bluuebunny/crossref_metadata_2025_split huggingface dataset:
Significance of interictal occipital epileptiform discharges in children
Authors: Wang Chunyang , Khurana Divya S. , Kothare Sanjeev V. , Legido Agustin , Harrison Geoffrey , Carvalho Karen S. , Valencia Ignacio 
Published: August 2019

ABSTRACTObjectiveInterictal occipital epileptiform abnormalities have not been well characterized. The objective of this pilot study was to assess their significance in children.MethodsA search was performed on the EEG database for the keywords “occipital”, “spike”, “sharp wave” and “epileptiform”. Patients were divided into two groups based on the absence of all (group 1) or presence of any (group 2) of the following criteria: mental retardation, cerebral palsy, neurological deficits, abnormal MRI and/or intractable epilepsy. Special attention was given to the spike/sharp wave amplitude/duration and background slowing.ResultsA total of 44 children (eight months to 15 years) were studied. Groups 1 and 2 were each composed of 22 children. Background slowing was more frequent in group 2 (10/22, 45%) compared to group 1 (1/22, 4.5%; p = 0.002). In group 2, 8/22 (36%) had spikes or sharp waves with amplitudes below 50 μV or above 150 μV with a positive predictive value of 89%, and a negative predictive value of 39%. Only 1/22 (4.5%) in group 1 had epileptiform activity outside of the 50‐150 μV range.ConclusionsThe presence of very high or low‐amplitude occipital epileptiform abnormalities or background slowing may be indicative of encephalopathy.
Prevalence of epilepsy in northeast Turkey
Authors: Velioglu Sibel K. , Bakirdemir Metin , Can Gamze , Topbas Murat 
Published: August 2019

ABSTRACTThe aim of this study was to determine the prevalence and clinical and socio‐demographic characteristics of active epilepsy in the population, aged 15 and over, in the province of Trabzon in northern Turkey. We surveyed households and identified 34 epileptic patients (prevalence of 6/1,000), 28 of whom had active epilepsy (prevalence of 5/1,000). Only one case of hot water epilepsy was established among the 5,254 participants. Of the various seizure types, the most common were partial seizures (63%), over half of which were secondary generalised seizures. The largest syndromic category was that of localisation‐related symptomatic cases (46%). Forty‐six percent of cases were of unknown cause, and 16% were resistant to medication. The prevalence rate of active epilepsy in Trabzon is low compared to other parts of Turkey and other developing countries. This may be attributable to several factors, and particularly to variations among socio‐economic factors. The population of Trabzon is regarded as relatively stable and homogenous, and socio‐demographic and health data for the province of Trabzon are much better than those for the rest of the country.
A distinct variant of focal cortical dysplasia type I characterised by magnetic resonance imaging and neuropathological examination in children with severe epilepsies
Authors: Blümcke Ingmar , Pieper Tom , Pauli Elisabeth , Hildebrandt Michelle , Kudernatsch Manfred , Winkler Peter , Karlmeier Anja , Holthausen Hans 
Published: August 2019

ABSTRACTFocal Cortical Dysplasias (FCDs) present with a large clinicopathological spectrum. FCDs are believed to relate directly to an epileptogenic condition, although seizure control by surgical resection is variable. This applies in particular to young children with multilobar FCDs, suffering from severe epilepsies and psychomotor retardation. Herein, we performed a comparative analysis of presurgically available data and microscopic inspection of resected cortical specimens to further characterise the pathomorphological spectrum of FCD. Multilobar resection procedures were performed in a consecutive series of 18 young children (mean 7.6 years) with severe pharmaco‐resistant epilepsies following extensive presurgical surface‐/invasive video‐EEG monitoring intraoperative electro‐corticography (iECoG), as well as high resolution MRI. In all cases, systematic neuropathological examination of surgical specimens was performed with respect to architectural abnormalities and cell density measurements. These histomorphological data were compared with volumetric MRI analysis. Histopathological examination revealed increased neuronal densities correlating with decreased cortical thickness and abundance of neuronal microcolumns in all cases. Intriguingly, the affected cerebral hemisphere was significantly smaller, relative to the non‐epileptogenic contralateral side, in 16 children of our patient series. In conclusion, hypoplastic neocortex and columnar architectural disorganisation point to compromised cortical development, and appear as distinct FCD subtype in children suffering from severe epilepsies and psychomotor retardation.
Epilepsia partialis continua as the presenting symptom in probable sporadic Creutzfeldt‐Jakob disease
Authors: Taskiran Aslihan , Tezer F. Irsel , Saygi Serap 
Published: August 2019

ABSTRACTCreutzfeldt‐Jakob disease (CJD) is a rare form of rapidly progressive neurodegenerative disorder. Literature survey reveals only four reported cases of CJD with epilepsia partialis continua. Here, we present a review and a case study, with video‐EEG sequences and characteristic MRI findings, of a fifth patient who presented with epilepsia partialis continua in the very early stage of the disease and followed a fatal course which was faster than expected.[Published with video sequences]
Propofol withdrawal seizures: non‐epileptic nature of seizures in a patient with recently controlled status epilepticus
Authors: Zubair Salman , Patton Thomas , Smithson Kenneth , Sonmezturk Hasan H. , Arain Amir , Abou‐Khalil Bassel 
Published: August 2019

ABSTRACTSeizures or seizure‐like phenomena which are mostly convulsive have been observed during the induction, maintenance and withdrawal phases of propofol administration. The nature and mechanism of this phenomenon are not well understood and several case reports on these phenomena have presented only indirect evidence. We report on a patient who was administered propofol in order to control status epilepticus with success. However, every attempt at propofol withdrawal was followed by convulsive seizure‐like activity. Continuous EEG monitoring showed muscle artefacts without any ictal discharges. Based on this finding, the propofol treatment was withdrawn and the seizure‐like activity eventually attenuated and resolved. We propose that seizure‐like phenomena associated with propofol withdrawal may not be ictal in nature and should not lead to unnecessary resumption of propofol infusion without documentation of an epileptic origin by EEG.
Impact of side of lesion, seizure outcome and interictal epileptiform discharges on attention and memory after surgery in temporal lobe epilepsy
Authors: Wisniewski Ilona , Wendling Anne‐Sophie , Steinhoff Bernhard J. 
Published: August 2019

ABSTRACTPurposeTo determine the impact of side of surgery, seizure outcome and interictal epileptiform discharges (IEDs) on attention and memory in a cohort of patients with temporal lobe epilepsy who had undergone unilateral mesial temporal lobe resection.Material and methods. Ninety‐four patients were investigated pre‐ and postoperatively by means of a standardised neuropsychological battery measuring subcomponents of attention, as well as short‐term, working and long‐term memory. The side of epilepsy surgery, seizure outcome and the presence of postoperative IEDs, as well as their possible relationship to the neuropsychological changes, were assessed. Statistical data were analysed using a repeated‐measures MANOVA.ResultsThe absence of seizures following surgery had a positive effect on short‐term memory and attentional control. The occurrence of IEDs was found in patients with impaired figural learning. In terms of attentional control and working memory, patients who continued to present IEDs had also scored lower in these domains prior to surgery.ConclusionIEDs had an effect independent of seizure presence, but were found to have a “supplementary negative effect” when the two variables were combined.
Asymmetric hemispheric representation of periictal heart rate modulation is individually lateralised
Authors: Panchani Jaina , Adjei Patrick , Henneberger Christian , Scott Catherine A. , Thompson Alan J. , Diehl Beate , Walker Matthew C. , Surges Rainer 
Published: August 2019

ABSTRACTAsymmetric cortical representation of cardiac function is a matter of debate and large inter‐individual variability of cortical autonomic networks and different study designs may contribute to this controversy. Lateralised seizure activity in individual patients may provide valuable insights into cortical regulation of cardiac function. We report two patients with focal epilepsy who had seizures arising from both hemispheres. In Patient 1, heart rate increased over two‐fold with seizures arising from the right hemisphere, whereas heart rate increased invariably less with seizures arising from the left hemisphere. In Patient 2, heart rate increased 1.3 fold or less with seizures arising from the left hemisphere, whereas a seizure with right‐sided onset was followed by bradycardia and asystole. Our findings support the notion that effects on autonomic function are lateralised, although lateralisation varies from patient to patient. This may partially explain the difficulty in determining cortical representation of cardiac autonomic function.
Transient parietal hand syndrome after cortical resection
Authors: Oster Joel M. , Apetauerova Diana , Thomas Christine , Cosgrove G. Rees 
Published: August 2019

ABSTRACTThis case study with video recording describes a brief neurological examination of a rare post‐surgical finding in a patient with intractable seizures who had a right parietal topectomy in order to cure focal and disabling epilepsy. Contemporaneously during the resection and while awake intraoperatively, the patient developed features characterised by involuntary, purposeless, and almost ballistic movements of the contralateral left upper extremity. These involved the shoulder and, more distally, the arm with less involvement of the hand itself and some clonic movement at the elbow, persisting for approximately 24 hours after surgery. Although identified in only one case in our series, we have named the resulting clinical phenomenology “parietal hand syndrome”. A Medline search does not reveal any other such case in the English literature with the clinical elements and actual video documentation of neurological examination noted in our case report in the immediate post‐operative setting. In this regard, this is a unique clinical report. [Published with video sequences]
A case imitating Panayiotopoulos syndrome
Authors: Bolukbasi Feray , Hamamcioglu Irem , Demirbilek Veysi , Dogangun Burak , Dervent Aysin 
Published: August 2019

ABSTRACTPanayiotopoulos syndrome is one of the most common childhood‐specific epileptic disorders. It is characterized by autonomic‐symptoms; the most common being emesis. However, the presence of emetic symptoms may cause misdiagnosis, for example with some organic disorders which have similar autonomic features. On the other hand, since this syndrome has been recently well documented, the tendency for early recognition may lead to overdiagnosis. Here, we present a case which could be mistaken for Panayiotopoulos syndrome based on anamnesis, however, with the aid of ictal video‐EEG monitoring, the patient was shown to present with a non‐epileptic psychogenic seizure. This report is an example of careful evaluation in order to avoid over‐ or underdiagnosis of this benign disorder. [Published with video sequences]
Bilaterally independent epileptic spasms in a case of Aicardi syndrome
Authors: Kobayashi Katsuhiro , Watanabe Kiyoko , Yoshinaga Harumi , Ohtsuka Yoko 
Published: August 2019

ABSTRACTA girl with Aicardi syndrome was observed to have two distinct types of asymmetric epileptic spasms, as detected by ictal video‐EEG recording at three months of age. When the two types of spasm concurred, they showed no mutual interactions based on either clinical or EEG aspects. This observation does not support the hypothesis that the brainstem always plays an initiating role in generating spasms. [Published with video sequences]
Lamotrigine is favourable for startle‐induced seizures
Authors: Ikeda Hiroko , Imai Katsumi , Ikeda Hitoshi , Shigematsu Hideo , Shishido Takeo , Takayama Rumiko , Fujiwara Tateki , Takahashi Yukitoshi , Inoue Yushi 
Published: August 2019

ABSTRACTFalling due to startle‐induced seizures (SISs) often leads to injury. The triggers of SIS are mostly unexpected auditory stimuli, which are too common to avoid in daily life. As SISs are often refractory to conventional medications, effective therapeutic options have to be established. We report a small series of six patients treated with lamotrigine (LTG) as add‐on therapy. Seizure control was improved greatly in three of the six patients, resulting in less restricted daily life, but no effect was observed in two and a skin rash developed in one. Patient 1 was a 19‐year‐old man. His seizure comprised of a sudden tonic extension of the extremities induced by auditory or visual stimulus. He fell down due to SISs, five to ten times a day, with frequent injuries. After adding LTG to treatment with valproate (VPA) and clobazam (CLB), SISs were reduced to once a month. Patient 2 was a 51‐year‐old woman. Sudden tonic extension of all limbs induced by unexpected sounds frequently threw her down onto the floor. Addition of LTG to treatment with CLB, zonisamide and phenytoin reduced her SISs from several to less than once a day. Patient 3 was a seven‐year‐old girl with post‐encephalitic epilepsy. After adjunctive treatment of LTG to VPA, the severity of SISs became milder thus avoiding injury, although seizure frequency did not decrease. LTG is potentially effective for the treatment of SISs and may prevent falling. The addition of LTG treatment dramatically improved the lives of the patients presented here and should be considered as an option for startle‐induced seizures.
Early‐onset childhood absence epilepsy: is it a distinct entity?
Authors: Farooque Pue , Goraya Jatinder , Valencia Ignacio , Carvalho Karen S , Hardison H Huntley , Legido Agustin , Khurana Divya S 
Published: August 2019

ABSTRACTChildhood absence epilepsy (CAE) typically starts between four and seven years of age. Onset before three years is rare and has not been previously reported from North America. We retrospectively reviewed the electroencephalography laboratory database and paediatric neurology clinic records (from January 2000 to June 2009) at our institution in order to identify patients with absence seizures beginning before age three. Information was collected for age, gender, neurodevelopment, antiepileptic drugs (AEDs) used, seizure control, follow‐up, and side effects. Of 12 patients identified, mean age at onset was 20.5 months (range:11 months to two years; follow‐up: six months to 11 years). Seven of 12 patients had normal neurodevelopment and five had speech delay. Four patients were seizure‐free without AEDs, three were seizure‐free with a single AED, and five still had seizures with multiple AEDs. Three patients had recurrences after medication withdrawal. Other previously published series have identified better seizure control than that reported here, however, 16% of the 130 patients so far documented are reported to have poorly controlled epilepsy, indicating that early‐onset CAE is not a homogeneous condition. The debate as to whether early‐onset CAE is a distinct epilepsy syndrome therefore continues. We believe that early‐onset CAE may be a distinct epilepsy syndrome, with some features that overlap with those of typical CAE, as well as unique distinguishing features. Large prospective multicentric studies would be necessary to definitely resolve this matter.
Coexistence of two distinct benign EEG variants in the same subject
Authors: Brigo Francesco , Manganotti Paolo , Fiaschi Antonio , Bongiovanni Luigi Giuseppe 
Published: August 2019

ABSTRACTRhythmic temporal theta bursts of drowsiness (RTTD), also known as “psychomotor variant”, and subclinical rhythmic EEG discharge of adults (SREDA) are two EEG patterns of uncertain significance that occur without any correlation with epilepsy. Each of these patterns has been described to occur alone and in the literature there are no previous reports of co‐occurrence of the two distinct benign EEG variants in the same patient. We describe the coexistence of RTTD and SREDA in EEG recordings from the same subject. Although the coexistence of two distinct EEG variants in the same patient is a rarity, these patterns are not so infrequently encountered when present alone and should thus be promptly recognised in order to avoid misdiagnosis of epilepsy due to an over‐interpretation of normal sharp patterns.
Analysis of acylcarnitine levels by tandem mass spectrometry in epileptic children receiving valproate and oxcarbazepine
Authors: Cansu Ali , Serdaroglu Ayse , Biberoglu Gursel , Tumer Leyla , Hirfanoglu Tugba Luleci , Ezgu Fatih Suheyl , Hasanoglu Alev 
Published: August 2019

ABSTRACTThis prospective study was designed to investigate whether or not monotherapy with sodium valproate (VPA) or oxcarbazepine (OXC) affects plasma levels of fatty acylcarnitine esters in children with epilepsy. A total of 56 children with idiopathic partial or generalised epilepsy were included in the study. Patients were assigned to receive either VPA or OXC monotherapy. Free carnitine (C0) and acylcarnitine profiles of the patients were investigated using tandem mass spectrometry at baseline and at six and 18 months after commencement of therapy. For patients receiving VPA or OXC monotherapy, there were no significant differences in plasma levels of C0, compared with baseline, at six and 18 months (p>0.05). Treatment with VPA for six and 18 months correlated with a significant increase in 3‐hydroxy‐isovalerylcarnitine (C5‐OH) (six months: +23%; 18 months: +73%), and significant decreases in the following acylcarnitines: C6‐acylcarnitine (six months: ‐60%; 18 months: ‐66%), C14‐acylcarnitine (six months: ‐25%; 18 months: ‐38%), C16‐acylcarnitine (six months: ‐73%; 18 months:‐73%), and C18:1‐OH‐acylcarnitine (six months:‐60%; 18 months: ‐70%), compared with baseline (p<0.05). In patients receiving OXC monotherapy, on the other hand, plasma concentrations (μmol/L) of acylcarnitines (from C2 to C18:1‐OH) fell within the normal reference range. The results of this study indicate that there are significant biochemical changes in acylcarnitines in ambulatory children on VPA monotherapy but these are not clinically significant. OXC monotherapy had no effect on acylcarnitine metabolism in ambulatory children.
Starting at the beginning: the neuropsychological status of children with new‐onset epilepsies*
Authors: Hermann Bruce P , Jones Jana E , Jackson Daren C , Seidenberg Michael 
Published: August 2019

ABSTRACTThis review examines the neurodevelopmental contribution to the cognitive and behavioural complications of epilepsy. Following a brief review of the lifespan complications of childhood epilepsies, attention turns to cognitive, psychiatric and social correlates of childhood epilepsies reported in population‐based and tertiary care studies. The focus then becomes the neurobehavioural status of children with new‐onset epilepsy; a point in time not confounded by the effects of years of recurrent seizures, medications, and social reactions to epilepsy. Recent research shows that abnormalities in cognition, brain structure and behaviour are present at or near the time of diagnosis. Further, careful history taking indicates that neurobehavioural problems may be present in advance of the first seizure suggesting the potential influence of epileptogenesis, antecedent neuro‐developmental abnormalities, genetic and environmental susceptibilities, and other risk factors. This becomes the substrate upon which to characterise the effects of epilepsy and its treatment on subsequent neurodevelopment. The review concludes with suggestions for future clinical care and research.
PET follow‐up in a case of anti‐NMDAR encephalitis: arguments for cingulate limbic encephalitis
Authors: Chanson Jean‐Baptiste , Diaconu Mihaela , Honnorat Jérôme , Martin Thierry , De Seze Jérôme , Namer Izzie‐Jacques , Hirsch Edouard 
Published: August 2019

ABSTRACTBackground. The lack of specific MRI abnormalities in anti‐NMDA receptor encephalitis makes the identification of the most affected areas difficult. Functional neuroimaging could be useful to identify brain dysfunction associated with psychiatric symptoms, but few precise data are available up to now. Case study. A 27‐year‐old woman was referred for recent behavioural changes and jerks of the right foot. Serial left fronto‐temporal seizures were recorded. Identification of anti‐NMDA receptor antibodies in CSF indicated a diagnosis of anti‐NMDA receptor encephalitis. Two foci of hypermetabolism, in the left prefrontal cortex and the anterior cingulate cortex, were identified using 18‐fluorodeoxyglucose PET and both disappeared after treatment. Brain MRI was normal, except for a mild left prefrontal hypersignal. Conclusions. The increase in marker uptake in motor and premotor regions in our case probably corresponds to epileptic activity. Our data suggest that the anterior cingulate cortex could play an important role in psychiatric symptoms. Other studies are needed to better understand the pathophysiology of anti‐NMDA receptor encephalitis.
Ambulatory non‐convulsive status epilepticus evolving into a malignant form
Authors: Fernández‐Torre José L , Kaplan Peter W , Rebollo Mariano , Gutiérrez Agustín , Hernández‐Hernández Miguel A , Vázquez‐Higuera José L 
Published: August 2019

ABSTRACTWe retrospectively analysed the clinical characteristics, electroencephalogram (EEG) records, brain magnetic resonance imaging (MRI) scans, antiepileptic therapy and prognosis of a case series with ambulatory non‐convulsive status epilepticus (NCSE) which evolved into a malignant form (mNCSE). mNCSE was defined as NCSE that continues or recurs five days or more after the onset of general anaesthesia, including those cases where NCSE recurs upon reduction or withdrawal of anaesthetic therapy. Four women and two men were studied. The mean age was 42.8 years (range: 19‐63 years). Two of six patients had a previous diagnosis of epilepsy. Four patients died. Two patients had a good outcome with return to consciousness and activities of daily life, and one of these subjects developed temporal lobe epilepsy. In four patients, the cause of mNCSE was encephalitis. Clinical presentation was complex partial status epilepticus (CPSE) in three patients and generalised NCSE (GNCSE) in the other three. Two of the latter had encephalitis and one was diagnosed with progressive myoclonus epilepsy (PME) of Lafora type. The mean duration of the NCSE episode was 47.5 days (range: 9‐139 days; SD: 53.1). In three patients, continuous or rhythmic focal epileptiform discharges were the EEG pattern at onset. In the other three, continuous rhythmic generalised spike‐wave, polyspike‐wave or sharp slow‐wave complexes were observed. Antiepileptic treatment was heterogeneous and patients were treated with an average of 6.0 AEDs (range: 3‐10; SD: 3.0). In summary, mNCSE is a severe epileptic condition that occurs often in the context of encephalitis or pre‐existing epilepsy. Both CPSE and GNCSE may occur. Although high mortality is associated with mNCSE, some patients may survive with normal or good recovery.