Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jun 2024
Case ReportsExcellent response to anti-CD38 therapy with daratumumab in a patient with severe refractory CANOMAD.
Intravenous immunoglobulin (IVIG) and rituximab are considered the first-line and second-line treatments for Chronic Ataxic Neuropathy and Ophthalmoplegia with IgM-paraprotein, cold Agglutinins, and anti-Disialosyl antibodies (CANOMAD), with an overall clinical response around 50%. New anti-CD38 daratumumab, targeting long-lived plasma cells, has been reported as a promising therapy for treatment-refractory antibody-mediated disorders. We report the first case of a severe refractory CANOMAD, successfully treated with daratumumab. ⋯ The first patient with aggressive treatment-refractory CANOMAD treated with daratumumab provides proof-of-principle evidence that daratumumab may be an effective treatment in IgM-related neuropathies.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2024
Prevalence and burden of pain across the entire spectrum of Huntington's disease.
Pain is an important symptom in Huntington's disease (HD), however, not systematically studied and understood. The objective of the current study is to assess the prevalence of pain, pain interference in daily activities, painful conditions, analgesic use and the severity of the pain burden across different disease stages and 'Age at symptom Onset' groups. Additionally, the association between pain and disease burden was investigated. ⋯ Pain is a prevalent and important symptom in HD. Severe pain burden in the HD population is present and positively associated with disease burden. Risk for undertreatment with analgesics is nevertheless present. Awareness of pain in HD needs to be increased, both clinically and scientifically.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2024
Meta AnalysisIncidence and determinants of seizures in multiple sclerosis: a meta-analysis of randomised clinical trials.
Seizures are reported to be more prevalent in individuals with multiple sclerosis (MS) compared with the general population. Existing data predominantly originate from population-based studies, which introduce variability in methodologies and are vulnerable to selection and reporting biases. ⋯ Patients with MS face a nearly twofold higher seizure risk compared with the general population. This risk appears to be associated not only with disease burden but also with S1PR modulators. Our findings underscore epilepsy as a significant comorbidity in MS and emphasise the necessity for further research into its triggers, preventive measures and treatment strategies.
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J. Neurol. Neurosurg. Psychiatr. · Jun 2024
ReviewNICE guideline on ME/CFS: robust advice based on a thorough review of the evidence.
In 2021, the National Institute for Health and Care Excellence produced an evidence-based guideline on the diagnosis and management of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), a disabling long-term condition of unknown cause. The guideline provides clear support for people living with ME/CFS, their families and carers, and for clinicians. A recent opinion piece published in the journal suggested that there were anomalies in the processing and interpretation of the evidence when developing the guideline and proposed eight areas where these anomalies were thought to have occurred. We outline how these opinions are based on a misreading or misunderstanding of the guideline process or the guideline, which provides a balanced and reasoned approach to the diagnosis and management of this challenging condition.