bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinico‐pathological investigations of Rasmussen encephalitis suggest multifocal disease progression and associated focal cortical dysplasia
Authors: Wang Dandan , Blümcke Ingmar , Gui Qiuping , Zhou Wenjing , Zuo Huancong , Lin Jiuluan , Luo Yang 
Published: August 2019

ABSTRACTRasmussen encephalitis is a devastating neurological disorder characterised by seizures, brain inflammation, and progressive hemispheric atrophy. The objective of the current study was to systematically characterise patterns of structural lesions in children with Rasmussen encephalitis, referred for modified anatomical hemispherectomy at the Tsinghua University Epilepsy Center in Beijing. Seven consecutive patients were investigated with a mean age at operation of 4.5 years, who suffered from medically intractable seizures for a mean of 1.6 years. Foci of abnormally increased T2 signal intensity were observed in all patients. With the exception of one child, all patients presented with progressive unilateral cerebral atrophy. FDG‐PET imaging revealed extensive regions of hypometabolism within the affected cerebral hemisphere in 3 of 4 patients. Diagnosis of Rasmussen encephalitis was confirmed histologically, demonstrating CD68 positive microglial nodules, as well as CD3 and CD8 positive T lymphocytes invading the cerebral parenchyma. An intriguing observation was the heterogenous distribution of patterns of lesions throughout the affected hemisphere, suggesting multifocal manifestation and distinct sequences of disease progression, from discrete foci of inflammatory infiltrates (stage 1) to extensive cortical destruction (stage 4). Atypical hippocampal sclerosis (HS), with neuronal cell loss affecting most prominently the CA4 region (HS type 3 or end folium sclerosis), was evident in 5 of 7 cases. Four hippocampi also showed chronic inflammation. In addition, we observed associated focal cortical dysplasia (FCD; ILAE type IIId) in 4 of 7 children, supporting the concept of acquired and postmigratory FCD pathomechanisms. Postsurgical seizure freedom was achieved in all children with a mean follow‐up period of 2.7 years and continuous antiepileptic medication.
Syncope with atypical trunk convulsions in a patient with malignant arrhythmia
Authors: Doležalová Irena , Brázdil Milan , Rektor Ivan , Tyrlíková Ivana , Kuba Robert 
Published: August 2019

ABSTRACTSyncope is a condition often misdiagnosed as epilepsy. Syncope caused by cardiac disturbance is a life‐threatening condition and accurate diagnosis is crucial for patient outcome. We present a case study of a 71‐year‐old woman who was referred to our epilepsy centre with a diagnosis of refractory epilepsy. We diagnosed convulsive syncope caused by malignant cardiac arrhythmia based on the presence of cardiac asystole lasting for 20‐30 seconds, which was caused by sick sinus syndrome combined with third‐degree atrioventricular block. The most prominent feature of this syncope was atypical trunk (abdominal or thoracoabdominal) convulsions, which were accompanied by other motor signs (head and eye deviation and brief jerks of the extremities). In the periods between attacks, all investigations, including standard 12‐lead ECG and 24‐hour ECG monitoring, were normal. This case study highlights the challenge in differential diagnosis of sudden loss of consciousness. [Published with video sequences]
Antiepileptic treatment for anti‐NMDA receptor encephalitis: the need for video‐EEG monitoring
Authors: Dericioglu Nese , Vural Atay , Acar Pinar , Agayeva Nergiz , Ismailova Vefa , Kurne Asli , Saka Esen , Arsava Ethem Murat , Topcuoglu Mehmet Akif 
Published: August 2019

ABSTRACTAnti‐NMDA receptor encephalitis is a severe disorder characterised clinically by seizures, autonomic instability, and severe disturbances of memory, behaviour, and cognition. Due to the severity of symptoms, many patients are admitted to the intensive care unit. For some patients, the presence of various movement disorders and abnormal autonomic signs, as well as a history of seizures, lead to a false impression of status epilepticus, which is reported in 6% of the cases. Here, we present two young female patients, one of whom had ovarian teratoma. Both patients were referred to our neurological intensive care unit with a diagnosis of status epilepticus. However, prolonged video‐EEG findings were compatible with encephalopathy. We avoided aggressive treatment with intravenous anaesthetics and both patients recovered after immunotherapy, one of whom received surgery. Physicians should be cautious in interpreting abnormal movements and autonomic signs in such patients and video‐EEG monitoring is advised when status epilepticus is suspected. [Published with video sequences]
Reflex myoclonic epilepsy in infancy: a critical review
Authors: Verrotti Alberto , Matricardi Sara , Pavone Piero , Marino Raffaella , Curatolo Paolo 
Published: August 2019

ABSTRACTBenign myoclonic epilepsy in infancy, classified among the generalised idiopathic epilepsies, is characterised by the occurrence of myoclonic seizures in the first three years of life in otherwise normal infants. Some authors have described cases of myoclonic seizures as a reflex response to sudden unexpected tactile or acoustic stimuli and this clinical entity has been proposed as a separate nosographic syndrome, referred to as “reflex myoclonic epilepsy in infancy” (RMEI). We reviewed all published articles and case reports on RMEI in order to clarify clinical and electroencephalographic findings, with particular attention to outcome and treatment. RMEI appears to be a benign variant of idiopathic myoclonic epilepsy in infancy with specific features that occur in neurologically and developmentally normal children. This rare clinical entity is often under‐described and under‐diagnosed, and for this reason should be brought to the attention of paediatricians in order to avoid extensive investigations and reassure parents of the lack of long‐term complications.
Adherence to treatment and influencing factors in a sample of Chinese epilepsy patients
Authors: Liu Jianming , Liu Zhiliang , Ding Hu , Yang Xiaohong 
Published: August 2019

ABSTRACTAimTo assess adherence to antiepileptic drugs (AEDs) and factors associated with non‐adherence in a sample of Chinese patients with epilepsy.MethodsA cross‐sectional descriptive study was carried out on patients who had no change in treatment regimen over the last six months. Data on adherence to medication and related factors for each patient were gathered using a questionnaire.ResultsOf a total of 368 patients studied, 48.1% of patients were non‐adherent with regards to AEDs. There were no demographic differences (based on gender, age, seizure type, and rural or urban location) between adherent and non‐adherent patients. Adherence was positively and significantly correlated with duration of illness (p=0.007). The primary reason for non‐adherence was forgetfulness or not having medication on hand (69.6%), followed by a negative attitude (12.8%), a bad patient‐prescriber relationship (9.5%), side effects (5.4%), inability to buy drugs (1.9%), and other reasons (0.8%).ConclusionThe non‐adherence of epilepsy patients is common in China. Targeted management programs and communication strategies are necessary to improve adherence to AED treatments in patients with epilepsy and avoid the clinical consequences of poor adherence.
Anorgasmia during pregabalin add‐on therapy for partial seizures
Authors: Calabrò Rocco Salvatore , De Luca Rosaria , Pollicino Patrizia , Bramanti Placido 
Published: August 2019

ABSTRACTAnorgasmia is the inability to reach orgasm during sexual intercourse, and, although it is believed that around 90% of anorgasmia problems are related to psychological issues, the use of serotoninergic drugs, including antidepressants and atypical antipsychotics, is a common cause of situational anorgasmia. Pregabalin is a new antiepileptic drug, structurally related to gabapentin, and commonly used as adjunctive therapy for partial epilepsy and treatment of neuropathic pain in adults. Herein, we describe three men with epilepsy, who experienced severe anorgasmia after pregabalin add‐on treatment.
Newly‐diagnosed epileptic seizures in three populations: Geneva (EPIGEN), Martinique (EPIMART), and the Reunion Island (EPIREUN)
Authors: Bhalla Devender , Preux Pierre‐Marie , Mignard Claude , Smadja Didier , Tchalla Achille Edem , Marin Benoît , Jallon Pierre 
Published: August 2019

ABSTRACTAimThe objective was to analyse and discuss data from three studies of newly‐diagnosed epileptic seizures (provoked and unprovoked) conducted in Geneva, Martinique, and the Reunion Island, in which the same methodology was used.MethodsWe extracted data from three studies in which the incidence of seizures was estimated and aetiologies identified. Data was extracted and analysed using STATA. Group comparison was performed firstly for each study as a single group, and secondly by considering Martinique and the Reunion Island as an overseas group, in comparison with Geneva, considered as a mainland group. Uncorrected χ2was used and statistical significance (two‐sided, p=0.05) was determined for each aetiology per cohort.ResultsThe incidence of newly‐diagnosed epileptic seizures per100,000 was 71.0, 80.6, and 100.4 in Geneva, Martinique, and the Reunion Island, respectively. A bimodal distribution and predominance of generalised seizures was noted. The male to female ratio was higher in Martinique (~2.0) than other populations (~1.5). Status epilepticus was noted in Geneva and more so in the Reunion Island. The incidence of provoked seizures per 100,000 was 25.2,16.4, and 17.7, and for unprovoked seizures was 45.6,64.1, and 81.2 in Geneva, Martinique, and the Reunion Island, respectively. There was a greater risk of provoked seizures in Geneva relative to the overseas group, which was due to tumours, use of toxic substances, and drug abuse. The risk of unprovoked seizures in Geneva was due to trauma and infections. In Martinique, alcoholism and HIV were foremost factors for provoked and unprovoked seizures, and stroke was an important aetiology in both Martinique (provoked seizures) and the Reunion Island (unprovoked seizures).ConclusionThe risk of provoked seizures was greatest in Geneva and risk of unprovoked seizures was greatest in the Reunion Island. Toxic substances, alcohol, infection, and trauma constituted major factors for epileptic seizures in Geneva, while alcoholism, HIV, and stroke were major factors in the overseas group. Relative eradication of tropical infections has paved a way for the emergence of non‐communicable aetiologies (stroke, alcoholism). Males from Martinique demonstrated the greatest risk of epileptic seizures, signifying the importance of alcoholism, HIV, etc. Three steps should follow: follow‐up studies (mortality), strong mechanisms for prevention (or control) of risk factors, guidelines on whether to treat or not.
Torsional nystagmus in hypothalamic hamartoma
Authors: Shaikh Aasef G. 
Published: August 2019

ABSTRACTTorsional nystagmus was noted in a patient with hypothalamic hamartoma. Magnetic resonance imaging revealed an exophytic hypothalamic mass extending into the pre‐pontine cistern and abutting ventral mesencephalon. The quickphase of the torsional nystagmus was directed towards the left side, ipsilateral to the side of compression by the hamartoma. Ipsi‐lesionally directed pure torsional nystagmus in this case is attributed to the compressive lesion of ocular motor structures responsible for the neural integration of torsional and vertical eye movements, the interstitial nucleus of Cajal. [Published with video sequences]
Usefulness of bilateral bispectral index (BIS) monitoring in a comatose patient with myoclonic status epilepticus secondary to cefepime
Authors: Fernández‐Torre José L , Hernández‐Hernández Miguel A , Rodríguez‐Borregán Juan C , González‐Quintanilla Vicente 
Published: August 2019

ABSTRACTBackground. Status epilepticus, particularly non‐convulsive status epilepticus (NCSE), is a frequent complication in patients with altered renal function receiving treatment with intravenous cefepime. To the best of our knowledge, we report the first case, illustrated by video‐EEG, of a critically ill patient receiving treatment with cefepime who developed an episode of confirmed symptomatic myoclonic status epilepticus (MSE). Methods. Case report and video‐EEG. Results. A 60‐year‐old man, who had received a liver transplant due to alcoholic cirrhosis one year ago, was admitted to our intensive care unit due to septic shock. Computed tomography revealed a prostatic abscess as cause of his sepsis. On Day 27, a respiratory infection due to Pseudomona aeruginosa was diagnosed, and treatment with intravenous cefepime (2 g/8 hours) was initiated. On Day 32, his mental status deteriorated and he developed inattention, a reduced level of consciousness, and multifocal and generalised continuous myoclonic jerks. A video‐EEG study was compatible with the diagnosis of symptomatic MSE. On Day 35, cefepime was stopped and general anaesthesia with midazolam was started in order to achieve a faster clinical improvement. We used the BIS‐Vista™ monitor to guide general anaesthesia and detect potential episodes of NCSE. On Day 40, an EEG confirmed the existence of moderate diffuse encephalopathy. Finally, the patient died as a consequence of severe heart failure. Conclusions. Cefepime may be a cause of MSE in non‐anoxic comatose patients. Clinicians should be aware of this possibility when evaluating comatose patients on cephalosporin therapy in order to establish a correct diagnostic approach and accurate prognosis. [Published with video sequences]
Cortical network dysfunction in musicogenic epilepsy reflecting the role of snowballing emotional processes in seizure generation: an fMRI‐EEG study
Authors: Diekmann Volker , Hoppner Anselm Cornelius 
Published: August 2019

ABSTRACTAim. Patients suffering from musicogenic epilepsy have focal seizures triggered by auditory stimuli. In some of these patients, the emotions associated with the music appear to play a role in the process triggering the seizure, however, the significance of these emotions and the brain regions involved are unclear. In order to shed some light on this, we conducted fMRI and EEG in a case of musicogenic epilepsy.Methods. In a 32‐year‐old male patient with seizures induced by a specific piece of Russian music, we performed video‐EEG monitoring as well as simultaneous fMRI and EEG registration.Results. Video‐EEG monitoring revealed a left temporo‐frontal epileptogenic focus. During fMRI‐EEG co‐registration, BOLD signal alterations were not only found in the epileptogenic focus but also in areas known for their role in the processing of emotions. Prior to a seizure in some of these areas, BOLD contrasts exponentially increased or decreased.Conclusion. These results suggest that in our case, dysfunction of the regulation processes of the musically‐induced emotions, and not the musical stimulus itself, led to the seizures.
Visual and auditory socio‐cognitive perception in unilateral temporal lobe epilepsy in children and adolescents: a prospective controlled study
Authors: Laurent Agathe , Arzimanoglou Alexis , Panagiotakaki Eleni , Sfaello Ignacio , Kahane Philippe , Ryvlin Philippe , Hirsch Edouard , de Schonen Scania 
Published: August 2019

Aim. A high rate of abnormal social behavioural traits or perceptual deficits is observed in children with unilateral temporal lobe epilepsy. In the present study, perception of auditory and visual social signals, carried by faces and voices, was evaluated in children or adolescents with temporal lobe epilepsy.Methods. We prospectively investigated a sample of 62 children with focal non‐idiopathic epilepsy early in the course of the disorder. The present analysis included 39 children with a confirmed diagnosis of temporal lobe epilepsy. Control participants (72), distributed across 10 age groups, served as a control group. Our socio‐perceptual evaluation protocol comprised three socio‐visual tasks (face identity, facial emotion and gaze direction recognition), two socio‐auditory tasks (voice identity and emotional prosody recognition), and three control tasks (lip reading, geometrical pattern and linguistic intonation recognition). All 39 patients also benefited from a neuropsychological examination.Results. As a group, children with temporal lobe epilepsy performed at a significantly lower level compared to the control group with regards to recognition of facial identity, direction of eye gaze, and emotional facial expressions. We found no relationship between the type of visual deficit and age at first seizure, duration of epilepsy, or the epilepsy‐affected cerebral hemisphere. Deficits in socio‐perceptual tasks could be found independently of the presence of deficits in visual or auditory episodic memory, visual non‐facial pattern processing (control tasks), or speech perception. A normal FSIQ did not exempt some of the patients from an underlying deficit in some of the socio‐perceptual tasks.Conclusion. Temporal lobe epilepsy not only impairs development of emotion recognition, but can also impair development of perception of other socio‐perceptual signals in children with or without intellectual deficiency. Prospective studies need to be designed to evaluate the results of appropriate re‐education programs in children presenting with deficits in social cue processing.
A child with ictal vocalizations and generalized epilepsy
Authors: Kurian Mary , Barras Anne‐Chantal Heritier , Korff Christian M 
Published: August 2019

AbstractIctal vocalizations in the form of both articulate speech and non‐speech vocalizations have been described in focal epilepsies, with seizures originating mainly from the frontal and temporal lobe, however, this phenomenon has not been described in generalized epilepsies. We report the case of an adolescent boy with juvenile‐onset generalized epilepsy who presented with ictal “ovine vocalizations” (resembling the bleating of sheep). The ictal EEG revealed a clear correlate of vocalizations with time‐locked generalized spikes and polyspike discharges. The 3T cerebral MRI ruled out any focal lesion. The boy is currently seizure‐free under valproic acid, after twelve months of follow‐up. We conclude that ictal non‐speech vocalizations may be observed not only in focal or structural epilepsies, but also in generalized epilepsies; the exact underlying mechanism of this phenomenon needs to be further delineated. [Published with video sequence]
Neuroimaging in neonatal seizures
Authors: Weeke Lauren C , Van Rooij Linda GM , Toet Mona C , Groenendaal Floris , de Vries Linda S 
Published: August 2019

AbstractSeizures are the most common sign of neurological dysfunction in full‐term neonates, with an incidence estimated at 0.15–3.5/1,000 live births. Neonatal seizures often reflect severe underlying brain injury and are associated with high rates of mortality and morbidity. Prognosis is primarily determined by the nature, site and extent of the underlying aetiology, making accurate diagnosis and identification of associated brain lesions essential. Data on neuroimaging in newborns presenting with seizures is limited and most studies report on MRI findings in infants with a specific underlying problem, such as hypoxic‐ischaemic encephalopathy, stroke or metabolic disorders. The aim of this review is to discuss the spectrum of neuroimaging findings in full‐term newborns presenting with seizures, divided into subgroups with different underlying aetiologies. A standard neonatal MRI protocol is presented.
Periodic eye opening and upward eye deviation accompanied by burst‐suppression, as an isolated clinical manifestation of acute post‐hypoxic myoclonus
Authors: Dericioglu Nese , Arsava Ethem Murat , Topcuoglu Mehmet Akif 
Published: August 2019

AbstractVarious movement disorders have been described following hypoxic‐ischaemic brain injury. Here, we present a 72‐year‐old female patient who developed periodic opening and upward deviation of the eyes as an isolated clinical finding, within 24 hours after cardio‐pulmonary arrest. These movements were accompanied by burst‐suppression on EEG, and both clinical and electrophysiological findings were suppressed 18 hours after intravenous levetiracetam infusion. The strictly periodic nature of both EEG discharges and eye opening with vertical deviation suggest a cause due to either activation of a subcortical/brainstem pacemaker reciprocally stimulating the cortex, or, alternatively, post‐anoxic burst activity of viable cortical neural networks, somehow stimulating the relevant oculomotor nuclei. Together with previous similar cases, our case expands the spectrum of post‐resuscitation myoclonus syndromes with the addition of this rare isolated oculopalpebral subtype. [Published with video sequence]
Panayiotopoulos syndrome and diffuse paroxysms as the first EEG manifestation at clinical onset: a study of nine patients
Authors: Caraballo Roberto Horacio , Pasteris María Constanza , Portuondo Ernesto , Fortini Pablo Sebastian 
Published: August 2019

AbstractAim. To present a retrospective study of nine children with Panayiotopoulos syndrome associated with diffuse spikes and waves as the sole EEG manifestation at onset.Methods. Charts of children with typical clinical criteria of Panayiotopoulos syndrome, electroclinically followed between February 2000 and February 2012, were reviewed.Results. Among 150 patients who met the electroclinical criteria of Panayiotopoulos syndrome, we identified nine children who presented with the typical clinical manifestations but who, on EEG, only had diffuse paroxysms at onset that continued along the course of the syndrome. In three, in addition to the diffuse paroxysms, focal spikes appeared later. From a clinical point of view, other features were otherwise unremarkable. Diffuse spike‐and‐wave discharges were observed in all patients when awake and during sleep (100%). Later, three children also had focal spikes during sleep, which were occipital in one, frontal in one, and temporo‐occipital in the remaining patient. Spikes were activated by sleep in all three cases. During disease evolution, no particular electroclinical pattern was observed. Two patients who received clobazam and carbamazepine, respectively, did not respond well to the drugs and valproic acid was added with excellent seizure control. Outcome was good.Conclusions. We present evidence that patients with Panayiotopoulos syndrome may have diffuse discharges at onset as the sole EEG manifestation, which last throughout the course of the syndrome. In some, focal paroxysms developed later. The course was benign. In our group of patients, clinical features and evolution were similar to those of typical cases of Panayiotopoulos syndrome.