Neurology Alert
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Patterns of Weakness in Amyotrophic Lateral Sclerosis
Unusual patterns of weakness in the various motor neuron disorders may point to a specific diagnosis of amyotrophic lateral sclerosis, if supported by electrophysiological evidence of chronic denervation.
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The Natural History of Leigh Syndrome
The natural history of Leigh syndrome is characterized by quantifiable disease progression in fewer than three years, with the poorest outcomes predicted by surfeit locus protein 1 (SURF1) pathogenic variants, bilateral caudate involvement on magnetic resonance imaging, and rapid increases in yearly Newcastle Paediatric Mitochondrial Disease Scale scores.
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White Matter Hyperintensities and Progression of Parkinsonism in Older Adults
Both higher levels of cerebral white matter hyperintensities and cerebrovascular disease pathologies may be associated with a more rapid progression of parkinsonism in older adults.
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Rituximab Treatment for AChR Myasthenia Gravis: Results of the BeatMG Study
The B-Cell Targeted Treatment In Myasthenia Gravis (BeatMG) study was the first prospective, randomized, double-blind, placebo-controlled clinical trial to explore the role of targeted B-cell depletion in myasthenia patients. Although rituximab was found to be safe and well-tolerated, rituximab treatment did not confer a significant steroid-sparing effect compared to placebo. Results of a futility analysis suggested rituximab treatment would be unlikely to show a clinically meaningful improvement of 30% over placebo in a larger Phase III trial of mild to moderately symptomatic acetylcholine receptor autoantibody-positive generalized myasthenia gravis patients.
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Plasma Biomarkers for Neurodegenerative Dementias: We Are Getting Closer
Two recent multicenter studies suggest that plasma biomarkers, including phosphorylated-tau181, could be used as cost-effective and more readily accessible biomarkers for the diagnosis and management of individuals with neurodegenerative dementias.
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Is Physical Activity Associated with Mortality Risk in Parkinson’s Disease?
In individuals with Parkinson’s disease (PD), physical activity (PA) at all intensities is associated with reduced all-cause mortality, with the greatest reduction seen in individuals who maintained PA before and after PD diagnosis.
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The Effect of Disease-Modifying Therapies for Multiple Sclerosis on the Immune Responses to COVID-19 Vaccination
An analysis of a large group of patients with multiple sclerosis taking various disease-modifying therapies showed that the response to COVID-19 vaccination was not uniform across the therapies. Patients taking anti-CD20 therapies and fingolimod had attenuated responses to vaccines.
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Diet and the Risk of Dementia
Specific dietary patterns, such as the Mediterranean-style diet, have been shown to promote brain health, mitigate neuro-inflammation, and reduce the risk of dementia. This prospective cohort study demonstrated an association between a high inflammatory potential diet and increased risk for incident dementia.
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Acute Muscle Weakness in Children: Acute Flaccid Myelitis or Guillain-Barré Syndrome?
Compared to children with Guillain-Barré syndrome, children with acute flaccid myelitis have a more rapid presentation to nadir of weakness, fewer sensory abnormalities, and an inflammatory spinal fluid early in the course.
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The Effect of Epilepsy on Patients with Brain Tumors
Cancer cells form direct synapses with neurons, whose electrical activity stimulates brain tumor growth. Accumulating evidence suggests that epilepsy enhances the proliferation of malignant brain tumors and that improved management of epilepsy may reduce morbidity and mortality in these patients.