Epileptic Origin Research Articles

Ictal Cotard delusion as a manifestation of nonconvulsive status epilepticus: A case report and commentary.

Psychosis of epileptic origin can present a wide range of cognitive and affective symptoms and is often underrecognized. Usually occurring in the inter- and postictal phase, epileptic psychosis is mostly related to temporal lobe epilepsy. Here, we describe the clinical presentation and diagnostic workup including routine EEG recording and brain MRI of a 63-year-old woman expressing isolated nihilistic delusions comprising belief of being dead and denial of self-existence. EEG showed an ictal pattern fulfilling the Salzburg criteria of nonconvulsive status epilepticus and brain MRI revealed extensive peri-ictal hyperperfusion. Delusional symptoms and EEG abnormalities subsided after acute antiseizure treatment. Our case illustrates how nihilistic delusions can occur as a direct clinical correlate of seizure activity, thereby expanding the spectrum of ictal neuropsychiatric phenomena in temporal lobe epilepsy and highlighting the need to consider an epileptic origin in patients presenting with psychotic symptoms.

Clinical features and outcome of 10 dogs with suspected idiopathic vestibular epilepsy.

In humans, vestibular epilepsy (VE) is described as focal seizures with transient signs of vestibular disease. In dogs, 2 cases of vestibular episodes, called vestibular paroxysmia, are reported. The objective of this study was to define the clinical features, phenotypical manifestation, and outcome of suspected VE in dogs. Ten dogs with recurrent vestibular episodes. Retrospective study. Medical records between 2009 and 2023 were reviewed, and dogs with a normal neurological examination, a history of transient signs of vestibular disease, absence of abnormalities detected on blood exams and brain magnetic resonance imaging (MRI) or computed tomography (CT), besides a minimum 10-month follow-up were included. Clinical improvement was defined as a ≥50% reduction in frequency or the cessation of clinical signs after the onset of antiseizure medications (ASMs). Pugs were the most prevalent breed (5/10; 50%). In 2 cases, additional generalized tonic-clonic (GTC) seizures were reported. MRI exam was performed in most cases (9/10; 90%), whereas 1 dog underwent a CT scan (1/10; 10%). Electroencephalography (EEG) was carried out in 3 dogs that showed interictal spikes in the fronto-temporal and fronto-parietal areas. All cases received ASMs, with clinical improvement in 10/10 dogs (100%). The presence of GTC seizures, EEG interictal spikes, and responsiveness to ASMs supported the hypothesis of an epileptic origin of vestibular episodes and thus the existence of VE in these dogs, with a presumed idiopathic cause and apparent favorable outcome.

Mental disorders in cerebral paroxysms of epileptic origin

Mental disorders in cerebral paroxysms of epileptic origin

A novel GABRG2 variant in Sunflower syndrome: a case report and video EEG monitoring.

Sunflower syndrome is a unique photosensitive epilepsy, characterized by heliotropism and stereotyped seizures associated with handwaving. These handwaving events (HWE) are thought to be an ictal phenomenon, although current data are contrasting. Photosensitive epilepsy occurs in 2-5% of the epilepsy forms and several pathogenic gene variants have been associated with photosensitive epilepsy. However, the genetic etiology of Sunflower syndrome remains unknown. Antiseizure medications (ASM) efficacious in treating photosensitive epilepsy are valproic acid (VPA) and levetiracetam (LEV) although some forms, such as Sunflower syndrome, can be drug-resistant. Here, we report an 8-year-old boy with an early onset of episodes of HWE that was initially categorized as behavioral problems for which risperidone was started. However, the medical history was suggestive of Sunflower syndrome and subsequent video EEG showed focal mostly temporal and fronto-temporal (right and left) epileptiform activity and confirmed the epileptic nature of the HWE. Thus, VPA was started and initially led to seizure frequency reduction. Molecular analyses showed a pathogenic variant in GABRG2 (c.1287G>A p.(Trp429Ter)), which has been associated with photosensitive and generalized epilepsy. Overall, clinicians worldwide should be cautious by interpreting HWE and/or other tic-like movements, since an epileptic origin cannot be ruled out. A prompt and correct diagnosis can be made by performing a video EEG early on in the diagnostic process when epileptic seizures are part of the differential diagnosis. Even though the genetic etiology of Sunflower syndrome remains poorly understood, this constellation supports further genetic testing since the detection of a pathogenic variant can help in making correct decisions regarding ASM management.

1001 A Case of Unusual Nocturnal Movements Consistent with Paroxysmal Hypnogenic Dyskinesia

Abstract Introduction Paroxysmal hypnogenic dyskinesia (PHD) is a rare subset of paroxysmal dyskinesias characterized by recurring involuntary dystonic or choreoathetoid movements that occur during sleep or immediately after an arousal from sleep. These episodes often occur without loss of consciousness or trigger and lack association with epileptiform electroencephalography (EEG) activity. Report of case(s) A 61-year-old male with obstructive sleep apnea compliant with PAP was referred for abnormal nighttime movements for the past 9 years. The episodes were characterized by stereotyped involuntary dystonic posturing involving his upper extremities or his entire body lasting up to 15 seconds. The onset of the events occurred invariable during nighttime sleep and resulted in awakenings from sleep. He denied dream enactment as well as other symptoms suggestive of sleep-related movement disorders or parasomnias. Sleep deprived EEG demonstrated left temporal slowing during wakefulness and drowsiness and captured a typical event that occurred during NREM stage II sleep and had no ictal correlate. An MRI of the brain was unremarkable. During overnight polysomnogram, his current PAP settings were found to be effective, and three episodes of body stiffening consistent with his typical events were captured. These episodes were found to occur after awakening from REM sleep and lasted less than 10 seconds each. There was no EEG correlate, and REM atonia was preserved throughout. The patient was started on oxcarbazepine for the nocturnal movements, which reduced the frequency of events from 12 to 4 events per night. Oxcarbazepine was tapered and discontinued due to side effects and incomplete control of the events. Genetic testing for epilepsy syndromes is pending. Conclusion PHD is a rare syndrome of involuntary movements which begin during sleep or cause awakenings from sleep and can be misdiagnosed as a sleep disorder. It is crucial for the sleep physician to be aware of possible neurological causes of sleep-related movement disorders. Furthermore, our patient’s partial response to oxcarbazepine may further support that PHD may have an epileptic origin although not well characterized. Support (if any) Advancing Innovation in Residency Education (AIRE) Pilot Program Training Grant

Evaluating whole-brain tissue-property changes in MRI-negative pharmacoresistant focal epilepsies using MR fingerprinting.

We aim to quantify whole-brain tissue-property changes in patients with magnetic resonance imaging (MRI)-negative pharmacoresistant focal epilepsy by three-dimensional (3D) magnetic resonance fingerprinting (MRF). We included 30 patients with pharmacoresistant focal epilepsy and negative MRI by official radiology report, as well as 40 age- and gender-matched healthy controls (HCs). MRF scans were obtained with 1 mm3 isotropic resolution. Quantitative T1 and T2 relaxometry maps were reconstructed from MRF and registered to the Montreal Neurological Institute (MNI) space. A two-sample t test was performed in Functional Magnetic Resonance Imaging of the Brain (FMRIB) Software Library (FSL) to evaluate significant abnormalities in patients comparing to HCs, with correction by the threshold-free cluster enhancement (TFCE) method. Subgroups analyses were performed for extra-temporal epilepsy/temporal epilepsy (ETLE/TLE), and for those with/without subtle abnormalities detected by morphometric analysis program (MAP), to investigate each subgroup's pattern of MRF changes. Correlation analyses were performed between the mean MRF values in each significant cluster and seizure-related clinical variables. Compared to HCs, patients exhibited significant group-level T1 increase ipsilateral to the epileptic origin, in the mesial temporal gray matter (GM) and white matter (WM), temporal pole GM, orbitofrontal GM, hippocampus, and amygdala, with scattered clusters in the neocortical temporal and insular GM. No significant T2 changes were detected. The ETLE subgroup showed a T1-increase pattern similar to the overall cohort, with additional involvement of the ipsilateral anterior cingulate GM. The subgroup of MAP+ patients also showed a T1-increase pattern similar to the overall cohort, with additional cluster in the ipsilateral lateral orbitofrontal GM. Higher T1 was associated with younger seizure-onset age, longer epilepsy duration, and higher seizure frequency. MRF revealed group-level T1 increase in limbic/paralimbic structures ipsilateral to the epileptic origin, in patients with pharmacoresistant focal epilepsy and no apparent lesions on MRI, suggesting that these regions may be commonly affected by seizures in the epileptic brain. The significant association between T1 increase and higher seizure burden may reflect progressive tissue damage.

Joubert Sendromu ve Biot Solunumunun Epilepsi Olarak Yanlış Teşhisi

Joubert syndrome (JS) is a rare ciliopathy characterized by neurodevelopmental delay and irregular breathing patterns. Here we present a patient with JS, who was misdiagnosed as having epilepsy. A five year-old girl with JS was referred due to excessive daytime sleepiness. She was diagnosed as having apneic seizures, and given antiseizure medications with no benefit. Apneas were present during wakefulness lasting for more than one minute, with sudden myoclonic jerks characterized by the extension of the body and the flexion of the limbs at the resume of respiration with paradoxical breathing, hyperventilation and tachypnea. A full-night polysomnography (PSG) revealed pediatric obstructive and central sleep apnea syndromes. The patient was effectively treated by adaptive servo ventilation therapy. Respiratory control disturbances are commonly encountered in the children with JS. Although routine electroencephalography may be helpful in differentiation of the apneas of epileptic origin, polygraphic recordings and/or PSG better delineate the pathophysiology.

Vanishing hyperintensity in MRI: Vascular or epileptic origin?

Status Epilepticus and epilepsy-related MRI vanishing changes have been reported in the literature since the 1980s; hypoxia and hypoperfusion have been related to these image modifications. These alterations and their disappearing characteristics can cause trouble among work up and their diagnosis, especially if there is no exact etiology of what is causing seizures. We present a case of a 37-year-old righthanded man with a 6-year history followed up after seizure debut, with no exact etiology at the first event, considering an ischemic event as etiology. After a six-year follow-up seizure-free and no sequels, the patient newly developed aphasia, seizures, and status epilepticus, with a now evident vascular abnormality (cavernous cerebral malformation) etiology in MRI imaging. The cumbersomeness of the clinical picture in its presentation, the seriousness to which it reached, and the complete resolution of the problem with medical treatment make this clinical case especially attractive.

A case of epiletic “belly dancing”

The “belly dancing” phenomenon is featured by undulating movements of the abdominal wall. Although several aetiologies have been reported, the epileptic origin is the most commonly described. Previously reported patients were known to have epilepsy, were already receiving antiepileptic drugs, presented with focal status epilepticus, and/or their seizure motor phenomenology also included the eyes, face, limbs, and even loss of awareness. Isolated epileptic “belly dancing” has been very rarely described so far.

Early-onset eyelid stereotypies are a frequent and distinctive feature in Dravet syndrome

Dravet syndrome (DS) is a severe infantile-onset epilepsy syndrome featuring drug resistant epilepsy, global developmental delay and intellectual disability. In addition to ataxia and progressive crouch gait, Parkinsonism has recently been reported as characteristic in young adults with DS. We describe 5 patients out of a series of 23 patients with DS who present between 12 and 24 months of age with repetitive episodes of eyelid closure, sometimes as fast as eye blinking or flickering. Consistent lack of any EEG correlate in serial video-EEG ruled out an epileptic origin. We propose that this movement disorder, namely ‘eyelid stereotypies’, might be an early motor trait of SCN1A-associated DS.

Epileptic seizures in patients with COVID-19: A systematic review of early evidences

Background: Global emergence of SARS-CoV-2 surfaced neurological complications amongst the patients. COVID-19 resembles with other coronavirus strains follows a trend of neurological complication, damage and encephalopathy, which entails considerable risks, requires attention for the neurologists. This is, to our knowledge, the first systematic review of the literature to investigate solely to elucidate the seizure spectrum by unfolding epileptogenicity of the SARS CoV-2 and potential pathways of neuroinvasion.
 Methods: A systematic literature search was performed in PubMed and Embase database following standard guidelines, using specific keywords based on epileptic seizure onset described from December 01, 2019, to July 17, 2020
 Results: A total of 17 studies were included ranging from case reports, series of cases, multicentre cross-sectional study with the first-time onset of seizure associated with an epileptic origin. We excavated causes of complex COVID-19 related neurological manifestations, e.g., cerebrovascular diseases, encephalitis, demyelinating lesions, cytokine storm and proposed routes of SARS-CoV-2 entry into the nervous system to understand the mechanism of an epileptic seizure.
 Conclusion: COVID-19 is a potent neuropathogen which causes the new onset of epileptic seizures should get diagnostic recognition to evade possible deterioration of neurological conditions. However, more shreds of evidence from the future will further elucidate the epileptogenic potential of the pandemic.

Intracranial correlates of small sharp spikes

ObjectiveTo identify cortical correlates of scalp small sharp spikes (SSS) using simultaneous scalp and intracranial EEG recordings. MethodsPatients were retrospectively evaluated based on a database of intracranial long-term recordings at the Epilepsy Center Freiburg. Inclusion criteria were: simultaneous recordings with intracranial and scalp EEGs and the presence of at least five unequivocal SSS in the scalp EEG.Intracranial recordings were analyzed regarding the co-occurring intracranial potentials during scalp SSS. Results33 patients, aged 9-60y, 17 females, fulfilled the above-mentioned criteria. Almost all patients had intracranial SSS correlates in the form of spike/polyspike-waves in the temporal lobe, predominantly in the hippocampus (24/28), less frequently involving the amygdala (5/29), temporal basal (3/18), lateral neocortical (4/32), entorhinal cortices (1/12), and the parietal lobe (2/13). Amplitudes of intrahippocampal spikes or polyspikes co-occurring with SSS were significantly higher than intracranial discharges without scalp correlates. In 45% of patients, intracranial spikes accompanying SSS were located within the seizure onset zone (SOZ). ConclusionsOur results strongly support an epileptic origin of SSS and provide evidence about their heterogenous generators. SignificanceThis study suggests that SSS cannot with certainty be classified as “benign” but rather considered as one of the EEG manifestations of focal epilepsy.

Sandifer Syndrome

Sandifer syndrome, named after neurologist Paul Sandifer, was first reported by Marcel Kinsbourne in 1962, who noted an upper gastrointestinal disorder that occurs in children and adolescents with neurological manifestations. Sandifer syndrome is a neurobehavioral disorder that causes a series of paroxysmal dystonic movements in association with gastroesophageal reflux and, in some cases, with hiatal hernia. It is characterized by esophagitis, iron deficiency anemia, and is often mistaken for a seizure of epileptic origin.

Plasma-lactate levels in simulated seizures – An observational study

PurposeDifferentiating between epileptic seizures, convulsive syncope or non-epileptic seizures is a common diagnostic challenge in the acute setting. Plasma-lactate levels have previously been proposed as a tool to aid in differentiating between epileptic and non-epileptic seizures, with lower levels of lactate suggesting a non-epileptic origin. The aim of this study was to investigate levels of lactate in non-epileptic seizures. MethodsHealthy subjects were asked to perform a simulated seizure lasting no more than 5 min. Venous blood samples were taken before and immediately after the simulated seizure and analyzed using an ABL90 FLEX yielding information about lactate, pH, pO2,pCO2, electrolytes and plasma glucose. Results8 people participated in the study: 6 men and 2 women aged 27–45. The average pre-simulation lactate was 1.1 mmol/L while the average pH was 7.39. The average post-simulation lactate was 10.2 mmol/L while the average pH was 7.25. This means an average increase in plasma-lactate of 9.1 mmol/L and an average drop of 0.15 in pH. ConclusionOur data indicate that high rises in lactate levels are not specific for an epileptic origin. Further study of lactate as a marker for epileptic seizures is warrented.

Depersonalization- and derealization-like phenomena of epileptic origin.

ObjectiveDepersonalization refers to the sensation of being detached from one’s body, often associated with feelings of loss of control over one’s own body, actions, or thoughts. Derealization refers to the altered perception of one’s surroundings that is experienced as unreal. Although usually reported by psychiatric patients suffering from depression or anxiety, single case reports and small case series have described depersonalization‐ and derealization‐like symptoms in the context of epilepsy.MethodsWe investigated the brain mechanisms of ictal depersonalization– and derealization like symptoms by analyzing clinical and neuropsychological data as well as the epileptogenic zone based on a multimodal approach in a group of patients reporting depersonalization‐ (n = 9) and derealization‐like symptoms (n = 7), from a single presurgical epilepsy center with focal epilepsy. We compared them with a group of control patients with experiential phenomena due to temporal lobe epilepsy (n = 28).ResultsWe show that all patients with ictal depersonalization‐like symptoms report altered self‐identification with their body and mostly suffer from frontal lobe epilepsy with the epileptogenic zone in the dorsal premotor cortex, while patients with derealization‐like symptoms suffer from temporal lobe epilepsy. This finding is supported by post‐ictal neuropsychological deficits, showing that depersonalization‐like symptoms were significantly more often associated with frontal lobe dysfunction as compared to the control patients and patients with derealization‐like symptoms.ConclusionWe argue that depersonalization of epileptic origin constitutes a distinct disorder due to frontal lobe epilepsy. We discuss these findings with respect to earlier accounts of depersonalization and the recent concept of bodily self‐consciousness.

Editors' note: Clinical Reasoning: A 54-year-old woman with confusion and visual disturbances

Rossi et al. presented the unusual case of a 54-year-old woman with cirrhosis who developed oculomotor apraxia, optic ataxia, impaired smooth pursuit, and horizontal nystagmus in all directions of gaze. The neuroimaging and electrographic diagnosis was nonconvulsive status epilepticus resulting in Balint syndrome. Dr. Pollak also suspects an epileptic origin of the horizontal, alternating nystagmus pattern, given the bilateral MRI and EEG findings. However, Dr. Pollack notes that a normal optokinetic nystagmus would be unusual during seizure activity. Rossi et al. attribute this to the fluctuating nature of the patient's condition and the intermittent epileptiform activity on EEG. Resolution of the cortical diffusion abnormalities on MRI would also have supported seizures as the cause of the patient's symptoms, as Dr. Pollak writes. Unfortunately, this could not be confirmed as the patient was lost to follow-up. IRossi et al. presented the unusual case of a 54-year-old woman with cirrhosis who developed oculomotor apraxia, optic ataxia, impaired smooth pursuit, and horizontal nystagmus in all directions of gaze. The neuroimaging and electrographic diagnosis was nonconvulsive status epilepticus resulting in Balint syndrome. Dr. Pollak also suspects an epileptic origin of the horizontal, alternating nystagmus pattern, given the bilateral MRI and EEG findings.

Classification of neonatal epileptic seizures using support vector machine

Neonates are infants who are in their first 28 days of life. The diagnoses of neonatal seizures have been advocated by the use of clinical observations and electroencephalography (EEG). The continuous monitoring of neonatal EEGs in neonatal intensive care units is tedious and involves experts' intervention. The use of clinical decision support systems into the neonatal intensive care units has proved to produce aid to neonatal staff. The neonatal seizures of epileptic origin are more common and we recommend an approach to aid in the classification of the same using EEG signals of the neonates. Daubechies wavelet transform is used for the task of separation of frequency bands and the extraction of features. The theta rhythm of EEG reflects rightly the occurrence of epileptic seizures in neonates. The features taken into consideration for the classification are mean, variance, skewness and kurtosis. The support vector machine (SVM)-based classification is adopted for the development of the system which detects the presence or absence of epileptic seizures. The performance of this diagnostic aid system has been studied and the system has a sensitivity of 94% and specificity of 96%. The receiver operating characteristic curve is also used in the performance assessment.

Classification of neonatal epileptic seizures using support vector machine

Neonates are infants who are in their first 28 days of life. The diagnoses of neonatal seizures have been advocated by the use of clinical observations and electroencephalography (EEG). The continuous monitoring of neonatal EEGs in neonatal intensive care units is tedious and involves experts' intervention. The use of clinical decision support systems into the neonatal intensive care units has proved to produce aid to neonatal staff. The neonatal seizures of epileptic origin are more common and we recommend an approach to aid in the classification of the same using EEG signals of the neonates. Daubechies wavelet transform is used for the task of separation of frequency bands and the extraction of features. The theta rhythm of EEG reflects rightly the occurrence of epileptic seizures in neonates. The features taken into consideration for the classification are mean, variance, skewness and kurtosis. The support vector machine (SVM)-based classification is adopted for the development of the system which detects the presence or absence of epileptic seizures. The performance of this diagnostic aid system has been studied and the system has a sensitivity of 94% and specificity of 96%. The receiver operating characteristic curve is also used in the performance assessment.

Interictal spike connectivity in human epileptic neocortex

ObjectiveInterictal spikes are a biomarker of epilepsy, yet their precise roles are poorly understood. Using long-term neocortical recordings from epileptic patients, we investigated the spatial-temporal propagation patterns of interictal spiking. MethodsInterictal spikes were detected in 10 epileptic patients. Short time direct directed transfer function was used to map the spatial-temporal patterns of interictal spike onset and propagation across different cortical topographies. ResultsEach patient had unique interictal spike propagation pattern that was highly consistent across times, regardless of the frequency band. High spiking brain regions were often not spike onset regions. We observed frequent spike propagations to shorter distances and that the central sulcus forms a strong barrier to spike propagation. Spike onset and seizure onset seemed to be distinct networks in most cases. ConclusionsPatients in epilepsy have distinct and unique network of causal propagation pattern which are very consistent revealing the underlying epileptic network. Although spike are epileptic biomarkers, spike origin and seizure onset seems to be distinct in most cases. SignificanceUnderstanding patterns of interictal spike propagation could lead to the identification patient-specific epileptic networks amenable to surgical or other treatments.

A Prediction Model to Determine Childhood Epilepsy After 1 or More Paroxysmal Events.

The clinical profile of children who had possible seizures is heterogeneous, and accuracy of diagnostic testing is limited. We aimed to develop and validate a prediction model that determines the risk of childhood epilepsy by combining available information at first consultation. We retrospectively collected data of 451 children who visited our outpatient department for diagnostic workup related to 1 or more paroxysmal event(s). At least 1 year of follow-up was available for all children who were diagnosed with epilepsy or in whom diagnosis remained inconclusive. Clinical characteristics (sex, age of first seizure, event description, medical history) and EEG report were used as predictor variables for building a multivariate logistic regression model. Performance was validated in an external cohort (n = 187). Model discrimination was excellent, with an area under the receiver operating characteristic curve of 0.86 (95% confidence interval [CI]; 0.80-0.92), a positive predictive value of 0.93 (95% CI 0.83-0.97) and a negative predictive value of 0.76 (95% CI 0.70-0.80). Model discrimination in a selective subpopulation of children with uncertain diagnosis after initial clinical workup was good, with an area under the receiver operating characteristic curve of 0.73 (95% CI 0.58-0.87). This model may prove to be valuable because predictor variables together with a first interictal EEG can be available at first consultation. A Web application is provided (http://epilepsypredictiontools.info/first-consultation) to facilitate the diagnostic process for clinicians who are confronted with children with paroxysmal events, suspected of having an epileptic origin.