Neurologia最新文献

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. 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.

Introduction

Rafts are function-structural cell membrane nano-domains. They contribute to explain the efficiency of signal transduction at the low physiological membrane concentrations of the signaling partners by their clustering inside specialized signaling domains.

Development

In this article, we review the current model of the membrane rafts and their physio-pathological relevance in the nervous system, including their role in Parkinson, Alzheimer, and Huntington diseases.

Conclusions

Rafts disruption/dysfunction has been shown to relate diverse neurological diseases. Therefore, it has been suggested that preservation of membrane rafts may represent a strategy to prevent or delay neuronal dysfunctions in several diseases.

Introduction

The COVID-19 pandemic has caused an unexpected boost to telemedicine. We analyse the impact of the pandemic on telemedicine applied in Spanish headache consultations, review the literature, and issue recommendations for the implementation of telemedicine in consultations.

Method

The study comprised 3 phases: 1) review of the MEDLINE database since 1958 (first reported experience with telemedicine); 2) Google Forms survey sent to all members of the Spanish Society of Neurology's Headache Study Group (GECSEN); and 3) online consensus of GECSEN experts to issue recommendations for the implementation of telemedicine in Spain.

Results

COVID-19 has increased waiting times for face-to-face consultations, increasing the use of all telemedicine modalities: landline telephone (from 75% before April 2020 to 97% after), mobile telephone (from 9% to 27%), e-mail (from 30% to 36%), and video consultation (from 3% to 21%). Neurologists are aware of the need to expand the availability of video consultations, which are clearly growing, and other e-health and m-health tools.

Conclusions

The GECSEN recommends and encourages all neurologists who assist patients with headaches to implement telemedicine resources, with the optimal objective of offering video consultation to patients under 60-65 years of age and telephone calls to older patients, although each case must be considered on an individual basis. Prior approval and advice must be sought from legal and IT services and the centre's management. Most patients with stable headache and/or neuralgia are eligible for telemedicine follow-up, after a first consultation that must always be held in person.

Introduction

Microvascular decompression is considered to be the most effective and only etiological surgical treatment for classical trigeminal neuralgia, relieving the neurovascular compression found in up to 95% of cases. This study aims to report the long-term outcomes and to identify prognostic factors in a series of patients with trigeminal neuralgia treated by microvascular decompression.

Methods

A retrospective observational study of 152 consecutive patients operated by microvascular decompression with at least six months of follow-up. The surgical results, including pain relief according to the Barrow Neurological Institute pain scale, complications and the medical treatment during the follow-up period were reviewed. Binary regression analysis was performed to identify factors associated with a good long-term outcome.

Results

A total of 152 patients with a mean age of 60 years and a mean follow-up of 43 months were included. At the final follow-up visit, 83% of the patients had achieved significant relief of the pain and 63% could reduce the absolute drug doses by 50% or more. The most frequent complications were wound infection (4.5%) and CSF fistula (7%). Being over 70 years of age and having paroxysmal pain were associated with a long-term pain relief.

Conclusions

Our results support the notion that microvascular decompression is an effective and safe therapy in patients with trigeminal neuralgia. A multidisciplinary approach with an early referral to a neurosurgical unit many be beneficial in patients who are refractory to pharmacological treatment.

Introduction

The Fototest and Mini-Cog include all the domains that are necessary in a cognitive assessment. This study aims to evaluate the diagnostic accuracy of the combined use of both instruments for detecting cognitive impairment.

Methods

We performed a phase iii diagnostic accuracy study with 2 independent samples: STUDY, which included 448 participants randomly allocated to 2 datasets (BASE [80%] and TEST [20%]); and EXTERNAL, which included 61 participants. The index test was consecutive administration of the Fototest and Mini-Cog, and the reference test was formal cognitive assessment. We evaluated the diagnostic accuracy of two-step vs. consecutive application of the tests and simple (Comb-Simple), logistic regression (Comb-LR), and random decision tree (Comb-RDT) models of their combined use for detecting cognitive impairment (Global Deterioration Scale score ≥ 3). We performed an exploratory analysis of the BASE dataset, selecting criteria that maximise accuracy; a pre-specified analysis was used to evaluate the selected criteria in the TEST and EXTERNAL datasets.

Results

The diagnostic accuracy (95% confidence interval) of the combined models in the BASE dataset (Comb-Simple: 88.3 [88.5-91.4]; Comb-LR: 91.6 [88.2-94.3]; Comb-RDT 95.2 [92.5-97.2]) was significantly higher than the individual values observed for the Mini-Cog and Fototest (81.6 [77.1-85.4] and 84.9 [80.8-88.5], respectively). These results were replicated in the TEST (Comb-Simple: 88.9; Comb-LR: 95.6; Comb-RDT: 92.2) and EXTERNAL datasets (Comb-Simple: 91.8; Comb-LR: 90.2; Comb-RDT: 88.5). Two-step application had the same diagnostic accuracy than consecutive application but required less time (mean [SD] of 197.3 s [56.7] vs. 233.9 s [45.2]; P<.0001).

Conclusions

Combined application of the Fototest and Mini-Cog takes less than 4 minutes and improves the diagnostic accuracy of both instruments. Two-step application is more efficient as it requires less time while maintaining the same diagnostic accuracy.

Introduction

This study aimed to determine whether the administration of antiepileptic drugs (AED) alters the likelihood of detecting epileptiform abnormalities in electroencephalographies (EEG) performed early after a first epileptic seizure.

Method

We performed a retrospective, observational study including patients with a first seizure attended at our centre's emergency department between July 2014 and November 2019. We collected clinical data, as well as technical data on the acquisition and interpretation of the EEG performed within the first 72 hours after the seizure, and the factors related with seizure recurrence.

Results

We recruited 155 patients with a mean (SD) age of 48.6 (22.5) years; 61.3% were men. Regarding seizure type, 51% presented tonic-clonic seizures of unknown onset and 12% presented focal to bilateral tonic-clonic seizures. Thirty-nine patients (25.2%) received AED treatment before the EEG was performed: 33 received a non-benzodiazepine AED and 6 received a benzodiazepine. Epileptiform abnormalities were observed in 29.7% of patients. Previous administration of AEDs was not significantly associated with the probability of detecting interictal epileptiform abnormalities (P=.25) or with the risk of recurrence within 6 months (P=.63).

Conclusions

Administration of AEDs before an early EEG following a first seizure does not decrease the likelihood of detecting epileptiform abnormalities. These findings suggest that starting AED treatment immediately in patients with a high risk of early recurrence does not imply a reduction in the diagnostic accuracy of the test.