418 results match your criteria pns involvement

The Neuropathy In Hereditary Transthyretin Amyloidosis (Attrv): A Narrative Review.

J Peripher Nerv Syst 2021 May 7. Epub 2021 May 7.

Department of Neuroscience, Reproductive and Odontostomatological Science, University of Naples "Federico II", Naples, Italy.

Hereditary transthyretin amyloidosis (ATTRv) is a condition with adult-onset caused by mutation of transthyretin (TTR) gene and characterized by extracellular deposition of amyloid fibrils in tissue, expecially in peripheral nervous system (PNS) and heart. The PNS involvement lead to a rapidly progressive and disabling sensory-motor axonal neuropathy. Although awareness among neurologist raised in last years thank to new treatment options, ATTRv is frequently misdiagnosed and thus correct diagnosis can be delayed by several years. Read More

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Monocentric study of 28 cases of chronic inflammatory demyelinating polyneuropathy: first Tunisian study.

Neurol Sci 2021 May 4. Epub 2021 May 4.

Department of Neurology, Military Hospital, Tunis, Tunisia.

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare affection of the peripheral nervous system. Its diagnostic criteria have evolved since 1975. The aim of our work is to study the epidemiological, clinical, and paraclinical aspects of CIDP. Read More

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Neurological impact of eosinophilic granulomatosis with polyangiitis.

Acta Neurol Belg 2021 Apr 27. Epub 2021 Apr 27.

Neurology Department, Coimbra University Hospital Centre, Praceta Mota Pinto, 3000-075, Coimbra, Portugal.

Nervous system (NS) affection may occur in Eosinophilic Granulomatosis with Polyangiitis (EGPA), but its clinical manifestations and pathophysiology are rarely described. Our aims are to characterize central and peripheral NS (CNS/PNS) involvement and compare biological markers in EGPA patients with and without neurological manifestations. Retrospective observational study, including EGPA patients with and without neurological manifestations. Read More

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Antibodies to the Caspr1/contactin-1 complex in chronic inflammatory demyelinating polyradiculoneuropathy.

Brain 2021 May;144(4):1183-1196

Neuromuscular Diseases Unit, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Spain.

Previous studies have described the clinical, serological and pathological features of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and antibodies directed against the paranodal proteins neurofascin-155, contactin-1 (CNTN1), contactin-associated protein-1 (Caspr1), or nodal forms of neurofascin. Such antibodies are useful for diagnosis and potentially treatment selection. However, antibodies targeting Caspr1 only or the Caspr1/CNTN1 complex have been reported in few patients with CIDP. Read More

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Peripheral Nervous System Involvement in Sjögren's Syndrome: Analysis of a Cohort From the Italian Research Group on Sjögren's Syndrome.

Front Immunol 2021 24;12:615656. Epub 2021 Mar 24.

Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy.

Purpose: The prevalence of peripheral nervous system (PNS) involvement in primary Sjögren's syndrome (pSS) has been reported to range from 2% to over 50%. Bias in study designs, including low number of patients and unclearly defined rheumatological and neurological diagnosis could explain such variability. Consequently, the exact depiction of PNS involvement in pSS is still lacking. Read More

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CNS involvement in systemic vasculitides.

Neil Scolding

J Neurol Sci 2021 May 27;424:117423. Epub 2021 Mar 27.

Burden Professor of Clinical Neurosciences, University of Bristol Institute of Clinical Neurosciences, UK, Gulu University Faculty of Medicine, Uganda. Electronic address:

Both the CNS and the PNS can be involved in almost all of the vasculitides - including the primary systemic vasculitic disorders, such as microscopic polyangiitis and polyarteritis nodosa, and in non-vasculitic systemic disorders, such as systemic lupus erythematosis and sarcoidosis. The latter diseases also include infections and toxininduced disorders - particularly drugs of abuse such as cocaine and amphetamines. Here we will summarise the spectrum of these disorders as they affect the CNS, concentrating in particular on their distinguishing clinical and investigational features. Read More

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Clinical and Pathophysiologic Spectrum of Neuro-COVID.

Mol Neurobiol 2021 Apr 8. Epub 2021 Apr 8.

Disciplina de Neurociência, Universidade Federal de São Paulo/Escola Paulista de Medicina (UNIFESP/EPM), São Paulo, Brasil.

Though the lungs are predominantly affected in SARS-CoV-2-infected patients, extra-pulmonary manifestations can occur. Extra-pulmonary manifestations of the central and peripheral nervous system need to be recognised as they can strongly determine the outcome. This mini-review summarises and discusses previous and recent findings about neuro-COVID. Read More

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COVID-19 and Neurological Impairment: Hypothalamic Circuits and Beyond.

Viruses 2021 03 17;13(3). Epub 2021 Mar 17.

Department of Medicine, Austin Health, University of Melbourne, Heidelberg 3084, Australia.

In December 2019, a novel coronavirus known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) emerged in Wuhan, the capital of Hubei, China. The virus infection, coronavirus disease 2019 (COVID-19), represents a global concern, as almost all countries around the world are affected. Clinical reports have confirmed several neurological manifestations in COVID-19 patients such as headaches, vomiting, and nausea, indicating the involvement of the central nervous system (CNS) and peripheral nervous system (PNS). Read More

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Delayed diagnosis of cranial neuropathies from perineural spread of skin cancer.

Taiwan J Ophthalmol 2021 Jan-Mar;11(1):86-88. Epub 2020 Nov 19.

Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia, USA.

Perineural spread (PNS) to cranial nerves (CNs) by cutaneous malignancies is difficult to diagnose given the indolent course and often late or absent findings on brain imaging. A 68-year-old white man with multiple cranial neuropathies secondary to PNS by squamous cell carcinoma had negative high-quality neuroimaging for 5.25 years. Read More

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November 2020

Functional Importance of Transient Receptor Potential (TRP) Channels in Neurological Disorders.

Front Cell Dev Biol 2021 4;9:611773. Epub 2021 Mar 4.

Gachon Pain Center and Department of Physiology, Gachon University College of Medicine, Incheon, South Korea.

Transient receptor potential (TRP) channels are transmembrane protein complexes that play important roles in the physiology and pathophysiology of both the central nervous system (CNS) and the peripheral nerve system (PNS). TRP channels function as non-selective cation channels that are activated by several chemical, mechanical, and thermal stimuli as well as by pH, osmolarity, and several endogenous or exogenous ligands, second messengers, and signaling molecules. On the pathophysiological side, these channels have been shown to play essential roles in the reproductive system, kidney, pancreas, lung, bone, intestine, as well as in neuropathic pain in both the CNS and PNS. Read More

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Shear wave elastography evaluation of brachial plexus in multiple sclerosis.

Acta Radiol 2021 Mar 17:2841851211002828. Epub 2021 Mar 17.

Department of Radiology, Gazi University, Ankara, Turkey.

Background: Multiple sclerosis (MS) is a chronic neuroinflammatory disease characterized by inflammation involving the peripheral nerves. Shear wave elastography (SWE) is potentially a method of choice for detecting peripheral nerve involvement.

Purpose: To compare the degree of thickening and nerve elasticity of brachial plexus (BP) nerve roots and evaluate the usefulness of sonoelastography in patients with clinically diagnosed MS without brachial plexopathy. Read More

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From PNS to CNS: characteristics of anti-neurofascin 186 neuropathy in 16 cases.

Neurol Sci 2021 Mar 15. Epub 2021 Mar 15.

Department of Neurology, Renji Hospital, Shanghai Jiaotong University School of Medicine, 160 Pujian Road, Pudong, Shanghai, 200127, China.

Introduction: Neurofascin (NF) is critical for the formation and maintenance of Ranvier nodes. NF186, the neuronal form of NF, localizes in the initial segment of axon and Ranvier node. NF186 antibody has been detected in demyelinating diseases of both central nervous system (CNS) and peripheral nervous system (PNS). Read More

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[Clinical characteristics of "classical" and "non-classical" paraneoplastic neurological syndrome].

Zhonghua Yi Xue Za Zhi 2021 Mar;101(9):615-619

Department of Neurology, the First Affiliated Hospital of Anhui Medical University, Hefei 230022, China.

To explore the clinical features of classical and non-classical paraneoplastic neurological syndrome (PNS). From 2015 to 2020, 48 cases of definite PNS admitted to the First Affiliated Hospital of University of Science and Technology of China were retrospectively collected, and classification, clinical characteristics, onconeural antibodies and primary tumors were analyzed. The included cases were divided into classical and non-classical groups according to Graus criteria, and the differences of clinical characteristics, onconeural antibodies, combined tumors, time of diagnosis and mortality were compared between the two groups. Read More

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Peculiar Presentation of COVID-19: A Case Report of Concurrent Stroke and Guillain-Barré Syndrome.

Case Rep Neurol Med 2021 15;2021:8824512. Epub 2021 Feb 15.

Isfahan Neuroscience Research Center, Department of Neurology, Al Zahra Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran.

Background: Coronavirus disease 2019 (COVID-19) is a newly recognized infectious disease that has turned into a pandemic. There are few studies reporting Guillain-Barré syndrome (GBS) and stroke separately associated with COVID-19. In this study, we report an unusual case of COVID-19 with stroke and GBS concurrently. Read More

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February 2021

The use of eye-movement recording in patients with anti-Hu antibody-associated paraneoplastic neurological syndromes to objectively determine extent and course of disease.

Eur J Neurol 2021 Mar 5. Epub 2021 Mar 5.

Department of Neuroscience, Erasmus MC, Rotterdam, the Netherlands.

Background And Purpose: Paraneoplastic neurological syndromes with Hu-antibodies (Hu-PNS) are immune-mediated disorders in patients with malignancies, most frequently small cell lung cancer, affecting both the peripheral and central nervous system (CNS). In Hu-PNS, brainstem and cerebellar involvement are common. Here, we assessed whether eye-movement disturbances can be used for diagnosis and monitoring of CNS involvement in Hu-PNS. Read More

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[Neuroendorine paraneoplastic syndromes].

M Böhm R Gellner

Hautarzt 2021 Apr 4;72(4):299-306. Epub 2021 Mar 4.

Medizinische Klinik B, Universitätsklinikum Münster, Münster, Deutschland.

Skin is commonly affected by neuroendorine paraneoplastic syndromes (PNS). This is due to the expression of receptors in the skin by which abnormally secreted neuroendocrine hormones and mediators elicit directly, and indirectly, cutaneous key signs and thus facilitate early diagnosis of these diseases. In acromegaly, induction of the growth hormone-insulin-like growth factor‑1 axis results in trophic changes of the acral portions of the skin and mucosal membranes including cutis verticis gyrata. Read More

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A Rare Case of Wobbly, Psychotic Patient with Frozen Eyes - Anti-AMPA Receptor Encephalitis.

Neurol India 2021 Jan-Feb;69(1):149-152

Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.

Background: Anti α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis is a rare autoimmune encephalitis. They present with memory, confusion or behavioral changes.

Objective: The aim of this study was to describe unusual clinical features in a patient with AMPAR-associated encephalitis. Read More

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Neurological Involvement in Patients With Primary Sjögren's Syndrome.

J Clin Rheumatol 2021 Mar;27(2):50-55

Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University.

Background/objective: The neurological involvement associated with primary Sjögren's syndrome (pSS) can be life threatening. However, the specific characteristics of pSS-related neurological involvement remain obscure. This study aimed at determining the clinical characteristics of this neurological involvement in patients with pSS. Read More

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Lymphatic muscle cells contribute to dysfunction of the synovial lymphatic system in inflammatory arthritis in mice.

Arthritis Res Ther 2021 02 19;23(1):58. Epub 2021 Feb 19.

Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, NY, 14642, USA.

Background: Our previous studies reveal that impaired draining function of the synovial lymphatic vessel (LV) contributes to the pathogenesis of inflammatory arthritis, but the cellular and molecular mechanisms involved are not fully understood.

Objective: To investigate the involvement of lymphatic muscle cells (LMCs) in mediating impaired LV function in inflammatory arthritis.

Methods: TNF transgenic (TNF-Tg) arthritic mice were used. Read More

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February 2021

Peripheral Nervous System Manifestations Associated with COVID-19.

Curr Neurol Neurosci Rep 2021 02 14;21(3). Epub 2021 Feb 14.

Department of Neurology, School of Medicine, University of New Mexico, NM, Albuquerque, NM, 87131, USA.

Purpose Of Review: The present review discusses the peripheral nervous system (PNS) manifestations associated with coronavirus disease 2019 (COVID-19).

Recent Findings: Nerve pain and skeletal muscle injury, Guillain-Barré syndrome, cranial polyneuritis, neuromuscular junction disorders, neuro-ophthalmological disorders, neurosensory hearing loss, and dysautonomia have been reported as PNS manifestations in patients with COVID-19. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes COVID-19. Read More

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February 2021

The Neuroimmunology of Guillain-Barré Syndrome and the Potential Role of an Aging Immune System.

Front Aging Neurosci 2020 13;12:613628. Epub 2021 Jan 13.

Departments of Clinical Neurosciences and Cell Biology and Anatomy, Hotchkiss Brain Institute, University of Calgary, Calgary, AB, Canada.

Guillain-Barré syndrome (GBS) is a paralyzing autoimmune condition affecting the peripheral nervous system (PNS). Within GBS there are several variants affecting different aspects of the peripheral nerve. In general, there appears to be a role for T cells, macrophages, B cells, and complement in initiating and perpetuating attacks on gangliosides of Schwann cells and axons. Read More

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January 2021

Childhood-Onset Multifocal Motor Neuropathy with IgM Antibodies to Gangliosides GM1: A Case Report with Poor Outcome.

Neuropediatrics 2021 Jan 28. Epub 2021 Jan 28.

Child Neurology Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Background:  Multifocal motor neuropathy (MMN) is a slowly progressive motor neuropathy characterized by asymmetric muscle weakness without sensory involvement. Typically, MMN respond completely to treatment with intravenous immunoglobulin (IVIg). MMN is even rarer in the pediatric population, where only five patients have been reported up to now. Read More

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January 2021

SARS-CoV-2: its potential neurological manifestations and plausible mechanism: a review article.

Acta Neurol Belg 2021 Apr 19;121(2):331-339. Epub 2021 Jan 19.

Department of Radiology, University of British Columbia, Vancouver, BC, Canada.

Coronavirus disease (COVID-19) was first identified in late December 2019. The disease began in Wuhan, Hubei province in China and since then it has spread quickly to many countries all over the world. COVID-19 is caused by a novel coronavirus, named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Read More

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How to Build and to Protect the Neuromuscular Junction: The Role of the Glial Cell Line-Derived Neurotrophic Factor.

Int J Mol Sci 2020 Dec 24;22(1). Epub 2020 Dec 24.

Institute of Neuroscience (IoNS), Université Catholique de Louvain (UCLouvain), 1200 Bruxelles, Belgium.

The neuromuscular junction (NMJ) is at the crossroad between the nervous system (NS) and the muscle. Following neurotransmitter release from the motor neurons (MNs), muscle contraction occurs and movement is generated. Besides eliciting muscle contraction, the NMJ represents a site of chemical bidirectional interplay between nerve and muscle with the active participation of Schwann cells. Read More

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December 2020

New evidence for secondary axonal degeneration in demyelinating neuropathies.

Neurosci Lett 2021 01 24;744:135595. Epub 2020 Dec 24.

Department of Neurology, Neuromuscular Division, Johns Hopkins School of Medicine, Baltimore, MD, United States. Electronic address:

Development of peripheral nervous system (PNS) myelin involves a coordinated series of events between growing axons and the Schwann cell (SC) progenitors that will eventually ensheath them. Myelin sheaths have evolved out of necessity to maintain rapid impulse propagation while accounting for body space constraints. However, myelinating SCs perform additional critical functions that are required to preserve axonal integrity including mitigating energy consumption by establishing the nodal architecture, regulating axon caliber by organizing axonal cytoskeleton networks, providing trophic and potentially metabolic support, possibly supplying genetic translation materials and protecting axons from toxic insults. Read More

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January 2021

MOG antibody-related disease with recurrent optic neuritis and sensory polyradiculoneuropathy: A case report.

Mult Scler Relat Disord 2020 Nov 21;46:102597. Epub 2020 Oct 21.

Department of Neurology and Strokology, Nagasaki University Hospital, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan.

Myelin oligodendrocyte glycoprotein (MOG) antibody-related diseases are inflammatory demyelinating conditions of the central nervous system (CNS) that induce a broad spectrum of symptoms. Since MOG is expressed exclusively in the CNS, the lesions are thought to be confined to the CNS. However, few cases of MOG antibody-related disease involve the peripheral nervous system (PNS); the mechanisms underlying such PNS involvement remain unclear. Read More

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November 2020

Overlapping central and peripheral nervous system syndromes in MOG antibody-associated disorders.

Neurol Neuroimmunol Neuroinflamm 2021 01 3;8(1). Epub 2020 Dec 3.

From the Inflammatory Neuropathy Group (S. Rinaldi, A.D., J.F.), Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital; University of Oxford; Department of Neurology (S. Rinaldi, S.R.I.), Oxford University Hospitals NHS Foundation Trust, UK; Department of Neurology (H.N.B., M.H.B.), Royal Prince Alfred Hospital, Sydney; Brain and Mind Centre (H.N.B., T.A.H., M.H.B., S.W.R., F.B., R.C.D.), University of Sydney; Department of Neurology (J.W.), St George Hospital, Sydney; Department of Neurology (T.A.H., S.W.R., S. Ramanathan), Concord Repatriation General Hospital, Sydney; Menzies Institute of Health Queensland (S.A.B.), Griffith University; Department of Neurology (S.A.B.), Gold Coast University Hospital, Australia; Autoimmune Neurology Group (P.W., S.R.I., S. Ramanathan), Nuffield Department of Clinical Neuroscience, John Radcliffe Hospital; University of Oxford, UK; Brain Autoimmunity and Clinical Neuroimmunology Groups (F.B., R.C.D., S. Ramanathan), Kids Neuroscience Centre, Kids Research at the Children's Hospital at Westmead, Sydney; Faculty of Medicine and Health (F.B., R.C.D., S. Ramanathan), University of Sydney; School of Medical Sciences (F.B.), Discipline of Applied Medical Science, Faculty of Medicine and Health, University of Sydney, Australia; and TY Nelson Department of Paediatric Neurology (R.C.D.), Children's Hospital at Westmead, Sydney, Australia.

Objective: Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with CNS demyelination inclusive of optic neuritis (ON) and transverse myelitis (TM). To examine whether peripheral nervous system (PNS) involvement is associated with MOG antibody-associated disorders (MOGAD), we performed detailed characterization of an Australasian MOGAD cohort.

Methods: Using a live cell-based assay, we diagnosed 271 adults with MOGAD (2013-2018) and performed detailed clinical and immunologic characterization on those with likely PNS involvement. Read More

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January 2021

Neurotransmitter and neuropeptide regulation of mast cell function: a systematic review.

J Neuroinflammation 2020 Nov 25;17(1):356. Epub 2020 Nov 25.

Department of Pathogen Biology and Immunology, School of Basic Medical Sciences, Nanchang University, 461 Bayi Avenue, Nanchang, 330006, Jiangxi Province, People's Republic of China.

The existence of the neural control of mast cell functions has long been proposed. Mast cells (MCs) are localized in association with the peripheral nervous system (PNS) and the brain, where they are closely aligned, anatomically and functionally, with neurons and neuronal processes throughout the body. They express receptors for and are regulated by various neurotransmitters, neuropeptides, and other neuromodulators. Read More

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November 2020

Is MS affecting the CNS only? Lessons from clinic to myelin pathophysiology.

Neurol Neuroimmunol Neuroinflamm 2021 01 24;8(1). Epub 2020 Nov 24.

From the Department of Anatomy and Neurosciences (E.O., A.L., J.J.G.G., A.-M.v.D.), Department of Neurology (E.M.M.S.), and Department of Child Neurology (E.O.), Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam Neuroscience, the Netherlands.

MS is regarded as a disease of the CNS where a combination of demyelination, inflammation, and axonal degeneration results in neurologic disability. However, various studies have also shown that the peripheral nervous system (PNS) can be involved in MS, expanding the consequences of this disorder outside the brain and spinal cord, and providing food for thought to the still unanswered questions about MS origin and treatment. Here, we review the emerging concept of PNS involvement in MS by looking at it from a clinical, molecular, and biochemical point of view. Read More

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January 2021

Prolonged Neuropsychological Deficits, Central Nervous System Involvement, and Brain Stem Affection After COVID-19-A Case Series.

Front Neurol 2020 5;11:574004. Epub 2020 Nov 5.

Department of Neurology, Medical Faculty, Heinrich-Heine University, Düsseldorf, Germany.

The affection of both the peripheral (PNS) and central nervous system (CNS) by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been assumed to play a direct role in the respiratory failure of patients with Corona virus disease 2019 (COVID-19) through affection of medullary cardiorespiratory centers resulting in neurological complications and sequelae. We used a multimodal electrophysiological approach combined with neuropsychological investigations to study functional alteration of both the PNS and CNS in four patients with severe COVID-19. We found electrophysiological evidence for affection of both the PNS and CNS, and particularly affection of brain stem function. Read More

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November 2020