Philippe Gélisse , Carlos Gallegos , Annacarmen Nilo , Greta Macorig , Pierre Genton , Arielle Crespel
{"title":"Epilepsy with eyelid myoclonia (Jeavons syndrome): Generalized, focal, or combined generalized and focal epilepsy syndrome?","authors":"Philippe Gélisse , Carlos Gallegos , Annacarmen Nilo , Greta Macorig , Pierre Genton , Arielle Crespel","doi":"10.1016/j.neucli.2024.102947","DOIUrl":"https://doi.org/10.1016/j.neucli.2024.102947","url":null,"abstract":"<div><p>Epilepsy with eyelid myoclonia (EM) or Jeavons syndrome (JS) is an epileptic syndrome related to the spectrum of genetic generalized epilepsies (GGE). We report two untreated children on which EEGs were performed several hours after a generalized tonic-clonic seizure (GTCS). These showed a unilateral, nearly continuous posterior slowing. This slow-wave activity was associated with contralateral epileptiform activity in one case, while in the second case, it was associated with an ipsilateral activity. However, in the latter child, a few months later an independent focus on the contralateral side was observed. A diagnosis of focal occipital lobe epilepsy was proposed in both cases, and one child underwent a left occipital lobectomy at 3.5 years of age. Despite surgery, absences with EM persisted in this child, and a marked photosensitivity to photic stimulation was observed two years later. The focal slow wave activity of one occipital lobe several hours after a GTCS in these two subjects was in favor of a focal onset preceding the generalization. The EEG evidence for independent left and right posterior focus in these two cases, the persistence of EM, and the development of a marked photosensitivity to photic stimulation in the child who underwent an occipital lobectomy, allow us to suggest that JS is associated with a network of bi-occipital hyperexcitability that rapidly engages bilaterally to produce generalized seizures.</p></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 3","pages":"Article 102947"},"PeriodicalIF":3.0,"publicationDate":"2024-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0987705324000054/pdfft?md5=7d04720d1f9396d9d6400d77de918871&pid=1-s2.0-S0987705324000054-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139986330","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yun-Ru Lai , Chih-Cheng Huang , Wen-Chan Chiu , Ben-Chung Cheng , Ting-Yin Lin , Hui-Ching Chiang , Cheng-Hsien Lu
{"title":"Predictive value of heart rate variability and electrochemical skin conductance measurements for cardiovascular autonomic neuropathy persistence in type 2 diabetes and prediabetes: A 3-year follow-up study","authors":"Yun-Ru Lai , Chih-Cheng Huang , Wen-Chan Chiu , Ben-Chung Cheng , Ting-Yin Lin , Hui-Ching Chiang , Cheng-Hsien Lu","doi":"10.1016/j.neucli.2024.102946","DOIUrl":"https://doi.org/10.1016/j.neucli.2024.102946","url":null,"abstract":"<div><h3>Objective</h3><p>The study aimed to explore risk stratification approaches for cardiovascular autonomic neuropathy (CAN) in individuals with prediabetes and type 2 diabetes (T2DM) over a three-year follow-up period.</p></div><div><h3>Methods</h3><p>Participants underwent evaluations of autonomic function encompassing cardiovascular autonomic reflex tests (CARTs), baroreflex sensitivity (BRS), heart rate variability (HRV) in time domains (standard deviation of all normal RR intervals (SDNN)) and frequency domains (high frequency/low frequency ratio), and electrochemical skin conductance (ESC). The diagnosis of CAN relied on abnormal CART results. Subjects were categorized into 4 groups, based on their assessment of cardiac autonomic function at 3-year follow-up, relative to the presence or absence of CAN at baseline assessment: Persistent absence of CAN; Resolution of CAN; Progression to CAN; and Persistent CAN.</p></div><div><h3>Results</h3><p>Participants with T2DM/prediabetes (<em>n</em> = 91/7) were categorized as: Persistent absence of CAN (<em>n</em> = 25), Resolution of CAN (<em>n</em> = 10), Progression to CAN (<em>n</em> = 18), and Persistent CAN (<em>n</em> = 45) groups. The Persistent absence of CAN group showed significant associations with SDNN. The Resolution of CAN group exhibited notable associations with mean HbA1C (follow-up), while the Progression to CAN group displayed a significant link with baseline estimated glomerular filtration rate. The Persistent CAN group demonstrated significant associations with SDNN and Sudoscan CAN risk score. Screening recommendations involve biennial to annual assessments based on risk levels, aiding in CAN detection and subsequent comprehensive and time-intensive autonomic function tests for confirmation. The study's findings offer improved risk categorization approaches for detecting CAN, which has relevance for shaping public health strategies.</p></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 3","pages":"Article 102946"},"PeriodicalIF":3.0,"publicationDate":"2024-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139986331","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Investigation of small fiber neuropathy in patients with diabetes mellitus by corneal confocal microscopy","authors":"Hatice Kaplan , Sema Yüzbaşıoğlu , Gönül Vural , Şadiye Gümüşyayla","doi":"10.1016/j.neucli.2024.102955","DOIUrl":"https://doi.org/10.1016/j.neucli.2024.102955","url":null,"abstract":"<div><h3>Objectives</h3><p>Corneal confocal microscopy (CCM) is a non-invasive technique that examines the corneal cellular structure. Its use in the detection of small fiber neuropathy is being researched. In our study, we examined the role of CCM in the detection of small fiber neuropathy in diabetic patients, as well as the differences between CCM findings in diabetic patients with and without overt polyneuropathy with neuropathic symptoms.</p></div><div><h3>Methods</h3><p>56 Diabetes Mellitus (DM) patients and 18 healthy controls were included in the study. The individuals included in the study were divided into three groups. Patients with diabetes who were found to have polyneuropathy according to electrophysiological diagnostic criteria were classified as Group 1, patients with diabetes and neuropathic symptoms without overt polyneuropathy according to electrophysiological diagnostic criteria were classified as Group 2, and healthy individuals were classified as Group 3. Electrophysiological examination and corneal imaging with CCM were performed in all groups.</p></div><div><h3>Results</h3><p>The CNFD and CNFL values of individuals in the diabetic group were discovered to be lower. CNFD values differ statistically between the groups (<em>p</em> = 0.047). Group 1-Group 3 differs from Group 2-Group 3 (respectively; <em>p</em> = 0.018, <em>p</em> = 0.048).</p></div><div><h3>Conclusion</h3><p>Our study demonstrates that CCM can be used in patients with neuropathic symptoms and no polyneuropathy detected in EMG and thought to have small fiber neuropathy. CCM provides an opportunity for early diagnosis in small fiber neuropathy.</p></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 4","pages":"Article 102955"},"PeriodicalIF":3.0,"publicationDate":"2024-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139985674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Correlation with sympathetic skin response, 123I-MIBG scintigraphy, and 123I-FP-CIT SPECT in Parkinson's disease","authors":"Masakazu Ozawa , Ryo Morishima , Toshio Shimizu , Kazushi Takahashi","doi":"10.1016/j.neucli.2024.102956","DOIUrl":"https://doi.org/10.1016/j.neucli.2024.102956","url":null,"abstract":"<div><h3>Background</h3><p>Parkinson's disease (PD), and other parkinsonian syndromes are known to cause striatonigral dopaminergic system dysfunction and autonomic disturbances, including the vasomotor and sudomotor nervous systems. The detection of <sup>123</sup>I-FP-CIT SPECT (DaT scan) imaging and autonomic dysfunction helps differentiate PD from multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). The sympathetic skin response (SSR) is a simple, non-invasive electrophysiological test that assesses the sympathetic sudomotor nervous system. It is reported that the SSR is impaired in patients with PD, MSA, and PSP.</p></div><div><h3>Objective</h3><p>To study the relationship between SSR, <sup>123</sup>I-metaiodobenzylguanidine (MIBG) cardiac scintigraphy and DaT scan imaging parameters in patients with PD, MSA, and PSP.</p></div><div><h3>Methods</h3><p>The study included 62, 25, and 19 patients with PD, MSA, and PSP, respectively. The SSR, MIBG cardiac scintigraphy, and DaT scan imaging were examined. The amplitude and latency of the SSR were measured in all limbs and were compared with the results of MIBG cardiac scintigraphy and DAT scan imaging.</p></div><div><h3>Results</h3><p>The SSR amplitudes were lower than reported normal subjects' reference values in PD, MSA, and PSP. The SSR amplitude only correlated with MIBG cardiac scintigraphy and DaT scan imaging parameters in PD. Multiple regression analyses also showed a significant relationship between the amplitudes of SSR and DaT scan imaging in PD.</p></div><div><h3>Conclusion</h3><p>Unlike MSA, and PSP, the sudomotor nervous system is parallelly involved with cardiac sympathetic and central dopaminergic dysfunction from the early stage of PD.</p></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 4","pages":"Article 102956"},"PeriodicalIF":3.0,"publicationDate":"2024-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139992625","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vincent P. Martin , Christophe Gauld , Jacques Taillard , Laure Peter-Derex , Régis Lopez , Jean-Arthur Micoulaud-Franchi
{"title":"Sleepiness should be reinvestigated through the lens of clinical neurophysiology: A mixed expertal and big-data Natural Language Processing approach","authors":"Vincent P. Martin , Christophe Gauld , Jacques Taillard , Laure Peter-Derex , Régis Lopez , Jean-Arthur Micoulaud-Franchi","doi":"10.1016/j.neucli.2023.102937","DOIUrl":"https://doi.org/10.1016/j.neucli.2023.102937","url":null,"abstract":"<div><p>Historically, the field of sleep medicine has revolved around electrophysiological tools. However, the use of these tools as a neurophysiological method of investigation seems to be underrepresented today, from both international recommendations and sleep centers, in contrast to behavioral and psychometric tools. The aim of this article is to combine a data-driven approach and neurophysiological and sleep medicine expertise to confirm or refute the hypothesis that neurophysiology has declined in favor of behavioral or self-reported dimensions in sleep medicine for the investigation of sleepiness, despite the use of electrophysiological tools. Using Natural Language Processing methods, we analyzed the abstracts of the 18,370 articles indexed by PubMed containing the terms ‘sleepiness’ or ‘sleepy’ in the title, abstract, or keywords. For this purpose, we examined these abstracts using two methods: a lexical network, enabling the identification of concepts (neurophysiological or clinical) related to sleepiness in these articles and their interconnections; furthermore, we analyzed the temporal evolution of these concepts to extract historical trends. These results confirm the hypothesis that neurophysiology has declined in favor of behavioral or self-reported dimensions in sleep medicine for the investigation of sleepiness. In order to bring sleepiness measurements closer to brain functioning and to reintroduce neurophysiology into sleep medicine, we discuss two strategies: the first is reanalyzing electrophysiological signals collected during the standard sleep electrophysiological test; the second takes advantage of the current trend towards dimensional models of sleepiness to situate clinical neurophysiology at the heart of the redefinition of sleepiness.</p></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 2","pages":"Article 102937"},"PeriodicalIF":3.0,"publicationDate":"2024-02-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139941704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jacques Taillard , Jean Arthur Micoulaud-Franchi , Vincent P. Martin , Laure Peter-Derex , Marie Françoise Vecchierini
{"title":"Objective evaluation of excessive daytime sleepiness","authors":"Jacques Taillard , Jean Arthur Micoulaud-Franchi , Vincent P. Martin , Laure Peter-Derex , Marie Françoise Vecchierini","doi":"10.1016/j.neucli.2023.102938","DOIUrl":"https://doi.org/10.1016/j.neucli.2023.102938","url":null,"abstract":"<div><p>Excessive daytime sleepiness (EDS) is multifactorial. It combines, among other things, an excessive propensity to fall asleep (“physiological sleepiness”) and a continuous non-imperative sleepiness (or drowsiness/hypo-arousal) leading to difficulties remaining awake and maintaining sustained attention and vigilance over the long term (“manifest sleepiness”). There is no stand-alone biological measure of EDS. EDS measures can either capture the severity of physiological sleepiness, which corresponds to the propensity to fall asleep, or the severity of manifest sleepiness, which corresponds to behavioral consequences of sleepiness and reduced vigilance. Neuropsychological tests (The psychomotor vigilance task (PVT), Oxford Sleep Resistance Test (OSLeR), Sustained Attention to Response Task (SART)) explore manifest sleepiness through several sustained attention tests but the lack of normative values and standardized protocols make the results difficult to interpret and use in clinical practice. Neurophysiological tests explore the two main aspects of EDS, i.e. the propensity to fall asleep (Multiple sleep latency test, MSLT) and the capacity to remain awake (Maintenance of wakefulness test, MWT). The MSLT and the MWT are widely used in clinical practice. The MSLT is recognized as the “gold standard” test for measuring the severity of the propensity to fall asleep and it is a diagnostic criterion for narcolepsy. The MWT measures the ability to stay awake. The MWT is not a diagnostic test as it is recommended only to evaluate the evolution of EDS and efficacy of EDS treatment. Even if some efforts to standardize the protocols for administration of these tests have been ongoing, MSLT and MWT have numerous limitations: age effect, floor or ceiling effects, binding protocol, no normal or cutoff value (or determined in small samples), and no or low test-retest values in some pathologies. Moreover, the recommended electrophysiological set-up and the determination of sleep onset using the 30‑<em>sec</em> epochs scoring rule show some limitations. New, more precise neurophysiological techniques should aim to detect very brief periods of physiological sleepiness and, in the future, the brain local phenomenon of sleepiness likely to underpin drowsiness, which could be called “physiological drowsiness”.</p></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 2","pages":"Article 102938"},"PeriodicalIF":3.0,"publicationDate":"2024-02-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139935376","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"From physiological awakening to pathological sleep inertia: Neurophysiological and behavioural characteristics of the sleep-to-wake transition✰","authors":"Perrine Ruby , Elisa Evangelista , Hélène Bastuji , Laure Peter-Derex","doi":"10.1016/j.neucli.2023.102934","DOIUrl":"https://doi.org/10.1016/j.neucli.2023.102934","url":null,"abstract":"<div><p>Sleep inertia refers to the transient physiological state of hypoarousal upon awakening, associated with various degrees of impaired neurobehavioral performance, confusion, a desire to return to sleep and often a negative emotional state. Scalp and intracranial electro-encephalography as well as functional imaging studies have provided evidence that the sleep inertia phenomenon is underpinned by an heterogenous cerebral state mixing local sleep and local wake patterns of activity, at the neuronal and network levels. Sleep inertia is modulated by homeostasis and circadian processes, sleep stage upon awakening, and individual factors; this translates into a huge variability in its intensity even under physiological conditions. In sleep disorders, especially in hypersomnolence disorders such as idiopathic hypersomnia, sleep inertia may be a daily, serious and long-lasting symptom leading to severe impairment. To date, few tools have been developed to assess sleep inertia in clinical practice. They include mainly questionnaires and behavioral tests such as the psychomotor vigilance task. Only one neurophysiological protocol has been evaluated in hypersomnia, the forced awakening test which is based on an event-related potentials paradigm upon awakening. This contrasts with the major functional consequences of sleep inertia and its potentially dangerous consequences in subjects required to perform safety-critical tasks soon after awakening. There is a great need to identify reproducible biomarkers correlated with sleep inertia-associated cognitive and behavioral impairment. These biomarkers will aim at better understanding and measuring sleep inertia in physiological and pathological conditions, as well as objectively evaluating wake-promoting treatments or non-pharmacological countermeasures to reduce this phenomenon.</p></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 2","pages":"Article 102934"},"PeriodicalIF":3.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139935294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Long sleep time and excessive need for sleep: State of the art and perspectives","authors":"Elisa Evangelista , Smaranda Leu-Semenescu , Fabio Pizza , Giuseppe Plazzi , Yves Dauvilliers , Lucie Barateau , Isabelle Lambert","doi":"10.1016/j.neucli.2024.102949","DOIUrl":"10.1016/j.neucli.2024.102949","url":null,"abstract":"<div><p>The mechanisms underlying the individual need for sleep are unclear. Sleep duration is indeed influenced by multiple factors, such as genetic background, circadian and homeostatic processes, environmental factors, and sometimes transient disturbances such as infections. In some cases, the need for sleep dramatically and chronically increases, inducing a daily-life disability. This “excessive need for sleep” (ENS) was recently proposed and defined in a European Position Paper as a dimension of the hypersomnolence spectrum, “hypersomnia” being the objectified complaint of ENS. The most severe form of ENS has been described in Idiopathic Hypersomnia, a rare neurological disorder, but this disabling symptom can be also found in other hypersomnolence conditions. Because ENS has been defined recently, it remains a symptom poorly investigated and understood. However, protocols of long-term polysomnography recordings have been reported by expert centers in the last decades and open the way to a better understanding of ENS through a neurophysiological approach. In this narrative review, we will 1) present data related to the physiological and pathological variability of sleep duration and their mechanisms, 2) describe the published long-term polysomnography recording protocols, and 3) describe current neurophysiological tools to study sleep microstructure and discuss perspectives for a better understanding of ENS.</p></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 2","pages":"Article 102949"},"PeriodicalIF":3.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139932213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"It is time to personalize rTMS targeting for the treatment of pain","authors":"Jean-Pascal Lefaucheur","doi":"10.1016/j.neucli.2024.102950","DOIUrl":"https://doi.org/10.1016/j.neucli.2024.102950","url":null,"abstract":"","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"54 1","pages":"Article 102950"},"PeriodicalIF":3.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139914531","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}