Lancet neurology
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Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins (PrPSc). Effective therapeutics are currently not available and accurate diagnosis can be challenging. Clinical diagnostic criteria use a combination of characteristic neuropsychiatric symptoms, CSF proteins 14-3-3, MRI, and EEG. ⋯ Development and clinical application of disease-specific protein aggregation and amplification assays, such as real-time quaking induced conversion (RT-QuIC), have constituted major breakthroughs for the confident pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. Updated criteria for the diagnosis of sporadic Creutzfeldt-Jakob disease, including application of RT-QuIC, should improve early clinical confirmation, surveillance, assessment of PrPSc seeding activity in different tissues, and trial monitoring. Moreover, emerging blood-based, prognostic, and potentially pre-symptomatic biomarker candidates are under investigation.
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The field of acquired CNS neuroimmune demyelination in children is transforming. Progress in assay development, refinement of diagnostic criteria, increased biological insights provided by advanced neuroimaging techniques, and high-level evidence for the therapeutic efficacy of biological agents are redefining diagnosis and care. ⋯ However, doing clinical trials is challenging because of the rarity of these conditions in the paediatric age group, necessitating new approaches to trial design, including age-based trajectory modelling based on phase 3 studies in adults. Despite these limitations, the future for children and adolescents living with multiple sclerosis, MOGAD, or AQP4-NMOSD is far brighter than in years past, and will be brighter still if successful therapies to promote remyelination, enhance neuroprotection, and remediate cognitive deficits can be further accelerated.
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Poor quality diet and nutrition is strongly associated with risk of first stroke, and adherence to a Mediterranean-style diet has been reported to reduce the risk of first stroke. The association between diet quality and the risk of recurrent stroke is less certain and there is no reliable evidence that improving diet quality or dietary supplementation reduces recurrent stroke risk. ⋯ In the absence of reliable evidence from randomised clinical trials, the GRADE (Grading of Recommendations Assessment, Development, and Evaluation) approach can be used to assess the potential causal role of diet quality and interventions in reducing recurrent stroke, and to provide guidance for clinical practice and directions for future research. Further work is needed to identify and develop the most promising dietary interventions for evaluation by large randomised clinical trials.
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Review Case Reports
SARS-CoV-2 and the risk of Parkinson's disease: facts and fantasy.
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Neuromyelitis optica spectrum disorder is an autoimmune disease of the CNS that primarily affects the optic nerves and spinal cord. Most patients have serum antibodies targeting the aquaporin-4 water channel expressed on the end-feet of astrocytes. Although the prevalence of neuromyelitis optica spectrum disorder is limited to around 1-2 people per 100 000, severe immune-mediated attacks can quickly lead to blindness and paralysis if undiagnosed and untreated. However, diagnosis is straightforward when the highly specific serum aquaporin-4 antibodies are detected with cell-based assays. ⋯ Four randomised controlled trials have tested the efficacy of three new therapies (eculizumab, satralizumab, and inebilizumab) for patients with neuromyelitis optica spectrum disorder that all showed a benefit in preventing future attacks. These therapies have different targets within the immune pathogenic process, and the four trials have similarities and differences that mean they might change the therapeutic landscape for people with neuromyelitis optica spectrum disorder in different ways. Efficacy, safety, tolerability, and practical considerations, including potential cost, differ for each drug and might affect the rate of use in real-world populations of patients with neuromyelitis optica spectrum disorder. WHERE NEXT?: Despite the rarity of neuromyelitis optica spectrum disorder, a relative abundance of preventive treatment options now exists. In the future, trials should focus on areas of unmet need, including aquaporin-4 seronegative disease, and on development of treatments for acute relapses and for recovery from autoimmune attacks in the CNS.