bluuebunny/crossref_metadata_2025_split huggingface dataset:
Electroconvulsive therapy for psychosis in a patient with epilepsy related to hypothalamic hamartoma
Authors: Ruppert Elisabeth , Lalanne Laurence , Foucher Jack , Zimmermann Marie‐Agathe , Hirsch Edouard , Vidailhet Pierre 
Published: August 2019

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

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

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

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

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

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

AbstractWe investigated the anatomo‐electro‐clinical features and clinical outcome of surgical resections strictly confined to the parietal lobe in 40 consecutive patients who received surgery for pharmacoresistant seizures. The population was subcategorized into a paediatric (11 subjects; mean age at surgery: 7.2+/−3.7 years) and an adult group (29 patients; mean age at surgery: 30+/−10.8 years). The paediatric group more frequently exhibited personal antecedents, neurological impairment, high seizure frequency, and dysplastic lesions. Nonetheless, compared with adults, children had better outcome and more frequently reached definitive drug discontinuation after surgery.After a mean follow‐up of 9.4 years (range: 3.1–16.7), 30 subjects (75%) were classified as Engel Class I. The presence of multiple types of aura in the same patient, as well as a high incidence of secondary generalization, represented a characteristic feature of parietal seizures and did not correlate negatively with surgical outcome. A total resection of the epileptogenic zone and a localizing/regional interictal EEG were statistically significant predictive factors of outcome. Intracerebral investigation, performed in 55% of cases, contributed to complete tailored resections of the epileptogenic area and determination of prognosis. Frequent subjective manifestations of parietal lobe seizures, such as vertiginous, cephalic and visual‐moving sensations, underscore their potential misdiagnosis as non‐epileptic events.
Anomia produced by direct cortical stimulation of the pre‐supplementary motor area in a patient undergoing preoperative language mapping
Authors: Rozanski Verena Eveline , Peraud Aurelia , Noachtar Soheyl 
Published: August 2019

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

AbstractAim. Convulsive status epilepticus is the most common childhood neurological emergency in developing countries, where poor healthcare organisation could play a negative role in the management of the condition. Unavailability of second‐line injectable anticonvulsants is an additional hindering factor in Georgia. This report reflects the results of the first study aimed at evaluating the epidemiological features of convulsive status epilepticus, as well as identifying obstacles influencing the management of patients with convulsive status epilepticus in Georgia.Methods. A prospective, hospital‐based study was performed. Paediatric patients with convulsive status epilepticus, admitted to the emergency department of a referral academic hospital from 2007 to 2012, were included in the study.Results. Forty‐eight paediatric patients admitted to hospital met the criteria for convulsive status epilepticus. Seizure duration was significantly shorter among the group with adequate and timely pre‐hospital intervention. Moreover, patients with appropriate pre‐hospital treatment less frequently required mechanical ventilation (p=0.039). Four deaths were detected during the follow‐up period, thus the case fatality rate was 8%. Only 31% of patients received treatment with intravenous phenytoin.Conclusion. The study results show that adequate and timely intervention could improve outcome of convulsive status epilepticus and decrease the need for mechanical ventilation. Mortality parameters were comparable to the results from other resource‐limited countries. More than one third of patients did not receive appropriate treatment due to unavailability of phenytoin.
Treatable newborn and infant seizures due to inborn errors of metabolism
Authors: Campistol Jaume , Plecko Barbara 
Published: August 2019

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

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

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

AbstractAims. To evaluate the efficacy and safety of mega‐dose phenobarbital (MDPB; enteral or parenteral phenobarbital >10 mg/kg/day) for treating super‐refractory status epilepticus (SRSE; continuous or recurrent status epilepticus for ≥24 hours after the onset of continuous anaesthetic treatment) in adult patients.Methods. Adult patients with SRSE who were treated with MDPB in our institution from March 2005 to September 2014 were reviewed. We collected data on basic demographics, clinical features, functional status, anticonvulsant treatment, and possible adverse events. SRSE outcome was divided into six categories: successful therapy, initial failure, breakthrough seizures, withdrawal seizures, intolerable side effects, and death during treatment.Results. Ten adult patients with SRSE received MDPB. Median age at seizure onset was 38 years (range: 18‐59), and half were male. All patients had no history of seizures and had symptoms suggestive of viral encephalitis. Median duration of status epilepticus was 17.5 days (range: 6–60) and anaesthetics were used for a median of 14.0 days (range: 2–54) before MDPB. Successful control of SRSE was achieved in half of the patients, however, only one of ten patients was able to fully recover at discharge. Median duration of the MDPB was 45.5 days and the maximum serum phenobarbital level reached a median of 151.5 μg/ml. Patients with successful MDPB therapy had normal brain imaging (80% vs. 0%; p=0.048) and better functional outcome at discharge and after three months of follow‐up. Infection was the most critical complication, along with cardiorespiratory depression.Conclusion. MDPB is a therapeutic option for control of SRSE when other choices are exhausted.
Characterization of ictal slow waves in epileptic spasmsCharacterization of ictal slow waves in epileptic spasms
Authors: Honda Ryoko , Saito Yoshiaki , Okumura Akihisa , Abe Shinpei , Saito Takashi , Nakagawa Eiji , Sugai Kenji , Sasaki Masayuki 
Published: August 2019

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

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

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

AbstractAim. Lennox‐Gastaut syndrome (LGS) is characterized by interictal epileptiform discharges (IEDs) occurring during sleep. The aim of this study was to determine whether sleep influences not only the frequency of seizures and IEDs, but also the time‐dependent evolution that may support the hypothesis of homeostatic influences on epileptic threshold.Methods. Video polysomnography data from our database were reviewed to identify adult LGS patients with at least seven hours of nocturnal recording. Thirteen patients were identified and a second polysomnography was available for nine. The number, duration and index of IEDs, relative to total sleep, sleep stages, and time during the night, were calculated.Results. The majority of IEDs occurred during non‐rapid eye movement sleep, mainly in stage 2 and slow‐wave sleep. Adjusting for time spent in each sleep stage, we found 45 IEDs/hour in stage 1, 123/hour in stage 2, 106/hour in slow‐wave sleep, and 26/hour in rapid eye movement sleep. The temporal distribution of IEDs showed a significant rise in the first three hours of sleep, followed by a progressive decrease at the end of the night (F=85.6; p<0.0001).Conclusion. Interictal epileptiform discharges occurrence in adult LGS is facilitated by non‐rapid eye movement sleep with an evident effect of stage 2 and slow‐wave sleep. The significant IED occurrence in the first part of the night and the subsequent decline suggests a link between epileptic threshold and homeostatic sleep mechanisms. The latter should be considered regarding choice of therapy.