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Current Opinion in Neurology

Renato Mantegazza, Pia Bernasconi, Paola Cavalcante
PURPOSE OF REVIEW: The current article reviews the recent advances in the field of myasthenia gravis, which span from autoantibody profiling and pathogenic mechanisms to therapy innovation. The overview is highlighting specifically the data and the needs of targeted treatments in the light of precision medicine in myasthenia gravis. RECENT FINDINGS: Novel data published recently further increased our knowledge on myasthenia gravis. The use of cell-based assays has greatly improved autoantibody detection in myasthenia gravis patients, and the mechanisms of action of these antibodies have been described...
October 2018: Current Opinion in Neurology
H Stephan Goedee, W Ludo van der Pol, Jeroen Hendrikse, Leonard H van den Berg
PURPOSE OF REVIEW: This review summarizes the most relevant developments in the fields of nerve ultrasound and MRI in the diagnosis of treatable inflammatory neuropathies over the last 18 months. RECENT FINDINGS: MRI and nerve ultrasound can accurately identify potentially treatable neuropathies and thereby help to improve diagnosis. Advanced MRI techniques also show potential to dissect pathophysiology. The apparent mismatch between nerve function and morphology is not surprising and reflects different dimensions of the disease process in neuropathies...
October 2018: Current Opinion in Neurology
Stephen Keddie, Michael P Lunn
PURPOSE OF REVIEW: To provide an overview of polyneuropathy organomegaly endocrinopathy M-protein and skin changes (POEMS) syndrome, detailing new insights into pathogenesis, prognostic factors, treatments, and outcome scores. RECENT FINDINGS: With the development of large multicentre national cohorts of patients, POEMS syndrome is evolving into a well characterized multisystem hematoneurological syndrome. Without early diagnosis significant disability results from the neuropathy...
October 2018: Current Opinion in Neurology
Katie Beadon, Raquel Guimarães-Costa, Jean-Marc Léger
PURPOSE OF REVIEW: Multifocal motor neuropathy (MMN) has specific clinical and electrophysiologic features but can be difficult to diagnose if cases are not typical. Intravenous immunoglobulin (IVIg) remains the core initial and long-term treatment. In this review, recent advances in the diagnosis, monitoring and treatment of MMN are discussed. RECENT FINDINGS: The pathology of MMN likely depends on immune-mediated attack of the nodes of Ranvier and paranodal regions leading to conduction block...
October 2018: Current Opinion in Neurology
Stephan Kröger
PURPOSE OF REVIEW: Muscle spindles are encapsulated mechanosensory receptors within skeletal muscle tissue that inform the central nervous system about the contractile status of each muscle. This information is required for any coordinated movement and for stable posture. This review summarizes recent findings regarding novel functions for proprioceptive feedback information, muscle spindle disease and the molecular basis of mechanoreception. RECENT FINDINGS: Muscle spindle function is not limited to regulating motor control but is also required for appropriate realignment of fractured bones, successful regeneration of spinal cord axons after injury and spinal alignment...
October 2018: Current Opinion in Neurology
Robert H Baloh, Jonathan D Glass, Clive N Svendsen
PURPOSE OF REVIEW: This review analyses the recent efforts to develop therapeutics using transplantation of stem cells for amyotrophic lateral sclerosis (ALS). RECENT FINDINGS: Stem cells are considered as a potential therapeutic for a variety of neurodegenerative diseases, in an effort to either replace cells that are lost, or to enhance the survival of the remaining cells. In ALS, meaningful attempts to verify the safety and feasibility of many cell transplantation approaches have only recently been completed or are underway...
October 2018: Current Opinion in Neurology
Mamatha Pasnoor, Richard J Barohn, Mazen M Dimachkie
PURPOSE OF REVIEW: Our aim is to highlight major advances reported in the last few years in drug-induced muscle toxicity. RECENT FINDINGS: Our focus is on myopathies induced by statins and immune checkpoint inhibitors with a brief overview of rare steroid myopathies. Statin muscle injury is frequently because of direct toxicity rather than an autoimmune mechanism. Laboratory testing and muscle pathologic features distinguish these two conditions. Statin-associated necrotizing autoimmune myopathy (SANAM) is associated with an autoantibody in 66% of cases targeting the HMGCR enzyme...
October 2018: Current Opinion in Neurology
Claudia Sommer
PURPOSE OF REVIEW: To give an overview of recent data on the use of nerve and skin biopsy as a diagnostic tool in neuropathies. RECENT FINDINGS: Axonal damage in a biopsy from a patient with chronic inflammatory demyelinating polyradiculoneuropathy may point to the presence of autoantibodies to paranodal proteins. In nonsystemic vasculitis of the peripheral nervous system, nerve biopsy is still the only means to make a definite diagnosis. Increased autophagy has been found in idiopathic neuropathy and may also be a common final pathway in various types of neuropathy...
October 2018: Current Opinion in Neurology
Claire L Wood, Volker Straub
PURPOSE OF REVIEW: Muscle and bone are intrinsically linked, and therefore, it is not surprising that many muscular dystrophies are associated with impaired bone health and increased risk of osteoporosis. Osteoporotic fracture is an important and preventable cause of morbidity and mortality. This article will firstly review the general causes of impaired bone health in muscular dystrophies and then focus on the evidence available for the diagnosis and treatment of osteoporosis in specific conditions...
October 2018: Current Opinion in Neurology
Steve Vucic, Seward B Rutkove
PURPOSE OF REVIEW: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder of the motor neurons, characterized by upper motor neuron (UMN) and lower motor neuron (LMN) dysfunction. There have been significant technological advances in the development of neurophysiological biomarkers of UMN and LMN dysfunction in ALS. In this review, we discuss major advances in development of neurophysiological biomarkers in ALS, critiquing their potential in diagnosis and prognosis of ALS, as well as utility in monitoring treatment effects...
October 2018: Current Opinion in Neurology
Alex Y Doets, Bart C Jacobs, Pieter A van Doorn
PURPOSE OF REVIEW: The clinical presentation of Guillain-Barré syndrome (GBS) is highly variable, which can make the diagnosis challenging. Intravenous immunoglobulin (IVIg) and plasma exchange are the cornerstones of treatment since decades. But despite these treatments, 25% initially progress in muscle weakness, 25% require artificial ventilation, 20% is still not able to walk independently after 6 months, and 2-5% die, emphasizing the need for better treatment. We summarize new developments regarding the diagnosis, prognosis, and management of GBS...
October 2018: Current Opinion in Neurology
Carla A Wicki, James V M Hanson, Sven Schippling
PURPOSE OF REVIEW: Optical coherence tomography (OCT) is a noninvasive in-vivo imaging tool that enables the quantification of the various retinal layer thicknesses. Given the frequent involvement of the visual pathway in multiple sclerosis, OCT has become an important tool in clinical practice, research and clinical trials. In this review, the role of OCT as a means to investigate visual pathway damage in multiple sclerosis is discussed. RECENT FINDINGS: Evidence from recent OCT studies suggests that the peripapillary retinal nerve fibre layer (pRNFL) appears to be an ideal marker of axonal integrity, whereas the macular ganglion cell and inner plexiform layer (GCIP) thickness enables early detection of neuronal degeneration in multiple sclerosis...
October 2018: Current Opinion in Neurology
Jason R Thonhoff, Ericka P Simpson, Stanley H Appel
PURPOSE OF REVIEW: Neuroinflammation is increasingly recognized as an important mediator of disease progression in patients with amyotrophic lateral sclerosis (ALS), and is characterized by reactive central nervous system (CNS) microglia and astroglia as well as infiltrating peripheral monocytes and lymphocytes. Anti-inflammatory and neuroprotective factors sustain the early phase of the disease whereas inflammation becomes proinflammatory and neurotoxic as disease progression accelerates...
October 2018: Current Opinion in Neurology
Jordi Díaz-Manera, Sabina Luna, Carles Roig
PURPOSE OF REVIEW: The current article provides a brief summary of the clinical approach to congenital and acquired ptosis. An increasing number of publications analyze causes of ptosis or describe diagnostic tests or advances in ptosis genetics. The aim of our work is to summarize these findings and provide an updated algorithm for the diagnosis and treatment of patients with ptosis. This review covers important clinical research and studies relevant for neurologists recently published...
October 2018: Current Opinion in Neurology
Emanuele Rastelli, Federica Montagnese, Roberto Massa, Benedikt Schoser
PURPOSE OF REVIEW: Myotonic dystrophies are the most frequent muscular dystrophies in adulthood; however, myotonic dystrophy type 2 (DM2) is by far less prevalent than myotonic dystrophy type 1 (DM1). Consequently, studies on large cohorts are lacking and disease-specific outcome measures have not been developed (see video abstract, Supplemental Digital Content 1, aim of this review is to systematically evaluate the outcome measures applied in patients with DM2 and to identify tests adopted from other neuromuscular disorders potentially suitable for DM2...
October 2018: Current Opinion in Neurology
Pietro E Doneddu, Eduardo Nobile-Orazio
PURPOSE OF REVIEW: To review the recent advances in the management and treatment of chronic inflammatory demyelinating polyradiculopathy (CIDP). RECENT FINDINGS: Recent studies confirm the efficacy/safety of long-term intravenous immunoglobulin (IVIg) and short-term subcutaneous immunoglobulin (SCIg) therapy in CIDP. New outcome measures have been recently proposed and further studies evaluated the properties of those already in use. The presence of antibodies against proteins at the node of Ranvier was associated with specific clinical features and treatment response...
October 2018: Current Opinion in Neurology
Nicolai Preisler, Mette Cathrine Orngreen
PURPOSE OF REVIEW: Regular exercise improves muscle and cardiovascular function, which is why exercise is used as an adjuvant treatment in myopathies. In this review, we provide an update on recent exercise studies (from 2016) performed in humans with inherited myopathy. RECENT FINDINGS: Several studies provide new and interesting insight in the field of exercise in myopathies. A retrospective cohort study suggests that exercise may actually increase rate of disease progression in dysferlinopathy, and high intensity exercise, which is normally discouraged in muscle disorders because of the risk of muscle damage, is demonstrated to be an efficient time saving mode of exercise to train patients with facioscapulohumeral muscular dystrophy...
October 2018: Current Opinion in Neurology
Cindy V Ly, Timothy M Miller
PURPOSE OF REVIEW: Amyotrophic lateral sclerosis (ALS) is a rapidly fatal disease for which there is currently no effective therapy. The present review describes the current progress of existing molecular therapies in the clinical trial pipeline and highlights promising future antisense oligonucleotide (ASO) and viral therapeutic strategies for treating ALS. RECENT FINDINGS: The immense progress in the design of clinical trials and generation of ASO therapies directed towards superoxide dismutase-1 (SOD1) and chromosome 9 open reading frame 72 (C9orf72) repeat expansion related disease have been propelled by fundamental work to identify the genetic underpinnings of familial ALS and develop relevant disease models...
October 2018: Current Opinion in Neurology
Nens van Alfen, Kaj Gijsbertse, Chris L de Korte
PURPOSE OF REVIEW: This review focuses on developments in muscle ultrasound as a noninvasive and accurate tool for the diagnosis and follow-up of neuromuscular disease. It discusses current muscle ultrasound applications with already proven clinical value, and highlights recent technical developments that may further advance muscle ultrasounds' diagnostic qualities. RECENT FINDINGS: The sensitivity and specificity of muscle ultrasound for detecting a neuromuscular disorder are high (90-95%), and quantitative ultrasound is well suited to monitor disease progression in several disorders...
October 2018: Current Opinion in Neurology
Benedikt Schoser
No abstract text is available yet for this article.
October 2018: Current Opinion in Neurology
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