Tick-borne encephalitis (TBE) virus is a major cause of central nervous system infections in endemic countries. Here, we present clinical and laboratory characteristics of a large international cohort of patients with confirmed TBE using a uniform clinical protocol. Patients were recruited in eight centers from six European countries between 2010 and 2017. A detailed description of clinical signs and symptoms was recorded. The obtained information enabled a reliable classification in 553 of 555 patients: 207 (37.3%) had meningitis, 273 (49.2%) meningoencephalitis, 15 (2.7%) meningomyelitis, and 58 (10.5%) meningoencephalomyelitis; 41 (7.4%) patients had a peripheral paresis of extremities, 13 (2.3%) a central paresis of extremities, and 25 (4.5%) had single or multiple cranial nerve palsies. Five (0.9%) patients died during acute illness. Outcome at discharge was recorded in 298 patients. Of 176 (59.1%) patients with incomplete recovery, 80 (27%) displayed persisting symptoms or signs without recovery expectation. This study provides further evidence that TBE is a severe disease with a large proportion of patients with incomplete recovery. We suggest monitoring TBE in endemic European countries using a uniform protocol to record the full clinical spectrum of the disease.
Background: Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular transmission. Exacerbations may involve increasing bulbar weakness and/or sudden respiratory failure, both of which can be critically disabling. Management of MG exacerbations includes plasma exchange and intravenous immunoglobulin (IVIG); they are equally effective, but patients experience fewer side effects with IVIG. The objective of this study was to assess the efficacy and safety of immune globulin caprylate/chromatography purified (IGIV-C) in subjects with MG exacerbations. Methods: This prospective, open-label, non-controlled 28-day clinical trial was conducted in adults with MG Foundation of America class IVb or V status. Subjects received IGIV-C 2 g/kg over 2 consecutive days (1 g/kg/day) and were assessed for efficacy/safety on Days 7, 14, 21, and 28. The primary efficacy endpoint was the change from Baseline in quantitative MG (QMG) score to Day 14. Secondary endpoints of clinical response, Baseline to Day 14, included at least a 3-point decrease in QMG and MG Composite and a 2-point decrease in MG-activities of daily living (MG-ADL). Results: Forty-nine subjects enrolled. The change in QMG score at Day 14 was significant (p < 0.001) in the Evaluable (–6.4, n = 43) and Safety (–6.7, n = 49) populations. Among evaluable subjects, Day 14 response rates were 77, 86, and 88% for QMG, MG Composite, and MG-ADL, respectively. IGIV-C showed good tolerability with no serious adverse events. Conclusions: The results of this study show that IGIV-C was effective, safe, and well tolerated in the treatment of MG exacerbations.
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.
Diffusion tensor imaging (DTI) is an MRI analysis method that could help assess cognitive impairment (CI) in the ageing population more accurately. In this research, we evaluated fractional anisotropy (FA) of whole brain (WB) and corpus callosum (CC) in patients with normal cognition (NC), mild cognitive impairment (MCI), and moderate/severe cognitive impairment (SCI). In total, 41 participants were included in a cross-sectional study and divided into groups based on Montreal Cognitive Assessment (MoCA) scores (NC group, nine participants, MCI group, sixteen participants, and SCI group, sixteen participants). All participants underwent an MRI examination that included a DTI sequence. FA values between the groups were assessed by analysing FA value and age normative percentile. We did not find statistically significant differences between the groups when analysing CC FA values. Both approaches showed statistically significant differences in WB FA values between the MCI-SCI and MCI-NC groups, where the MCI group participants showed the highest mean FA and highest mean FA normative percentile results in WB.
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.
Advances in magnetic resonance imaging, particularly diffusion imaging, have allowed researchers to analyze brain connectivity. Identification of structural connectivity differences between patients with normal cognition, cognitive impairment, and dementia could lead to new biomarker discoveries that could improve dementia diagnostics. In our study, we analyzed 22 patients (11 control group patients, 11 dementia group patients) that underwent 3T MRI diffusion tensor imaging (DTI) scans and the Montreal Cognitive Assessment (MoCA) test. We reconstructed DTI images and used the Desikan–Killiany–Tourville cortical parcellation atlas. The connectivity matrix was calculated, and graph theoretical analysis was conducted using DSI Studio. We found statistically significant differences between groups in the graph density, network characteristic path length, small-worldness, global efficiency, and rich club organization. We did not find statistically significant differences between groups in the average clustering coefficient and the assortativity coefficient. These statistically significant graph theory measures could potentially be used as quantitative biomarkers in cognitive impairment and dementia diagnostics.