Journal of Neurology最新文献

Background: Brain health may be impaired years after hospitalization for critical illness, and similar impairments occur after hospitalization for COVID-19. However, it remains unclear which patients are most likely to experience long-term brain health consequences and whether these adverse events differ between non-COVID critical illness and COVID-19.

Methods: In a prospective observational study, we enrolled patients hospitalized for (1) non-COVID critical illness (pneumonia, myocardial infarction, or ICU-requiring conditions) or for (2) COVID-19, from March 2020 to June 2021. Brain health was assessed at 18-month follow-up with cognitive, psychiatric, and neurological tests. We used both logistic regression and prediction models to test for associations between different variables and brain health.

Results: We included 245 patients: 125 hospitalized for non-COVID critical illness and 120 for COVID-19 [mean age 61.2 (± 13.6) years, 42% women]. Brain health was impaired in 76% of patients (72% critical illness, 81% COVID-19; p = 0.14) at 18-month follow-up. The strongest predictive factors associated with impaired brain health were education < 13 years, age ≥ 70 years, and neuroticism traits in the best performing model (AUC = 0.63). When analyzing non-COVID critical illness and COVID-19 patients separately, low education was one of the few factors associated with impaired brain health in both groups (AUCs for best models: 0.66 and 0.69).

Conclusion: Brain health is comparably impaired after hospitalization for critical illness and COVID-19. Factors like higher age, lower education and neuroticism may help identifying vulnerable individuals, who could benefit from close monitoring to improve brain health after critical illness, regardless of the underlying disease etiology.

Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive and fatal prion disease with significant public health implications. Survival is heterogenous, posing challenges for prognostication and care planning. We developed a survival model using diagnostic data from comprehensive UK sCJD surveillance.

Methods: Using national CJD surveillance data from the United Kingdom (UK), we included 655 cases of probable or definite sCJD according to 2017 international consensus diagnostic criteria between 01/2017 and 01/2022. Data included symptoms at diagnosis, CSF RT-QuIC and 14-3-3, MRI and EEG findings, as well as sex, age, PRNP codon 129 polymorphism, CSF total protein and S100b. An artificial neural network based multitask logistic regression was used for survival analysis. Model-agnostic interpretation methods was used to assess the contribution of individual features on model outcome.

Results: Our algorithm had a c-index of 0.732, IBS of 0.079, and AUC at 5 and 10 months of 0.866 and 0.872, respectively. This modestly improved on Cox proportional hazard model (c-index 0.730, IBS 0.083, AUC 0.852 and 0863) but was not statistically significant. Both models identified codon 129 polymorphism and CSF 14-3-3 to be significant predictive features.

Conclusions: sCJD survival can be predicted using routinely collected clinical data at diagnosis. Our analysis pipeline has similar levels of performance to classical methods and provide clinically meaningful interpretation which help deepen clinical understanding of the condition. Further development and clinical validation will facilitate improvements in prognostication, care planning, and stratification to clinical trials.

Isolated rapid-eye-movement sleep behavior disorder (iRBD) is a strong predictor of Parkinson's disease and Dementia with Lewy bodies. Previous studies indicate that cortical atrophy in iRBD patients may be linked to cognitive impairment, but the pattern of atrophy is inconsistently reported. This study aimed to elucidate cortical atrophy patterns in a cognitively unimpaired iRBD cohort, focusing on regions associated with cognitive functions, particularly the cuneus/precuneus, and evaluated the predictive value for future phenoconversion. We conducted voxel-based morphometry and region of interest (ROI) analysis of structural MRI scans of 36 healthy controls and 19 iRBD patients, nine of whom also received a 3-year follow-up MRI scan. The iRBD patients were followed clinically for 8 years, and time-to-event analyses, using Cox regression, were performed based on baseline ROI volumes. The iRBD patients had lower gray-matter volume in the cuneus/precuneus region as well as in subcortical structures (caudate nuclei and putamen) compared to controls. Eight iRBD patients developed either Parkinson's disease (N = 4) or Dementia with Lewy bodies (N = 4) during the follow-up period. Time-to-event analyses showed that lower right cuneus volume was associated with a higher risk of phenoconversion to alpha-synuclein-linked Parkinsonism in the iRBD patients (Hazard ratio = 13.0, CI: 1.53-110), and correlated with shorter time to conversion. In addition, lower volumes of the bilateral precuneus trended to indicate a higher risk of phenoconversion. These findings suggest a potential predictive value of cuneus and precuneus volumes in identifying iRBD patients at risk of disease progression, even before the onset of cognitive impairment.

Objective: This study aims to evaluate the methodological quality of guidelines concerning the prophylactic use of antiseizure medications (ASMs) in neurosurgery and to summarize relevant recommendations.

Methods: PubMed, Embase, MEDLINE, Web of Science, China National Knowledge Infrastructure (CNKI), Wanfang, China Science and Technology Journal Database (VIP), National Guideline Clearinghouse (NGC), Guidelines International Network (GIN), and other guideline repositories and official organizations were searched from 2004 to 2023 (20 years). The extracted information consisted of the guideline characteristics, relevant recommendations, levels of evidence, and strength of recommendations. Using the Guideline Research and Evaluation Tool II (AGREE II), five reviewers assessed the methodological quality of the guidelines, and the intraclass correlation coefficient (ICC) is used to assess the inter-reviewer consistency.

Results: Of 27 eligible guidelines, AGREE II scores varied with higher scores in Clarity of Presentation (88.89%), Scope and Purpose (83.33%), and Editorial Independence (72.92%), but lower in Rigor of Development (59.17%), Stakeholder Involvement (46.67%), and Applicability (41.67%). ICC ranged from 0.51 to 0.92. Nine guidelines were recommended, eight with modifications, and ten not recommended. ASMs prophylaxis are recommended for patients with a seizure history, which means secondary prophylaxis, and specific high-risk groups, but not recommended for primary prophylaxis routinely for those without a history of seizure.

Conclusions: Guidelines in neurosurgical perioperative prophylactic use of ASMs are of moderate quality with domains for enhancement. Guidelines lack detailed guidance on medication initiation, dosage, and duration, highlighting the need for more high-quality clinical trials comparing newer and classical ASMs.

Background: Focal seizures may encompass vestibular sensations in their symptomatology. When these manifestations occur in isolation or constitute the predominant symptom, they prompt consideration for diagnosing recurrent paroxysmal vertigo. However, the characterization of "vestibular epilepsy" remains debated and underexplored. Our objective is to characterize the clinical and electrophysiological criteria of vestibular epilepsy.

Methods: We retrospectively analyzed data from a cohort of outpatients treated in the epileptology department of Marseille University Hospital. The study focused on patients presenting with vestibular symptoms without focal abnormalities on brain MRI, and with interictal epileptic abnormalities on wake or sleep EEG.

Results: 31 patients (15 men and 16 women) were included in the study. Visual, auditory, and dysautonomic symptoms were frequently associated with vestibular symptoms. The mean time to diagnosis was 3 years. The duration of attacks was generally short, ranging from a few seconds to a few minutes, with variable frequency. Most patients responded well to antiseizure medication. Some patients showed interictal phenomena, such as permanent instability, raising the possibility of inter/postictal disturbances. Seizures could be triggered by peripheral vestibular stimuli. Interictal EEG abnormalities were observed only during sleep in 25% of patients and predominated in the posterior temporoparietal regions.

Discussion: We propose clinical-electro-radiological criteria for defining vestibular epilepsy. These diagnostic criteria overlap with the criteria for vestibular paroxysmia, suggesting the possibility of a single nosological entity.

Background: Stroke is the second leading cause of disability worldwide. Stroke results in focal neurological deficit and often leads to auditory problems due to its impact on the auditory pathway. Altered connections in the auditory pathway, caused by stroke, can result in hearing difficulties ranging from impaired sound detection to altered auditory perception. A better understanding of how stroke affects these early sound processing mechanisms will provide valuable insights into stroke recovery and rehabilitation options.

Methods: We recruited forty consecutive adult patients with stroke (30 males, 10 females) due to ischemic or intracerebral hemorrhage > 3 and up to 12 months after stroke (subacute stage). Brain MRIs were performed on all patients, and we calculated a central auditory nervous system stroke severity index (CANS SSI) according to number of CANS areas involved and an extended CANS definition of auditory responsive areas. All patients underwent cognitive screening assessment, basic audiological assessments, and a hierarchical central auditory processing assessment battery with the Queen Square Tests of Auditory Cognition (early perceptual processing, apperceptive processing, semantic Processing) and Gaps in Noise tests.

Results: When comparing patients with auditory responsive cortical lesions and with versus without Heschl's gyrus involvement (primary auditory cortex), patients with Heschl's gyrus involvement exhibited worse early perceptual scores. The CANS SSI showed a significant negative correlation with early perceptual test scores.

Conclusion: This study demonstrates a correlation between stroke severity, characterized by a higher number of lesions involving auditory areas in patients with subacute stroke, and worse early perceptual scores. Heschl's gyrus involvement is associated with poorer early perceptual score.

Background and objectives: The term rapid progressive dementia (RPD) may be applied to patients who develop dementia within 1 year or complete incapacitation within 2 years of the first symptom of impairment. However, in select cases, cognitive impairment may emerge abruptly, with symptoms evolving across hours or days. We sought to determine the frequency, etiologies, and factors that associated with ultra-RPD.

Methods: Ultra-RPD was defined as persistent dementia (global Clinical Dementia Rating^® ≥ 1), developing within 7 days of initial symptoms. Patients with ultra-RPD were identified via case review of patients enrolled in a prospective study of RPD at two tertiary care centers (February 2016-September 2023) followed by a systematic review of multiple English-language databases, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (completed January 2024).

Results: Three of 188 patients with RPD enrolled in our prospective series met the proposed definition for ultra-RPD (frequency = 1.6%). Systematic review yielded 57 additional cases from 47 publications (60 total cases). Ultra-RPD was attributed to vascular (40%), toxic/metabolic (22%), autoimmune/inflammatory (20%), and iatrogenic/structural (12%) causes. Lesions within the Papez circuit were detected in 52/59 (88%) of patients on neuroimaging. Twelve patients (20%) had potentially treatable causes of ultra-RPD.

Discussion: Patients with ultra-RPD were rarely encountered in our prospective series, representing < 2% of cases of RPD, and rarely reported in the extant literature. The evaluation of patients with ultra-RPD should prioritize testing for vascular, toxic/metabolic, and autoimmune/inflammatory conditions that affect neuroanatomical structures or networks critical for memory formation and retrieval.