α-synuclein antibody 5G4 identifies idiopathic REM-sleep behavior disorder in abdominal skin biopsies.

INTRODUCTION: Idiopathic REM-sleep behavior disorder (iRBD) is considered the most specific prodromal marker of Parkinson's disease (PD). With the need to improve early detection of prodromal α-synucleinopathies, several methods to identify peripheral α-synuclein (α-syn) pathology have been exploited in manifest and prodromal PD with varying diagnostic accuracy. Recently, a disease specific 5G4 antibody has been evaluated in skin biopsies of manifest PD patients. The aim of our study was to analyze the 5G4 α-syn immunoreactivity in skin biopsies of deeply phenotyped subjects with iRBD and controls. METHODS: The study cohort consisted of 28 patients with PD, 24 subjects with iRBD and 27 healthy controls, recruited from the CEGEMOD, PDBIOM and PARCAS cohorts. All subjects were deeply phenotyped and assessed for prodromal PD (pPD) probability based on MDS research criteria. Abdominal skin punch biopsies were processed and stained using a conformation specific 5G4 α-syn antibody as well as axonal markers SMI-31 and S100. RESULTS: 5G4-positivity was identified in 23/28 PD patients, 20/24 iRBD subjects and 8/27 healthy controls. Compared to healthy controls, sensitivity and specificity reached 83.33 % and 70.37 % for iRBD; and 82.14 % and 70.37 % for PD, respectively. 5G4-positivity rate in our study was irrespective of the calculated pPD probability of iRBD subjects. CONCLUSIONS: This work establishes the diagnostic yield of conformation specific 5G4 α-syn antibody testing in skin biopsies of subjects with pPD, specifically iRBD. The diagnostic accuracy for this method seems to be similar for both manifest and prodromal PD and is not dependent on the pPD probability ratios.

Predictors of Good Clinical Outcome After Endovascular Treatment for Acute Ischemic Stroke due to Tandem Lesion in Anterior Circulation: Results from the ASCENT Study.

PURPOSE: Endovascular treatment (EVT) of tandem lesion (TL) in anterior circulation (AC) acute ischemic stroke (AIS) represents still a clinical challenge. We aimed to evaluate selected factors related to EVT and assess other possible predictors of good clinical outcome besides the generally known ones. METHODS: AIS patients with TL in AC treated with EVT were enrolled in the multicenter retrospective ASCENT study. A good three-month clinical outcome was scored as 0-2 points in modified Rankin Scale (mRS) and achieved recanalization using the TICI scale. Symptomatic intracerebral hemorrhage (SICH) was assessed using the SITS-MOST criteria. Logistic regression analysis was used for the assessment of possible predictors of mRS 0-2 with adjustment for potential confounders. RESULTS: In total, 300 (68.7% males, mean age 67.3 ± 10.2 years) patients with median of admission NIHSS 17 were analyzed. Recanalization (TICI 2b-3) was achieved in 290 (96.7%) patients and 176 (58.7%) had mRS 0-2. Besides the age, admission NIHSS and SICH, admission glycemia (p = 0.005, OR: 0.884) the stent patency within the first 30 days after EVT (p = 0.0003, OR: 0.219), dual antiplatelet therapy (DAPT) started within 12 h after EVT (p < 0.0001, OR: 5.006) and statin therapy started within 24 h after stenting (p < 0.0001, OR: 5.558) were found as other predictors. CONCLUSION: Admission glycemia, start of DAPT within 12 h and statin therapy within 24 h after EVT, and stent patency within the first 30 days after EVT were found as other predictors of good three-month clinical outcome in AIS patients treated with EVT for TL.

Steroid responsive encephalopathy associated with autoimmune thyroiditis as a cause of acuteencephalopathy.

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), known as Hashimoto's encephalopathy (HE), represents a heterogeneous group of neurological and neuropsychiatric symptoms associated with a presence of antithyroid antibodies in case of other causes of encephalopathy were excluded. Clinical symptoms most commonly includes acute onset of encephalopathy, behaviour changes and cognitive dysfunction, epileptic seizures as well as cerebellar and extrapyramidal symptoms. Corticoids provides rapid and sustained therapeutic benefit in most patients and only a few patients require other immunosuppressive therapy such as plasmapheresis, intravenous immunoglobulins, or others. We present the cases of two patients with acute onset of encephalopathy, status epilepticus based on SREAT, with rapid improvement after steroid treatment.

Concomitant occurrence of Wernicke's encephalopathy and sarcopenia due to vitamin D depletion in patients with alcohol use disorder: a case report.

Individuals with alcohol use disorder frequently suffer from vitamin D deficiency, in addition to deficiencies in vitamins B12, folic acid and B1. This is due to inadequate dietary intake and behavioural changes. Each of these deficiencies results in different clinical symptoms. Subacute spinal cord degeneration, together with radicular and sensorimotor peripheral neuropathy, arises from B12 vitamin and folic acid deficiencies. B1 vitamin deficiency leads to Wernicke's encephalopathy, which can include the classical triad of symptoms (i.e. cognitive changes, ataxia and ophthalmoplegia). Sarcopenia is a consequence of a long-term deficiency of vitamin D. This current case report describes a 43-year-old female patient with alcohol use disorder who complained of dizziness, postural disturbance and episodes of intermittent paraesthesia. She was subsequently shown to have concomitant Wernicke's encephalopathy and sarcopenia due to vitamin D deficiency. This case report presents the diagnostic process undertaken to exclude conditions related to ataxia and paraparesis other than vitamins D and B1 deficiencies. It also emphasizes the importance of concomitant replacement of the depleted vitamins because the vitamin deficiency may occur simultaneously, which causes the accompanying manifestations of several clinical syndromes.

Annual Plasma Neurofilament Dynamics Is a Sensitive Biomarker of Disease Activity in Patients with Multiple Sclerosis.

Background and Objectives: Neurofilament light chain (NfL) is a sensitive biomarker of neuroaxonal damage. This study aimed to assess the relationship between the annual change in plasma NfL (pNfL) and disease activity in the past year, as defined by the concept no evidence of disease activity (NEDA) in a cohort of multiple sclerosis (MS) patients. Materials and Methods: Levels of pNfL (SIMOA) were examined in 141 MS patients and analyzed in relationship to the NEDA-3 status (absence of relapse, disability worsening, and MRI activity) and NEDA-4 (NEDA-3 extended by brain volume loss ≤ 0.4%) during the last 12 months. Patients were divided into two groups: annual pNfL change with an increase of less than 10% (group 1), and pNfL increases of more than 10% (group 2). Results: The mean age of the study participants (n = 141, 61% females) was 42.33 years (SD, 10.17), and the median disability score was 4.0 (3.5-5.0). The ROC analysis showed that a pNfL annual change ≥ 10% correlates with the absence of the NEDA-3 status (p < 0.001; AUC: 0.92), and the absence of the NEDA-4 status (p < 0.001; AUC: 0.839). Conclusions: Annual plasma NfL increases of more than 10% appear to be a useful tool for assessing disease activity in treated MS patients.

Early versus Later Anticoagulation for Stroke with Atrial Fibrillation.

BACKGROUND: The effect of early as compared with later initiation of direct oral anticoagulants (DOACs) in persons with atrial fibrillation who have had an acute ischemic stroke is unclear. METHODS: We performed an investigator-initiated, open-label trial at 103 sites in 15 countries. Participants were randomly assigned in a 1:1 ratio to early anticoagulation (within 48 hours after a minor or moderate stroke or on day 6 or 7 after a major stroke) or later anticoagulation (day 3 or 4 after a minor stroke, day 6 or 7 after a moderate stroke, or day 12, 13, or 14 after a major stroke). Assessors were unaware of the trial-group assignments. The primary outcome was a composite of recurrent ischemic stroke, systemic embolism, major extracranial bleeding, symptomatic intracranial hemorrhage, or vascular death within 30 days after randomization. Secondary outcomes included the components of the composite primary outcome at 30 and 90 days. RESULTS: Of 2013 participants (37% with minor stroke, 40% with moderate stroke, and 23% with major stroke), 1006 were assigned to early anticoagulation and 1007 to later anticoagulation. A primary-outcome event occurred in 29 participants (2.9%) in the early-treatment group and 41 participants (4.1%) in the later-treatment group (risk difference, -1.18 percentage points; 95% confidence interval [CI], -2.84 to 0.47) by 30 days. Recurrent ischemic stroke occurred in 14 participants (1.4%) in the early-treatment group and 25 participants (2.5%) in the later-treatment group (odds ratio, 0.57; 95% CI, 0.29 to 1.07) by 30 days and in 18 participants (1.9%) and 30 participants (3.1%), respectively, by 90 days (odds ratio, 0.60; 95% CI, 0.33 to 1.06). Symptomatic intracranial hemorrhage occurred in 2 participants (0.2%) in both groups by 30 days. CONCLUSIONS: In this trial, the incidence of recurrent ischemic stroke, systemic embolism, major extracranial bleeding, symptomatic intracranial hemorrhage, or vascular death at 30 days was estimated to range from 2.8 percentage points lower to 0.5 percentage points higher (based on the 95% confidence interval) with early than with later use of DOACs. (Funded by the Swiss National Science Foundation and others; ELAN ClinicalTrials.gov number, NCT03148457.).

Sex differences in outcome after carotid revascularization in symptomatic and asymptomatic carotid artery stenosis.

OBJECTIVE: Sex differences regarding the safety and efficacy of carotid revascularization in carotid artery stenosis have been addressed in several studies with conflicting results. Moreover, women are underrepresented in clinical trials, leading to limited conclusions regarding the safety and efficacy of acute stroke treatments. METHODS: A systematic review and meta-analysis was performed by literature search including four databases from January 1985 to December 2021. Sex differences in the efficacy and safety of revascularization procedures, including carotid endarterectomy (CEA) and carotid artery stenting (CAS), for symptomatic and asymptomatic carotid artery stenoses were analyzed. RESULTS: Regarding CEA in symptomatic carotid artery stenosis, the stroke risk in men (3.6%) and women (3.9%) based on 99,495 patients (30 studies) did not differ (P = .16). There was also no difference in the stroke risk by different time frames up to 10 years. Compared with men, women treated with CEA had a significantly higher stroke or death rate at 4 months (2 studies, 2565 patients; 7.2% vs 5.0%; odds ratio [OR], 1.49; 95% confidence interval [CI], 1.04-2.12; I2 = 0%; P = .03), and a significantly higher rate of restenosis (1 study, 615; 17.2% vs 6.7%; OR, 2.81; 95% CI, 1.66-4.75; P = .0001). For CAS in symptomatic artery stenosis, data showed a non-significant tendency toward higher peri-procedural stroke in women, whereas for asymptomatic carotid artery stenosis, data based on 332,344 patients showed that women (compared with men) after CEA had similar rates of stroke, stroke or death, and the composite outcome stroke/death/myocardial infarction. The rate of restenosis at 1 year was significantly higher in women compared with men (1 study, 372 patients; 10.8% vs 3.2%; OR, 3.71; 95% CI, 1.49-9.2; P = .005). Furthermore, CAS in asymptomatic patients was associated with low risk of a postprocedural stroke in both sexes, but a significantly higher risk of in-hospital myocardial infarction in women than men (8445 patients, 1.2% vs 0.6%; OR, 2.01; 95% CI, 1.23-3.28; I2 = 0%; P = .005). CONCLUSIONS: A few sex-differences in short-term outcomes after carotid revascularization for symptomatic and asymptomatic carotid artery stenosis were found, although there were no significant differences in the overall stroke. This indicates a need for larger multicenter prospective studies to evaluate these sex-specific differences. More women, including those aged over 80 years, need to be enrolled in randomized controlled trials, to better understand if sex differences exist and to tailor carotid revascularization accordingly.

Importance of Retesting for the Final Diagnosis of Post-Stroke Cognitive Impairment.

Background and Objectives: Post-stroke cognitive impairment (PSCI) has been defined as all problems in cognitive function that occur following a stroke. Studies published thus far on the prevalence of PSCI and post-stroke dementia (PSD) have shown conflicting estimates. The aim of this study was screening for cognitive impairment (CogI) in patients with an ischaemic stroke and finding the relationship between CogI (and its changes) and cardiovascular risk factors and imaging procedures-CT/MRI. Materials and Methods: We prospectively included patients with an ischaemic stroke admitted in the period from October 2019 to May 2022. In this period, 1328 patients were admitted, 305 of whom met the established inclusion criteria and underwent an examination of cognitive functions using the Montreal Cognitive Assessment (MoCA). Of these, 50 patients appeared for the control examination after 6 months. Results: In the retested group, CogI at discharge was diagnosed in 37 patients (74%). In follow-up testing after 6 months, CogI was present in 30 patients (60%). Only arterial hypertension (OR: 15; 95% CI; Pearson r: 0.001), lower education level (less than 13 years) (OR: 9.7; 95% CI 2.0-48.5; Pearson r: 0.002), and higher age were significantly associated with CogI after stroke. Conclusions: We established the prevalence of CogI and its course after 6 months in a well-defined group of patients after a mild ischaemic stroke (mean NIHSS: 2 and mean mRS: 1 at the discharge). Our results show that the prevalence of CogI after an ischaemic stroke at discharge is relatively high (74%), and it tends to be a spontaneous reduction. Cognitive functions were changed in 35% of patients. The definition of PSCI was completed in only 24% of individuals. Only an examination several months after a stroke can give us more accurate information about the true prevalence of persistent CogI after a stroke.

Heart Rate Variability in evaluation of autonomic dysfunction in idiopathic REM-sleep behaviour disorder.

INTRODUCTION: Nearly 80% of people diagnosed with idiopathic REM sleep behaviour disorder (iRBD) via video-polysomnography (v-PSG) are expected to be in the prodromal stage of an alpha-synucleinopathy. Signs of autonomic dysfunction can appear earlier than motor or cognitive alpha-synucleinopathy symptoms. Heart Rate Variability (HRV) can potentially be an objective measurement of autonomic dysfunction, and furthermore can be obtained directly from v-PSG. OBJECTIVES: The aim of this study was to evaluate dysautonomia in iRBD subjects using HRV obtained during different sleep stages and wakefulness from v-PSG. MATERIAL AND METHODS: Subjects positively screened by an RBD screening questionnaire (RBD-SQ) underwent v-PSG to diagnose RBD. HRV obtained from v-PSG recordings was correlated to dysautonomia evaluated from a Non-Motor Symptoms Scale (NMSS) questionnaire. Optimal cut-off values of HRV parameters to predict dysautonomia were calculated using receiver operating characteristics (ROC) - area under the curve (AUC) analysis. The effect of confounder variables was predicted with binomial logistic regression and multiple regression analyses. RESULTS: Out of 72 positively screened subjects, 29 subjects were diagnosed as iRBD (mean age 66 ± 7.7 years) by v-PSG. Eighty-three per cent of the iRBD subjects in our cohort were at the time of diagnosis classified as having possible or probable prodromal Parkinson's Disease (pPD) compared to zero subjects being positively screened in the control group. The iRBD-positive subjects showed significant inverse correlations of NMSS score, particularly to log low-frequency (LF) component of HRV during wakefulness: r = -0.59 (p = 0.001). Based on ROC analysis and correlation between NMSS score, log LF during wakefulness (AUC 0.74, cut-off 4.69, sensitivity 91.7%, specificity 64.7%, p = 0.028) was considered as the most accurate predictor of dysautonomia in the iRBD group. Apnoea-hypopnoea index (AHI) negatively predicted dysautonomia in the iRBD group. None of the HRV components was able to predict the presence of iRBD in the full cohort. Age, gender, and PSG variables were significant confounders of HRV prediction. CONCLUSIONS: The presented study did not confirm the possibility of using HRV from v-PSG records of patients with iRBD to predict dysautonomia expressed by questionnaire methods. This is probably due to several confounding factors capable of influencing HRV in such a cohort.

How stroke care has changed in Slovakia in the last 5 years.

Introduction: Although stroke patients in Slovakia had been treated according to European recommendations, no network of primary and comprehensive stroke centers had been officially established; the ESO recommended quality parameters had not been fulfilled. Therefore, the Slovak Stroke Society decided to change the stroke management concept and introduced mandatory evaluation of quality parameters. This article focuses on key success factors of the change in stroke management in Slovakia and presents the 5-year results and perspectives for the future. Material and methods: We processed data from the stroke register at the National Health Information Center, which is mandatory in Slovakia for all hospitals designated as primary and secondary stroke care centers. Results: Since 2016, we have started to change stroke management. New National Guideline for Stroke Care was prepared in 2017 and published in 2018 as a Recommendation of the Ministry of Health of the Slovak Republic. The recommendation included pre-hospital as well as in-hospital stroke care, a network of primary stroke centers (hospitals administering intravenous thrombolysis - 37), and secondary stroke centers (hospitals treating with intravenous thrombolysis + endovascular treatment (ET) - 6). A stroke priority was instituted, having equally high priority as myocardial infarction. More efficient in-hospital workflow and pre-hospital patient triage shortened the time to treatment. Prenotification became mandatory in all hospitals. Non-contrast CT, and CT angiography is mandatory in all hospitals. In patients with suspected proximal large-vessel occlusion the EMS stays at the CT facility in primary stroke centers until the CT angiography is finished. If LVO is confirmed, the patient is transported to an EVT secondary stroke center by the same EMS. From 2019 all secondary stroke centers offer endovascular thrombectomy in a 24/7/365 system. We consider the introduction of quality control one of the most critical steps in stroke management. The result of these activities is 25.2% of patients treated with IVT and 10.2% by endovascular treatment, and median DNT 30 min. Number of patients screened for dysphagia increased from 26.4% in 2019 to 85.9% in 2020. In the most of the hospitals the proportion of ischemic stroke patients discharged with antiplatelets and in case of AF with anticoagulants was >85%. Discussion: Our results indicate that it is possible to change stroke management at a single hospital and national level. For continuous and further improvement, regular quality monitoring is necessary; therefore, the results of stroke hospital management are presented regularly once a year at national and international level. Collaboration with the "Second for Life" patient organization is very important for the "time is brain" campaign in Slovakia. Conclusion: Due to the change in stroke management over the last 5 years, we have reduced the time for acute stroke treatment and improved the proportion of patients with acute treatment, and in this area, we have achieved and exceeded the goals of the Stroke Action Plan for Europe for 2018-2030. Nevertheless, we still have many insufficiencies in stroke rehabilitation and post-stroke nursing that need to be addressed.

Early versus Late initiation of direct oral Anticoagulants in post-ischaemic stroke patients with atrial fibrillatioN (ELAN): Protocol for an international, multicentre, randomised-controlled, two-arm, open, assessor-blinded trial.

Rationale: Direct oral anticoagulants (DOAC) are highly effective in preventing ischaemic strokes in people with atrial fibrillation (AF). However, it is unclear how soon they should be started after acute ischaemic stroke (AIS). Early initiation may reduce early risk of recurrence but might increase the risk of haemorrhagic complications. Aim: To estimate the safety and efficacy of early initiation of DOACs compared to late guideline-based initiation in people with AIS related to AF. Methods and design: An international, multicentre, randomised (1:1) controlled, two-arm, open, assessor-blinded trial is being conducted. Early treatment is defined as DOAC initiation within 48 h of a minor or moderate stroke, or at day 6-7 following major stroke. Late treatment is defined as DOAC initiation after day 3-4 following minor stroke, after day 6-7 following moderate stroke and after day 12-14 following major stroke. Severity of stroke is defined according to imaging assessment of infarct size. Sample size: ELAN will randomise 2000 participants 1:1 to early versus late initiation of DOACs. This assumes a risk difference of 0.5% favouring the early arm, allowing an upper limit of the 95% confidence interval up to 1.5% based on the Miettinen & Nurminen formula. Outcomes: The primary outcome is a composite of symptomatic intracranial haemorrhage, major extracranial bleeding, recurrent ischaemic stroke, systemic embolism or vascular death at 30 ± 3 days after randomisation. Secondary outcomes include the individual components of the primary outcome at 30 ± 3 and 90 ± 7 days and functional status at 90 ± 7 days. Discussion: ELAN will estimate whether there is a clinically important difference in safety and efficacy outcomes following early anticoagulation with a DOAC compared to late guideline-based treatment in neuroimaging-selected people with an AIS due to AF.

Aspirin for Primary Stroke Prevention; Evidence for a Differential Effect in Men and Women.

Background: The use of aspirin for primary prevention of cardiovascular events in men and women remains controversial. Our study aimed to investigate the role of aspirin in primary stroke prevention in men and women and the effect of aspirin on risk of ischemic stroke in patients with covert cerebral small vessel disease (ccSVD). Methods: We performed systematic searches of the PubMed, and Cochrane Library databases, covering the period from the inception of each database to May 2021. The incidence of any ischemic stroke (IS) or hemorrhagic stroke (HS) was the main outcome. The incidence of stroke overall, both ischemic (IS) and hemorrhagic (HS), was the main outcome. Results: From 531 abstracts, 11 randomized control trials which assessed primary prevention of cardiovascular events in men and women were included. Only one study assessed the risk of aspirin in people with ccSVD. In women, there was significant decrease in the risk of stroke (OR 0.85 [95% CI 0.73, 0.99], p = 0.03) and IS (OR 0.76 [0.63, 0.93], p = 0.008) with aspirin compared to placebo while no increase in the risk of HS was found (OR 1.78 [0.61, 5.19], p = 0.29). In men, aspirin did not affect the risk of stroke (OR 1.13 [0.97, 1.31], p = 0.12) and IS (OR 0.94 [0.67, 1.32], p = 0.72) but increased the risk of HS with borderline statistical significance (OR 1.99 [0.99, 4.03], p = 0.05) compared to placebo. Aspirin significantly increased major bleedings in both sexes (p < 0.05). We found no evidence to support the use of aspirin in patients with ccSVD. Conclusion: Our meta-analysis suggests aspirin is effective in primary prevention of stroke and IS in women with no clear increased risk of HS. However, it was associated with an overall increased risk of bleeding. Aspirin is not recommended in ccSVD.

European Stroke Organisation guidelines on stroke in women: Management of menopause, pregnancy and postpartum.

Pregnancy, postpartum and menopause are regarded as periods women are more vulnerable to ischaemic events. There are conflicting results regarding stroke risk and hormone replacement therapy (HRT) during menopause. Stroke in pregnancy is generally increasing with serious consequences for mother and child; therefore, recommendations for acute treatment with intravenous thrombolysis (IVT) and/or mechanical thrombectomy (MT) are needed. The aim of this guideline is to support and guide clinicians in treatment decisions in stroke in women. Following the "Grading of Recommendations and Assessment, Development and Evaluation (GRADE)" approach, the guidelines were developed according to the European Stroke Organisation (ESO) Standard Operating Procedure. Systematic reviews and metanalyses were performed. Based on available evidence, recommendations were provided. Where there was a lack of evidence, an expert consensus statement was given. Low quality of evidence was found to suggest against the use of HRT to reduce the risk of stroke (ischaemic and haemorrhagic) in postmenopausal women. No data was available on the outcome of women with stroke when treated with HRT. No sufficient evidence was found to provide recommendations for treatment with IVT or MT during pregnancy, postpartum and menstruation. The majority of members suggested that pregnant women can be treated with IVT after assessing the benefit/risk profile on an individual basis, all members suggested treatment with IVT during postpartum and menstruation. All members suggested treatment with MT during pregnancy. The guidelines highlight the need to identify evidence for stroke prevention and acute treatment in women in more vulnerable periods of their lifetime to generate reliable data for future guidelines.

Did the severity of the COVID-19 outbreak affect the quality of acute stroke care? (Data from across the country).

Introduction: The aim of our study was to determine whether the severity of the COVID-19 pandemic affected the quality of acute care of stroke. Methods: Data from the stroke register at the National Health Information Centre were analysed. Clinical data from two time periods (the first wave: March-April 2020; the second wave: October-November 2020) were compared using an independent sample t-test and the Wilcoxon-Mann-Whitney two sample rank-sum test. Results: The total number of patients admitted with stroke during the second wave of COVID-19 was 1848, versus 1698 in the first wave. The proportion of patients treated by IVT was similar in both waves (275 (20.7%) vs 333 (22.1%), p = 1, difference in location: -0,0003, 95% CI: -5.0 to 5.95). We found no difference in time from the onset of symptoms to treatment (median = 130 min in both waves, p = 0.52, difference in location: 3.99, 95% CI: -6.0 to 14.0), nor in the door-to-needle time (median = 29 vs 30 min, p = 0.08, difference in location: -2.99, 95% CI: -5.0 to 0.008) between the first and the second waves of the pandemic. We found no difference in NIHSS (median = 3 vs 4, p = 0.51, difference in location: 0.00007, 95% CI: -0.9 to 0.000006) and mRS (median = 3 in both waves, p = 0.60, difference in location: -0.00004, 95% CI: -0.00004 to 0.00003) at discharge from hospital between the two periods. Conclusion: The severity of the COVID-19 outbreak did not affect the quality of acute stroke care in Slovakia.

Lower-limb progressive paraparesis management and diagnosis overview in a pregnant woman with vertebral haemangioma.

Vertebral haemangioma is a benign vascular tumour mostly seen in the thoracic region of the spine. Spinal haemangiomas are usually asymptomatic and are discovered incidentally. In a few patients, however, aggressive vertebral haemangiomas can cause local pain, radicular pain or neurologic deficits, which result from neural compression. The aetiology of the origin is unclear and is probably multifactorial. Hormonal and biological changes in pregnant women can lead to accelerated vascular growth of haemangioma. In our report, we present the case of a pregnant patient who was diagnosed with an aggressive vertebral haemangioma that further led to progressive paraparesis. We had to take the fact that she was pregnant into account in the diagnostic procedure, in the choice of examination method and also in the method of therapy. The goal of this case report is threefold: (1) provide an overview of the possible methods of management, specifically imaging, which will aid in diagnosis and based on that, (2) determining the appropriate therapy and (3) review the risks and benefits of each will be presented when choosing individual approaches.

Prevalence of Fabry Disease among Patients with Parkinson's Disease.

BACKGROUND: An increased prevalence of Parkinson's disease (PD) disease has been previously reported in subjects with Fabry disease (FD) carrying alpha-galactosidase (GLA) mutations and their first-line relatives. Moreover, decreased alpha-galactosidase A (AGLA) enzymatic activity has been reported among cases with PD compared to controls. OBJECTIVE: The aim of our study was to determine the prevalence of FD among patients with PD. METHODS: We recruited 236 consecutive patients with PD from February 2018 to December 2020. Clinical and sociodemographic data, including the MDS-UPDRS-III scores and HY stage (the Hoehn and Yahr scale), were collected, and in-depth phenotyping was performed in subjects with identified GLA variants. A multistep approach, including standard determination of AGLA activity and LysoGb3 in males, and next-generation based GLA sequencing in all females and males with abnormal AGLA levels was performed in a routine diagnostic setting. RESULTS: The mean age of our patients was 68.9 ± 8.9 years, 130 were men (55.1%), and the mean disease duration was 7.77 ± 5.35 years. Among 130 men, AGLA levels were low in 20 patients (15%), and subsequent Lyso-Gb3 testing showed values within the reference range for all tested subjects. In 126 subsequently genetically tested patients, four heterozygous p.(Asp313Tyr) GLA variants (3.2%, MAF 0.016) were identified; all were females. None of the 4 GLA variant carriers identified had any clinical manifestation suggestive of FD. CONCLUSIONS: The results of this study suggest a possible relationship between FD and PD in a small proportion of cases. Nevertheless, the GLA variant found in our cohort is classified as a variant of unknown significance. Therefore, its pathogenic causative role in the context of PD needs further elucidation, and these findings should be interpreted with caution.

Excessive supraventricular activity and risk of atrial fibrillation in patients with cryptogenic ischaemic stroke.

INTRODUCTION: Atrial fibrillation (AF) is one of the leading causes of ischaemic stroke. However, screening for AF is often time-consuming in clinical practice. Therefore, the determination of an appropriate marker to detect the presence of AF would improve the diagnostic process. OBJECTIVE: The aim of the current study was to evaluate the efficacy of prolonged and early inpatient event Holter monitoring in the detection of AF in patients with ESUS-related cryptogenic ischaemic stroke (CIS), and to determine the possible relationship between excessive supraventricular activity and AF detection. MATERIAL AND METHODS: All consecutive patients with documented cerebral or cerebellar infarction were included. The diagnostic work-up included brain neuroimaging (CT/MRI), ultrasound of the carotid and vertebral arteries, admission ECG followed by 24 hours of Holter monitoring, and transthoracic echocardiography. The 24-hour Holter ECG was analysed, and supraventricular ectopic activity (supraventricular extrasystoles, runs and pairs of supraventricular extrasystoles) was recorded in all patients. If these examinations did not reveal the cause of ischaemic stroke, the patients underwent subsequent prlonged 14-day event Holter recorder monitoring. RESULTS: We included 48 patients (mean age 69.9 ± 8.5 years, 60.4% men) who had been diagnosed with CIS. Of these 48 patients, atrial fibrillation was detected in seven (14.6%) during the prolonged 14-day Holter event monitoring. Patients with newly diagnosed atrial fibrillation had a higher burden of supraventricular ectopic activity. The number of supraventricular extrasystoles (SVES) per hour, as well as the number of SV pairs and SV runs, was significantly higher in patients with new onset AF (p < 0.022; p < 0.043; p < 0.022). CONCLUSIONS: In our study, we confirmed that prolonged ECG event Holter monitoring in patients with CIS-ESUS subtype led to a higher rate of AF detection. Likewise, frequent supraventricular ectopic activity predicted the development of AF.

Cognitive Event-Related Potentials-The P300 Wave Is a Prognostic Factor of Long-Term Disability Progression in Patients With Multiple Sclerosis.

PURPOSE: Multiple sclerosis (MS) is a chronic disorder with a variable course. The aim of our study was to find out whether cognitive event-related potentials are prognostic for patient disability at the 15-year follow-up. METHODS: In the observed cohort of patients with MS, we examined the event-related potentials at baseline (2003). Functional status (Expanded Disability Status Scale score) was then assessed 15 years later, and the prognostic model was developed using binary logistic regression analysis. The independent variables included demographic (age, sex, and education), clinical (disability in 2003), radiologic (MRI lesion load), and event-related potentials parameters. The prognostic accuracy of the proposed model was evaluated by calculating the area under the receiver-operating characteristics curve. RESULTS: The study sample consisted of 85 patients with MS. The mean age was 35.5 (SD, 11.2) years, and the median disability score was 3.0 (1-7) in 2003 and 5.0 (1.5-9.5) in 2018. The significant prognostic factors of poor Expanded Disability Status Scale are higher baseline Expanded Disability Status Scale, longer MS duration, and prolonged P300 latency. The sensitivity and specificity of the cutoff at 5.0 for the disability score were 94% and 89%, respectively, with the area under the receiver-operating characteristics curve 0.94 (95% confidence interval, 0.889-0.984; P < 0.001). CONCLUSIONS: The results show that out of event-related potentials, the P300 wave latency is a prognostic of long-term disability progression in patients with MS.

Associations between neurofilament light chain levels, disease activity and brain atrophy in progressive multiple sclerosis.

BACKGROUND: Neurofilament light chain is a promising biomarker of disease activity and treatment response in relapsing-remitting multiple sclerosis (MS). Its role in progressive MS is less clear. AIM: The aim of the study was to assess the relationship between plasma neurofilament light chain (pNfL) and disease activity as defined by the concept NEDA-3 (No Evident Disease Activity), and brain volumetry, in a cohort of patients with the progressive disease form (PMS). METHODS: Levels of pNfL (SIMOA technology) were examined in 52 PMS patients and analysed in relationship to NEDA-3 status and annual brain volume loss (BVL) during the last 12 months. The statistical model was developed using logistic regression analysis, including demographic, clinical and magnetic resonance imaging (MRI) data as independent variables. Dependent variables were NEDA-3 status and BVL. RESULTS: The mean age of the study participants (n=52, 50% females) was 45.85 (SD, 9.82) and the median disability score was 5.0 (IQR: 5.0-5.5). ROC analysis showed that pNfL predicts NEDA-3 (the sensitivity and specificity of the model were 77.8% and 87.6%, respectively, P<0.001) and abnormal BVL (the sensitivity and specificity were 96.6% and 68.2%, respectively, P<0.001). CONCLUSIONS: The results show that pNfL levels are a useful biomarker of disease activity determined by NEDA-3 status, including brain MRI-volumetry, in patients with the progressive form of MS.