Journal of Neurology最新文献

Implanted cortical neuroprosthetics (ICNs) are medical devices developed to replace dysfunctional neural pathways by creating information exchange between the brain and a digital system which can facilitate interaction with the external world. Over the last decade, researchers have explored the application of ICNs for diverse conditions including blindness, aphasia, and paralysis. Both transcranial and endovascular approaches have been used to record neural activity in humans, and in a laboratory setting, high-performance decoding of the signals associated with speech intention has been demonstrated. Particular progress towards a device which can move into clinical practice has been made with ICNs focussed on the restoration of speech and movement. This article provides an overview of contemporary ICNs for speech and movement restoration, their mechanisms of action and the unique ethical challenges raised by the field.

Background: Optic neuritis (ON) is a common manifestation of multiple sclerosis (MS) and myelin-oligodendrocyte-glycoprotein IgG-associated disease (MOGAD). This study evaluated the applicability of optical coherence tomography (OCT) for differentiating between both diseases in two independent cohorts.

Methods: One hundred sixty two patients from seven sites underwent standard OCT and high-contrast visual acuity (HCVA) testing at least 6 months after first ON. Of these, 100 patients (32 MOGAD, 68 MS) comprised the primary investigational cohort, while 62 patients (31 MOGAD, 31 MS) formed a validation cohort. A composite score distinguishing between MOGAD and MS was developed using multivariate logistic regression.

Results: Bilateral simultaneous ON occurred more frequently in MOGAD compared to MS (46.9 vs. 11.8%, p < 0.001). OCT revealed more peripapillary retinal nerve fiber layer (pRNFL) atrophy in all segments in MOGAD compared to predominantly temporal pRNFL atrophy in MS (p < 0.001). HCVA was better preserved in MS (p = 0.007). pRNFL thickness in all except for temporal segments was suitable for differentiating MOGAD and MS. Simultaneous bilateral ON and critical atrophy in nasal (< 58.5 µm) and temporal superior (< 105.5 µm) segments were included into the composite score as three independent predictors for MOGAD. The composite score distinguished MOGAD from MS with 75% sensitivity and 90% specificity in the investigational cohort, and 68% sensitivity and 87% specificity in the validation cohort.

Conclusion: Following a single ON-episode, MOGAD exhibits more pronounced global pRNFL atrophy and lower visual acuity after ON compared to MS. The introduced OCT-based composite score enabled differentiation between the two entities across both cohorts.

Recently, the α-synuclein origin and connectome model described two types of Parkinson's disease: "brain-first" and "body-first" subtypes. We aimed to investigate the role of periodic limb movements during sleep (PLMS) in identifying these subtypes starting from a prodromal stage of α-synucleinopathies. 191 patients with isolated REM sleep behavior disorder (iRBD) underwent video-polysomnography (vPSG), questionnaires, clinical interview, and neuropsychological battery. Patients who presented PLMS index (PLMSi) > 15 were compared with patients presenting PLMSi ≤ 15 on clinical questionnaires, vPSG, and neuropsychological domains with age as a covariate. Correlations were performed between PLMSi and vPSG and neuropsychological domains in both groups of iRBD. 48.2% of patients presented PLMSi > 15. iRBD subgroup with PLMSi > 15 performed better than the iRBD subgroup with PLMSi ≤ 15 in the executive function domain. In patients with PLMSi > 15 negative correlations were observed between PLMSi and some neuropsychological domains (memory, language, and executive function). Moreover, this subgroup was older and their PLMSi was positively correlated with wake-after-sleep onset and inversely correlated with total sleep time and sleep efficiency, suggesting a detrimental effect of PLM on sleep also in this cohort. Patients with PLMSi > 15 are characterized by a more preserved cognitive status, despite a more disrupted sleep. PLMSi could be explored in longitudinal studies concerning the "brain-first" and "body-first" model.

This review investigated the effectiveness of robotic-assisted gait training (RAGT) in improving gait and balance performance in adults with multiple sclerosis (MS). Databases and registers were searched from inception to December 2023 to identify randomized controlled trials (RCTs) that analyzed the effects of RAGT on gait speed, function, balance, fatigue, and adverse events. Three reviewers screened studies for eligibility and extracted key information such as participants and intervention characteristics, as well as outcomes of interest. The reviewers assessed the risk of bias of included studies using Cochrane Risk of Bias tool. From the 948 records identified, 8 RCTs were included, involving 335 participants. The studies have demonstrated significant heterogeneity in patient characteristics, intervention protocols, and outcomes measured. The risk of bias assessment revealed concerns, mainly in terms of performance and detection bias. The evidence is uncertain on the effectiveness of RAGT on balance and gait in people with MS, but a multimodal rehabilitation approach, including RAGT, should be encouraged. No serious adverse events seem to be associated with RAGT, suggesting that these interventions are generally safe for use in people with MS. Further studies of higher methodological quality should be led to confirm these positive results.