Hyperventilation maneuver during EEG in children with epilepsy after the COVID-19 pandemic. Is a routine procedure necessary?

OBJECTIVE: Hyperventilation (HV) is one of the main and basic activation methods during ambulatory electroencephalogram (EEG), unless medical reasons contraindicate it. During the COVID-19 pandemic, with the high risk of human-to-human infection, local guidelines and recommendations have been developed that suggest not to perform the HV maneuver routinely. Our objective was to characterize patients who present positive HV in an epilepsy center. METHODS: We analyzed retrospectively all the ambulatory EEGs performed during one year in our specialized ambulatory child and adolescent epilepsy center, and describe patients with positive maneuver. RESULTS: A total of 305 EEGs were performed. Patients under 3 years and 11 months were excluded as well as all patients that did not fill up the criteria for epilepsy diagnosis. From the 252 EEGs that were included in the study, 194 EEGs (77%) were classified as abnormal and 58 (23%) as normal. From these same 252 EEGs, 150 EEG finished correctly the HV maneuver. Physiological slowing response was found in 54 EEGs (36%), no changes (negative) in 83 (55%), and abnormal response (positive) in 13 EEGs (9%). The 13 HV-positive EEGs showed 4 patients with an increase of epileptiform activity, 3 patients experienced an increase of basal preregistered abnormal slowing, and 6 EEGs showed trigger of bilaterally synchronous and symmetric 2-4 Hz spike-and-slow wave discharges and absences. None of these last 6 patients needed more than 3 minutes to elicit the paroxysmal discharge. SIGNIFICANCE: Based on these findings and according with other studies, the low positivity and high specificity of the HV maneuver support the idea that HV could be excluded during the COVID-19 pandemic situation, and also reevaluate whether it could be changed to a complementary maneuver, restricted only for cases where absence epilepsy is suspected. Larger studies will be needed to reaffirm this proposal.

[Parkinson's disease and it's look-alike].

The diagnosis of Parkinson's disease (PD) requires the exclusion of other diseases using various methods. However, it is difficult to differentiate these diseases based only on clinical symptoms, and information regarding responses to drugs and several imaging examinations are often needed for a diagnosis. In recent years, various neurological signs and symptoms have been reported that are particularly useful in neurological examinations for differentiating PD, progressive supranuclear palsy, and multiple system atrophy. Currently, diagnosis using imaging techniques and artificial intelligence are being developed, but systematic neurological examinations will continue to be important in diagnosing these diseases.

[Second look: practical diagnostic and therapeutic checks in neurorehabilitation]. / Der zweite Blick: praktische diagnostische und therapeutische Checks in der rehabilitativen Neurologie.

Post-acute inpatient neurorehabilitation facilities are increasingly treating patients who are not only severely ill and multimorbid but who are also referred from non-neurological departments. These patients are still often medically unstable so that the previous diagnostics and treatment must be reevaluated and when necessary adapted or supplemented. Certain interdisciplinary diagnostic and therapeutic problems, such as antithrombotic therapy, regularly reoccur. This article presents these problems in a checklist fashion, which should provide indications in individual cases when previously carried out measures need to be questioned and adapted.

Functional movement disorders.

Functional movement disorders (FMD) represent a complex and disabling entity characterized by a broad range of clinical symptoms not explained by a classical neurological disease. In 2013, the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) added a clinical criterion based on incongruence and inconsistency, supported by recent literature highlighting the role of "positive clinical signs". These clinical signs allow a "rule-in" procedure in making a diagnosis of FMD so that the diagnosis is no longer a "rule-out" or "by default" diagnosis made after exclusion of other neurological conditions. This review summarizes current evidence on common clinical features and highlights bedside signs in FMD, such as tremor, dystonia, myoclonus and parkinsonism. Tics, chorea and hemiballism are also briefly discussed.

Diagnostic test accuracy meta-analysis of PRE-DELIRIC (PREdiction of DELIRium in ICu patients): A delirium prediction model in intensive care practice.

OBJECTIVES: To review and examine the evidence on diagnostic test accuracy of PRE-DELIRIC (PREdiction of DELIRium in ICu patients) for predicting delirium risk in critically ill patients. RESEARCH METHODOLOGY: This meta-analysis included studies reporting the diagnostic performance of PRE-DELIRIC between 2012 and 2019. The Cochrane Library, MEDLINE, Embase, CINAHL and Chinese Electronic Periodical Services databases were searched for eligible diagnostic studies. Risk of bias was assessed using a standard procedure according to the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) criteria. RESULTS: We included seven studies involving a total of 7941 critically ill patients in intensive care units settings. Results indicated that PRE-DELIRIC had a summary sensitivity of 0.76 (95% CI 0.60-0.87), and specificity of 0.66 (95% CI 0.45-0.82), suggesting that diagnostic performance of PRE-DELIRIC is useful to predict delirium risk in ICU patients. The area under the summary receiver operator characteristics (SROC) curve was 0.78 (95% CI 0.74-0.81), which also confirmed good accuracy of PRE-DELIRIC. CONCLUSION: We suggest that the PRE-DELIRIC model can be applied in the intensive care unit according to its good diagnostic test accuracy. However, this finding should be interpreted with caution due to the heterogeneity of this meta-analysis.

Modern neuro-ophthalmological evaluation of patients with pituitary disorders.

Pituitary adenomas can manifest as ophthalmological symptoms, such as decreased vision, impaired visual field or diplopia. It is important to recognize these neuro-ophthalmological syndromes to achieve early diagnosis and treatment and to improve prognosis. Currently, ophthalmological examination includes precise measuring instruments, such as optical coherence tomography (OCT), which allows the evaluation of optic atrophy related to compression of the anterior optic tract. These measurements are reproducible and are useful for diagnostic and prognostic evaluation. In this review, we describe the ophthalmological syndromes associated with pituitary tumours: anterior optic pathway compression, followed by oculomotor disorders and pituitary apoplexy.

Toward a motor signature in autism: Studies from human-machine interaction.

BACKGROUND: Autism spectrum disorder (ASD) is a heterogeneous group of neurodevelopmental disorders which core symptoms are impairments in socio-communication and repetitive symptoms and stereotypies. Although not cardinal symptoms per se, motor impairments are fundamental aspects of ASD. These impairments are associated with postural and motor control disabilities that we investigated using computational modeling and developmental robotics through human-machine interaction paradigms. METHOD: First, in a set of studies involving a human-robot posture imitation, we explored the impact of 3 different groups of partners (including a group of children with ASD) on robot learning by imitation. Second, using an ecological task, i.e. a real-time motor imitation with a tightrope walker (TW) avatar, we investigated interpersonal synchronization, motor coordination and motor control during the task in children with ASD (n=29), TD children (n=39) and children with developmental coordination disorder (n=17, DCD). RESULTS: From the human-robot experiments, we evidenced that motor signature at both groups' and individuals' levels had a key influence on imitation learning, posture recognition and identity recognition. From the more dynamic motor imitation paradigm with a TW avatar, we found that interpersonal synchronization, motor coordination and motor control were more impaired in children with ASD compared to both TD children and children with DCD. Taken together these results confirm the motor peculiarities of children with ASD despite imitation tasks were adequately performed. DISCUSSION: Studies from human-machine interaction support the idea of a behavioral signature in children with ASD. However, several issues need to be addressed. Is this behavioral signature motoric in essence? Is it possible to ascertain that these peculiarities occur during all motor tasks (e.g. posture, voluntary movement)? Could this motor signature be considered as specific to autism, notably in comparison to DCD that also display poor motor coordination skills? We suggest that more work comparing the two conditions should be implemented, including analysis of kinematics and movement smoothness with sufficient measurement quality to allow spectral analysis.

Would a Reasonable Person Now Accept the 1968 Harvard Brain Death Report? A Short History of Brain Death.

When The Ad Hoc Committee of Harvard Medical School to Examine the Definition of Brain Death began meeting in 1967, I was a graduate student, with committee member Ralph Potter and committee chair Henry Beecher as my mentors. The question of when to stop life support on a severely compromised patient was not clearly differentiated from the question of when someone was dead. A serious clinical problem arose when physicians realized that a patient's condition was hopeless but life support perpetuated body function. Thus, the committee stated that its first purpose was to deal with the burdens on patients and families as well as on hospitals and on patients needing hospital beds occupied by comatose patients. They intuited the strategy of "defining" these patients as dead, thus legitimating treatment stoppage. They noted that this would also serve a second purpose. Although the dead donor rule had not yet been clearly articulated, they claimed that defining patients as dead would also address controversy over obtaining organs for transplant. My mentors' discussions related to my interest in the intersection between questions primarily of medical fact (When has a human brain irreversibly ceased functioning?) and nonmedical questions of social policy (Should we treat individuals with dead brains and beating hearts as dead humans?). It quickly became clear that most committee members did not appreciate the interplay of these questions.

The 2016 World Health Organization classification of tumours of the central nervous system.

The 2016 WHO classification of tumours of the central nervous system represents the new paradigm among the specialists in the brain tumours and proposes a new approach combining histopathological and molecular features into diagnosis named 'integrated diagnosis'. The aim of this challenge is to overstep the interobserver variability of diagnosis based on previous classifications in order to ensure homogenous biological entities with a more accurate clinical significance. Over the last two decades, several molecular aberrations into gliomagenesis were highlighted and then confirmed as emerging biomarkers through prognostic stratification. In particular, IDH1/IDH2 genes mutations, 1p/19q codeletion and mutations in genes encoding histone H3 variants drastically changed the knowledge about diffuse gliomas inducing the WHO working group to consider the phenotype-genotype approach. In the present review, the historical development of the diagnosis of brain tumours from the 3D spatial configuration to the integration of multidisciplinary data up to recent molecular alterations is discussed. At the national level, the RENOCLIP network (supported by the National Cancer Institute) contributes to improve the standardization of histological diagnosis and the facilitation of access to molecular biology platforms for the detection of genetic aberrations necessary for integrated diagnosis. Importantly, the French POLA cohort allowed to test the clinical impact of the new criteria introduced by 2016 WHO classification of CNS tumours confirming the high accuracy in predicting clinical behaviour for diffuse gliomas.

Genes important for otoneurological diagnostic purposes - current status and future prospects.

SUMMARY: This review focuses on the current knowledge of the genes responsible for non-syndromic hearing loss that can be useful for otoneurological diagnostic purposes. From among a large number of genes that have been associated with non-syndromic hearing impairment, we selected several best-known genes, including the COCH gene, GJB2, GJB6 and SLC26A4, and we describe their role and effects of mutations and prevalence of mutations in various populations. Next, we focus on genes associated with tinnitus. Important areas for further research include assessment of genes potentially involved in pathophysiology of tinnitus and vertigo, which have traditionally been considered as being of otological aetiology, while advances in neuroimaging techniques have increasingly shifted studies toward neurological correlations.

Making psychiatric semiology great again: A semiologic, not nosologic challenge.

This article analyzes whether psychiatric disorders can be considered different from non-psychiatric disorders on a nosologic or semiologic point of view. The supposed difference between psychiatric and non-psychiatric disorders relates to the fact that the individuation of psychiatric disorders seems more complex than for non-psychiatric disorders. This individuation process can be related to nosologic and semiologic considerations. The first part of the article analyzes whether the ways of constructing classifications of psychiatric disorders are different than for non-psychiatric disorders. The ways of establishing the boundaries between the normal and the pathologic, and of classifying the signs and symptoms in different categories of disorder, are analyzed. Rather than highlighting the specificity of psychiatric disorders, nosologic investigation reveals conceptual notions that apply to the entire field of medicine when we seek to establish the boundaries between the normal and the pathologic and between different disorders. Psychiatry is thus very important in medicine because it exemplifies the inherent problem of the construction of cognitive schemes imposed on clinical and scientific medical information to delineate a classification of disorders and increase its comprehensibility and utility. The second part of this article assesses whether the clinical manifestations of psychiatric disorders (semiology) are specific to the point that they are entities that are different from non-psychiatric disorders. The attribution of clinical manifestations in the different classifications (Research Diagnostic Criteria, Diagnostic Statistic Manual, Research Domain Criteria) is analyzed. Then the two principal models on signs and symptoms, i.e. the latent variable model and the causal network model, are assessed. Unlike nosologic investigation, semiologic analysis is able to reveal specific psychiatric features in a patient. The challenge, therefore, is to better define and classify signs and symptoms in psychiatry based on a dual and mutually interactive biological and psychological perspective, and to incorporate semiologic psychiatry into an integrative, multilevel and multisystem brain and cognitive approach.

[Biomarkers for dementia and other neurodegenerative diseases : Current developments]. / Biomarker bei Demenzen und anderen neurodegenerativen Erkrankungen : Aktuelle Entwicklungen.

Cerebrospinal fluid-based neurochemical dementia diagnostics (CSF-NDD) support the early and differential diagnosis of dementia, most importantly the diagnosis of early or preclinical Alzheimer's dementia (AD). Meanwhile CSF-NDD are now recommended for improved exclusion and positive diagnostics of AD by the German national neuropsychiatry S3 dementia guidelines ( www.DGPPN.de ). Meta-analyses of independent international multicenter studies have shown that a combined CSF analysis of amyloid-beta 1-42 (Aß 1-42, decreased), total tau proteins (increased) and phospho-tau proteins (increased) offers a sensitivity and specificity of 80-90 % for the early and differential diagnosis of AD (AD versus all other). Generally, CSF-NDD should be combined with blood-based routine diagnostics and should be part of routine CSF diagnostics, e. g. cell count and cell differentiation (if applicable), intrathecal antibody synthesis and blood-CSF barrier analysis. The CSF-NDD are most valuable for the improved differentiation between reversible dementia syndromes and irreversible neurodegenerative dementia, e. g. cognitive deficits due to late onset depression (pseudodementia due to depression) or AD. Combined with extended psychometric neuropsychological evaluation and neuroimaging methods, such as magnetic resonance imaging (MRI), dopamine transporter scanning (DaTscan) by single photon emission computed tomography (SPECT), 18F-fluorodeoxyglucose positron emission tomography (glucose-PET) and amyloid-PET, CSF-NDD also significantly improve the differential diagnostics within the heterogeneous group of primary neurodegenerative dementias. Meanwhile, several independent studies have indicated that the Aß 1-42:Aß 1-40 ratio is superior to the determination of Aß 1-42 alone. Currently, several international research initiatives have been launched to further harmonize and optimize preanalytical procedures and CSF-NDD biomarker assays.

[Cerebrospinal fluid diagnostics in Germany since 1950 : Developments in the GDR and FRG in the context of society and science]. / Liquordiagnostik in Deutschland nach 1950 : Entwicklungen im Kontext von Wissenschaft und Gesellschaft in DDR und BRD.

The 40 years of separated development in two countries with extremely different political and social utopias allow consideration of the connection between science and society. The society-dependent development of cerebrospinal fluid (CSF) diagnostics in the German Democratic Republic (GDR) and the Federal Republic of Germany (FRG) is shown in the context of the international scientific development of the post-war era with new paradigms in physics, biology and genetics. As part of this contribution to the philosophy of science the consequences of the complex life science for a new view of disease research are discussed in contrast to the currently dominating, reductionistic medical industrial complex.

Transcranial magnetic stimulation (TMS) coupled with electroencephalography (EEG): Biomarker of the future.

INTRODUCTION: In recent years, a number of novel brain-stimulation techniques have been developed (such as TMS-EEG, TMS-fMRI and TMS-NIRS), yet they remain underutilized in the field of epilepsy. Accumulating evidence suggests that transcranial magnetic stimulation (TMS) combined with electroencephalography (TMS-EEG) is a highly relevant technique for exploration of the pathophysiology of human epilepsies as well as a promising biomarker with diagnostic and prognostic potential. RESULTS: In genetic generalized epilepsies, TMS-EEG has provided pathophysiological insight by revealing quasi-stable, covert states of excitability, a subclass of which is associated with the generation of TMS-induced epileptiform discharges (EDs). In focal epilepsy, TMS-induced EDs were successfully employed to identify the epileptogenic zone. In addition, TMS trains applied during focal EDs can terminate them, and appear to restore the effective connectivity of the brain network significantly altered by EDs. This abortive effect of TMS on EDs may possibly serve as a biomarker of response to invasive neuromodulatory techniques. CONCLUSION: TMS-EEG-based stimulation paradigms can provide insight into the mechanisms underlying human epilepsies and, thus, warrant further study as diagnostic and prognostic biomarkers.

XVIII Reunión Anual de la Sociedad Iberoamericana de Enfermedad Cerebrovascular. Comunicaciones / XVIII Reunión Anual de la Sociedad Iberoamericana de Enfermedad Cerebrovascular. Communications

No disponible

Estudio de la asistencia neurológica ambulatoria en la Comunidad de Madrid: impacto del modelo de libre elección de hospital / Study of outpatient neurological care in the Region of Madrid: The impact of implementing free choice of hospital

Introducción: En la Comunidad de Madrid se ha desarrollado un nuevo sistema de libertad de elección de área sanitaria, que puede suponer un cambio en la asistencia sanitaria neurológica y su gestión. El objetivo de este estudio fue analizar las primeras visitas de Neurología general atendidas en un área sanitaria de Madrid, teniendo en cuenta el área sanitaria de procedencia del paciente. Métodos: Estudio observacional, prospectivo, de una cohorte de primeras visitas de neurología ambulatoria realizadas entre el 16 de septiembre del 2013 y el 16 de enero del 2014. Resultados: Se incluyó a 1.109 pacientes (63,8% mujeres, edad media 55,2 ± 20,5 años). Las categorías diagnósticas más frecuentes fueron cefalea periódica, trastornos cognitivos y patología neuromuscular. El 1,1% se consideró patología no neurológica. El tiempo medio de demora fue de 7,2 ± 5,1 días. El 73,8% perteneció a la propia área sanitaria del hospital, mientras que el 26,2% procedió de otra área por libertad de elección. De estos, el 59,5% acudió por excelencia, mientras que el 39,7% por una menor demora. Los pacientes que acudieron por libre elección tuvieron una edad media menor (50,7 vs. 57,3 años; p < 0,0001) y una menor tasa de altas en la primera visita (16,4% vs. 30,1%; p < 0,0001). Conclusión: El modelo de libre elección de la asistencia neurológica implica un cambio relevante en la gestión sanitaria. La búsqueda de centros a los que se atribuye mayor excelencia y con menor demora son motivos para la libertad de elección, asociándose los pacientes de otra área a una mayor complejidad

Introduction: A new model permitting free choice of hospital has been introduced in the Region of Madrid. This may result in changes in how outpatient neurological care is provided and managed. The purpose of this study is to analyse initial visits to a general neurology department in the Region of Madrid and record the health district corresponding to each patient's residence. Methods: Observational and prospective study of a cohort of patients making initial outpatient visits to a neurology department between 16 September 2013 and 16 January 2014. Results: The study included 1109 patients (63.8% women, mean age 55.2 ± 20.5). The most frequent diagnostic groups were periodic headache, cognitive disorders, and neuromuscular diseases. Non-neurological diseases were diagnosed in 1.1% of the cases. The mean time of delay was 7.2 ± 5.1 days. Residents within the hospital's health district made up 73.8% of the total, while 26.2% chose a hospital outside of the health district corresponding to their residences. In the latter group, 59.5% made the choice based on the level of care offered, while 39.7% changed hospitals due to shorter times to consultation. The patients who came from another health district were younger (50.7 vs 57.3, P <.0001) and had a lower rate of discharges on the first visit (16.4% vs 30.1%, P <.0001). Conclusion: The model of free choice of hospital delivers significant changes in healthcare management and organisation. Reasons given for choosing another hospital are more ample experience and shorter delays with respect to the home district hospital. Management of patients from outside the health district is associated with greater complexity

Evolution of diagnostic criteria for multiple sclerosis.

Multiple sclerosis is a chronic demyelinating disease of the central nervous system that occurs primarily in young adults. There is no single diagnostic test to recognize the disease. The diagnostic criteria, based on clinical examination and laboratory tests, have changed considerably over time. The first guidelines involved only the results of the patient's neurological examination. The diagnostic criteria developed by Poser in 1983 were based largely on the results of additional tests, including visual evoked potentials and analysis of cerebrospinal fluid. The McDonald criteria, developed in 2001 and updated in 2005 and 2010, reflected the diagnostic breakthrough caused by widespread use of magnetic resonance imaging (MRI). Currently, the diagnosis depends largely on the results of the MRI examination. An early diagnosis is particularly important for starting disease-modifying treatments.

Neurological morbidity of monochorionic twins / Morbilidad neurológica en gemelos monocoriónicos

Monochorionic twins (MC) are at increased risk for morbidity. The unique placenta with vascular anastomoses may create imbalance with either acute or chronic hypotension or cardiac insufficiency that will eventually affect the fetal brain. It appears that these characteristics of the MC placenta add to the already higher frequency of brain anomalies observed among MZ twins. TOPS, TAPS, and TTTS require not only inter-twin anastomoses but also two live twins with an unbalanced shunt of blood. The death of the co-twin may prompt a sudden hypotension in the surviving twin with the consequent brain lesions. The more frequently occurring velamentous cord insertion in this kind of pregnancies is related to severe selective intrauterine growth restriction that may cause cerebral compromise. Finally, the preterm birth rate among MC twins is ten times higher than in singletons, and prematurity is a key factor for neurological morbidity as well. In this paper the various aspects of neurological morbidity in MC twins will be discussed (AU)

Los gemelos monocoriónicos (MC) poseen mayor riesgo de presentar un mal pronóstico, especialmente en la morbilidad neurológica. La existencia de una única placenta con anastomosis vascular crea un desequilibrio hemodinámico que causa hipotensión aguda o crónica, o insuficiencia cardiaca, lo que eventualmente afectará al cerebro fetal. Parece que estas características de la placenta MC se suman a la ya mayor frecuencia de anomalías cerebrales observadas entre los gemelos MZ. La secuencia oligodramnio y polidramnio (TOPS), las secuencias anemia-policitemia (SAP) y el síndrome de transfusión fetal-fetal (STFF) requieren no solo anastomosis intergemelar, sino que además deben existir 2 gemelos vivos con un desequilibrio en el intercambio sanguíneo. La muerte de uno de los cogemelos puede provocar una hipotensión brusca en el gemelo superviviente con las consiguientes lesiones cerebrales. La inserción velamentosa del cordón, que ocurre con mayor frecuencia en este tipo de embarazos, está relacionada con una restricción grave del crecimiento intrauterino selectivo que puede comprometer la función cerebral. Por último, la tasa de nacimientos prematuros entre gemelos MC es 10 veces superior a la de los embarazos simple s, y la prematuridad también es un factor clave para la morbilidad neurológica. En este artículo se examinarán los distintos aspectos de la morbilidad neurológica en los gemelos MC (AU)