Progress in the therapy of myasthenia gravis: getting closer to effective targeted immunotherapies
Purpose of review
To provide an update on immunomodulating and immunosuppressive therapies in myasthenia gravis and highlight newly approved, or pending approval, therapies with new biologics.
Recent findings
Preoperative IVIg is not needed to prevent myasthenic crisis in stable myasthenia gravis patients scheduled for surgery under general anesthesia, based on controlled data. Rituximab, if initiated early in new-onset myasthenia gravis, can lead to faster and more sustained remission even without immunotherapies in 35% of patients at 2 years. Biomarkers determining the timing for follow-up infusions in Rituximab-res...
Source: Current Opinion in Neurology - September 4, 2020 Category: Neurology Tags: PERIPHERAL NERVE AND NEURO-MUSCULAR JUNCTION DISEASE: Edited by Claudia Sommer Source Type: research
Editorial: Management of rare neuromuscular diseases
No abstract available (Source: Current Opinion in Neurology)
Source: Current Opinion in Neurology - September 4, 2020 Category: Neurology Tags: PERIPHERAL NERVE AND NEURO-MUSCULAR JUNCTION DISEASE: Edited by Claudia Sommer Source Type: research
Editorial introductions
No abstract available (Source: Current Opinion in Neurology)
Source: Current Opinion in Neurology - September 4, 2020 Category: Neurology Tags: EDITORIAL INTRODUCTIONS Source Type: research
Wilson disease
Purpose of review
The aim of this article is to review recent developments in the areas of the disease features and treatment of Wilson disease, and survey disorders that share its pathophysiology or clinical symptoms.
Recent findings
Knowledge of the clinical spectrum of Wilson disease has expanded with recognition of patients who present in atypical age groups – patients with very early onset ( (Source: Current Opinion in Neurology)
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Nutan Sharma Source Type: research
Immunotherapy in progressive supranuclear palsy
Purpose of review
Progressive supranuclear palsy (PSP) is a progressive adult-onset neurodegenerative disease. Abnormally, phosphorylated forms of the microtubule-associated protein tau containing four repeat domains (4R-tau) aggregate in neurons. Additionally, increasing evidence suggests that secretion and uptake of fragments of abnormal 4R-tau may play a role in disease progression. This extracellular tau is a natural target for immunotherapy.
Recent findings
Three monoclonal antibodies targeting extracellular tau are in clinical stages of development. ABBV-8E12 and BIIB092 were safe in Phase 1, but both Phase two ...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Nutan Sharma Source Type: research
Recent therapeutic prospects for Machado–Joseph disease
Purpose of review
Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is a fatal, dominantly inherited, neurodegenerative disease caused by expansion of a CAG repeat in the coding region of the ATXN3 gene. No disease-modifying treatment is yet available for MJD/SCA3. This review discusses recently developed therapeutic strategies that hold promise as future effective treatments for this incurable disease.
Recent findings
As a result of the exploration of multiple therapeutic approaches over the last decade, the MJD/SCA3 field is finally starting to see options for disease-modifying trea...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Nutan Sharma Source Type: research
Therapeutic strategies for Huntington's disease
Purpose of review
Huntington's disease is a fatal autosomal dominant neurodegenerative disorder caused by a trinucleotide expansion in the HTT gene, and current therapies focus on symptomatic treatment. This review explores therapeutic approaches that directly target the pathogenic mutation, disrupt HTT mRNA or its translation.
Recent findings
Zinc-finger transcription repressors and CRISPR-Cas9 therapies target HTT DNA, thereby preventing all downstream pathogenic mechanisms. These therapies, together with RNA interference (RNAi), require intraparenchymal delivery to the brain in viral vectors, with only a single del...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Nutan Sharma Source Type: research
Recent genetic advances in early-onset dystonia
Purpose of review
The discovery of new disease-causing genes and availability of next-generation sequencing platforms have both progressed rapidly over the last few years. For the practicing neurologist, this presents an increasingly bewildering array both of potential diagnoses and of means to investigate them. We review the latest newly described genetic conditions associated with dystonia, and also address how the changing landscape of gene discovery and genetic testing can best be approached, from both a research and a clinical perspective.
Recent findings
Several new genetic causes for disorders in which dystonia...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Nutan Sharma Source Type: research
Editorial: Advances in rare movement disorders
No abstract available (Source: Current Opinion in Neurology)
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: MOVEMENT DISORDERS: Edited by Nutan Sharma Source Type: research
Magnetic resonance-guided focused ultrasound for movement disorders: clinical and neuroimaging advances
Purpose of review
To describe the path of technological developments that have led to the actual application of MRI-guided focused ultrasound in multiple neurological disorders and to update the more recent technical advances in the field. An insight into the latest clinical achievements in movement disorders will be provided, together with the neuroimaging advances for the screening, planning, and outcome evaluation.
Recent findings
Developments, such as phased array transducers and MRI guidance have allowed the use of focused ultrasound to successfully perform incisionless therapeutic ablation in deep brain structur...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: NEUROIMAGING: Edited by Stéphane Lehéricy Source Type: research
Prediction of motor recovery after stroke: being pragmatic or innovative?
Purpose of review
This review considers both pragmatic and cutting-edge approaches for predicting motor stroke recovery over the period 2017–2019. It focuses on the predictive value of clinical scores and biomarkers including Transcranial Magnetic Stimulation (TMS) and MRI as well as more innovative alternatives.
Recent findings
Clinical scores combined with corticospinal tract (CST) integrity as assessed by both TMS-induced motor-evoked potential (MEP) and MRI predict motor recovery with an accuracy of about 75%. Therefore, research on novel biomarkers is still needed to improve the accuracy of these models.
Summa...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: NEUROIMAGING: Edited by Stéphane Lehéricy Source Type: research
Tremor pathophysiology: lessons from neuroimaging
Purpose of review
We discuss the latest neuroimaging studies investigating the pathophysiology of Parkinson's tremor, essential tremor, dystonic tremor and Holmes tremor.
Recent findings
Parkinson's tremor is associated with increased activity in the cerebello-thalamo-cortical circuit, with interindividual differences depending on the clinical dopamine response of the tremor. Although dopamine-resistant Parkinson's tremor arises from a larger contribution of the (dopamine-insensitive) cerebellum, dopamine-responsive tremor may be explained by thalamic dopamine depletion. In essential tremor, deep brain stimulation nor...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: NEUROIMAGING: Edited by Stéphane Lehéricy Source Type: research
MRI of neurodegeneration with brain iron accumulation
This article details the contribution of MRI in the diagnosis by summarizing and comparing MRI patterns of the various NBIA subtypes.
Recent findings
MRI almost always shows characteristic changes combining iron accumulation and additional neuroimaging abnormalities. Iron-sensitive MRI shows iron deposition in the basal ganglia, particularly in bilateral globus pallidus and substantia nigra. Other regions may be affected depending on the NBIA subtypes including the cerebellum and dentate nucleus, the midbrain, the striatum, the thalamus, and the cortex. Atrophy of the cerebellum, brainstem, corpus callosum and cortex, a...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: NEUROIMAGING: Edited by Stéphane Lehéricy Source Type: research
MR imaging and spectroscopy in degenerative ataxias: toward multimodal, multisite, multistage monitoring of neurodegeneration
Purpose of review
Degenerative ataxias are rare and currently untreatable movement disorders, primarily characterized by neurodegeneration in the cerebellum and brainstem. We highlight MRI studies with the most potential for utility in pending ataxia trials and underscore advances in disease characterization and diagnostics in the field.
Recent findings
With availability of advanced MRI acquisition methods and specialized software dedicated to the analysis of MRI of the cerebellum, patterns of cerebellar atrophy in different degenerative ataxias are increasingly well defined. The field further embraced rigorous multim...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: NEUROIMAGING: Edited by Stéphane Lehéricy Source Type: research
Machine learning for classification and prediction of brain diseases: recent advances and upcoming challenges
Purpose of review
Machine learning is an artificial intelligence technique that allows computers to perform a task without being explicitly programmed. Machine learning can be used to assist diagnosis and prognosis of brain disorders. Although the earliest articles date from more than ten years ago, research increases at a very fast pace.
Recent findings
Recent works using machine learning for diagnosis have moved from classification of a given disease versus controls to differential diagnosis. Intense research has been devoted to the prediction of the future patient state. Although a lot of earlier works focused on n...
Source: Current Opinion in Neurology - July 30, 2020 Category: Neurology Tags: NEUROIMAGING: Edited by Stéphane Lehéricy Source Type: research
