Advances in epileptic network findings of hypothalamic hamartomas.

Hypothalamic hamartomas (HHs) are congenital developmental malformations located in the hypothalamus. They are associated with a characteristic clinical manifestation known as gelastic seizures (GS). However, the traditional understanding of HHs has been limited, resulting in insufficient treatment options and high recurrence rates of seizures after surgery. This is consistent with the network hypothesis of focal epilepsy that the epileptogenic zone is not only limited to HH but may also involve the distant cerebral cortex external to the HH mass. The epilepsy network theory, on the other hand, provides a new perspective. In this study, we aim to explore HH-related epilepsy as a network disease, challenging the conventional notion of being a focal lesional disease. We analyze various aspects of HHs, including genes and signaling pathways, local circuits, the whole-brain level, phenotypical expression in terms of seizure semiology, and comorbidities. By examining HHs through the lens of network theory, we can enhance our understanding of the condition and potentially identify novel approaches for more effective management and treatment of epilepsy associated with HHs.

Hypothalamic hamartomas (HHs) are unusual brain malformations present from birth in the hypothalamus region. They often lead to a distinctive type of seizures known as GSs. However, our current understanding of HHs is limited, and this has made it challenging to treat them effectively. Many patients continue to experience seizures even after surgery. We've typically considered HH-related epilepsy as a localized problem, but a new theory suggests that it may involve a network of brain areas. In our study, we aim to change the way we view HH-related epilepsy. Instead of thinking of it as a single lesion in the brain, we explore the idea that it's a network disease. To do this, we'll investigate various aspects of HHs, such as the genes and pathways involved, how different parts of the brain interact, the impact on the whole brain, the types of seizures experienced, and any related health issues. By looking at HHs through this network theory, we hope to gain a deeper understanding of the condition and potentially discover new ways to manage and treat epilepsy associated with HHs. This shift in perspective could offer hope to those living with HH-related epilepsy and lead to more effective treatments, ultimately improving their quality of life.

Deep learning approaches for seizure video analysis: A review.

Seizure events can manifest as transient disruptions in the control of movements which may be organized in distinct behavioral sequences, accompanied or not by other observable features such as altered facial expressions. The analysis of these clinical signs, referred to as semiology, is subject to observer variations when specialists evaluate video-recorded events in the clinical setting. To enhance the accuracy and consistency of evaluations, computer-aided video analysis of seizures has emerged as a natural avenue. In the field of medical applications, deep learning and computer vision approaches have driven substantial advancements. Historically, these approaches have been used for disease detection, classification, and prediction using diagnostic data; however, there has been limited exploration of their application in evaluating video-based motion detection in the clinical epileptology setting. While vision-based technologies do not aim to replace clinical expertise, they can significantly contribute to medical decision-making and patient care by providing quantitative evidence and decision support. Behavior monitoring tools offer several advantages such as providing objective information, detecting challenging-to-observe events, reducing documentation efforts, and extending assessment capabilities to areas with limited expertise. The main applications of these could be (1) improved seizure detection methods; (2) refined semiology analysis for predicting seizure type and cerebral localization. In this paper, we detail the foundation technologies used in vision-based systems in the analysis of seizure videos, highlighting their success in semiology detection and analysis, focusing on work published in the last 7 years. We systematically present these methods and indicate how the adoption of deep learning for the analysis of video recordings of seizures could be approached. Additionally, we illustrate how existing technologies can be interconnected through an integrated system for video-based semiology analysis. Each module can be customized and improved by adapting more accurate and robust deep learning approaches as these evolve. Finally, we discuss challenges and research directions for future studies.

Neurodevelopmental outcome of neonatal seizures: A longitudinal study.

INTRODUCTION: Neonatal seizures (NS) are the most common neurological emergency in the neonatal period. The International League Against Epilepsy (ILAE) proposed a new classification of NS based on semiology and highlighted the correlation between semiology and aetiology. However, neurodevelopmental outcomes have not been comprehensively evaluated based on this new classification. AIMS: To evaluate neurodevelopmental outcomes and potential risk factors for severe outcomes in NS. METHODS: Patients with video electroencephalogram confirmed NS were evaluated. Seizure aetiology, cerebral magnetic resonance imaging (MRI) data, background electroencephalograms data, general movements, and neurodevelopmental outcomes were analysed. Severe outcomes were one of the following: death, cerebral palsy, Griffiths developmental quotient <70, epilepsy, deafness, or blindness. RESULTS: A total of 74 neonates were evaluated: 62 (83.8 %) with acute provoked NS (primarily hypoxic-ischaemic encephalopathy), and 12 (16.2 %) with neonatal-onset epilepsies (self-limited neonatal epilepsy, developmental and epileptic encephalopathy, cerebral malformations). Of these, 32 (43.2 %) had electrographic seizures, while 42 (56.7 %) had electroclinical seizures - 38 (90.5 %) were motor (42.1 % clonic) and 4 (9.5 %) were non-motor phenomena. Severe outcomes occurred in 33 of the 74 (44.6 %) participants. In multivariate analysis, neonatal-onset epilepsies (odds ratio [OR]: 1.3; 95 % confidence interval [CI]: 1.1-1.6), status epilepticus (OR: 5.4; 95 % CI: 1.5-19.9), and abnormal general movements (OR: 3.4; 95 % CI: 1.9-7.6) were associated with severe outcomes. CONCLUSIONS: At present, hypoxic-ischaemic encephalopathy remains the most frequent aetiology of NS. The prognosis of neonatal-onset epilepsies was worse than that of acute provoked NS, and status epilepticus was the most predictive factor for adverse outcomes.

"Amity Seizures": A previously unreported semiology localizing to a circuit between the right hippocampus and orbitofrontal area.

We describe a case of focal epilepsy with a semiology consisting of behaviors indicating an enthusiastic desire for those around him to get along and engage in friendly relations, which we refer to as "amity seizures". The patient was a 41-year-old right-handed male with seizures since age 26. Semiology consisted of stereotyped enthusiastic behaviors such as expressing "Peace! Peace!… Come on, we all on the same team, right?!", and giving hugs, kisses, and high-fives to those around him. On SEEG evaluation, 2 independent areas of seizure onset were identified, the right hippocampus and right posterior orbitofrontal area. Locally confined seizures had bland manifestation. However, spread from right hippocampus to right orbitofrontal area, or vice versa, elicited his typical amity seizure semiology. To our knowledge this is the first report of the seizure semiology we have coined "Amity seizures". While emotions were once thought to localize to discrete brain regions, they are now accepted to arise from networks across multiple brain regions. The fact that this behavior only occurred when seizures spread from either of 2 onset zones to the other suggests that this semiology results from network engagement between, and likely beyond, either onset zone.

Focal electroclinical features in generalized tonic-clonic seizures: Decision flowchart for a diagnostic challenge.

OBJECTIVE: Bilateral tonic-clonic seizures with focal semiology or focal interictal electroencephalography (EEG) can occur in both focal and generalized epilepsy types, leading to diagnostic errors and inappropriate therapy. We investigated the prevalence and prognostic values of focal features in patients with idiopathic generalized epilepsy (IGE), and we propose a decision flowchart to distinguish between focal and generalized epilepsy in patients with bilateral tonic-clonic seizures and focal EEG or semiology. METHODS: We retrospectively analyzed video-EEG recordings of 101 bilateral tonic-clonic seizures from 60 patients (18 with IGE, 42 with focal epilepsy). Diagnosis and therapeutic response were extracted after ≥1-year follow-up. The decision flowchart was based on previous observations and assessed concordance between interictal and ictal EEG. RESULTS: Focal semiology in IGE was observed in 75% of seizures and 77.8% of patients, most often corresponding to forced head version (66.7%). In patients with multiple seizures, direction of head version was consistent across seizures. Focal interictal epileptiform discharges (IEDs) were observed in 61.1% of patients with IGE, whereas focal ictal EEG onset only occurred in 13% of seizures and 16.7% of patients. However, later during the seizures, a reproducible pattern of 7-Hz lateralized ictal rhythm was observed in 56% of seizures, associated with contralateral head version. We did not find correlation between presence of focal features and therapeutic response in IGE patients. Our decision flowchart distinguished between focal and generalized epilepsy in patients with bilateral tonic-clonic seizures and focal features with an accuracy of 96.6%. SIGNIFICANCE: Focal semiology associated with bilateral tonic-clonic seizures and focal IEDs are common features in patients with IGE, but focal ictal EEG onset is rare. None of these focal findings appears to influence therapeutic response. By assessing the concordance between interictal and ictal EEG findings, one can accurately distinguish between focal and generalized epilepsies.

How Can a Focal Seizure Lead to a Dacrystic Behavior? A Case Analyzed with Functional Connectivity in Stereoelectroencephalography.

We present a case of a patient with focal non-motor emotional seizures with dacrystic expression in the context of drug-resistant magnetic resonance imaging negative epilepsy. The pre-surgical evaluation suggested a hypothesis of a right fronto-temporal epileptogenic zone. Stereoelectroencephalography recorded dacrystic seizures arising from the right anterior operculo-insular (pars orbitalis) area with secondary propagation to temporal and parietal cortices during the dacrystic behavior. We analyzed functional connectivity during the ictal dacrystic behavior and found an increase of the functional connectivity within a large right fronto-temporo-insular network, broadly similar to the "emotional excitatory" network. It suggests that focal seizure, potentially, from various origins but leading to disorganization of these physiological networks may generate dacrystic behavior.

Ictal hypersalivation as a prominent symptom in a girl with insulo-opercular epilepsy.

Introduction: Hypersalivation has been associated with Rolandic epilepsy and other childhood epilepsy syndromes. However, pure salivatory seizures are a rare type of focal seizure in which ictal hypersalivation is the dominant feature throughout the seizures. Case presentation: We present a case of pure salivatory seizures originating from the right post-central operculum cortex, confirmed by the favorable surgical outcome. We attempt to analyze the symptom from behavioral and neural network perspectives and propose a possible mechanism to generate ictal hypersalivation and pure salivatory seizures. Conclusion: Based on previous reports in the literature and our case, we emphasize the importance of the operculum in patients with ictal hypersalivation, particularly in patients with pure salivatory seizures.

Validez y pertinencia de la semiología de las psicosis en Castilla del Pino / Validity and Pertinence of the Semiology of Psychosis in Castilla del Pino

En las últimas décadas, la psicopatología y, con ella, la semiología psiquiátrica han sido sustituidas por las nosografías predominantes (CIE y DSM). En contraposición, existen modelos alternativos que ofrecen explicaciones de la conducta humana en el contexto relacional. Uno de ellos, el Modelo Judicativo de la Conducta, de Castilla del Pino, supone una herramienta válida para establecer los criterios que deben cumplir los actos de conducta calificados de psicóticos. En este artículo se describen los conceptos fundamentales de este modelo con el fin de objetivar las formaciones psicóticas, capturar los síntomas, diferenciarlos y establecer su validez en el diagnóstico de las conductas consideradas psicóticas, así como en los estados previos y en la deconstrucción. Se expliitan los aspectos necesitados de actualización y desarrollo, y se correlacionan conceptos de otros campos teóricos: niveles semiológicos, metáfora-metonimia, saliencia, saliencia aberrante e hiperreflexividad.

In recent decades, psychopathology and, with it, psychiatric semiology have been replaced by the predominant nosographies (ICD and DSM). In contrast, there are al-ternative models that offer explanations of human behavior in the relational context. One of them, the Castilla del Pino ́s Modelo Judicativo de Conducta, is a valid tool to establish the criteria that behaviors classified as psychotic must meet. The fundamental concepts of this model are described to objectify the psychotic formations, capture the symptoms, differentiate them, and establish their validity in the diagnosis of behaviors considered as psychotic, as well as in the previous states and in the deconstruction. Aspects in need of updating and development are made explicit and correlations with concepts from other theoretical fields, such as, semiological levels, metaphor-metonymy, salience, aberrant salience and hyperreflexivity, are examined. (AU)

[Prospective controlled study evaluating teleconsultation and tele-semiology for the management of renal colic]. / Étude prospective contrôlée évaluant la téléconsultation et la télé-sémiologie pour la prise en charge d'une colique néphrétique.

INTRODUCTION: Physical examination appears to be a limiting factor of teleconsultation (TC). We evaluated the feasibility of self-percussion of the lumbar fossae (sPLF) and TC for simple renal colic (SRC) in comparison with a face-to-face consultation (FC). MATERIAL AND METHODS: We performed a comparative prospective study in two steps. First: evaluation of the quality of an sPLF on a standardized patient in TC, without and with tutorial. Secondarily: evaluation of a TC and a FC for a SRC with a standardized patient in real conditions. Evaluation using objective clinical scores and qualitative scales by an observer, the standardized patient and the practitioner himself. RESULTS: Forty-two practitioners were included in the study. In the absence of a tutorial, the sPLF was most often "poorly done". The tutorial led to a significant improvement in the quality of sPLF. There was no difference in diagnostic and therapeutic performance among senior physicians between TC and FC. The therapeutic performances of the interns were significantly lower in TC without his being aware of it. The qualitative scores were significantly lower in TC vs FC according to the practitioners, the standardized patient and the observer. CONCLUSION: An sPLF is feasible but its practice should be taught. Unlike interns, senior physicians were able to perform a TC comparable to FC for the management of SRC. TC and telesemiology therefore require dedicated training and an experienced practitioner.

Semiologic differences and primary dysmenorrhea.

Dysmenorrhea is a condition that manifests itself in women before, during, and after menstruation, and it is accompanied by very intense pelvic pain. Moreover, women experience moderate to intense menstrual cramps year after year, decreasing their quality of life. The purpose of the study was to establish semiological differences associated with primary dysmenorrhea. Women who start their menstrual cycles with this symptomatology assume that it is normal and that they do not need specialized medical assistance. Medical professionals have established general practice guidelines on clinical disorders. However, there are no specific recommendations for the menstrual cycle that can effectively support the prompt diagnosis and treatment of primary dysmenorrhea, particularly at 3870 msnm. The uniqueness of our research is that semiological distinctions can change depending on height.

Towards a better identification of ictal semiology patterns in insular epilepsies: A stereo-EEG study.

OBJECTIVE: To describe pure insular ictal semiology and patterns of extra-insular spread demonstrated by stereoelectroencephalography (SEEG) according to a classification based on the insular cytoarchitecture. METHODS: We investigated the ictal semiology in 17 patients undergoing SEEG for insular epilepsy. The insular cortex was divided into three regions roughly overlapping with the agranular, dysgranular and granular regions. Ictal semiology was described accordingly: anterior insula (AI, short anterior and middle gyri), middle insula (MI, short posterior and long anterior gyri) and posterior insula (PI, long posterior gyrus). RESULTS: Awareness impairment occurred secondarily to extra-insular ictal spread. Subjective manifestations were constant. AI seizures (n = 3) presented with autonomic (increased heart rate [HR], respiratory changes), oropharyngeal (mainly throat sensations), emotional (fear, anguish) semiology and the "hand-to-throat" sign followed by frontal-like semiology. MI seizures (n = 8) presented with mainly non-painful paresthesia, some autonomic (respiratory, increased HR), oropharyngeal or thermic symptoms and early motor features with spread to the opercular cortex. PI seizures (n = 6) were characterized by somatosensory semiology, mainly paresthesia potentially painful, and cephalic sensations. CONCLUSIONS: Cytoarchitectonic-based classification and the corresponding ictal features support the antero-posterior grading of insular seizures and highlight specific ictal symptoms. SIGNIFICANCE: This refinement of insular semiology can help optimize the planning of SEEG for presumed insular epilepsy.

Exploratory Investigation of Handwriting Disorders in School-Aged Children from First to Fifth Grade.

Handwriting disorders (HDs) are prevalent in school-aged children, with significant interference with academic performances. The current study offers a transdisciplinary approach with the use of normed and standardized clinical assessments of neuropsychomotor, neuropsychological and oculomotor functions. The aim is to provide objective data for a better understanding of the nature and the etiology of HDs. Data from these clinical assessments were analyzed for 27 school-aged children with HD (first to fifth grade). The results underline a high heterogeneity of the children presenting HDs, with many co-occurrences often unknown. However, it was possible to highlight three levels of HDs based on BHK scores: mild HD not detected by the BHK test (26% of children), moderate HD (33%) and dysgraphia (41% of children). The mild nature of the HDs not detected by the BHK test appears to occur at a relatively low frequency of the associated disorders identified during clinical evaluations. On the contrary, dysgraphia appears to be associated with a high frequency of co-occurring disorders identified in the clinical assessment, with a predominance of oculomotor disorders (55% of children), leading to visual-perceptual difficulties and a high level of handwriting deterioration. Finally, children with moderate HD have fewer co-occurrences than children with dysgraphia, but have more difficulties than children with mild HD. This highlights the importance of differentiating between different degrees of HDs that do not respond to the same semiologies. Our findings support the interest in performing a transdisciplinary and standardized clinical examination with developmental standards (neuropsychomotor, neuropsychological and oculomotor) in children with HD. Indeed, HDs can therefore be associated with a multitude of disorders of different natures ranging from poor coordination of the graphomotor gesture to a more general and more complex impairment affecting perceptual-motor, cognitive and/or psycho-affective functions.

Somatosensory Auras in Epilepsy: A Narrative Review of the Literature.

An aura is a subjective experience felt in the initial phase of a seizure. Studying auras is relevant as they can be warning signs for people with epilepsy. The incidence of aura tends to be underestimated due to misdiagnosis or underrecognition by patients unless it progresses to motor features. Also, auras are associated with seizure remission after epilepsy surgery and are an important prognostic factor, guiding the resection site and improving surgical outcomes. Somatosensory auras (SSAs) are characterized by abnormal sensations on one or more body parts that may spread to other parts following a somatotopic pattern. The occurrence of SSAs among individuals with epilepsy can range from 1.42% to 80%. The upper extremities are more commonly affected in SSAs, followed by the lower extremities and the face. The most common type of somatosensory aura is paresthetic, followed by painful and thermal auras. In the primary somatosensory auras, sensations occur more commonly contralaterally, while the secondary somatosensory auras can be ipsilateral or bilateral. Despite the high localizing features of somatosensory areas, cortical stimulation studies have shown overlapping sensations originating in the insula and the supplementary sensorimotor area.

Motor seizure semiology.

Motor semiology is a major component of epilepsy evaluation, which provides essential information on seizure classification and helps in seizure localization. The typical motor seizures include tonic, clonic, tonic-clonic, myoclonic, atonic, epileptic spasms, automatisms, and hyperkinetic seizures. Compared to the "positive" motor signs, negative motor phenomena, for example, atonic seizures and Todd's paralysis are also crucial in seizure analysis. Several motor signs, for example, version, unilateral dystonia, figure 4 sign, M2e sign, and asymmetric clonic ending, are commonly observed and have significant clinical value in seizure localization. The purpose of this chapter is to review the localization value and pathophysiology associated with the well-defined motor seizure semiology using updated knowledge from intracranial electroencephalographic recordings, particularly stereoelectroencephalography.

When in doubt, return to fundamentals (seizure semiology).

This case of drug resistant focal epilepsy highlights several invaluable teaching points. Hypotheses grounded in seizure semiology provide the best framework to ensure accurate interpretation of diagnostic testing. Without a hypothesis, information gathered from tests can be difficult to linearly piece together and can lead to poor patient outcomes. The case also provides a real-world conundrum of discordant test results that were ultimately rectified by re-visiting the initial hypothesis and cross-testing. Perinatal stroke continues to be a common etiology of drug resistant epilepsy. Patients can achieve seizure freedom and good quality of life with appropriate workup and in appropriate cases, surgery.

Seizures Provoked by Greasy Pork in a Patient With Refractory Focal Epilepsy.

Seizures have been reported to be directly triggered by certain foods in some people with epilepsy. On the other hand, eating epilepsy has been mentioned in the literature as a rare disorder characterized by clinical and EEG findings that vary from patient to patient and are interestingly prevalent in certain geographic areas. Epilepsy in these patients is either idiopathic or due to underlying brain pathology. We present a case of refractory focal epilepsy in which the patient reports seizures provoked by eating greasy pork. During the admission to the epilepsy monitoring unit (EMU), the patient did not have seizures during the first three days of the admission despite antiepileptic medication withdrawal, sleep deprivation, and photic stimulation. However, when he consumed greasy pork, he had tonic-clonic convulsions about five hours after eating. On the following day, he had another tonic-clonic seizure after eating greasy pork.

[Non-epileptic psychogenic seizures]. / Les crises non épileptiques psychogènes.

Psychogenic non-epileptic seizures resemble epileptic seizures but their pathophysiological mechanisms differ. Despite being quite frequent, they are often misunderstood, regularly leading to a misdiagnosis and therapeutic errors. The objective of this article is to provide a short review of the current knowledge about this pathology. We will detail the main epidemiological data of psychogenic non-epileptic seizures. We will then discuss the diagnostic approach, emphasizing the semiological differences that exist between psychogenic non-epileptic seizures and epileptic seizures. Then, we will detail the main therapeutic actions and discuss the importance of communication between clinicians, the patient and his/her family. Finally, we will discuss the long-term prognosis of this pathology, which remains quite poor.

Les crises non épileptiques psychogènes ressemblent à des crises d'épilepsie, mais leurs mécanismes physiopathologiques diffèrent. Elles sont fréquentes, mais souvent méconnues, menant régulièrement à des erreurs diagnostiques et thérapeutiques. L'objectif de cet article est de dresser une synthèse des connaissances actuelles de cette pathologie. Nous y détaillerons les principales données épidémiologiques des crises non épileptiques psychogènes. Nous aborderons ensuite la démarche diagnostique en insistant sur les différences sémiologiques qui existent entre les crises non épileptiques psychogènes et les crises épileptiques. Nous présenterons ensuite les principales actions thérapeutiques existantes et parlerons de l'importance de la communication entre les cliniciens, le patient et son entourage. Enfin, nous discuterons du pronostic à long terme de cette pathologie, qui reste assez péjoratif.

Clinical and electroencephalography characteristics of 45 patients with neonatal seizures.

OBJECTIVES: The aim of our study was to retrospectively research the semiology of neonatal seizures (NSs) based on the 2021 classification scheme of the International League Against Epilepsy, and the relationship between etiology and electroclinical features. METHODS: Patients admitted to Children's Hospital of Chongqing Medical University from May 1, 2020 to March 30, 2022 and diagnosed with NSs were included to retrospectively investigate the etiology, seizure characteristics, prognosis, and ictal and interictal video electroencephalography (EEG) characteristics. RESULTS: Of the 45 patients, 73.3% had definite etiology. Twenty-seven patients had electro-clinical seizures, of which two had both electro-clinical and electrographic-only seizures. Electrographic-only seizures were reported in 18 patients. The tonic, clonic, and electrographic-only seizures were associated with various etiologies. Both tonic and clonic seizures occurred in acute symptomatic seizures and were associated with neonatal epilepsy. 50% of tonic seizures were related to genetic factors. Among the clonic seizures, 50.0% occurred in acute symptomatic seizures. Epileptic spasms always indicated neonatal epilepsy. There were few patients who experienced automatisms and sequential seizures, and these two seizure types were associated with brain malformation and genetic factors, respectively. Patients with a normal interictal EEG had acute symptomatic seizures. whereas the interictal EEG of patients with neonatal epilepsy mainly showed burst-suppression or multifocal discharges. The ictal EEG recordings were related to seizure semiology. CONCLUSION: Seizure semiology and video EEG are suggestive of potential causes but do not provide a definite etiology.