Neurologia最新文献

Introduction

Availability of adequate normative data is essential when performing neuropsychological evaluation; good methodological quality of the studies that propose these data ensures that their conclusions are reliable and valid. We present the methodological characteristics of the Neuronorma Colombia Project in order to analyse its contributions and limitations.

Method

We present the characteristics of the normative sample, inclusion and exclusion criteria, statistical analysis, the procedure for obtaining normative data, and the instruments used.

Results

We present graphical profiles of patient performance, based on the Neuronorma Work Unit, to illustrate the interpretation of the results obtained when evaluating patients with the Neuronorma Colombia Battery.

Discussion and conclusions

Our study presents several methodological advantages, such as its multicentre, co-normalised design and the availability of the Neuronorma Work Unit, which allows the creation of graphical profiles of patient performance, a fundamental tool for diagnosis and research. We present the findings of subsequent research based on the proposed normative data, which demonstrate the value of the battery. The contribution of this study is discussed in the context of its immediate background.

Background

Ischaemic stroke may be a major complication of SARS-CoV-2 infection. Studying and characterising the different aetiological subtypes, clinical characteristics, and functional outcomes may be valuable in guiding patient selection for optimal management and treatment.

Methods

Data were collected retrospectively on consecutive patients with COVID-19 who developed acute focal brain ischaemia (between 1 March and 19 April 2020) at a tertiary university hospital in Madrid (Spain).

Results

During the study period, 1594 patients were diagnosed with COVID-19. We found 22 patients with ischaemic stroke (1.38%), 6 of whom did not meet the inclusion criteria. The remaining 16 patients were included in the study (15 cases of ischaemic stroke and one case of transient ischaemic attack).

Median baseline National Institutes of Health Stroke Scale score was 9 (interquartile range: 16), and mean (standard deviation) age was 73 years (12.8). Twelve patients (75%) were men. Mean time from COVID-19 symptom onset to stroke onset was 13 days. Large vessel occlusion was identified in 12 patients (75%).

We detected elevated levels of D-dimer in 87.5% of patients and C-reactive protein in 81.2%. The main aetiology was atherothrombotic stroke (9 patients, 56.3%), with the predominant subtype being endoluminal thrombus (5 patients, 31.2%), involving the internal carotid artery in 4 cases and the aortic arch in one. The mortality rate in our series was 44% (7 of 16 patients).

Conclusions

In patients with COVID-19, the most frequent stroke aetiology was atherothrombosis, with a high proportion of endoluminal thrombus (31.2% of patients). Our clinical and laboratory data support COVID-19–associated coagulopathy as a relevant pathophysiological mechanism for ischaemic stroke in these patients.

Objectives

To identify possible predictors of seizure cluster or status epilepticus (SE) and to evaluate whether these patients receive greater interventions in emergency departments.

Methodology

We conducted a secondary analysis of the ACESUR Registry, a multipurpose, observational, prospective, multicentre registry of adult patients with seizures from 18 emergency departments. Clinical and care-related variables were collected. We identified risk factors and risk models for seizure cluster or SE and assessed the effect of interventions by prehospital emergency services and the hospital emergency department.

Results

We identified a total of 186 (28%) patients from the ACESUR registry with seizure cluster (126 [19%]) or SE (60 [9%]); the remaining 478 patients (72%) had isolated seizures. The risk model for seizure cluster or SE in the emergency department included Charlson Comorbidity Index scores ≥ 3 (OR: 1.60; 95% CI, 1.05–2.46; P = .030), ≥ 2 habitual antiepileptic drugs (OR: 2.29; 95% CI, 1.49–3.51; P < .001), and focal seizures (OR: 1.56; 95% CI, 1.05–2.32; P = .027). The area under the curve of the model was 0.735 (95% CI, 0.693–0.777; P = .021). Patients with seizure cluster and SE received more aggressive interventions both by prehospital emergency services (OR: 2.89; 95% CI, 1.91–4.36; P < .001) and at the emergency department (OR: 4.41; 95% CI, 2.69–7.22; P < .001).

Conclusions

This risk model may be of prognostic value in identifying adult patients at risk of presenting seizure cluster or SE in the emergency department. In our sample, these patients received more aggressive treatment than adult patients with isolated seizures before arriving at hospital, and even more so in the emergency department.

Introduction

Communication and language skills are among the most severely affected domains in individuals with autistic spectrum disorder (ASD). When a child diagnosed with ASD lives in a bilingual environment, the parents often express concerns about whether their child should learn both languages simultaneously, turning to specialists for advice. Despite the lack of evidence of any negative effect, some professionals disagree on this subject. In this systematic review we study whether bilingualism affects language development in children with ASD.

Methods

We reviewed the literature published in 4 different databases. After applying a series of selection criteria, we selected 12 scientific articles, including a total of 328 children diagnosed with ASD (169 bilingual and 159 monolingual), with ages ranging from 3 to 12 years. These patients were evaluated with different receptive and expressive language assessment instruments covering several areas. The assessments were performed directly on the children, although indirect assessment of parents was also performed in some studies.

Conclusions

There seems to be consensus regarding the assertion that bilingualism does not entail any additional difficulty for language development in children with ASD from the age of 3.

Introduction

Risk factors for dementia include genetic factors, aging, environmental factors, certain diseases, and unhealthy lifestyle; most types of dementia share a common chronic systemic inflammatory phenotype. Psoriasis is also considered to be a chronic systemic inflammatory disease. It has been suggested that psoriasis may also contribute to the risk of dementia. The aim of this study was to systematically review the literature on the association between psoriasis and dementia.

Development

Articles were selected according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the PubMed and Web of Science databases to identify articles published in peer-reviewed journals and studying the association between psoriasis and dementia. Studies meeting the inclusion criteria were reviewed. We used the Newcastle–Ottawa Scale to assess the quality of each study. After applying the inclusion and exclusion criteria, we included 8 studies for review, 3 of which were found to present a higher risk of bias. Six of the 8 studies supported the hypothesis that prior diagnosis of psoriasis increases the risk of dementia; one study including only a few cases reported that psoriasis decreased the risk of dementia, and one study including relatively young patients found no significant association between psoriasis and the risk of dementia.

Conclusion

Most studies included in this review supported the hypothesis that psoriasis constitutes a risk factor for dementia. However, well-designed stratified cohort studies assessing both psoriasis severity and treatment status are still required to determine the real effect of psoriasis on the risk of dementia and its subtypes.

Background

Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy that can lead to respiratory failure. In this study, we evaluate early clinical risk factors for respiratory failure at the time of hospital admission.

Methods

We studied a retrospective cohort of patients with GBS admitted to a tertiary care center. The potential risk factors studied were sociodemographic characteristics, GBS symptoms, overall and cervical muscle weakness (Medical Research Council [MRC] scores), electromyography findings, and cerebrospinal fluid analysis findings. Unadjusted odds ratios (OR) were calculated and exact logistic regression analysis (adjusted OR) performed to assess the association between baseline risk factors and respiratory failure.

Results

Overall, 13 of 113 (12%) patients included in the study developed respiratory failure. Unadjusted analyses showed that involvement of any cranial nerve (OR: 14.7; 95% CI, 1.8–117.1), facial palsy (OR: 17.3; 95% CI, 2.2–138.0), and bulbar weakness (OR: 10.7; 95% CI, 2.3–50.0) were associated with increased risk of respiratory failure. Lower MRC sum scores (for scores <30, OR: 14.0; 95% CI, 1.54–127.2) and neck MRC scores (for scores ≤3, OR: 21.0; 95% CI, 3.5–125.2) were associated with higher likelihood of respiratory failure. Adjusted analyses showed that presence of bulbar weakness (OR: 7.6; 95% CI, 1.3–43.0) and low neck MRC scores (scores ≤3, OR: 9.2; 95% CI, 3.5–125.2, vs scores >3) were independently associated with respiratory failure.

Conclusions

Bulbar and neck muscle weakness at admission are clinical predictors of increased risk of respiratory failure in patients with GBS. These findings could guide the adequate management of high-risk patients.

Parkinson’s disease (PD) is the second most prevalent neurodegenerative disease among adults worldwide. It is characterised by the death of dopaminergic neurons in the substantia nigra pars compacta and, in some cases, presence of intracytoplasmic inclusions of α-synuclein, called Lewy bodies, a pathognomonic sign of the disease. Clinical diagnosis of PD is based on the presence of motor alterations. The treatments currently available have no neuroprotective effect. The exact causes of PD are poorly understood. Therefore, more precise preclinical models have been developed in recent years that use induced pluripotent stem cells (iPSC). In vitro studies can provide new information on PD pathogenesis and may help to identify new therapeutic targets or to develop new drugs.

Background and objective

Multiple system atrophy is a rare and fatal neurodegenerative disorder, characterized by autonomic dysfunction in association with either parkinsonism or cerebellar signs. The pathologic hallmark is the presence of alpha-synuclein aggregates in oligodendrocytes, forming glial cytoplasmic inclusions. Clinically, it may be difficult to distinguish form other parkinsonisms or ataxias, particularly in the early stages of the disease. In this case series we aim to describe in detail the features of MSA patients.

Material and methods

Unified MSA Rating Scale (UMSARS) score, structural and functional imaging and cardiovascular autonomic testing, are summarized since early stages of the disease.

Results

UMSARS proved to be useful to perform a follow-up being longitudinal examination essential to stratify risk of poor outcome. Neuropathological diagnosis showed an overlap between parkinsonian and cerebellar subtypes, with some peculiarities that could help to distinguish from other subtypes.

Conclusion

A better description of MSA features with standardized test confirmed by means of neuropathological studies could help to increase sensitivity.