case of a girl with ring chromosome 17 and a normal neurological and general cognitive profile. She had had, from 9 years old, focal pharmacoresistant epilepsy associated with episodes of non‐convulsive status epilepticus with mainly autonomic features. Cytogenetic analysis revealed an abnormal karyotype characterised by the presence ofde novoring chromosome 17 in 19% of metaphases. The array CGH (100 KB) did not show any genetic deletion. The clinical and epilepsy phenotype was, to a certain degree, similar to that of ring 20 syndrome.