Journal of Neurology最新文献

Objectives: To synthesise evidence on prognosis, functional outcomes, and prognostic factors across functional neurological disorder (FND) subtypes and age groups.

Design: Systematic review with structured narrative synthesis (PRISMA 2020).

Data sources: MEDLINE (PubMed), Embase, PsycINFO, CINAHL, CENTRAL, Web of Science, and Scopus from inception to [insert last search date], plus citation searching.

Eligibility criteria: Systematic reviews/meta-analyses, cohort studies with follow-up >  = 3 months, and treatment-outcome studies with follow-up >  = 6 months in patients diagnosed with FND or equivalent historical labels.

Data extraction and synthesis: Two reviewers independently screened and extracted data. Risk of bias was assessed using AMSTAR-2 (reviews), QUIPS (prognostic cohorts), RoB 2 (RCTs), and ROBINS-I (non-randomised interventions). Outcomes were harmonised into remission, improvement, or persistent/unchanged/worse; functional outcomes and quality of life were synthesised descriptively.

Results: Across adult FND subtypes, long-term outcomes were generally unfavourable for functional motor disorder and functional seizures, with complete remission uncommon and persistent symptoms at follow-up in most cohorts. Functional outcomes (employment, disability status) and quality of life were frequently poor and did not consistently correlate with symptom change. Sensory-only and functional visual symptoms were associated with relatively more favourable symptomatic outcomes, although evidence was limited and heterogeneous. Paediatric cohorts demonstrated substantially better symptom outcomes than adult populations, with most children showing improvement or remission, but psychosocial burden remained common. Longer symptom duration prior to diagnosis was the most consistent predictor of poor outcome across subtypes. Treatment-outcome studies suggested benefit from specialist interventions in selected patients, particularly when delivered early, but remission rates remained modest.

Conclusions: Adult FND is often associated with persistent symptoms and long-term functional impairment, with heterogeneity by subtype and age. Early diagnosis and timely access to specialist, function-focussed care appear important for improving outcomes.

Background: Hypoxic ischemic encephalopathy (HIE) is a severe brain injury that can lead to death and long-term disability. HIE can be treated with therapeutic hypothermia, and various adjuvant treatments (such as melatonin) are also utilized. Adjuvant therapies are not recommended outside clinical trials, and therapeutic hypothermia is not universally available. This study aimed to investigate the effects of Edaravone on improving levels of consciousness, hemodynamic stability, and short-term clinical outcomes of adult patients with severe HIE. To the best of our knowledge, this study is the first randomized clinical trial investigating the effects of Edaravone in adult patients with severe HIE.

Methods: A double-blind clinical trial enrolled 72 severe HIE patients (aged > 18) within 24 h of onset who were diagnosed clinically and radiologically. Patients were randomized to Edaravone group (n = 20) and non-Edaravone group (n = 52). Measured parameters included level of consciousness, vital signs, Barthel index, and patient outcome (death or discharge). Statistical analysis was performed using SPSS version 27, with a significance level of P < 0.05.

Results: In short-term assessment of the patient's level of consciousness, the Edaravone group showed significant improvement in the Glasgow Coma Scale (GCS) post-intervention (p = 0.001). While the Edaravone group and non-Edaravone group showed no significant difference in outcome (p = 0.863) and Barthel score for discharged patients (P = 0.557). Vital signs showed significant differences between groups in temperature (P = 0.002). In the comparison of comorbidities between the Edaravone and non-Edaravone groups, only coronary artery bypass grafting was significantly different (P = 0.021).

Conclusion: Edaravone improved the short-term level of consciousness in severe HIE adult patients, but there was no significant effect on outcome and level of independence in performing activities of daily living. Further investigation into Edaravone's effectiveness is warranted, particularly in patients with milder forms of HIE, as well as longer follow-up periods.

Anarthria is a lack of verbal communication caused by physiological disturbances in the motor pathway. While affected individuals retain the ability to comprehend and produce speech, orofacial paralysis renders them unable to execute speech. Anarthria can be caused by amyotrophic lateral sclerosis, stroke, traumatic brain injury, and other etiologies that affect the descending motor pathway. A wide range of technologies has been developed and tested to improve communication efficiency for patients with anarthria and accompanying paralysis. This review evaluates three key eras of communication device development. First, before implantation devices gained traction, many communication devices revolved around blinks, head and eye tracking, and non-invasive brain recording. Second, implanted cortical neuroprosthetics were designed to improve accuracy and speed of communication. Finally, the review analyzes the future era, where accessibility, patient comfort, and broader applications of neural analysis elevate communication for patients with anarthria to match fluid communication. Restoring speech communication in patients with anarthria is vital to improve their quality of life. Therefore, understanding communication device efficiency and its future trajectory is of utmost clinical importance.

Objective: To synthesize adverse event (AE) data from randomized controlled trials (RCTs) of levetiracetam (LEV) and brivaracetam (BRV), compare their safety profiles, and identify moderators of AE risk.

Methods: We conducted a systematic review and meta-analysis of RCTs of LEV or BRV, searching PubMed, Web of Science, and ClinicalTrials.gov to August 2025. AE frequencies were summarized qualitatively, and random-effects meta-analyses compared LEV and BRV with placebo. Additional analyses compared LEV and BRV and assessed moderators.

Prospero: CRD42023491050, CRD420251003207.

Results: Ninety-six RCTs including 7145 patients exposed to LEV and 2549 to BRV were analyzed. Qualitative synthesis identified headache, somnolence, dizziness, and fatigue as the most common AEs, with higher frequencies of some psychiatric AEs (e.g., irritability, aggression) reported for LEV. Meta-analyses showed increased somnolence with both LEV (OR 1.80, 95% CI [1.41-2.30]) and BRV (OR 1.86, 95% CI [1.33-2.61]). LEV was additionally associated with irritability (OR 2.55, 95% CI [1.41-4.63]) and asthenia (OR 1.71, 95% CI [1.15-2.54]), whereas BRV was associated with dizziness (OR 1.75, 95% CI [1.24-2.46]) and fatigue (OR 2.14, 95% CI [1.43-3.20]). Few moderators of AE risk were identified, and no significant differences emerged between LEV and BRV in indirect comparisons.

Conclusion: LEV and BRV show favorable safety profiles that appear comparable based on meta-analytic findings, despite higher rates of some psychiatric AEs with LEV on a descriptive level. Both remain safe treatment options, though larger head-to-head trials are needed to provide definitive comparative evidence.

Background: Stimulation of the subthalamic nucleus (STN) and globus pallidus internus (GPi) is an effective target for treating blepharospasm-oromandibular dystonia, but the individual optimal remains unclear.

Methods: Thirteen patients with blepharospasm-oromandibular dystonia were enrolled. Bilateral STN and GPi electrodes were implanted and externalized, and the signal of the two targets was recorded simultaneously. The power spectrum and burst characteristics were analyzed to see how they were related to symptom severity.

Results: The 1 year follow-up revealed an overall improvement rate of 76.21% ± 12.51%. Those with GPi as final targets showed higher band power and longer average burst duration in the 5.5-12 Hz band in the GPi compared to the STN (P < 0.05). Conversely, those with STN as final targets showed higher band power and longer average burst duration in the 6-10 Hz band in the STN compared to the GPi (P < 0.05). Exclusion of shorter low-frequency bursts led to higher correlation coefficients with BFMDRS-M scores in both the STN and GPi groups (P < 0.01).

Conclusions: Our study suggests that low-frequency oscillatory features may be associated with therapeutic target selection for DBS in blepharospasm-oromandibular dystonia. These findings may help inform target selection strategies and warrant prospective validation for their value in supporting long-term clinical outcomes.

Objective: The effects of theta-burst stimulation (TBS) and repetitive transcranial magnetic stimulation (rTMS) on the motor symptoms in Parkinson's disease (PD) were evaluated using a network meta-analysis (NMA).

Methods: Studies (1 January 2003-1 January 2025) from four databases were screened for analyses. A Bayesian NMA model was employed, and the surface under the cumulative ranking curve (SUCRA) was used to predict the efficacy ranking of each intervention. The study protocol was registered on the International Prospective Register of Systematic Reviews platform (Identification number: CRD42025646363).

Results: A total of 28 randomized controlled trials were included. Subgroup analysis showed that multi-session cTBS was significantly superior to single-session cTBS in improving motor symptom. The main analysis (23 studies with multi-session protocols) revealed that, compared with sham stimulation, high-frequency (HF) rTMS-stimulating primary motor cortex (M1) and dorsolateral prefrontal cortex (DLPFC) and cTBS-stimulating supplementary motor area (SMA) significantly improved global motor function, ranking first and second in SUCRA values, respectively. For specific symptoms, HF + SMA showed the best improvement in Freezing of Gait Questionnaire and time up and go test (SUCRA: 0.774 and 0.982, respectively), low-frequency stimulating SMA ranked first for improving bradykinesia (SUCRA = 0.944), and HF + M1 significantly improved quality of life. All interventions were well tolerated.

Conclusion: Multi-session TBS protocols are superior to single-session protocols. HF + M1 + DLPFC and cTBS + SMA represent the most promising protocols for improving global motor function in PD. For different motor symptoms, corresponding target-specific stimulation should be prioritized.

Objective: The results of randomised controlled trials (RCTs) and meta-analyses in the literature on whether technologies used in Parkinson's disease (PD) improve health-related quality of life (HRQoL) are varied, making it difficult to reach a definitive conclusion. Therefore, the aim was to investigate the effect of technologies used in PD on HRQoL according to moderator variables.

Methods: The mean effect size was calculated using either the fixed-effects or random-effects model. Assessment timepoints, technology and scale types were used as moderator variables. Technology types: Mobile health (mHealth), robotic-assisted, telerehabilitation, virtual reality and wearable technologies. The Egger's regression method was used to assess publication bias. The quality assessment used the risk of bias (RoB) 2 method. The results of the meta-analysis were evaluated clinically and statistically.

Results: The meta-analysis included 34 RCTs. RCTs were published in the form of thesis and article publications between 2012 and 2025. The control group had 709 patients, the experimental group 759. The RoB 2 method indicates that the majority of RCTs are low risk of bias. According to the Egger's regression method, there is no publication bias. According to the results of the meta-analysis, mHealth, robotic-assisted, telerehabilitation, virtual reality and wearable technologies used in PD have the potential to improve patients' HRQoL.

Conclusion: The technologies used in PD have the potential to improve patients' HRQoL. These results could provide significant opportunities for effectively managing the disease and its treatment, as well as improving patients' daily living activities.

Background: The hypothalamus as one of the core structures in metabolic control is increasingly recognized to be morphologically altered in various neurodegenerative diseases.

Objective: The purpose of this study was to quantitatively investigate the hypothalamic volumes in patients with progressive supranuclear palsy (PSP) and to compare them with controls and Parkinson disease (PD) patients.

Methods: An automatic hypothalamic volume quantification method based on the use of convolutional neural networks (CNN) of U-Net architecture was applied to the automatic segmentation of the hypothalamus and intracranial volumes (ICV). This CNN-based volumetric analysis was performed in high resolution T1 weighted MRI in two PSP cohorts: cohort A with 78 PSP patients and 63 controls was recorded at 3.0 T at multiple sites; the single site cohort B consisted of 66 PSP patients, 66 PD patients, and 44 controls, recorded at 1.5 T.

Results: In cohort A, significant hypothalamic volume reduction was observed in PSP (774 ± 83 mm³) when compared to controls (817 ± 74 mm³). In cohort B, this result of significant hypothalamic volume reduction was confirmed in PSP (745 ± 102 mm³) when compared to controls (831 ± 81 mm³); no significant hypothalamic volume reduction was observed in PD (797 ± 98 mm³), in support of previous studies.

Conclusion: The CNN-based hypothalamus volume quantification study demonstrated significantly reduced hypothalamus volumes in PSP patients compared to controls and PD, respectively; future studies will address the metabolic profiles of PSP as potential functional correlates.

Objectives: Although the clinical features of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are well documented, their early clinical presentations from the perspective of initial clinical consultation remain less well understood. This study aimed to characterize the early clinical features of PSP and CBD to inform clinicians on diagnostic assessment and management strategies.

Methods: Data were obtained from the National Alzheimer's Coordinating Center (NACC) database (2005-March 2025 data freeze). Neuropathologically confirmed cases of PSP (n = 278) and CBD (n = 149) were included. Analyses were restricted to first clinical visits, focusing on demographic, neuropsychiatric, neurological, and neuropsychological variables.

Results: Memory symptoms were reported in more than half of both groups, whereas language impairment was more common in CBD (> 70%) and associated with higher odds of CBD diagnosis. Depression was frequent (~ 50%), with PSP showing higher odds for depressive symptoms but lower odds for disinhibition. Although 20-30% of both PSP and CBD patients exhibited no parkinsonian signs at presentation, gait disturbance, falls, and slowness were common and strongly associated with PSP. Approximately half of both groups presented with cognitive-predominant onset, and nearly one-fifth were initially misdiagnosed as Alzheimer's disease.

Conclusions: A substantial proportion of PSP and CBD patients lack parkinsonian signs at first presentation, and cognitive-onset presentations are frequent, leading to early diagnostic uncertainty. Clinicians should recognize these overlapping features when evaluating early atypical parkinsonian or cognitive syndromes.