• Harmon Madden opublikował 1 rok, 3 miesiące temu

    313, 95% CI 0.112-0.878, p = 0.027), higher MR Parkinsonism Index (HR 6.162, 95% CI 1.790-21.209, p = 0.004) and larger third ventricle width (HR 2.755, 95% CI 1.068-7.108, p = 0.036) were associated with higher risk of dependency on wheelchair. Conclusions Irrespective of disease features and other MRI parameters, reduced midbrain size is significantly associated with greater ocular motor dysfunction at the time of MRI and more rapid disease progression over follow up. This is the first comprehensive study to systematically assess the association of available midbrain-based MRI measures and cortical volumes with disease severity and progression in a large cohort of patients with PSP in a real-world setting.Objectives To estimate the jitter parameters (single-fiber electromyography) in myasthenia gravis patients mostly by electrical activation in Frontalis, Orbicularis Oculi, and Extensor Digitorum muscles using a concentric needle electrode. Methods Between 2009 and 2019, a total of 97 myasthenia gravis patients, 52 male, and mean age 54 years were included. Results Any abnormal jitter parameter in individual muscles was 90.5% (Frontalis), 88.5% (Orbicularis Oculi), and 86.6% (Extensor Digitorum). Any jitter parameter combining Orbicularis Oculi and Frontalis muscle was abnormal in 100% for the ocular, and in 92.9% for the generalized myasthenia gravis. The most abnormal muscle was Orbicularis Oculi for the generalized, and Frontalis for the ocular myasthenia gravis. The decrement was abnormal in 78.4%, 85.9% for the generalized, and 25% for the ocular myasthenia gravis. The mean jitter ranged from 14.2 to 86 μs (mean 33.3 μs) for the ocular myasthenia gravis and from 14.4 to 220.4 μs (mean 66.3 μs) for the generalized myasthenia gravis. The antibody titers tested positive in 86.6%, 91.8% for the generalized, and 50% for the ocular myasthenia gravis. Thymectomy was done in 48.5%, thymoma was found in 19.6%, and myasthenic crisis occurred by 21.6%. Conclusion The jitter parameters achieved a 100% abnormality in ocular myasthenia gravis if both the Orbicularis Oculi and Frontalis muscles were tested. There was a high jitter abnormality in generalized myasthenia gravis cases with one muscle tested, with about a 2% increase in sensitivity when a second is added. Concentric needle electrode jitter had high sensitivity similar to the single fiber electrode (93.8%), followed by antibody titers (86.6%), and abnormal decrement (78.4%).Cerebral venous outflow disturbance (CVOD) has begun to garner the attention of researches owing to a series of clinical symptoms that impose a significant impact on people’s quality of life. Herein, we aimed to investigate whether normobaric oxygen (NBO) can ameliorate CVOD-induced neurological symptoms. This was one part of the prospective trial registered in ClinicalTrials.gov (NCT03373292). A total of 37 CVOD patients were divided into the NBO group (5-8 L/min of oxygen inhalation, 1 h per time, 3 times daily, n = 19) and the control group (without oxygen inhalation, n = 18) randomly. The assessments were performed at admission, 1-week hospitalization, and 6-month follow-up. Quantitative electroencephalogram (qEEG) data were recorded prior to and post 1 h of NBO in some patients. R software was used for data analysis. No NBO-related adverse events were observed during the whole NBO intervention process. The 1-week Patient Global Impression of Change (PGIC) scale showed that the symptom improvement occurred in nine patients in the NBO group (47.4%) while none in the control group (p = 0.001). NBO could improve headache evaluated with visual analog scale (pre-NBO vs. post-NBO 4.70 ± 2.16 vs. 2.90 ± 2.03, p = 0.024) and Headache Impact Test-6 (53.40 ± 12.15 vs. 50.30 ± 13.04, p = 0.041). As for 6-month PGIC follow-up, eight out of 14 cases (57.1%) in the NBO group reported improvement, while only one out of 12 patients in the control group replied mild improvement (p = 0.014). The qEEG revealed that NBO reduced the ratio of theta to alpha power (0.65 ± 0.38 vs. 0.56 ± 0.35, p = 0.030) over the fronto-central electrodes. To sum up, NBO may be a safe and effective approach to attenuate CVOD-related symptoms (especially for headache) by brain functional improvement resulting from increasing oxygen supply to the brain tissues.Cases of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy who were initially diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) were rarely reported. Herein, we reported a 31-year-old woman who presented with 7 years of recurrent headache. Her clinical history, symptoms, brain MRI enhancement features, and response to treatment during each attack were reviewed. Her brain MRI 7 years ago demonstrated characteristic pepper-like enhancement of pontine and cerebellum and her symptoms resolved completely after taking a high-dose of steroids. She was suspected with the diagnosis of CLIPPERS, and she experienced five relapses once the oral steroid was tapered below 20 mg/day. During her last relapse, she experienced fever and psychosis, and GFAPα-antibodies were detected in her serum and cerebrospinal fluid by antigen-transfected HEK293 cell-based assay (indirect immunofluorescence assay). She obtained relief again after steroid therapy, and her diagnosis converted to autoimmune GFAP astrocytopathy. Autoimmune GFAP astrocytopathy may mimic CLIPPERS, both clinically and radiologically. Long-term follow-up is essential for necessary diagnosis revision at each new attack in patients with a diagnosis of CLIPPERS.On the verge of the ongoing coronavirus pandemic, in vitro data suggested that chloroquine, and its analog hydroxychloroquine, may be useful in controlling SARS-CoV-2 infection. Efforts are ongoing in order to test this hypothesis in clinical trials. Some studies demonstrated no evidence of efficacy, whereas in some cases results were retracted after reporting. Despite the lack of scientific validation, support for the use of these compounds continues from various influencers. At the cellular level, the lysosomotropic drug chloroquine accumulates in acidic organelles where it acts as an alkalizing agent with possible downstream effects on several cellular pathways. In this perspective, we discuss a possible modulatory role of these drugs in two shared features of neurodegenerative diseases, the cellular accumulation of aberrantly folded proteins and the contribution of neuroinflammation in this pathogenic process. Certainly, the decision on the use of chloroquine must be determined by its efficacy in the specific clinical situation. However, at an unprecedented time of a potential widespread use of chloroquine, we seek to raise awareness of its potential impact in ongoing clinical trials evaluating disease-modifying therapies in neurodegeneration.In acute ischemic stroke, early recanalization of the occluded artery is crucial for best outcome to be achieved. Recanalization aims at restoring blood flow to the ischemic tissue (reperfusion) and is achieved with pharmacological thrombolytic drugs, endovascular thrombectomy (EVT) devices, or both. The introduction of modern endovascular devices has led to tremendous anatomical and clinical success with rates of substantial reperfusion exceeding 80% and proven clinical benefit in patients with anterior circulation large vessel occlusions (LVOs). However, not every successful reperfusion procedure leads to the desired clinical outcome. In fact, the rate of non-disabled outcome at 3 months with current EVT treatment is ~1 out of 4. A constraint upon better outcomes is that reperfusion, though resolving ischemic stress, may not restore the anatomic structures and metabolic functions of ischemic tissue to their baseline states. In fact, ischemia triggers a complex cascade of destructive mechanisms that can sometimes be exacerbated rather than alleviated by reperfusion therapy. Such reperfusion injury may cause infarct progression, intracranial hemorrhage, and unfavorable outcome. Therapeutic hypothermia has been shown to have a favorable impact on the molecular elaboration of ischemic injury, but systemic hypothermia is limited by slow speed of attaining target temperatures and clinical complications. A novel approach is endovascular delivery of hypothermia to cool the affected brain tissue selectively and rapidly with tight local temperature control, features not available with systemic hypothermia devices. In this perspective article, we discuss the possible benefits of adjunctive selective endovascular brain hypothermia during interventional stroke treatment.Objective Initial randomized controlled trials (RCTs) and recently released systematic reviews have identified resistance training (RT) as a modality to manage motor symptoms and improve physical functioning in individuals with Parkinson’s disease (PD), although the effects are inconsistent. Therefore, we conducted an updated meta-analysis to reassess the evidence of the relationship. Methods We performed a systematic search of studies reporting the effects of RT in PD available through major electronic databases (PubMed, Medline, Embase, Ovid, Cochrane Library, CNKI, Wanfang) through 20 July 2020. Eligible RCTs were screened based on established inclusion criteria. We extracted data on the indicators of leg strength, balance, gait capacity, and quality of life (QoL) of lower limbs. Random and fixed effects models were used for the analysis of standard mean differences (SMD) or mean differences (MD) with their 95% confidence intervals (CI). Results Thirty-one papers from 25 independent trials compromising 1,2rength, QoL, and improve gait performance to a certain extent. RT also could improve balance capacity of patients, although a wide variety of tools were used, and further study is needed to confirm these findings.Orbitocranial penetrating injury (OPI) with multiple vascular invasions is a rare occurrence. To our knowledge, experience with its clinical treatment is rather limited, especially for infants. This case report describes an infant who fell from a 0.5 m high bed and landed on a toy with a keen-edged plastic rod. The fractured end of the rod was noted at the medial aspect of the left eyelid, and she was experiencing impaired consciousness. Computed tomography showed that the foreign body penetrated the cavernous sinus with internal carotid artery involvement, and compressed the transverse sinus through the cerebellum. Emergency surgery was performed with temporal occlusion of the left common carotid artery. The rod was removed from the orbital side, and bleeding from cavernous sinus region was effectively controlled under direct inspection via a sub-temporal approach. The patient was successfully treated and recovered consciousness after 17 days. This is the first report of successful management of OPI combined with multiple vascular injury in an infant. Herein, we highlight the anatomical imaging features of the injuries and also the individualized strategy concerning vascular invasion.Tau plays an important pathological role in a group of neurodegenerative diseases called tauopathies, including Alzheimer’s disease, Pick’s disease, chronic traumatic encephalopathy and corticobasal degeneration. In each disease, tau self-assembles abnormally to form filaments that deposit in the brain. Tau is a natively unfolded protein that can adopt distinct structures in different pathological disorders. Cryo-electron microscopy has recently provided a series of structures for the core of the filaments purified from brain tissue from patients with different tauopathies and revealed that they share a common core region, while differing in their specific conformation. This structurally resolvable part of the core is contained within a proteolytically stable core region from the repeat domain initially isolated from AD tau filaments. Tau has recently become an important target for therapy. Recent work has suggested that the prevention of tau self-assembly may be effective in slowing the progression of Alzheimer’s disease and other tauopathies.

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