bluuebunny/crossref_metadata_2025_split huggingface dataset:
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

ABSTRACTWe 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

ABSTRACTStigma 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

ABSTRACTWe 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

ABSTRACTThis 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

ABSTRACTObjectiveTo 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.MethodsRandomised 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.ResultsCRBMA 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.ConclusionThere 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

ABSTRACTBarbexaclone 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

ABSTRACTAim. 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

ABSTRACTTemporal 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

ABSTRACTNeonatal 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

ABSTRACTAnti‐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.
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.
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.
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

AbstractMyoclonic 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].