Novel pathophysiological insights in autoimmune myasthenia gravis
Purpose of review
This review summarizes recent insights into the immunopathogenesis of autoimmune myasthenia gravis (MG). Mechanistic understanding is presented according to MG disease subtypes and by leveraging the knowledge gained through the use of immunomodulating biological therapeutics.
Recent findings
The past two years of research on MG have led to a more accurate definition of the mechanisms through which muscle-specific tyrosine kinase (MuSK) autoantibodies induce pathology. Novel insights have also emerged from the collection of stronger evidence on the pathogenic capacity of low-density lipoprotein ...
Source: Current Opinion in Neurology - September 13, 2022 Category: Neurology Tags: PERIPHERAL NERVE AND NEURO-MUSCULAR JUNCTION DISEASE: Edited by Luis Querol Source Type: research
Autoimmune nodopathies, an emerging diagnostic category
Purpose of review
In the last decade, antibodies targeting cell adhesion molecules of the node of Ranvier were described in patients with autoimmune neuropathies. These nodal/paranodal antibodies associate with specific clinicopathological features that are different from classical chronic inflammatory demyelinating polyneuropathy (CIDP). In this review, we will summarize recent findings establishing autoimmune nodopathies (AN) as a new category of autoimmune neuropathies.
Recent findings
AN include anti-contactin 1, anti-contactin-associated protein 1, anti-neurofascin 155 and anti-pan-neurofascin antibody-medi...
Source: Current Opinion in Neurology - September 13, 2022 Category: Neurology Tags: PERIPHERAL NERVE AND NEURO-MUSCULAR JUNCTION DISEASE: Edited by Luis Querol Source Type: research
Guillain-Barré syndrome in an era of global infections and 21st century vaccination
Purpose of review
Guillain-Barre syndrome is sometimes a severe and disabling postinfectious neuromuscular paralysis that is causally associated with a number of well defined infections, and occasionally with immunization. The severe acute respiratory syndrome coronavirus 2 (Sars-CoV-2) pandemic and the worldwide immunization programme provoked fears of an epidemic of coronavirus disease 2019 (COVID-19) related disease. As we emerge from the pandemic this review summarises some of the huge volume of publications about Guillain-Barre syndrome (GBS), COVID-19 and immunisation against it.
Recent findings
In the ear...
Source: Current Opinion in Neurology - September 13, 2022 Category: Neurology Tags: PERIPHERAL NERVE AND NEURO-MUSCULAR JUNCTION DISEASE: Edited by Luis Querol Source Type: research
Hereditary motor neuropathies
Purpose of review
Distal hereditary motor neuropathies (dHMN) are a clinically and genetically diverse group of disorders that are characterized by length-dependent axonal degeneration of lower motor neurons. In this review, we will provide an overview of dHMN, and we will correlate the distinct clinical subtypes with their causative genes, focusing on the most recent advances in the field.
Recent findings
Despite the massive use of new-generation sequencing (NGS) and the discovery of new genes, only a third of dHMN patients receive a molecular diagnosis. Thanks to international cooperation between researchers, ...
Source: Current Opinion in Neurology - September 13, 2022 Category: Neurology Tags: PERIPHERAL NERVE AND NEURO-MUSCULAR JUNCTION DISEASE: Edited by Luis Querol Source Type: research
Sensory neuronopathies, diagnostic criteria and causes
Purpose of review
To stress on the diagnostic strategy of sensory neuronopathies (SNN), including new genes and antibodies.
Recent finding
SNN involve paraneoplastic, dysimmune, toxic, viral and genetic mechanisms. About one-third remains idiopathic. Recently, new antibodies and genes have reduced this proportion. Anti-FGFR3 and anti-AGO antibodies are not specific of SNN, although SNN is predominant and may occur with systemic autoimmune diseases. These antibodies are the only marker of an underlying dysimmune context in two-thirds (anti-FGFR3 antibodies) and one-third of the cases (anti-AGO antibodies), respec...
Source: Current Opinion in Neurology - September 13, 2022 Category: Neurology Tags: PERIPHERAL NERVE AND NEURO-MUSCULAR JUNCTION DISEASE: Edited by Luis Querol Source Type: research
Small fiber neuropathies: expanding their etiologies
Purpose of review
Several conditions have been associated with the development of small fiber neuropathy (SFN). The list of metabolic, immune-mediated, infectious, toxic, drugs-related, and hereditary conditions is still growing and various hypotheses are made about the underlying pathophysiological mechanisms. Understanding these processes is important to provide new targets for treatment. In addition, the specific SFN phenotype can provide direction for the underlying etiology. This review discusses the latest developments concerning the expanding etiologies in SFN.
Recent findings
In the past 18 months, spe...
Source: Current Opinion in Neurology - September 13, 2022 Category: Neurology Tags: PERIPHERAL NERVE AND NEURO-MUSCULAR JUNCTION DISEASE: Edited by Luis Querol Source Type: research
Editorial introductions
No abstract available (Source: Current Opinion in Neurology)
Source: Current Opinion in Neurology - September 13, 2022 Category: Neurology Tags: EDITORIAL INTRODUCTIONS Source Type: research
Historical development and contemporary use of neuromodulation in human spinal cord injury
Purpose of review
There is a long history of neuromodulation of the spinal cord after injury in humans with recent momentum of studies showing evidence for therapeutic potential. Nonrandomized, mechanistic, hypothesis-driven, small cohort, epidural stimulation proof of principle studies provide insight into the human spinal circuitry functionality and support the pathway toward clinical treatments.
Recent findings
Individuals living with spinal cord injury can recover motor, cardiovascular, and bladder function even years after injury using neuromodulation. Integration of continuous feedback from sensory informa...
Source: Current Opinion in Neurology - July 28, 2022 Category: Neurology Tags: SPECIAL COMMENTARY Source Type: research
Update in the clinical application of focused ultrasound
Purpose of review
To review the most recent evidence about the clinical applicability of transcranial MRI-guided focused ultrasound (MRgFUS), including clinical evidence and indications, recent technical developments for its use and future prospects.
Recent findings
Unilateral MRgFUS thalamotomy for both essential and parkinsonian tremors is an approved and well established therapy. Recent studies have focused on its long-term safety and efficacy as well as technical advances for refining the approach. Moreover, ultrasound has expanded its application in Parkinson's disease, with clinical trials successfully tar...
Source: Current Opinion in Neurology - July 28, 2022 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Marie Vidailhet Source Type: research
Connecting tremors – a circuits perspective
Purpose of review
Tremor is one of the most prevalent movement disorders in clinical practice. Here, we review new insights in the pathophysiology of tremor. We focus on the three most common tremor disorders: essential tremor (ET), dystonic tremor syndrome (DTS), and Parkinson's disease (PD) tremor.
Recent findings
Converging evidence suggests that ET, DTS, and PD tremor are all associated with (partly) overlapping cerebral networks involving the basal ganglia and cerebello-thalamo-cortical circuit. Recent studies have assessed the role of these networks in tremor by measuring tremor-related activity and connec...
Source: Current Opinion in Neurology - July 28, 2022 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Marie Vidailhet Source Type: research
Deep brain stimulation in dystonia: factors contributing to variability in outcome in short and long term follow-up
Purpose of review
Deep brain stimulation (DBS) is currently the most effective treatment for medically refractory dystonia with globus pallidus internus (GPi) usually the preferred target. Despite the overall success of DBS in dystonia, there remains variability in treatment outcome in both short and long-term follow-up, due to various factors. Factors contributing to variability in outcome comprise ’Dystonia Related’ including dystonia classification, semiology, duration, body distribution, orthopaedic deformity, aetiology and genetic cause. The majority of these factors are identifiable from clinical assessment, ...
Source: Current Opinion in Neurology - July 28, 2022 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Marie Vidailhet Source Type: research
The apparent paradox of phenotypic diversity and shared mechanisms across dystonia syndromes
We describe here how such mechanisms shared by different genetic forms can give rise to motor performance dysfunctions with a clinical aspect of dystonia.
Recent findings
The continuing discoveries of genetic causes for dystonia syndromes are transforming our view of these disorders. They share unexpectedly common underlying mechanisms, including dysregulation in neurotransmitter signaling, gene transcription, and quality control machinery. The field has further expanded to include forms recently associated with endolysosomal dysfunction.
Summary
The discovery of biological pathways shared between different m...
Source: Current Opinion in Neurology - July 28, 2022 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Marie Vidailhet Source Type: research
Movement disorders in COVID-19 times: impact on care in movement disorders and Parkinson disease
Purpose of review
The purpose of this review is to outline the impact of the COVID-19 pandemic on movement disorder holistic care, particularly in the care of people with Parkinson disease (PWP).
Recent findings
As the pandemic unfolds, a flurry of literature was published regarding the impact of COVID-19 on people with Parkinson disease including the direct impact of infection, availability of ambulatory care, loss of community-based team care, and acceptability of telemedicine.
Summary
COVID-19 has impacted the care of PWP in numerous ways. Recognizing infection in PWP poses challenges. Specific long-te...
Source: Current Opinion in Neurology - July 28, 2022 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Marie Vidailhet Source Type: research
The recent surge of functional movement disorders: social distress or greater awareness?
Purpose of review
To analyze the interrelated factors that have led to the striking increase in functional movement disorders in recent years, with a focus on functional tic-like behaviours (FTLB).
Recent findings
The sudden onset of FTLB has been widely observed in several countries since the beginning of the SARS-CoV-2 pandemic, whereas it was previously very rarely reported. Pandemic-related FTLB typically occur in young females, share complex, disabling and tic-lookalike patterns, and are usually triggered by the exposure to videos portraying tic-like behaviours on social media. Both pandemic-related FTLB an...
Source: Current Opinion in Neurology - July 28, 2022 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Marie Vidailhet Source Type: research
Editorial: Innovation and resilience in movement disorders: abundance of creative science despite the covid-19 period
No abstract available (Source: Current Opinion in Neurology)
Source: Current Opinion in Neurology - July 28, 2022 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Marie Vidailhet Source Type: research
