Journal of neurology, neurosurgery, and psychiatry
-
J. Neurol. Neurosurg. Psychiatr. · Dec 2024
Predictors of a relapsing course in myelin oligodendrocyte glycoprotein antibody-associated disease.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently described demyelinating disorder, and children represent about 50% of all cases. Almost half of the patients experience relapses, but very few studies have evaluated predictors of relapse risk, challenging clinical management. The study aimed to identify predictors at MOGAD onset that are associated with a relapsing course. ⋯ Sex and ethnicity are associated with relapsing MOGAD. Use of rituximab or IVIG therapy shortly after onset is associated with a lower risk of the second event. Preventive treatment after a first event could be considered for those with a higher relapse risk.
-
J. Neurol. Neurosurg. Psychiatr. · Dec 2024
Phenotypic spectrum of myelin protein zero-related neuropathies: a large cohort study from five mutation clusters across Italy.
We aimed to investigate the clinical features of a large cohort of patients with myelin protein zero (MPZ)-related neuropathy, focusing on the five main mutation clusters across Italy. ⋯ This is the largest MPZ (and late-onset CMT2) cohort ever collected, reporting clinical features and disease progression of 186 patients from five different clusters across Italy. Our findings corroborate the importance of differentiating between 'classical' childhood-onset demyelinating, late-onset axonal and mild MPZ-related neuropathy, characterised by different pathomechanisms, in view of different therapeutic targets.
-
J. Neurol. Neurosurg. Psychiatr. · Dec 2024
Sodium valproate is associated with cortical thinning of disease-specific areas in juvenile myoclonic epilepsy.
Juvenile myoclonic epilepsy (JME) is associated with cortical thinning of the motor areas. The relative contribution of antiseizure medication to cortical thickness is unknown. We aimed to investigate how valproate influences the cortical morphology of JME. ⋯ Valproate was associated with JME-specific and dose-dependent thinning of the cortical motor regions. This suggests that valproate is a key modulator of cortical morphology in JME, an effect that may underlie its high efficacy in this syndrome.
-
J. Neurol. Neurosurg. Psychiatr. · Dec 2024
First-ever seizure and eligibility for commercial motor vehicle driving.
After a first-ever seizure, 6 months of seizure freedom is usually required before returning to driving a private motor vehicle, after which the annual risk of seizure recurrence has fallen to ≤20%. Stricter criteria apply for commercial driver's licence (CDL) holders, and a longer period of seizure freedom sufficient for the annual risk of recurrence to be <2% is recommended. However, CDL guidelines are based on little data with few studies having long-term follow-up. ⋯ For unprovoked and acute symptomatic first-ever seizure and CDL, a higher-than-expected annual seizure risk persists beyond the currently recommended seizure-free periods, even in those without risk factors for recurrence. Our data can inform decisions regarding a return to driving for CDL holders after first-ever seizure.
-
J. Neurol. Neurosurg. Psychiatr. · Dec 2024
The relationship between corticospinal excitability and structural integrity in stroke patients.
Evaluation of the structural integrity and functional excitability of the corticospinal tract (CST) is likely to be important in predicting motor recovery after stroke. Previous reports are inconsistent regarding a possible link between CST structure and CST function in this setting. This study aims to investigate the structure‒function relationship of the CST at the acute phase of stroke (<7 days). ⋯ This large multimodal study on the structure/function of the CST and stroke recovery proposes a paradigm change for the MEP-positive patients phenotypes and refines the nature of the link between structural integrity and neurophysiological function, with implications for study design and prognostic information.