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ImportanceAcute symptomatic seizures occurring within 7 days after ischemic stroke may be associated with an increased mortality and risk of epilepsy. It is unknown whether the type of acute symptomatic seizure influences this risk.ObjectiveTo compare mortality and risk of epilepsy following different types of acute symptomatic seizures.Design, Setting, and ParticipantsThis cohort study analyzed data acquired from 2002 to 2019 from 9 tertiary referral centers. The derivation cohort included adults from 7 cohorts and 2 case-control studies with neuroimaging-confirmed ischemic stroke and without a history of seizures. Replication in 3 separate cohorts included adults with acute symptomatic status epilepticus after neuroimaging-confirmed ischemic stroke. The final data analysis was performed in July 2022.ExposuresType of acute symptomatic seizure.Main Outcomes and MeasuresAll-cause mortality and epilepsy (at least 1 unprovoked seizure presenting >7 days after stroke).ResultsA total of 4552 adults were included in the derivation cohort (2547 male participants [56%]; 2005 female [44%]; median age, 73 years [IQR, 62-81]). Acute symptomatic seizures occurred in 226 individuals (5%), of whom 8 (0.2%) presented with status epilepticus. In patients with acute symptomatic status epilepticus, 10-year mortality was 79% compared with 30% in those with short acute symptomatic seizures and 11% in those without seizures. The 10-year risk of epilepsy in stroke survivors with acute symptomatic status epilepticus was 81%, compared with 40% in survivors with short acute symptomatic seizures and 13% in survivors without seizures. In a replication cohort of 39 individuals with acute symptomatic status epilepticus after ischemic stroke (24 female; median age, 78 years), the 10-year risk of mortality and epilepsy was 76% and 88%, respectively. We updated a previously described prognostic model (SeLECT 2.0) with the type of acute symptomatic seizures as a covariate. SeLECT 2.0 successfully captured cases at high risk of poststroke epilepsy.Conclusions and RelevanceIn this study, individuals with stroke and acute symptomatic seizures presenting as status epilepticus had a higher mortality and risk of epilepsy compared with those with short acute symptomatic seizures or no seizures. The SeLECT 2.0 prognostic model adequately reflected the risk of epilepsy in high-risk cases and may inform decisions on the continuation of antiseizure medication treatment and the methods and frequency of follow-up.

Background and objectives Early-stage behavioural variant frontotemporal dementia (bvFTD) is often misdiagnosed, highlighting the need for new diagnostic instruments. Based on the revised diagnostic criteria for bvFTD, we developed the Behavioural Dysfunction Questionnaire (BDQ). In this explorative study, we aimed to determine the best scoring and analytical method for the BDQ to discriminate between bvFTD and non-bvFTD patients. Materials and methods 34 patients with early-stage bvFTD, 56 with early-stage Alzheimer's disease dementia (ADD) and 41 with major depressive disorder (MDD) were recruited. We calculated BDQ-items with or without inclusion of a time criterion: (a) without time criterion, (b) with 10 years’ time criterion (symptom presence less than 10 years), and (c) with 3 years’ time criterion (symptom presentation within the first 3 years). Using these three differently calculated items, we generated six variables, i.e. 3*2 [BDQ-Global Score (BDQ-GS; domains average score); BDQ-Global Domain Score (BDQ-GDS; domains categorical score)]. Then, we performed univariate and bivariate (BDQ-GS and BDQ-GDS combined) ROC analyses. Results Models including BDQ-GS, BDQ-GDS or both variables combined discriminated similarly between groups. In contrast, models without time criterion or with 10 years’ time criterion discriminated better than models including variables with 3 years’ time criterion. These models discriminated highly (AUC = 85.98–87.78) between bvFTD and MDD and bvFTD and ADD, respectively. Conclusion BDQ-scores without any time criterion discriminated highly between early-stage bvFTD and non-bvFTD groups, which could improve the early diagnosis of bvFTD. With its standardised procedure, the BDQ is also appropriate for repeated assessments.

Established cerebrospinal fluid (CSF) biomarkers allow for earlier and more accurate etiological diagnosis of cognitive impairment. Information and counselling are needed both before and after biomarker-supported diagnosis. The procedures for diagnostic lumbar punctures and pre-analytical sample handling should follow published consensus recommendations. The results must be interpreted in the context of the other available history information and assessments. Blood-based biomarkers and other non-invasive markers are expected to become available for clinical practice soon. Consequently, a broader usage of biomarkers is expected and may accelerate the development of individually tailored prevention and treatment approaches. This article provides the recommendations of the Swiss Memory Clinics for the use of biomarkers in clinical practice.Les marqueurs du liquide céphalorachidien établis permettent un diagnostic des troubles cognitifs plus précoce et précis. Il est nécessaire de conseiller les patients avant et après un examen des biomarqueurs. Les procédures de la ponction lombaire et de traitement préanalytique des échantillons doivent suivre des recommandations publiées. L’interprétation des résultats prendra en compte les antécédents médicaux et les autres résultats d’examen disponibles. Des marqueurs sanguins pourraient être disponibles dans un avenir proche. Cela pourrait conduire à une utilisation plus large des biomarqueurs et accélérer le développement d’approches personnalisées de prévention et de traitement. Cet article présente les recommandations de Swiss Memory Clinics concernant l’utilisation des biomarqueurs en pratique clinique.

Accidente cerebrovascular isquémico; Tratamiento de reperfusión; Factores de riesgo Ischemic Stroke; Reperfusion treatment; Risk factors Accident cerebrovascular isquèmic; Tractament de reperfusió; Factor de risc Objective The purpose of this study was to identify risk factors for acute symptomatic seizures and post-stroke epilepsy after acute ischemic stroke and evaluate the effects of reperfusion treatment. Methods We assessed the risk factors for post-stroke seizures using logistic or Cox regression in a multicenter study, including adults from 8 European referral centers with neuroimaging-confirmed ischemic stroke. We compared the risk of post-stroke seizures between participants with or without reperfusion treatment following propensity score matching to reduce confounding due to treatment selection. Results In the overall cohort of 4,229 participants (mean age 71 years, 57% men), a higher risk of acute symptomatic seizures was observed in those with more severe strokes, infarcts located in the posterior cerebral artery territory, and strokes caused by large-artery atherosclerosis. Strokes caused by small-vessel occlusion carried a small risk of acute symptomatic seizures. 6% developed post-stroke epilepsy. Risk factors for post-stroke epilepsy were acute symptomatic seizures, more severe strokes, infarcts involving the cerebral cortex, and strokes caused by large-artery atherosclerosis. Electroencephalography findings within 7 days of stroke onset were not independently associated with the risk of post-stroke epilepsy. There was no association between reperfusion treatments in general or only intravenous thrombolysis or mechanical thrombectomy with the time to post-stroke epilepsy or the risk of acute symptomatic seizures. Interpretation Post-stroke seizures are related to stroke severity, etiology, and location, whereas an early electroencephalogram was not predictive of epilepsy. We did not find an association of reperfusion treatment with risks of acute symptomatic seizures or post-stroke epilepsy.

Zusammenfassung. Molekulare Liquormarker der Alzheimer-Kernpathologie (Amyloidpathologie, Tau-Hyperphosphorylierung und neuronaler Zelluntergang) sind Bestandteil des diagnostischen Instrumentariums zur Abklärung kognitiver Störungen im Alter. Sie erlauben eine frühere und präzisere Diagnose und werden von Swiss Memory Clinics als Zusatzdiagnostik nach individuell gestellter Indikation empfohlen. Aufklärung und Beratung sind sowohl vor als auch nach der Biomarker-Diagnostik erforderlich. Die Durchführung der diagnostischen Lumbalpunktion und der präanalytische Umgang mit den Proben richtet sich nach publizierten Standards. Die Interpretation der Resultate muss sorgfältig und im Gesamtkontext aller anderen Befunde erfolgen. Dank bedeutender Fortschritte ist zu erwarten, dass Blutbiomarker und andere kostengünstige und leicht zugängliche Marker sowie spezifische Biomarker für weitere Demenzursachen in wenigen Jahren zur Verfügung stehen werden. Dieser Trend dürfte zu einem deutlich breiteren Einsatz von Biomarkern führen und die Entwicklung wirksamer und personalisiert anwendbarer Präventions- und Behandlungsansätze beschleunigen. Unser Beitrag bietet einen Überblick über den Stand der Entwicklung und beinhaltet Empfehlungen der Swiss Memory Clinics zum Einsatz von Liquormarkern im diagnostischen Prozess.

The Memory Centres of several Swiss hospitals have set up a national online registry for Alzheimer's research, called www.BHR-suisse.org. This type of registry already exists in the United States (www.brainhealthregistry.org/) and the Netherlands (https://hersenonderzoek.nl/). It contributes, as do these initiating sites, to the creation of a global database of research partnersLes centres de la mémoire de plusieurs hôpitaux suisses ont créé un Registre national suisse en ligne pour la recherche sur Alzheimer, intitulé www.bhr-suisse.org. Ce type de registre existe déjà aux États-Unis (www.brainhealthregistry.org/) et aux Pays-Bas (hersenonderzoek.nl/). Il contribue, au même titre que ces sites initiateurs, à constituer une base de données globale de partenaires de recherche

Description, Identifying blood-based physical biomarkers for detection of Alzheimer’s disease., Quantifying physical differences of protein aggregates implicated in Alzheimer’s disease (AD), in blood, could provide crucial information on disease stages. Here, red blood cells (RBCs) from 50 patients with neurocognitive complaints and 16 healthy individuals were profiled using an atomic force microscope (AFM). AFM measurements revealed patient age– and stage of neurocognitive disorder–dependent differences in size, shape, morphology, assembly, and prevalence of protein aggregates on RBCs, referred to as physical biomarkers. Crystals composed of fibrils were exclusively detected on RBCs for AD patients aged above 80 years. Fibril prevalence was negatively correlated with the cerebrospinal fluid (CSF) β-amyloid (Aβ) 42/40 ratio and was observed to be higher in the Aβ-positive patient category. Using a cutoff of ≥40% fibril prevalence, the CSF Aβ status was classified with 88% accuracy (sensitivity 100%, specificity 73%). The merits and challenges in integrating physical biomarkers in AD diagnosis are discussed.

While FDG-PET imaging of the brain for the differential diagnosis of dementia has been covered by the compulsory health insurance in Switzerland for more than a decade, beta-amyloid-PET just recently has been added to the catalogue of procedures that have been cleared for routine use, provided that a set of appropriate use criteria (AUC) be followed. To provide guidance to dementia care practitioners, the Swiss Society of Nuclear Medicine and the Swiss Memory Clinics jointly report a mini-review on beta-amyloid-PET and discuss the AUC set into effect by the Swiss Federal Office of Public Health, as well as their application and limitations.Die Beta-Amyloid-PET-Bildgebung des Gehirns ist mit der Zulassung für die klinische Routineanwendung durch Swissmedic von 3 EU-weit bereits länger kommerziell verfügbaren Amyloid-β-PET-Radiopharmaka nun auch in dem Nicht-EU-Land Schweiz allgemein verfügbar geworden. Seit dem 01.04.2020 unterliegt die Anwendung von Beta-Amyloid-PET der gesetzlichen Leistungspflicht durch die obligatorische Schweizer Krankenversicherung für die Differenzialdiagnose demenzieller Erkrankungen unter bestimmten Voraussetzungen.Im Namen der Schweizer Gesellschaft für Nuklearmedizin (SGNM) und der Schweizer Memory-Kliniken publiziert die AG Neuronuklearmedizin der SGNM die aktuell gültigen Anwendungskriterien im Rahmen der gesetzlichen Leistungspflicht in der Schweiz, um sowohl den verordnenden Demenzspezialisten, Gerontopsychiatern und Neurologen, als auch den durchführenden Nuklearmedizinern Handlungsempfehlung an die Hand zu geben und einen aktuellen Kurzüberblick über Möglichkeiten und Grenzen der Beta-Amyloid-Bildgebung im klinischen Alltagskontext zu geben.

Résumé. Le diagnostic précoce des atteintes cognitives, ressenties subjectivement ou rapportées par un tiers, est essentiel pour détecter des maladies neurodégénératives ou exclure des causes traitables telles que des pathologies de médecine interne, neurologiques ou psychiatriques. C’est la seule façon de garantir un traitement anticipé. Dans le cadre du projet 3.1 de la stratégie nationale en matière de démences 2014–2019 («Mise en place et extension d’un réseau de centres de compétences régionaux pour le diagnostic»), l’association Swiss Memory Clinics (SMC) s’est fixé pour objectif d’améliorer les normes de qualité en matière de diagnostic des démences et de soins de proximité dans ce domaine. Ces recommandations contiennent des directives d’ordre général sur le diagnostic et les différentes possibilités d’examens, et proposent des normes pour les procédures à appliquer. Elles expliquent en détail les différents éléments du diagnostic standard, tels que l’anamnèse, l’examen clinique, l’analyse de laboratoire, les tests neuropsychologiques et les procédures neuroradiologiques, et présentent des examens complémentaires pouvant alimenter les réflexions sur le diagnostic différentiel. Les principaux objectifs des recommandations SMC pour le diagnostic des démences sont les suivants: assurer l’accès à un diagnostic de haute qualité à un maximum de personnes atteintes, améliorer le diagnostic précoce de la démence, ainsi que proposer aux médecins de premier recours et aux collaborateurs de Memory Clinics un outil d’investigations diagnostiques utile.

The early diagnosis of subjectively perceived or externally anamnestically observed cognitive impairments is essential for proving neurodegenerative diseases or excluding treatable causes such as internal, neurological or psychiatric disorders. Only in this way is early treatment made possible. As part of the project 3.1 of the National Dementia Strategy 2014–2019 («Development and expansion of regional and networked centres of competence for diagnostics»), the association Swiss Memory Clinics (SMC) set itself the goal of developing quality standards for dementia clarification and improving the community-based care in this field. In these recommendations, general guidelines of diagnostics and individual examination possibilities are presented, and standards for the related processes are suggested. Individual areas such as anamnesis, clinical examination, laboratory examination, neuropsychological testing and neuroradiological procedures are discussed in detail as part of standard diagnostics, and supplementary examination methods for differential diagnosis considerations are portrayed. The most important goals of the SMC recommendations for the diagnosis of dementia are to give all those affected access to high-quality diagnostics, if possible, to improve early diagnosis of dementia and to offer the basic service providers and the employees of Memory Clinics a useful instrument for the clarification.

Zusammenfassung. Die Frühdiagnostik subjektiv wahrgenommener oder fremdanamnestisch beobachteter kognitiver Beeinträchtigungen ist essenziell, um neurodegenerative Erkrankungen nachzuweisen oder behandelbare Ursachen wie internistische, neurologische oder psychiatrische Störungen auszuschliessen. Nur dadurch wird eine frühzeitige Behandlung ermöglicht. Im Rahmen des Projekts 3.1 der Nationalen Demenzstrategie 2014–2019 («Auf- und Ausbau regionaler und vernetzter Kompetenzzentren für die Diagnostik») hat sich der Verein Swiss Memory Clinics (SMC) zum Ziel gesetzt, Qualitätsstandards für die Demenzabklärung zu entwickeln und die wohnortsnahe Versorgung in diesem Bereich zu verbessern. In den vorliegenden Empfehlungen werden allgemeine Richtlinien der Diagnostik und einzelne Untersuchungsmöglichkeiten vorgestellt, sowie Standards für die diesbezüglichen Abläufe vorgeschlagen. Einzelne Bereiche wie Anamneseerhebung, klinische Untersuchung, Laborparameter, neuropsychologische Testung und neuroradiologische Verfahren werden als Teil der Standarddiagnostik ausführlich diskutiert, ergänzende Untersuchungsmethoden für differenzialdiagnostische Überlegungen abgebildet. Die wichtigsten Ziele der SMC-Empfehlungen zur Diagnostik der Demenzerkrankungen sind, möglichst allen Betroffenen Zugang zu einer qualitativ hochstehenden Diagnostik zu ermöglichen, die Frühdiagnostik der Demenz zu verbessern und den Grundversorgern sowie den Mitarbeitenden der Memory Clinics ein nützliches Instrument für die Abklärung anzubieten.

Background Sudden onset of aphasia is usually due to stroke. Rapid diagnostic workup is necessary if reperfusion therapy is considered. Ictal aphasia is a rare condition but has to be excluded. Perfusion imaging may differentiate acute ischemia from other causes. In dubious cases, EEG is required but is time-consuming and laborious. We report a case where we considered de novo status epilepticus as a cause of aphasia without any lesion even at follow-up. A 62-year-old right-handed woman presented to the emergency department after nurses found her aphasic. She had undergone operative treatment of varicosis 3 days earlier. Apart from hypertension and obesity, no cardiovascular risk factors and no intake of medication other than paracetamol were reported. Neurological examination revealed global aphasia and right pronation in the upper extremity position test. Computed tomography with angiography and perfusion showed no abnormalities. Electroencephalogram performed after the CT scan showed left-sided slowing with high-voltage rhythmic 2/s delta waves but no clear ictal pattern. Intravenous lorazepam did improve EEG slightly, while aphasia did not change. Lumbar puncture was performed which likely excluded encephalitis. Magnetic resonance imaging showed cortical pathological diffusion imaging (restriction) and cortical hyperperfusion in the left parietal region. Intravenous anticonvulsant therapy under continuous EEG resolved neurological symptoms. The patient was kept on anticonvulsant therapy. Magnetic resonance imaging after 6 months showed no abnormalities along with no clinical abnormalities. Conclusions Magnetic resonance imaging findings were only subtle, and EEG was without clear ictal pattern, so the diagnosis of aphasic status remains with some uncertainty. However, status epilepticus can mimic stroke symptoms and has to be considered in patients with aphasia even when no previous stroke or structural lesions are detectable and EEG shows no epileptic discharges. Epileptic origin is favored when CT or MR imaging reveal no hypoperfusion. In this case, MRI was superior to CT in detecting hyperperfusion. This article is part of a Special Issue entitled “Status Epilepticus”.

Myokymia of the tongue is a very rare clinical condition and is much less common than facial or focal myokymia of the limbs. Radiation-induced delayed nerve damage is a well-known cause of myokymia, but other etiologies i.e. tumor recurrence should be considered as a differential diagnosis. We describe a case series of neurophysiologically proven unilateral tongue myokymia, which arose in two patients after radiotherapy of the neck/head and in one patient due to a space occupying meningioma of the cerebrospinal passage affecting the hypoglossal nerve. With this case series and a review of the literature we aim to raise clinical suspicion of tongue myokymia and highlight the clinical and electromyographic impact of myokymia in the diagnosis of malignancies and treatment-associated lesions of the hypoglossal nerve.

In seltenen Fällen liegen der erworbenen Schädigung des Nervus peronaeus mit konsekutiver Lähmung der innervierten Muskulatur intraneurale Ganglien zugrunde. Diese können im Gegensatz zu den druckbedingten Kompressionen chirurgisch im Rahmen der Erstdiagnose behandelt werden. Eine sorgfältige Anamnese in Kombination mit einer klinischen und elektrophysiologischen Untersuchung ist hierbei zielführend. Häufig passen einzelne Befunde nicht zu der klassischen Präsentation einer Druckläsion am Fibulakopf. Ergänzend stellt die hochfrequente dynamische Nervensonographie des N. peronaeus eine sensitive und kostengünstige bildgebende Untersuchungsmethode dar, um diese besondere Pathologie aufzudecken. Nachfolgend werden drei Patienten mit Paresen des N. peronaeus beschrieben, bei denen die Nervensonographie zur Diagnose von intraneuralen Ganglien des N. peronaeus führte.

Die Haufigkeit an Demenzerkrankungen nimmt im Rahmen der weltweit steigenden Lebenserwartung deutlich zu. Bisher gibt es keine medikamentose Prophylaxe. Etwa 50 % aller Demenzerkrankungen lassen sich auf beeinflussbare Risikofaktoren (Adipositas, Diabetes mellitus Typ 2 und arterielle Hypertonie im mittleren Lebensalter, Rauchen, korperliche Inaktivitat, Depression, niedriger Bildungsstand) zuruckfuhren. Allein die korperliche Inaktivitat wird in den Industrienationen fur etwa jede 5. Demenzerkrankung verantwortlich gemacht. Epidemiologische Studien in industrialisierten Landern zeigen, dass sich das Risiko einer Demenz (ohne atiologische Differenzierung) durch regelmasige korperliche Aktivitat um etwa ein Viertel, das einer Demenz vom Alzheimer-Typ um etwa ein Drittel und das fur ein leichtes kognitives Defizit um etwa die Halfte senken lasst. Erstaunlicherweise wurde bisher lediglich fur vaskulare Demenzen keine statistisch signifikante Risikoreduktion nachgewiesen.

153 mutations in the Cu/Zn superoxide dismutase (SOD1) gene have been claimed to be associated with amyotrophic lateral sclerosis (ALS) in familial and sporadic ALS in an autosomal dominant or autosomal recessive pattern with complete or reduced penetrance. The authors now report four ALS pedigrees from Finland, France, Germany and Sweden with either the D90A or E100K SOD1 mutations in some but not all affected members. After re-collecting DNA, the non-segregation of the SOD1 mutations with disease was confirmed by three independent laboratories using different PCR primers: while some of the affected patients carry SOD1 mutations, other affected family members have two wildtype/normal SOD1 genes. In addition, some unaffected members within the same families are carriers of SOD1 gene mutations. To exclude other known genetic causes, the authors ruled out mutations within the genes coding for VAPB, ANG, TDP43, FUS and DCTN1 in affected individuals in the four pedigrees.The authors find that the D90A and E100K SOD1 gene mutations found in some patients are not the exclusive cause of ALS in these pedigrees. Whether this is also the case for the other 151 SOD1 mutations reported in ALS pedigrees is unknown. The findings have consequences for genetic testing in clinical practice when diagnosing ALS and for genetic counselling in ALS. Some SOD1 mutations may be part of an oligo- or epigentic pattern of inheritance. Such a pattern of inheritance may model other oligo- or polygenetic traits responsible for other forms of ALS.

In selected cases acquired peroneal palsy is caused by intraneural ganglia. In contrast to the much more frequent "loco typico" lesion which is caused by external pressure, intraneural ganglia can be treated by microscopic nerve surgery as part of primary treatment strategy. A careful clinical history as well as a profound clinical and electrophysiological examination is required to disclose unusual findings. These are common in non-typical peroneal palsy. In this situation high resolution nerve sonography is a fast and sensitive method to detect intraneural ganglia. We report a case series of three patients with peroneal palsy caused by intraneural ganglia and give a review of the literature.In seltenen Fällen liegen der erworbenen Schädigung des Nervus peronaeus mit konsekutiver Lähmung der innervierten Muskulatur intraneurale Ganglien zugrunde. Diese können im Gegensatz zu den druckbedingten Kompressionen chirurgisch im Rahmen der Erstdiagnose behandelt werden. Eine sorgfältige Anamnese in Kombination mit einer klinischen und elektrophysiologischen Untersuchung ist hierbei zielführend. Häufig passen einzelne Befunde nicht zu der klassischen Präsentation einer Druckläsion am Fibulakopf. Ergänzend stellt die hochfrequente dynamische Nervensonographie des N. peronaeus eine sensitive und kostengünstige bildgebende Untersuchungsmethode dar, um diese besondere Pathologie aufzudecken. Nachfolgend werden drei Patienten mit Paresen des N. peronaeus beschrieben, bei denen die Nervensonographie zur Diagnose von intraneuralen Ganglien des N. peronaeus führte.Rarement la lésion du nerf péronier au niveau de la tête fibulaire suivie par une parésie des muscles innervés par ce nerf est causée par un ganglion appuyant sur les structures nerveuses. En ce cas une intervention chirurgicale peut souvant résondre le problème. Une anamnèse soigneusement menée, l'examen neurologique et des investigations neuromusculaires sont essentielles mais souvent atypiques comparés à la lésion péronière «loco typico». L'ultrasonographie à haute résolution est procédée facilement et plus sensible qu'une MRI. Nous allons décrire dans ce papier trois cas de parèsie du nerf péronier pour lesquels la diagnostic final a été posé par ultrasonographie.

Background Acute peroneal palsy is frequently caused by compression, trauma and surgery. Risk factors are weight loss, sitting with crossed legs, wrong positioning during surgery and diabetes mellitus. Focal demyelination is more frequent than axonal damage which in turn is related with an inferior prognosis. Rarely, acquired peroneal palsy is caused by intraneural ganglia. Methods We here report of three patients with untypical peroneal palsy caused by intraneural ganglia which were detected by nerve sonography (15 MHz Linear array transducer, GE Healthcare LOGIQ E9 Ultrasound). Results and conclusions A careful clinical history as well as a profound clinical and electrophysiological examination is required to disclose unusual findings. These are common in non-typical peroneal palsy. In this situation high resolution nerve sonography is a fast and sensitive method to detect intraneural ganglia. In contrast to the much more frequent “loco typico” lesion intraneural ganglia can be treated by microscopic nerve surgery as part of primary treatment strategy. Hence, detection of intraneural ganglia is meaningful for prognosis and guides therapy.

The interest on the impact of physical activity on cognition is growing due to increasing frequencies of dementia caused by the demographic changes. The present chapter analyses whether there is any impact of short-term or long-standing physical activity on cognition and, if any, in which way and to what extent. A meta-analytical synthesis of prospective cohort studies on the development of cognitive decline in elderly individuals depending on their previous amount of regular physical activity has been performed. The literature review indicates that both short-term as well as regular long-standing physical activity have a positive influence on cognitive functions in several aspects. However, the effects are small or even questionable in people with pre-existing cognitive impairment. The meta-analysis proves that regular physical activity – especially on an aerobic basis – lowers the risk of subsequent cognitive decline by about 25 % (undifferentiated dementia), 37 % (dementia of Alzheimer type), and 46 % (mild cognitive impairment). Merely the risk of vascular dementia has not been proven to be reduced significantly. Therefore, preventing cognitive decline may be another motivation for being regularly physically active – besides the numerous other constitutional effects.

We screened 217 patients from Germany (n = 213), Austria (n = 2) and Switzerland (n = 2) with a positive family history for amyotrophic lateral sclerosis (ALS) for mutations in the copper/zinc superoxide dismutase (SOD1) gene. We found that 13% of the families tested carried mutations. By analyzing inheritance, we detected a clear-cut co-segregation in 5 of the 28 families; however, in two families with an established mutation, co-segregation was absent. In Germany, the R115G mutation is comparatively frequent and exhibits a specific aggressive phenotype. The L144F mutation, which is the most prevalent mutation in the Balkan countries, and the D90A mutation which is the most frequent SOD1 mutation globally, seem to be the second most common disease-causing mutations in Germany.

Summary Background Stroke is one of the leading causes of acquired epilepsy in adults. An instrument to predict whether people are at high risk of developing post-stroke seizures is not available. We aimed to develop and validate a prognostic model of late (>7 days) seizures after ischaemic stroke. Methods In this multivariable prediction model development and validation study, we developed the SeLECT score based on five clinical predictors in 1200 participants who had an ischaemic stroke in Switzerland using backward elimination of a multivariable Cox proportional hazards model. We externally validated this score in 1169 participants from three independent international cohorts in Austria, Germany, and Italy, and assessed its performance with the concordance statistic and calibration plots. Findings Data were complete for 99·2% of the predictors (99·2% for Switzerland, 100% for Austria, 97% for Germany, and 99·7% for Italy) and 100% of the outcome parameters. Overall, the risk of late seizures was 4% (95% CI 4–5) 1 year after stroke and 8% (6–9) 5 years after stroke. The final model included five variables and was named SeLECT on the basis of the first letters of the included parameters (severity of stroke, large-artery atherosclerotic aetiology, early seizures, cortical involvement, and territory of middle cerebral artery involvement). The lowest SeLECT value (0 points) was associated with a 0·7% (95% CI 0·4–1·0) risk of late seizures within 1 year after stroke (1·3% [95% CI 0·7–1·8] within 5 years), whereas the highest value (9 points) predicted a 63% (42–77) risk of late seizures within 1 year (83% [62–93] within 5 years). The model had an overall concordance statistic of 0·77 (95% CI 0·71–0·82) in the validation cohorts. Calibration plots indicated high agreement of predicted and observed outcomes. Interpretation This easily applied instrument was shown to be a good predictor of the risk of late seizures after stroke in three external validation cohorts and is freely available as a smartphone app. The SeLECT score has the potential to identify individuals at high risk of seizures and is a step towards more personalised medicine. It can inform the selection of an enriched population for antiepileptogenic treatment trials and will guide the recruitment for biomarker studies of epileptogenesis. Funding None.