Journal of neurology, neurosurgery, and psychiatry
-
J. Neurol. Neurosurg. Psychiatr. · Jun 2023
Effectiveness of early cycles of fast-acting treatment in generalised myasthenia gravis.
Early fast-acting treatment (EFT) is the aggressive use of fast-acting therapies such as plasmapheresis, intravenous immunoglobulin and/or intravenous high-dose methylprednisolone (IVMP) from the early phases of treatment. EFT is reportedly beneficial for early achievement of minimal manifestations (MM) or better status with ≤5 mg/day prednisolone (MM5mg), a practical therapeutic target for myasthenia gravis (MG). ⋯ Early cycles of intervention with EFT and early use of calcineurin inhibitors provides long-term benefits in terms of achieving therapeutic targets for generalised MG, regardless of clinical subtype.
-
The pathogenic missense mutations of the gelsolin (GSN) gene lead to familial amyloidosis of the Finnish type (FAF); however, our previous study identified GSN frameshift mutations existed in patients with Alzheimer's disease (AD). The GSN genotype-phenotype heterogeneity and the role of GSN frameshift mutations in patients with AD are unclear. ⋯ GSN frameshift mutations may be associated with AD. An increase in plasma GSN is probably a compensatory reaction in AD, which is a potential biomarker for early AD.
-
J. Neurol. Neurosurg. Psychiatr. · Jun 2023
Ocrelizumab concentration and antidrug antibodies are associated with B-cell count in multiple sclerosis.
The majority of patients with multiple sclerosis on ocrelizumab have B-cell depletion after standard interval dosing of 26 weeks. With B-cell-guided dosing patients receive their next dose when B-cell repopulation occurs. Prediction of B-cell repopulation using ocrelizumab concentrations could aid in personalising treatment regimes. The objectives of this study were to evaluate the association between ocrelizumab drug concentration, antidrug antibodies (ADAs) and CD19 B-cell count, and to define a cut-off ocrelizumab concentration for start of B-cell repopulation (defined by ≥10 CD19+ B cells/µL). ⋯ Serum ocrelizumab concentration was strongly associated with B-cell count. Measurement of ocrelizumab drug concentrations and ADAs could play an important role to further personalise treatment and predict the start of B-cell repopulation.
-
J. Neurol. Neurosurg. Psychiatr. · Jun 2023
Elevated plasma p-tau181 levels unrelated to Alzheimer's disease pathology in amyotrophic lateral sclerosis.
Phosphorylated-tau181 (p-tau181), a specific marker of Alzheimer's disease (AD) pathology, was found elevated in plasma but not in cerebrospinal fluid (CSF) of patients with amyotrophic lateral sclerosis (ALS). We expanded these findings in a larger patient cohort, exploring clinical/electrophysiological associations, prognostic value and longitudinal trajectories of the biomarker. ⋯ Plasma p-tau181 is elevated in patients with ALS, independently from CSF levels, and is firmly associated with LMN dysfunction. The finding indicates that p-tau181 of putative peripheral origin might represent a confounding factor in using plasma p-tau181 for AD pathology screening, which deserves further investigation.
-
J. Neurol. Neurosurg. Psychiatr. · Jun 2023
ReviewStructural and functional changes in the retina in Parkinson's disease.
Parkinson's disease is caused by degeneration of dopaminergic neurons, originating in the substantia nigra pars compacta and characterised by bradykinesia, rest tremor and rigidity. In addition, visual disorders and retinal abnormalities are often present and can be identified by decreased visual acuity, abnormal spatial contrast sensitivity or even difficulty in complex visual task completion. Because of their early onset in patients with de novo Parkinson's disease, the anatomical retinal changes and electrophysiological modification could be valuable markers even at early stages of the disease. ⋯ We review the underlying chemical changes seen with loss of retinal dopaminergic neurons and the effect of levodopa treatment on the retina in Parkinson's disease. Finally, we consider whether retinal abnormalities in Parkinson's disease could have a role as potential markers of poorer outcomes and help stratify patients at early stages of the disease. We emphasise that retinal measures can be valuable, accessible and cost-effective methods in the early evaluation of Parkinson's disease pathogenesis with potential for patient stratification.