bluuebunny/crossref_metadata_2025 huggingface dataset:
Stimulus‐sensitive post‐anoxic focal motor seizures evolving into generalised myoclonic status epilepticus: a video‐EEG study
Authors: Fernández‐Torre José L. , Santos‐Sánchez Carlos , Casariego‐Pola Francisco , Calleja Jesús
Published: August 2019

We describe the case of a 62‐year‐old man who developed stimulus‐induced focal motor seizures after prolonged cardiac arrest. During a video‐EEG study, these focal motor seizures progressed into a generalised myoclonic status epilepticus. Both the severely decremented background activity on the EEG, and the absence of median and trigeminal somatosensory evoked potentials were in keeping with a devastating post‐hypoxic encephalopathy and the patient died. Our clinical and electrophysiology data suggest that generalised myoclonic status epilepticus may occur in patients in whom the existence of severe cerebral damage prevents a complete development of all phases of classic generalised tonic‐clonic seizures. [Published with video sequences]
Reversible sleep‐related stridor during vagus nerve stimulation
Authors: St. Louis Erik K. , Faber Kevin
Published: August 2019

A 23‐year‐old woman without history of antecedent vocal, respiratory, or sleep disorders received vagus nerve stimulation (VNS) therapy for refractory partial epilepsy and developed sleep‐related stridor during the course of parameter titration. Reduction of VNS current during polysomnography completely eliminated stridor. We conclude that VNS may cause sleep‐related stridor in rare cases, expanding the spectrum of known sleep‐disordered breathing disorders associated with VNS therapy. Parameter adjustment during polysomnography may resolve nocturnal stridor caused by VNS. [Published with video sequences]
Bathing epilepsy: report of two Caucasian cases
Authors: Franzoni Emilio , Gentile Valentina , Grosso Salvatore , Brunetto Daniela , Cordelli Duccio Maria , Balestri Paolo
Published: August 2019

Bathing epilepsy, also known as water‐immersion epilepsy, refers to a rare form of benign reflex epilepsy in which seizures are precipitated by the normal process of domestic bathing. This condition has been confused with true hot water epilepsy, even though bathing in water at normal temperature is the trigger. Focal seizures predominate with a staring gaze, pallor and generalised features followed by prolonged postictal somnolence. A variable percentage of patients may also show unprovoked seizures. The prognosis is usually favourable, and modifying bathing habits may prevent further seizures. We report two Caucasian patients with bathing epilepsy. In one, seizures were provoked by water immersion. In the other, we noted an unusual triggering factor; pouring of lukewarm water over the genitalia.
Long‐term and late seizure outcome after surgery for temporal lobe epilepsy
Authors: Pimentel José , Bentes Carla , Campos Alexandre , Ferreira A. Gonçalves , Group Epilepsy Surgery
Published: August 2019

Aim

Although surgery for temporal lobe epilepsy (TLE) harbours a good prognosis, post‐operative seizures may occur. Long‐term, postoperative seizure follow‐ups are rare but necessary to properly define outcome.

Methods

Longitudinal, long‐term, post‐operative seizure follow‐up in TLE patients with outcome analysed using Engel's classification. Three groups were considered according to the type of resection: isolated amygdalohippocampectomy (IAH), further divided into anterior and complete, AH plus focal neocortical resections (AH + FR) and focal neocortical resections (FR).

Results

Eighty‐nine patients were enrolled (61 in the IAH group, 24 in the AH + FR group, and four in the FR group), with a mean follow‐up time of 46.7 months. For the three groups together, 90.9% and 86.7% of the patients were in Engel class I for six months and five years, respectively. Kaplan‐Meir analysis of the IAH and AH + FR groups showed that, while 82.2% of patients of the IAH group tended to remain in class I within 84 months after surgery, 86.7% of the AH + FR group tended to remain in class I within 12 months. Kaplan‐Meier analysis of the IAH sub‐groups showed that more patients (91.0%) with anterior resection tended to remain in class I, although for a longer period of time (36 months), compared to those with complete resection (84.0% of patients and 12 months, respectively). For the IFR group, only three patients were in Engel class I for long‐term follow‐up.

Conclusions

High rates of seizure freedom were obtained and stably maintained for years. The reasons for better long‐term prognosis of the anterior IAH group are so far unclear, the IFR group was too small to draw any conclusive data.
Improvement of myoclonic epilepsy in Down syndrome treated with levetiracetam
Authors: Sangani Masoud , Shahid Asim , Amina Shahram , Koubeissi Mohamad
Published: August 2019

Late Onset Myoclonic Epilepsy in Down Syndrome (LOMEDS) is a recognized entity usually preceded by cognitive deterioration. We report two patients with LOMEDS and cognitive decline, aged 52 and 44 years. Continuous video‐EEG recording showed generalised spike and slow wave complexes as an ictal correlate of the myoclonic jerks in both patients. Low dose levetiracetam resulted in rapid, sustained seizure freedom in both patients with no reported adverse events. As for other myoclonic epilepsies, levetiracetam appears to be effective for the treatment of LOMEDS, and may be considered as a first line agent for this disorder.
Lateralized ictal dystonia of upper and lower limbs in patients with temporal lobe epilepsy
Authors: Kuba Robert , Tyrlíková Ivana , Brázdil Milan , Rektor Ivan
Published: August 2019

Purpose

To perform a retrospective study to determine the incidence and lateralizing value of ictal dystonia in patients with temporal lobe epilepsy.

Methods

The study included 142 patients (76 males, 66 females) with temporal lobe epilepsy, aged from 19 to 58 years with an average age of 33.1 ± 8.7 years. Overall, 454 seizures were analysed. The seizure onset zone was mesial in 112 patients (78.8%), and “non‐mesial” in 30 patients (21.2%).

Results

Ictal dystonia was present in 68 of the 142 patients (47.9%), and in 186 of 454 total seizures (40.9%). Upper limb dystonia was present in 94 sizures (50.5%) of 32 patients; hemidystonia in 84 seizures (45.2%) of 30 patients; and lower limb dystonia in eight seizures (4.3%) of six patients. For all cases, all types of ictal dystonia were contralateral to the seizure onset zone. Ictal dystonia was significantly more frequent in patients with a mesial seizure onset zone than in “non‐mesial” patients (58.1%vs7.7%; p < 0.001). Within the mesial group, ictal dystonia was significantly more frequent in patients with hippocampal sclerosis than in those patients with other lesions (66.1%vs41.1%; p = 0.023).

Conclusion

Ictal dystonia in temporal lobe epilepsy is a reliable lateralizing ictal sign. During almost half of the seizures studied, ictal dystonia was present in the form of hemidystonia, and isolated involvement of the lower limbs also occured. Combined data obtained from both noninvasive and invasive EEG showed that ictal dystonia tended to occur more often in mesial onset temporal lobe epilepsy, especially when hippocampal sclerosis was the epileptogenic lesion.
Refractory nonconvulsive status epilepticus in Creutzfeldt‐Jakob disease
Authors: Aiguabella Maria , Falip Mercè , Veciana Misericordia , Bruna Jordi , Palasí Antoni , Corral Luisa , Herrero Jose‐Ignacio , Boluda Susana , Mora Jaume , Iranzo Alex , Serrano Carme
Published: August 2019

Creutzfeldt‐Jakob disease (CJD) is a rare human transmissible spongiform subacute encephalopathy. The most common clinical manifestations of CJD include rapidly progressive dementia, behavioural changes, cerebellar dysfunction and myoclonus. Other seizure types are rare and nonconvulsive status epilepticus (SE) is exceptional. We report a case of a 44‐year‐old man who presented a psychotic episode followed by akinetic mutism and refractory nonconvulsive SE. The final diagnosis was CJD. Continuous video‐EEG monitoring revealed the ictal pattern of nonconvulsive SE to be periodic sharp wave complexes characteristic of CJD. [Published with video sequences]
Interictal epileptiform discharges and phasic phenomena of REM sleep
Authors: Busek Petr , Buskova Jitka , Nevsimalova Sona
Published: August 2019

The objective of this study was to assess the effect of phasic rapid eye movement (REM) sleep events on interictal epileptiform discharges (IEDs). Twelve patients with focal epilepsy and IEDs during REM sleep were examined by video‐EEG monitoring. The number of IEDs was calculated in different REM sleep episodes according to the rate of rapid eye movements. A negative correlation was identified between the occurrence of rapid eye movements and IEDs, indicating that the suppression of propagation of IEDs during REM sleep is enhanced by phasic REM sleep events, probably as a result of phasic activation of cholinergic neurons of the ponto‐mesencephalic tegmentum. This study demonstrates that the degree of EEG desynchronization and IEDs is influenced by REM density.
Benign infantile focal epilepsy with midline spikes and waves during sleep: a new epileptic syndrome or a variant of benign focal epilepsy?
Authors: Flesler Santiago , Sakr Diego , Cersósimo Ricardo , Caraballo Roberto
Published: August 2019

Objective

To analyze the electroclinical features and evolution of seven infants with benign infantile focal epilepsy with midline spikes and waves during sleep (BIMSE).

Material and methods

Seven patients were examined at our department between February 2003 and February 2009, with onset of seizures between six and 13 months of age (mean, 10.2 months; median, 11 months). Patients with cryptogenic and symptomatic focal epilepsies were excluded. Sex, age, familial history, type of seizures and AED treatment were noted and EEG monitoring, MRI and CT scanning, and developmental and psychomotor evolution were investigated.

Results

Patients included five males and two females. All patients suffered from seizures during wakefulness. Two of the patients (29%) did not have a recurrence. Five (71%) had sporadic seizures (ranging between two and five). One of the seven patients (14%) presented with seizures in clusters. During seizures, staring was observed in six (86%), motion arrest in five (71%), stiffening in five (71%), cyanosis in three (42%), automatisms in one (14%) and lateralizing signs in four (57%). Two patients (29%) had secondary generalisation. The duration of the seizures ranged between 30 seconds and five minutes. No status epilepticus was observed. The interictal EEG recording during sleep showed low‐voltage unilateral or bilateral spikes located in the central and vertex regions, followed by slow waves in all patients. Outcome was excellent in all patients.

Conclusion

We believe that BIMSE is a new syndrome rather than an early presentation of benign epilepsy of childhood with centrotemporal spikes, Panayiotopoulos syndrome, or a late presentation of benign focal infantile seizures.
Partial epilepsy complicated by convulsive and nonconvulsive episodes of status epilepticus in a patient with ring chromosome 14 syndrome
Authors: Giovannini Simona , Frattini Daniele , Scarano Angela , Fusco Carlo , Bertani Gianna , Giustina Elvio Della , Martinelli Paola , Orteschi Daniela , Zollino Marcella , Neri Giovanni , Gobbi Giuseppe
Published: August 2019

Epilepsy is the most common and serious neurological symptom in ring chromosome 14 syndrome, also characterised by mild dysmorphisms, acquired microcephaly, cognitive impairment, hypotonia and ocular abnormalities. Typically, early‐onset, polymorphous and drug‐resistant seizures are reported. Status epilepticus has not been previously reported. We describe a nine‐year‐old Caucasian boy with ring 14 syndrome who presented a severe early‐onset and drug‐resistant focal epilepsy with secondary generalised seizures and repetitive episodes of convulsive and non‐convulsive status epilepticus. The electro‐clinical evaluation of prolonged seizures and their long‐term consequences is important for the practical management of these patients and for a better comprehension of the syndrome.
Novel familial cases of ICCA (infantile convulsions with paroxysmal choreoathetosis) syndrome
Authors: Rochette Jacques , Roll Patrice , Fu Ying‐Hui , Lemoing Anne Gaëlle , Royer Barbara , Roubertie Agathe , Berquin Patrick , Motte Jacques , Wong Sau Wei , Hunter Alasdair , Robaglia‐Schlupp Andrée , Ptacek Louis J. , Szepetowski Pierre
Published: August 2019

Epilepsy and paroxysmal dyskinesia are two episodic cerebral disorders that can share a common genetic basis. Rare families with infantile seizures and paroxysmal dyskinesia [predominantly paroxysmal kinesigenic dyskinesia (PKD)], co‐inherited as a single autosomal dominant trait, have been described (infantile convulsions with paroxysmal choreoathetosis; ICCA syndrome) and a disease gene has been mapped at chromosome 16p12‐q12 (ICCA region). We report the clinical picture of seven previously unreported families with ICCA syndrome. The identification of novel ICCA families should contribute to better knowledge regarding the clinical manifestations of ICCA syndrome as well as the search for the underlying genetic defect(s).
The efficacy of bromides, stiripentol and levetiracetam in two patients with malignant migrating partial seizures in infancy
Authors: Djuric Milena , Kravljanac Ruzica , Kovacevic Gordana , Martic Jelena
Published: August 2019

The syndrome of malignant migrating partial seizures in infancy is a devastating, age‐specific, epileptic encephalopathy, which still presents an aetiological, pathophysiological and therapeutic problem. In this study, we present two patients who were diagnosed with the disease, based on electroclinical symptoms. The patients were treated with a combination of sodium bromide, stiripentol and levetiracetam. The first patient unequivocally responded, following a course of ineffective conventional drugs, and the second, who was diagnosed and treated immediately, showed a more significant therapeutic response. Antiepileptic drugs, previously reported to be beneficial in case reports, when given concomitantly, may substantially reduce the number and severity of seizures, without influence on psychomotor development.[Published with video sequences]
Focal seizure propagation illustrated by fMRI
Authors: Sonmezturk Hasan H. , Morgan Victoria , Abou‐Khalil Bassel
Published: August 2019

We report the pattern of seizure propagation as detected by functional MRI (fMRI) in a 24‐year‐old man with frequent recurrent electrographic seizures. The EEG seizure onset was left occipital with later spread to the left hemisphere and, to a lesser extent, the right hemisphere. The fMRI showed initial increase in blood oxygen level‐dependent (BOLD) signal in the left occipital pole. The increased signal then propagated to the right occipital‐posterotemporal region, and subsequently, the right and left mesial temporal regions. fMRI can be an effective tool to study seizure onset localization and seizure propagation in patients with frequent recurrent seizures.
Sturge‐Weber syndrome: a favourable surgical outcome in a case with contralateral seizure onset and myoclonic‐astatic seizures
Authors: Jiruska Premysl , Marusic Petr , Jefferys John G.R. , Krsek Pavel , Cmejla Roman , Sebronova Vera , Komarek Vladimir
Published: August 2019

Sturge‐Weber syndrome is a neurocutaneous disorder classically characterized by the presence of facial port‐wine stain and ipsilateral leptomeningeal angiomatosis. It is often associated with refractory epilepsy which requires surgical treatment. We present a case of a patient who initially presented with partial seizures of temporo‐occipital origin, ipsilateral to the pial angiomatosis. During the course of the disease, the patient developed medically refractory epilepsy with partial seizures originating predominantly from the contralateral temporo‐occipital area as well as myoclonic and myoclonicastatic seizures. Resection of the occipital and temporal lobe affected by the pial angioma resulted in favourable outcome. Bilateral dysfunction observed in Sturge‐Weber syndrome may result in an increased capability of focal discharges to generate synchronous epileptiform activity leading to an increased incidence of generalised seizures, most probably via a mechanism of secondary bilateral synchrony. [Published with video sequences]
Addressing overtreatment in patients with refractory epilepsy at a tertiary referral centre in Brazil
Authors: Alexandre Veriano , Monteiro Edna Almeida , Freitas‐Lima Priscila , Pinto Kylvia Dantas , Velasco Tonicarlo Rodrigues , Terra Vera Cristina , Pinheiro‐Martins Ana Paula , Souza Roberto Molina , Perucca Emilio , Sakamoto Americo Ceiki
Published: August 2019

Background

Patients with refractory epilepsy often have impaired quality of life (QOL) as a consequence of seizures and adverse effects of antiepileptic drugs. We assessed the impact of adverse effects on QOL and the utility of a structured instrument to help the physician manage adverse effects in patients with refractory epilepsy.

Methods

Clinical characteristics, drug treatment and adverse effects were evaluated in 102 patients with refractory epilepsy at a single tertiary referral centre. The Adverse Events Profile (AEP) and Quality of Life in Epilepsy‐31 (QOLIE‐31) questionnaires were completed at baseline and after six months. At baseline, patients with a high burden of adverse effects (AEP scores ≥45) were randomized to an intervention or control group. AEP scores in the intervention group were available to the physician as an instrument to help to reduce adverse effects.

Results

Ninety‐five patients (93.1%) were on polytherapy. Sixty‐six completed the questionnaires and, of these, 43 (65.1%) had a high AE burden and were randomized to the intervention and control group. QOLIE‐31 scores were inversely correlated with AEP scores at both visits. Among randomized patients, AEP scores tended to decrease between the baseline and the final visit without significant differences between groups (intervention group: 54.1 ± 6.1 vs 51.1 ± 9.1; control group: 55.8 ± 5.8 vs 50.5 ± 12.2). QOLIE‐31 scores did not change substantially between visits (intervention group: 45.9 ± 17.4 vs 48.4 ± 14; control group: 47.5 ± 15.7 vs 45.2 ± 18.9).

Conclusion

A significant proportion of patients had a high toxicity burden which had an impact on their QOL. Reduction of overtreatment is a difficult challenge which cannot be addressed solely by providing a structured assessment of adverse effects, but requires a more comprehensive approach aimed at optimizing the many components of the management strategy.
Late‐onset Rasmussen's encephalitis and long‐term remission
Authors: Kupila Laura , Jutila Leena , Immonen Arto , Vanninen Ritva , Mervaala Esa , Pateau Anders , Luostarinen Liisa , Kälviäinen Reetta
Published: August 2019

We describe an adult man with biopsy‐proven Rasmussen's encephalitis and intractable epilepsy, who underwent excellent recovery. To our knowledge, this is the first report of a patient with Rasmussen's encephalitis who has become completely symptomless, at least for three years, on enhanced antiepileptic and immunological medication.
Extraoccipital photoparoxysmal response in a case of focal encephalitis
Authors: Jukkarwala Anis , Hassan Haseeb , Nair Muralidharan , Iyer Rajesh S.
Published: August 2019

Photoparoxysmal response (PPR) is commonly associated with idiopathic generalised epilepsies. Most of the focal events induced by intermittent photic stimulation (IPS) are reported to be of occipital origin. Only six temporal lobe epilepsy patients have been reported in the literature with focal PPR at extraoccipital sites. We report a four‐year‐old girl with possible encephalitis who presented initially with epilepsia partialis continua of limbs on the right side. Interictally, she had left centro‐parietal periodic lateralized epileptiform discharges (PLEDs). She responded to medical treatment and was free of seizures and motor and cognitive deficits at six months follow‐up. Repeat EEG at follow‐up showed left centro‐parietal spikes accentuated by IPS. This is the first report of an extraoccipital, extratemporal focus showing PPR. The possible mechanism of PPR from this fronto‐parietal epileptogenic focus is discussed.
Association between ABCB1 polymorphism and response to sodium valproate treatment in Malaysian epilepsy patients
Authors: Haerian Batoul Sadat , Lim Kheng Seang , Tan Hui Jan , Mohamed Elsa Hanifa Mejia , Tan Chong Tin , Raymond Azman Ali , Wong Chee Piau , Wong Sau Wei , Omar Haslyna , Roslan Harun , Mohamed Zahurin
Published: August 2019

Over‐expression of P‐glycoprotein, encoded by the ABCB1 gene, is proposed to be involved in resistance to antiepileptic drugs in about 30% of patients with epilepsy. Here, we investigated the possible association between ABCB1 polymorphisms and sodium valproate (VPA) treatment in Malaysian epilepsy patients. Genotypes were assessed in 249 drug‐resistant and 256 drug‐responsive Malaysian patients for C1236T, G2677T/A, and C 5T polymorphisms in the ABCB1 gene. No genotypes, alleles, or haplotypes were associated with the response to VPA in either the overall group or Chinese, Indian, and Malay subgroups. Our data suggest that C1236T, G2677T/A, and C3435T polymorphisms in the ABCB1 gene do not contribute to the response to VPA in patients with epilepsy.
Reflex seizures induced by micturition and defecation, successfully treated with clobazam and phenytoin
Authors: Higuchi Tsukasa , Fukuyama Tetsuhiro , Misawa Yuka , Inaba Yuji , Ichikawa Motoki , Koike Kenichi
Published: August 2019

We report a six‐year‐old girl with seizures induced by both micturition and defecation. Several days after unprovoked generalised tonic‐clonic seizures, she developed reflex seizures characterised by the extension of both arms and rhythmic jerking of her upper body. No abnormal findings were noted on brain magnetic resonance imaging. Interictal electroencephalography (EEG) showed spike‐and‐wave activity on central electrode recording, and rhythmic fast activity was recorded by central electrodes during the ictal EEG upon micturition. The combination of clobazam and phenytoin was effective for both unprovoked and reflex seizures. Although some previous reports have described reflex seizures triggered by either micturition or defecation, this is the first case report of reflex seizures induced by both micturition and defecation in the same patient. Based on a comparison with previous cases of reflex seizures induced either by micturition or defecation, the neuronal pathway from the pelvic base musculature to the supplementary motor area may be responsible for the condition in our patient.
Malignant migrating partial seizures in a 4‐month‐old boy
Authors: Gilhuis H. Jacobus , Schieving Jolanda , Zwarts Machiel J.
Published: August 2019

Malignant migrating partial seizures in infancy is an epilepsy syndrome characterised by an onset before the age of six months, multifocal seizures and an EEG pattern consisting of seizures which occur independently and sequentially from both hemispheres. The clinical course of a four‐month‐old boy with this syndrome, illustrated by video material of the seizures and EEG recordings, is described. The possible neurophysiological mechanism of epileptogenic activity alternating or ‘migrating’ from one hemisphere to the other is discussed.
Non‐convulsive status epilepticus characterised exclusively by a language disorder induced by non‐ketotic hyperglycaemia
Authors: Pro Stefano , Randi Franco , Pulitano Patrizia , Vicenzini Edoardo , Mecarelli Oriano
Published: August 2019

Non‐ketotic hyperglycaemia is an endocrine emergency characterised by elevated blood glucose levels and high plasma osmolarity. While hypoglycaemia‐induced seizures are usually generalised, hyperglycaemia‐induced seizures are often focal and secondary to the presence of brain lesions. Moreover, in the few studies in which language disorders of epileptic origin have been reported as a clinical manifestation of non‐ketotic hyperglycaemia, the disorders were usually not isolated but were followed by partial motor seizures. We describe a patient who presented with non‐convulsive partial status epilepticus and whose only sign was a fluctuating language disorder induced by non‐ketotic hyperglycaemia. There were no accompanying brain lesions and the patient responded optimally to diazepam. Neurophysiological EEG evaluation was fundamental for the diagnosis.
Benzodiazepine receptor imaging in an adult with Rasmussen's encephalitis and epilepsia partialis continua
Authors: Kataoka Hiroshi , Shinkai Takayuki , Kiriyama Takao , Tonomura Yasuyo , Ueno Satoshi
Published: August 2019

We describe an adult with Rasmussen's encephalitis associated with widespread decreased accumulation of tracer on 123‐I iomazenil (IMZ) SPECT. The patient had a form of epilepsia partialis continua and neurological deterioration associated with hemiatrophy. Cranial MRI showed cerebral atrophy in the left frontal, parietal, and temporal lobes, without abnormal intensity. Delayed IMZ SPECT showed widespread decreased accumulation of tracer in the left temporal and frontal regions. An early scan showed mildly decreased accumulation in the left temporal and frontal regions. Three‐dimensional stereotactic surface projection (3D‐SSP) analysis showed that the decreased regions on delayed IMZ images were slightly larger as compared with the previous IMZ study performed. Some regions without decreased accumulation of tracer on Tc‐ECD or early IMZ images showed decreased accumulation on delayed IMZ images. This case study suggests that benzodiazepine receptors may be reduced in the affected hemisphere of patients with Rasmussen's encephalitis.
Extrapyramidal epilepsy
Authors: Karakis Ioannis , Cole Andrew J. , Hoch Daniel B. , Sharma Nutan
Published: August 2019

Non‐epileptic paroxysmal movement disorders are rare extrapyramidal diseases. The clinical signs are often excluded from differential diagnosis due to their paucity on clinical examination and low prevalence. The paroxysmal, stereotypical nature of non‐epileptic paroxysmal movement disorders, as well as the potential response to benzodiazepines, renders these disorders susceptible to misdiagnosis as epileptic seizures. We report a case of paroxysmal non‐kinesigenic dyskinesia which was misdiagnosed and treated as epilepsy for decades. The major pathophysiological, diagnostic and therapeutic hallmarks of the disease are summarised. Familiarity and inclusion of the disease in the list of conditions that mimic epilepsy, as well as prolonged video‐EEG recordings, may prevent diagnostic delays and unnecessary or belated treatments. [Published with video sequences]
Rhythmic heart beat artefacts mimicking an ictal EEG pattern with abundant interictal epileptiform activity in both mother and child
Authors: Van den Berg Petronella J. , Brekelmans Geert J.F.
Published: August 2019

We present the EEG findings in a two‐year‐old girl with cryptogenic localisation‐related epilepsy. The electroencephalogram showed fronto‐temporal spike‐wave discharges. In addition, the EEG repeatedly showed activity at different electrodes consisting of spikes with varying amplitude, rhythmicity and frequency, mimicking polyspikes. Both low‐ and high‐amplitude spikes were generated from the same electrodes, however, only the low‐amplitude spikes coincided with features of the child's electrocardiogram. Video monitoring showed the child's head resting on her mother's chest. This spike pattern was shown to be a heart beat artefact originating from the mother, as implied by the rhythmicity and the shift to other electrodes by moving the child's head, causing electrocardiogram artefacts on the posterior electrodes. This case study underscores the importance of routine use of simultaneous electroencephalogram and video monitoring. [Published with video sequences]
Infliximab‐related seizures: a first case study
Authors: Brigo Francesco , Bongiovanni Luigi Giuseppe , Cerini Roberto , Manganotti Paolo , Storti Monica , Fiaschi Antonio
Published: August 2019

Seizures following infliximab treatment are very rare and, to date, there is no detailed description of EEG abnormalities with cerebral radiological findings reported in cases with infliximab‐related seizures. We describe a patient who acutely developed seizures temporally related to infliximab treatment, which disappeared after drug withdrawal. MRI showed encephalopathy involving mainly cortical regions and EEGs showed focal paroxysmal activity which completely disappeared a few days after infliximab withdrawal. No other plausible cause of the seizures was identified. The clear temporal association between seizure onset and infliximab treatment as well as the clinical improvement and disappearance of focal epileptiform activity after drug withdrawal indicated an evident correlation between seizures and infliximab therapy. The coexistence of pathological findings on MRI suggested that seizures were secondary to the encephalopathy. Further studies are required to evaluate whether infliximab per se has an epileptogenic effect or whether the seizures are caused by encephalopathy involving cortico‐subcortical regions.
Superselective anterior temporal resection in mesial temporal lobe epilepsy
Authors: Cordeiro Joacir Graciolli , Wagner Kathrin , Trippel Michael , Zentner Josef , Schulze‐Bonhage Andreas
Published: August 2019

We report the case of a patient with pharmacoresistant mesial temporal lobe epilepsy presenting psychomotor seizures with onset at early childhood. MRI showed a blurred internal structure of the right hippocampus and right mammillary body atrophy. Neuropsychological testing revealed deficits in selective attention and visual planning. Non‐invasive recording was not sufficient to precisely detect the seizure onset zone. Invasive recording showed seizure onset in the temporo‐polar neocortex, with spread to the amygdalum and hippocampus. A superselective resection of the temporal pole and amygdalum was performed with preservation of the hippocampus. Histology revealed the presence of focal cortical dysplasia (Palmini type Ib). Seizure frequency was reduced after surgery, and seizure freedom for two years was achieved with optimisation of the antiepileptic drug regime. Memory functions were preserved, and selective attention and visual planning improved following limited resection. This case suggests that, in selected cases, highly targeted resections with preservation of memory‐relevant structures may be the best choice considering both seizure control and unimpaired cognitive functioning.
Seizures as a manifestation of multiple sclerosis
Authors: Sponsler Jeffrey L , Kendrick‐Adey Anastasia C
Published: August 2019

Background

The incidence of seizures is generally accepted to be greater in patients with multiple sclerosis (MS) than in the general population, and rarely, MS can initially present as seizure.

Objective

To present a case report of seizure as the initial symptom of MS, to quantify the occurrence of seizures among MS patients, and to classify patients according to when seizures occur relative to onset of MS.

Methods

The medical history of patients presenting with MS and seizure in our clinic was examined. In addition, 25 scientific papers were reviewed and the number and characteristics of patients with MS and seizure recorded. Data from the literature review and from our own clinical series were combined and examined.

Results

Of the MS patients, 1.95% experienced seizures at any time during life. Patients experiencing seizures before MS diagnosis were classified into three categories: (a) 25 (7.3% of patients with MS and seizures) with seizure as the initial presentation of MS; (b) 27 (7.9%) with seizures appearing with other signs and symptoms of MS; and (c) 68 (20%) with seizures occurring years or an unknown period of time before MS onset. Seizure occurring as a symptom of MS relapse was found in 29 patients.

Conclusion

The prevalence of seizures among MS patients was higher than that in the general population, indicating a relationship between seizures and MS. Seizures occurred before MS diagnosis in a small percentage of patients.
aPTT prolongation and skin eruption possibly associated with lamotrigine monotherapy in a paediatric patient
Authors: Yeom Jung Sook , Park Ji Sook , Seo Ji Hyun , Park Eun Sil , Lim Jae Young , Park Chan‐Hoo , Woo Hyang Ok , Youn Hee‐Shang
Published: August 2019

We report the case of a six‐year‐old female with childhood absence epilepsy who developed combined aPTT prolongation, not corrected by normal plasma, and atypical skin eruption six months after initiating lamotrigine treatment with dose increment. Two weeks after lamotrigine withdrawal, the skin eruption disappeared and aPTT normalised. To our knowledge, this is the first report of aPTT prolongation possibly due to factor inhibitors associated with lamotrigine monotherapy.
Serial EEG and MRI changes in status epilepticus‐induced excitotoxic neuronal necrosis
Authors: Arman Fehim , Kaya Dilaver , Dincer Alp , Sav Aydin , Pamir M. Necmettin
Published: August 2019

Prolonged status epilepticus may directly cause selective neuronal necrosis due to excitotoxic mechanisms, as observed in experimental models and described in case reports. A 36‐year‐old woman presented with right hemiplegia and aphasia following a generalised tonic‐clonic status epilepticus of two hours duration. Accompanying serial MRI with advanced imaging techniques, EEG and histopathology of the cortical tissue of the patient were all compatible with excitotoxic neuronal necrosis. In this histopathologically‐proven rare case of status epilepticus‐induced excitotoxic neuronal injury, the observation of delayed cortical laminar necrosis on MRI, together with paroxysmal lateralised epileptiform discharges on the EEG, suggests that these changes may be an early sign of impending and ongoing excitotoxic neuronal injury and delayed cell death caused by glutamate release due to excessive neuronal firing in status epilepticus.
Limited resection of focal cortical dysplasia and associated epileptogenic cortex may lead to positive surgical outcome
Authors: Garganis Kyriakos , Kokkinos Vasileios , Zountsas Basilios
Published: August 2019

A drug‐resistant epilepsy patient with premotor type IIb focal cortical dysplasia is described with a positive postoperative outcome following partial resection of the lesion and epileptogenic zone. Presurgical fMRI of the sensorimotor areas showed haemodynamic responses over the posterior border of the lesion and ictal EEG‐fMRI revealed activation of both the primary sensorimotor strip and premotor lesion area. Almost continuous 1‐2 Hz interictal spiking was recorded during a chronic ECoG study over the primary sensorimotor cortex. Following partial resection of the lesion, an acute ECoG revealed marked reduction of epileptic activity over the sensorimotor area. Post‐operatively, seizure control was significantly improved (class IV ILAE outcome). Although partial FCD resections predict a worse postoperative outcome, individual patients may still respond favourably. The type of lesion, resected portion, and histopathology may be included among factors related to successful outcome.
Intracranial localisation of ictal urinary urge epileptogenic zone to the non‐dominant temporal lobe
Authors: Gurgenashvili Khatuna , Massey Shavonne L , Grant Mitzie , Piatt Joseph , Legido Agustin , Valencia Ignacio
Published: August 2019

Ictal urinary urge is a rare symptom of focal epilepsy usually localising to the non‐dominant hemisphere, specifically, the temporal lobe. Lateralisation in previously described cases has been established using scalp video‐EEG monitoring or functional imaging. We report the case of a 19‐year‐old girl with refractory epilepsy and ictal urinary urge arising from the non‐dominant temporal lobe, confirmed by invasive, subdural EEG monitoring. Since undergoing a temporal lobectomy two and a half years ago, the patient has not experienced ictal urinary urge. To our knowledge, this is the first report demonstrating localisation of ictal urinary urge epileptogenic zone to the non‐dominant temporal lobe by invasive intracranial monitoring.
Mirth and laughter elicited during brain stimulation
Authors: Vaca Guadalupe Fernández‐Baca , Lüders Hans O , Basha Maysaa Merhi , Miller Jonathan P
Published: August 2019

There are few reports of laughter and/or mirth evoked by electrical stimulation of the brain. In this study, we present a patient with intractable epilepsy in whom mirth and laughter was consistently produced during stimulation of the left inferior frontal gyrus (opercular part) using stereotactically placed depth electrodes. A review of the literature shows that cortical sites that produce mirth when stimulated are located in the dominant hemisphere close to language areas or cortical negative motor areas.
Therapy of encephalopathy with status epilepticus during sleep (ESES/CSWS syndrome): an update
Authors: Veggiotti Pierangelo , Pera Maria Carmela , Teutonico Federica , Brazzo Daniela , Balottin Umberto , Tassinari Carlo Alberto
Published: August 2019

Electrical status epilepticus in sleep (ESES)/continuous spikes and waves during slow sleep (CSWS) is an age‐related, self‐limiting disorder characterised by epilepsy with different seizure types, global or selective neuropsychological regression, motor impairment, and a typical EEG pattern of continuous epileptiform activity for more than 85% of non‐rapid eye movement (NREM) sleep. Although the first description of ESES/CSWS dates back to 1971, an agreement about the optimal treatment for this condition is still lacking. ESES/CSWS is rare (incidence is 0.2‐0.5% of all childhood epilepsies) and no controlled clinical trials have been conducted to establish the efficacy of different antiepileptic drugs; only uncontrolled studies and case reports are reported in the literature. Treatment options for ESES/CSWS include some antiepileptic drugs (valproic acid, ethosuximide, levetiracetam, and benzodiazepines), steroids, immunoglobulins, the ketogenic diet, and surgery (multiple subpial transections). In this study, the comparative value of each of these treatments is reviewed and a personal therapeutic approach is proposed.
Parietal lobe epilepsy: the great imitator among focal epilepsies
Authors: Ristić Aleksandar J , Alexopoulos Andreas V , So Norman , Wong Chong , Najm Imad M
Published: August 2019

Aim. Comprising large areas of association cortex, the parietal lobe is part of an extensive synaptic network elaborately intertwined with other brain regions. We hypothesize that such widespread projections are responsible for producing inaccurate localisation readings on scalp EEG and clinical semiology in patients with parietal lobe epilepsies, as opposed to frontal or temporal lobe epilepsies. Methods. Our study included 50 patients with pharmacoresistant focal epilepsy, who were subsequently rendered seizure‐free for ≥12 months (median: 23 months) following resections limited to the frontal (n=17), temporal (n=17), or parietal (n=16) lobes. Interictal and ictal EEG data with accompanying seizure video recordings were extracted from archived files of scalp video‐EEG monitoring. Two blinded raters independently reviewed the EEG according to predetermined criteria. Videos of seizures were then observed, as raters formulated their final electroclinical impression (ECI), identifying patients' abnormal neuronal activities with parietal, temporal, and frontal lobe epilepsy, or unspecified localisation. Results. Groups did not differ significantly in demographics, age at epilepsy onset, or presence of MRI abnormalities. Interictal discharges in parietal lobe epilepsy showed the greatest magnitude of scatter outside the lobe of origin; the majority of patients with parietal lobe epilepsy had more than one spike population (p<0.045). Localised ictal EEG recognition was most frequent in temporal, followed by frontal and parietal lobe epilepsy cases (p=0.024). Whenever raters confidently limited their ECI to one lobar subtype, overall accuracy was excellent. Lobar classifications by ECI were highly accurate for temporal lobe epilepsy, vacillating in frontal lobe epilepsy, and least accurate in parietal lobe epilepsy subjects. Conclusion. Scalp EEG readings of parietal lobe epilepsy patients showed a more variable scatter of interictal discharges and a lower localisation value of ictal recordings compared to temporal and frontal lobe epilepsy subjects, suggesting an increased likelihood of misidentification and mislocalisation of parietal lobe epilepsy. Combining seizure semiology with scalp interictal and ictal EEG readings facilitates a more accurate lobar classification in patients with temporal and frontal, but not parietal, lobe epilepsy.
Bálint‐like syndrome as an unusual representation of non‐convulsive status epilepticus
Authors: Ristić Aleksandar J , Marjanović Ivan , Brajković Leposava , Wolgamuth Barbara R , Odalović Strahinja , Lavrnić Slobodan , Vojvodić Nikola , Janković Slavko , Baščarević Vladimir , Sokić agoslav
Published: August 2019

The clinical signs of posterior cortex dysfunction are, due to their paucity and subtlety, very often ignored as non‐specific during clinical evaluation of non‐convulsive status epilepticus. Therefore, focal non‐convulsive status epilepticus emerging from the posterior cortex, and especially the parietal lobes, can be fairly under‐recognised. We report a 66‐year‐old patient with focal non‐convulsive status epilepticus presenting as isolated Bálint‐like syndrome, successfully treated to full clinical and electrophysiological recovery. The diagnostic and pathophysiological features are discussed. Focal non‐convulsive status epilepticus can be associated with negative phenomena such as neuropsychological deficits mimicking those detected more often in degenerative and vascular brain diseases. [Published with video sequences]
Gaucher disease: successful treatment of myoclonic status epilepticus with levetiracetam
Authors: Vaca Guadalupe Fernandez‐Baca , Lenz Timothy , Knight Elia M Pestana , Tuxhorn Ingrid
Published: August 2019

We present the first reported case of a rapid clinical and electroencephalographic response to intravenous levetiracetam infusion of myoclonic status epilepticus in a patient with progressive myoclonus epilepsy due to Gaucher disease. Under continuous video‐EEG monitoring, the clinical myoclonic status and the electrographic ictal discharges resolved within 10 minutes after the infusion was initiated. The patient tolerated the treatment well without any reported side effects. This case suggests that levetiracetam may be a safe, effective, and well tolerated intravenous drug in patients with metabolic myoclonic status epilepticus such as Gaucher disease.
Carbamazepine‐induced non‐epileptic myoclonus and tic‐like movements
Authors: Magaudda Adriana , Di Rosa Gabriella
Published: August 2019

Carbamazepine‐induced abnormal movements have been reported in children and adult patients, and both non‐epileptic myoclonus and tic‐like movements have been reported in the same patient. Although a pathogenetic mechanism underlying carbamazepine‐induced epileptic negative myoclonus has been proposed, a causative role of carbamazepine for positive myoclonus has not been fully identified. Here, we describe the video‐documented case of an adult patient with non‐epileptic myoclonus and tic‐like movements persisting for 21 years, which appeared after he started carbamazepine treatment at 10 years of age. [Published with video‐sequences]
Gelastic seizures in ring chromosome 20 syndrome: a case report with video illustration
Authors: Dimova Petia , Boneva Iliyana , Todorova Albena , Minotti Lorella , Kahane Philippe
Published: August 2019

Although increasingly recognised, ring chromosome 20 (r[20]) syndrome is still diagnosed with delay, sometimes leading to inappropriate presurgical evaluation. The focal, presumed frontal, character of the seizures manifesting with fear and hypermotor behaviour and episodes of non‐convulsive status epilepticus (NCSE) are most typical, as well as cognitive impairment with behavioural problems and, sometimes, dysmorphic signs. We present a girl diagnosed at the age of 13 years who suffered from an atypical clinical presentation, with minimal cognitive problems, absence of dysmorphic symptoms, and hypermotor/gelastic seizures. [Published with video sequences]
Falsely pessimistic prognosis by EEG in post‐anoxic coma after cardiac arrest: the borderland of nonconvulsive status epilepticus
Authors: Sreedharan Jemeen , Gourlay Elizabeth , Evans Matthew R. , Koutroumanidis Michalis
Published: August 2019

Background

Prognostication following anoxic coma relies on clinical assessment and is assisted by neurophysiology. A non‐evolving EEG spike burst/isoelectric suppression pattern after the first 24 hours almost invariably indicates poor outcome, while an evolving pattern implies nonconvulsive status epilepticus (NCSE) that may “hide” surviving brain activity and is amenable to treatment.

Case study

We present the case of a 53‐year‐old woman who had a witnessed out‐of‐hospital ventricular fibrillation cardiac arrest, was resuscitated by paramedics, but remained comatose. An EEG, performed 36 hours post‐insult, showed an unremitting, non‐evolving, unresponsive 2‐6 Hz high‐voltage spike burst/isoelectric suppression pattern, which remained unchanged at 96 hours post‐insult, following therapeutic hypothermia. During this period, she was completely off sedation and taking triple antiepileptic treatment, without systemic confounding disorders. Although the initial pattern was indicative of poor neurological outcome, she eventually made meaningful functional recovery; the last EEG showed satisfactory background rhythms and stimulus‐induced epileptiform discharges without seizures.

Conclusion

In post‐anoxic coma, non‐evolving >2 Hz spike burst/isoelectric suppression pattern may still reflect NCSE and therefore should be considered in the diagnostic EEG criteria for NCSE. Such borderline patterns should not dissuade physicians from intensifying treatment until more confident prognostication can be made.
Epilepsy surgery in a developing country (Lebanon): ten years experience and predictors of outcome
Authors: Mikati Mohamad A. , Ataya Nour , El‐Ferezli Jessica , Shamseddine Alhan , Rahi Amal , Herlopian Aline , Kurdi Rana , Bhar Saleh , Hani Abeer , Comair Youssef G.
Published: August 2019

We present our 10‐year experience and preoperative predictors of outcome in 93 adults and children who underwent epilepsy surgery at the American University of Beirut. Presurgical evaluation included video‐EEG monitoring, MRI, neuropsychological assessment with invasive monitoring, and other tests (PET, SPECT, Wada). Surgeries included temporal (54%), extratemporal (22%), and multilobar resections (13%), hemispherectomy (4%), vagal nerve stimulation (6%), and corpus callosotomy (1%). Mesial temporal sclerosis was the most common aetiology (37%). After resective surgery, 70% had Engel class I, 9% class II, 14% class III, and 7% class IV. The number of antiepileptic drugs before surgery was the only preoperative factor associated with Engel class I (p=0.005). Despite the presence of financial and philanthropic aid, many patients could not be operated on for financial reasons. We conclude that advanced epilepsy presurgical workups, surgical procedures, and favourable outcomes, comparable to those of developed countries, are achievable in developing countries, but that issues of financial coverage remain to be addressed.