Involvement of the Nervous System in Systemic Sclerosis

Publisher Copyright: © 2022 Polunosika et al.Introduction: Scleroderma is a rare heterogeneous multisystem autoimmune disease. The disease is characterized by structural abnormalities of the small blood vessels. Scleroderma affects all organs of the body. Skin manifestations are commonly reported, but peripheral nervous system (PNS) and central nervous system (CNS) involvement has been less frequently reported. Neurological manifestations are broad, and it is challenging for clinicians to confirm a diagnosis of scleroderma. Case Presentation: In our case, a 53-year-old white woman had extensive clinical presentations: skin rashes and symptoms from internal organs, CNS, and PNS during the previous 11 years. She had not undergone any specific treatment because diagnosis has not been made in the early stages. Conclusion: It is important to make the diagnosis as early as possible and start treatment with immunomodulatory and immunosuppressant medication, as it affects the patient's disease progression, quality of life, and mortality. A detailed medical history, physical examination, and laboratory and radiology findings help us to identify and diagnose scleroderma. But unfortunately, it was late, and the patient died. This case guides us to be more careful and make the diagnosis of scleroderma earlier in the future.publishersversionPeer reviewe

Idarucizumab in Dabigatran-Treated Patients with Acute Ischemic Stroke Receiving Thrombolytic Therapy

Funding Information: This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. Publisher Copyright: © 2022 by the authors.Background and Objectives: Thrombolytic therapy with recombinant tissue-type plasminogen activator (rt-PA) is used to treat acute ischemic stroke. Dabigatran is a reversible thrombin inhibitor approved for stroke prevention in patients with nonvalvular atrial fibrillation. In such cases, thrombolytic therapy can be administered to certain patients after idarucizumab treatment. We evaluated the effectiveness of idarucizumab in dabigatran-treated patients receiving rt-PA. Materials and Methods: We included the data of nine idarucizumab-treated patients from the Riga East University Hospital Stroke Registry from 2018 to 2022 in our retrospective medical records analysis. We used the National Institutes of Health Stroke Scale (LV-NIHSS) score and modified Rankin scale (mRS) on admission and discharge to evaluate neurological deficit and functional outcomes. Results: We analyzed the data of nine patients (seven males and two females) with a mean age of 75.67 ± 8.59 years. The median door-to-needle time for all patients, including those who received idarucizumab before rt-PA, was 51 min (IQR = 43–133); the median LV-NIHSS score was 9 (IQR = 6.0–16.0) on admission and 4 (IQR = 2.5–4.0) at discharge; and the intrahospital mortality rate was 11.1% due to intracranial hemorrhage as a complication of rt-PA. Conclusions: Our study shows that idarucizumab as an antidote of dabigatran appears to be effective and safe in patients with acute ischemic stroke. Furthermore, the administration of idarucizumab slightly prolongs the door-to-needle time; however, the majority of cases showed clinical improvement after receiving therapy. Further randomized controlled trials should be performed to evaluate the safety and effectiveness of idarucizumab for acute ischemic stroke treatment.publishersversionPeer reviewe

Structural analysis of brain hub region volume and cortical thickness in patients with mild cognitive impairment and dementia

Publisher Copyright: © 2020 by the authors. Licensee MDPI, Basel, Switzerland. Copyright: Copyright 2020 Elsevier B.V., All rights reserved.Background and Objectives: A complex network of axonal pathways interlinks the human brain cortex. Brain networks are not distributed evenly, and brain regions making more connections with other parts are defined as brain hubs. Our objective was to analyze brain hub region volume and cortical thickness and determine the association with cognitive assessment scores in patients with mild cognitive impairment (MCI) and dementia. Materials and Methods: In this cross-sectional study, we included 11 patients (5 mild cognitive impairment; 6 dementia). All patients underwent neurological examination, and Montreal Cognitive Assessment (MoCA) test scores were recorded. Scans with a 3T MRI scanner were done, and cortical thickness and volumetric data were acquired using Freesurfer 7.1.0 software. Results: By analyzing differences between the MCI and dementia groups, MCI patients had higher hippocampal volumes (p <0.05) and left entorhinal cortex thickness (p <0.05). There was a significant positive correlation between MoCA test scores and left hippocampus volume (r = 0.767, p <0.01), right hippocampus volume (r = 0.785, p <0.01), right precuneus cortical thickness (r = 0.648, p <0.05), left entorhinal cortex thickness (r = 0.767, p <0.01), and right entorhinal cortex thickness (r = 0.612, p <0.05). Conclusions: In our study, hippocampal volume and entorhinal cortex showed significant differences in the MCI and dementia patient groups. Additionally, we found a statistically significant positive correlation between MoCA scores, hippocampal volume, entorhinal cortex thickness, and right precuneus. Although other brain hub regions did not show statistically significant differences, there should be additional research to evaluate the brain hub region association with MCI and dementia.publishersversionPeer reviewe

A fatal case of COVID-19-associated acute necrotizing encephalopathy

© 2021 European Academy of Neurology.Introduction: An increasing number of published reports on SARS-CoV-2 neurological manifestations have revealed a wide spectrum of symptoms, diagnostic features, and outcomes. We report a fatal case of a COVID-19-associated acute necrotizing encephalopathy (ANE). Case report: We report a 70-year-old man brought to the hospital after a generalized tonic-clonic seizure. He was confused and disoriented. Nasopharyngeal swab testing for SARS-CoV-2 was positive. A head computed tomography (CT) scan and cerebrospinal fluid (CSF) analysis showed no signs of acute pathology. After recurrent seizures, he was sedated and intubated. Throughout the days that followed he remained in a therapeutic coma. After discontinuation of sedatives, he remained unconscious. A repeated head CT scan showed signs of pontine edema, and brain magnetic resonance imaging (MRI) revealed inhomogeneous hyperintensities with microhemorrhages and small autonecrotic cavities in both thalami, brain stem, and cerebellar peduncles. With a high suspicion of a COVID-19-associated ANE, the patient was started on high-dose glucocorticoids; however, he died the next day. The CSF tested negative for SARS-CoV-2. Discussion: A variety of COVID-19 neurological manifestations have been reported to date, including various forms of encephalitis and encephalopathy. In our patient, encephalopathy with seizures was the presenting symptom of SARS-CoV-2 infection. The radiological findings on days 8 and 9 were consistent with an ANE. The precise pathogenesis of ANE remains unclear; however, an immune-mediated mechanism is suspected. Early diagnostics with prompt administration of immunomodulators may be lifesaving. Suspicion of a COVID-19-related encephalopathy/encephalitis should be raised in all patients with altered mental status, seizures, and/or coma.publishersversionPeer reviewe

Cerebellar Cortex and Cerebellar White Matter Volume in Normal Cognition, Mild Cognitive Impairment, and Dementia

Funding Information: Funding: The article has been developed with financing from the European Social Fund and the state budget within the project no. 8.2.2.0/20/I/004 “Support for involving doctoral students in sci‐ entific research and studies”. Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland.The cerebellum is commonly viewed as a structure that is primarily responsible for the coordination of voluntary movement, gait, posture, and speech. Recent research has shown evidence that the cerebellum is also responsible for cognition. We analyzed 28 participants divided into three groups (9 with normal cognition, 9 with mild cognitive impairment, and 10 with moderate/severe cognitive impairment) based on the Montreal Cognitive Assessment. We analyzed the cerebellar cortex and white matter volume and assessed differences between groups. Participants with normal cognition had higher average values in total cerebellar volume, cerebellar white matter volume, and cerebellar cortex volume in both hemispheres, but by performing the Kruskal–Wallis test, we did not find these values to be statistically significant.publishersversionPeer reviewe

Clinical Symptoms Influencing Parkinson's Patients' Quality of Life in Latvia : A Single-Center Cohort Study

Funding Information: Rīga Stradiņš University (RSU) supported covering the article processing fee. Publisher Copyright: © 2023 by the authors.Background and Objectives: Parkinson's disease (PD) is a chronic, progressive illness with a profound impact on health-related quality of life, and it is crucial to know what factors influence the quality of life throughout the course of the disease. This study aimed to evaluate PD patients' motor and non-motor symptoms to compare symptom severity between PD clinical phenotypes and to assess the impact of disease symptoms on quality of life in a cohort of Latvian patients. Materials and Methods: We evaluated 43 patients with Parkinson's disease. Fourteen patients had tremor dominant (TD) PD, twenty-five patients had postural instability/gait difficulty (PIGD), and four patients had a mixed phenotype. Results: The patients' mean age was 65.21 years, and the disease's mean duration was 7 years. The most common non-motor symptoms were fatigue (95.3%), sleep disturbance (83.7%), daytime sleepiness (83.7%), and pain and other sensations (81.4%). PIGD patients had a higher prevalence of depressed mood, daytime sleepiness, constipation, lightheadedness on standing, cognitive impairment, and severe gastrointestinal and urinary disturbances (as assessed using the SCOPA-AUT domains) compared with TD patients. A high prevalence of fatigue was assessed in both disease subtypes. Health-related quality of life significantly statistically correlated with MDS-UPDRS parts III and IV (r = 0.704), the Hoehn and Yahr scale (r = 0.723), as well as the SCOPA-AUT scale's gastrointestinal (r = 0.639), cardiovascular (r = 0.586), thermoregulatory (r = 0.566) and pupillomotor domains (r = 0.597). Conclusions: The severity of motor symptoms, as well as non-motor symptoms, such as fatigue, apathy, sleep problems and daytime sleepiness, pain, and disturbances in gastrointestinal and cardiovascular function, negatively affect PD patients' health-related quality of life. Thermoregulatory and pupillomotor symptoms also significantly affect PD patients' well-being.publishersversionPeer reviewe

Preferences of patients for discussing sudden unexpected death in epilepsy

Funding Information: The study was approved by the Medical and Biomedical Research Ethics Committee of the Riga East Clinical University Hospital. Publisher Copyright: © 2017 De Gruyter Open Ltd. All rights reserved.People with epilepsy have increased mortality rates, which is partially attributed to sudden unexpected death in epilepsy syndrome (SUDEP). Poor seizure control appears to be the strongest SUDEP risk factor. Management of epilepsy and adherence to therapy is critical to seizure control. The belief by caregivers of negative influence caused by being informed about the syndrome is the main reason SUDEP is not disclosed. There are no clear recommendations when to disclose the risk of SUDEP and how much information should be provided. We addressed the preferences of Latvian epilepsy patients for discussing SUDEP as well as awareness of the syndrome. Our study involved 55 epilepsy patients. We found that, as in other studies, our patients were relatively well informed about SUDEP. We found that a considerable proportion of patients preferred to receive information about SUDEP from a general practitioner. We note the belief of patients that the disclosure of SUDEP would either improve or have no effect on the quality of life. We were able to identify groups of patients with a self-reported belief of more frequent expected anxiety and poor adherence to medical treatment. Our data improves the understanding of preferences of patient for discussing the negative aspects of epilepsy.publishersversionPeer reviewe

Combined Score of Perivascular Space Dilatation and White Matter Hyperintensities in Patients with Normal Cognition, Mild Cognitive Impairment, and Dementia

Funding Information: Funding: This research has been developed with financing from the European Social Fund and Latvian state budget within the project no. 8.2.2.0/20/I/004 “Support for involving doctoral students in scientific research and studies” at R¯ıga Stradin,š University. Publisher Copyright: © 2022 by the authors. Licensee MDPI, Basel, Switzerland.Background and Objectives: Cerebral perivascular spaces (PVS) are part of the cerebral microvascular structure and play a role in lymphatic drainage and the removal of waste products from the brain. White matter hyperintensities (WMH) are hyperintense lesions on magnetic resonance imaging that are associated with cognitive impairment, dementia, and cerebral vascular disease. WMH and PVS are direct and indirect imaging biomarkers of cerebral microvascular integrity and health. In our research, we evaluated WMH and PVS enlargement in patients with normal cognition (NC), mild cognitive impairment (MCI), and dementia (D). Materials and Methods: In total, 57 participants were included in the study and divided into groups based on neurological evaluation and Montreal Cognitive Assessment results (NC group 16 participants, MCI group 29 participants, D group 12 participants). All participants underwent 3T magnetic resonance imaging. PVS were evaluated in the basal ganglia, centrum semiovale, and midbrain. WMHs were evaluated based on the Fazekas scale and the division between deep white matter (DWM) and periventricular white matter (PVWM). The combined score based on PVS and WMH was evaluated and correlated with the results of the MoCA. Results: We found statistically significant differences between groups on several measures. Centrum semiovale PVS dilatation was more severe in MCI and dementia group and statistically significant differences were found between D-MCI and D-NC pairs. PVWM was more severe in patients with MCI and dementia group, and statistically significant differences were found between D-MCI and D-NC pairs. Furthermore, we found statistically significant differences between the groups by analyzing the combined score of PVS dilatation and WMH. We did not find statistically significant differences between the groups in PVS dilation of the basal ganglia and midbrain and DWM hyperintensities. Conclusions: PVS assessment could become one of neuroimaging biomarkers for patients with cognitive decline. Furthermore, the combined score of WMH and PVS dilatation could facilitate diagnostics of cognitive impairment, but more research is needed with a larger cohort to determine the use of PVS dilatation and the combined score.publishersversionPeer reviewe

The Riga East University Hospital Stroke Registry-An Analysis of 4915 Consecutive Patients with Acute Stroke

Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland.Background and Objectives: A hospital-based stroke registry is a useful tool for systematic analyses of the epidemiology, clinical characteristics, and natural course of stroke. Analyses of stroke registry data can provide information that can be used by health services to improve the quality of care for patients with this disease. Materials and Methods: Data were collected from the Riga East University Hospital (REUH) Stroke Registry in order to evaluate the etiology, risk factors, clinical manifestations, treatment, functional outcomes, and other relevant data for acute stroke during the period 2016-2020. Results: During a five-year period, 4915 patients (3039 females and 1876 males) with acute stroke were registered in the REUH Stroke Registry. The causative factors of stroke were cardioembolism (45.7%), atherosclerosis (29.9%), lacunar stroke (5.3%), stroke of undetermined etiology (1.2%), and stroke of other determined causes (1.2%). The most frequent localizations of intracerebral hemorrhage were subcortical (40.0%), lobar (18.9%), and brainstem (9.3%). The most prevalent risk factors for stroke were hypertension (88.8%), congestive heart failure (71.2%), dyslipidemia (46.7%), and atrial fibrillation (44.2%). In addition, 1018 (20.7%) patients were receiving antiplatelet drugs, 574 (11.7%) were taking statins, and 382 (7.7%) were taking anticoagulants. At discharge, 35.5% of the patients were completely independent (mRS (modified Rankin Scale) score: 0-2), while 49.5% required some form of assistance (mRS score: 3-5). The intrahospital mortality rate was 13.7%, although it was higher in the hemorrhage group (30.9%). Conclusions: Our stroke registry data are comparable to those of other major registries. Analysis of stroke registry data is important for improving stroke care and obtaining additional information for stroke studies.publishersversionPeer reviewe

Electrocardiographic Abnormalities and Mortality in Epilepsy Patients

Background and Objectives: People with epilepsy (PWE) have a 2-3 times higher mortality rate than the general population. Sudden unexpected death in epilepsy (SUDEP) comprises a significant proportion of premature deaths, whereas sudden cardiac death (SCD) is among the leading causes of sudden death in the general population. Cardiac pathologies are significantly more prevalent in PWE. Whether electrocardiographic (ECG) parameters are associated with remote death in PWE has yet to be elucidated. The study objective was to assess whether interictal ECG parameters are associated with mortality in the long-term. Materials and Methods: The study involved 471 epilepsy patients who were hospitalized after a bilateral tonic-clonic seizure(s). ECG parameters were obtained on the day of hospitalization (heart rate, PQ interval, QRS complex, QT interval, heart rate corrected QT interval (QTc), ST segment and T wave changes), as well as reported ECG abnormalities. Mortality data were obtained from the Latvian National Cause-of-Death database 3-11, mean 7.0 years after hospitalization. The association between the ECG parameters and the long-term clinical outcome were examined. Results: At the time of assessment, 75.4% of patients were alive and 24.6% were deceased. Short QTc interval (odds ratio (OR) 4.780; 95% confidence interval (CI) 1.668-13.698; p = 0.004) was associated with a remote death. After the exclusion of known comorbidities with high mortality rates, short QTc (OR 4.631) and ECG signs of left ventricular hypertrophy (OR 5.009) were associated with a remote death. Conclusions: The association between routine 12-lead rest ECG parameters-short QTc interval and a pattern of left ventricular hypertrophy-and remote death in epilepsy patients was found. To the best of our knowledge, this is the first study to associate rest ECG parameters with remote death in an epileptic population.publishersversionPeer reviewe