Neurological Sciences最新文献

Since it first appeared, Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) has had a significant and lasting negative impact on the health and economies of millions of individuals all over the globe. At the level of individual health too, many patients are not recovering fully and experiencing a long-term condition now commonly termed 'long-COVID'. Long-COVID is a collection of symptoms which must last more than 12 weeks following initial COVID infection, and which cannot be adequately explained by alternate diagnoses. The neurological and psychosocial impact of long-COVID is itself now a global health crisis and therefore preventing, diagnosing, and managing these patients is of paramount importance. This review focuses primarily on: neurological functioning deficits; mental health impacts; long-term mood problems; and associated psychosocial issues, among patients suffering from long-COVID with an eye towards the neurological basis of these symptoms. A concise account of the clinical relevance of the neurological and psychosocial impacts of long-COVID, the effects on long-term morbidity, and varied approaches in managing patients with significant chronic neurological symptoms and conditions was extracted from the literature, analysed and reported. A comprehensive account of plausible pathophysiological mechanisms involved in the development of long-COVID, its management, and future research needs have been discussed.

Background: Guillain-Barré Syndrome (GBS) remains a significant contributor to acute flaccid paralysis in pediatric patients worldwide. Despite its impact, studies focusing on pediatric GBS requiring intensive care unit (ICU) management are limited. This study aimed to address this gap by exploring the clinical and outcome characteristics of pediatric GBS necessitating ICU care.

Methods: This retrospective observational study, spanning a decade, analyzed the records of 75 pediatric GBS patients admitted to the Neuro-ICU of a tertiary care center in South India. Data included demographics, prodromal symptoms, clinical features, investigations, treatment modalities, and outcomes.

Results: The majority (55/75) of patients were male, with a median age of 12 years. The highest incidence of GBS requiring ICU admission was in the monsoon season. Prodromal symptoms were observed in 56%. Most patients (93.33%) presented with typical GBS symptoms, and 40% had respiratory distress on ICU admission. Acute motor axonal neuropathy (AMAN) was the most common subtype. Approximately 80% required mechanical ventilation, with a median duration of 22.5 days. No in-hospital mortality was recorded. At discharge, most patients had a GBS disability score of 4, improving to 2 at a median follow -up of 228 days.

Conclusions: Pediatric GBS patients requiring ICU care exhibit distinctive characteristics, including a higher prevalence of AMAN subtype, seasonal clustering, and favorable outcomes with intensive treatment. The absence of in-hospital mortality underscores the effectiveness of prompt ICU admission and dedicated Neuro-intensive care.

Objectives: Benign fasciculation syndrome (BFS) is a challenging clinical condition that causes great concern to patients, as the sudden onset of fasciculations often raises suspicion of the presence or future development of motor neuron diseases. This article hence aims to provide a systematic literature review of clinical studies that investigated the clinical progression of BFS over time.

Methods: We conducted an electronic search of PubMed, Scopus, and Web of Science using the keyword "benign fasciculation syndrome" in article title, abstract, and keywords, with no time or language restrictions, to identify all possible studies with a minimum number of 10 patients that examined the clinical progression of BFS over time.

Results: Three articles with 180 patients, predominantly men (140/180; 78%), could be included in our analysis. In 98.3% of all patients fasciculations persisted over a period of 8 months to several years after the initial diagnosis of BFS, but no patient developed motor neuron dysfunction at follow-up. In the two studies providing details on clinical evolution of symptoms, fasciculations improved in 51.7% of patients and worsened in 4.1%. These results confirm the almost benign nature of BFS, with progression to overt motor neuron disease described only in specific case reports.

Conclusion: Despite its benign nature, BFS does not appear to resolve over time, as fasciculations persist in the vast majority of BFS cases, albeit with some improvements in more than half of patients.

Background: After stroke, patients must learn to use residual motor function correctly. Consistently, motor learning is crucial in stroke motor recovery. We assessed motor performance, practice-dependent on-line motor learning, and factors potentially affecting them in stroke patients.

Methods: This is a cross-sectional observational study. Twenty-six patients with first brain stroke leading to upper limb motor deficit in the subacute or chronic timeframe were enrolled. They performed a Finger Tapping Task (FTT) with both the affected and unaffected limbs. We assessed how patients learn to perform motor tasks despite the motor deficit and the differences in performance between the unaffected and affected limbs. Furthermore, by randomizing the order, we evaluated the possible inter-limb transfer of motor learning (i.e. transfer of a motor skill learned in one limb to the opposite one). Moreover, sleep, attention, anxiety, and depression were assessed through specific tests and questionnaires.

Results: Improved FTT accuracy and completed sequences for the affected limb were observed, even if lower than for the unaffected one. Furthermore, when patients initially performed the FTT with the unaffected limb, they showed higher accuracy in subsequent task completion with the affected limb than subjects who started with the affected limb. Only anxiety and attentional abilities showed significant correlations with motor performance.

Conclusions: This work provides relevant insights into motor learning in stroke. Practice-dependent on-line motor learning is preserved in stroke survivors, and an inter-limb transfer effect can be observed. Attentional abilities and anxiety can affect learning after stroke, even if the effect of other factors cannot be excluded.

Objective: To evaluate the long-term efficacy and safety of tacrolimus in patients with muscle-specific kinase antibody-positive myasthenia gravis (MuSK-MG).

Methods: We performed a retrospective, single-center, and cross-sectional study analyzing medical records of 18 MuSK-MG patients treated with tacrolimus for more than 1 year. The efficacy and safety of tacrolimus were evaluated by modified Osserman scale, Myasthenia Gravis Foundation of America post-intervention status, prednisone dosage, quantitative MG (QMG) scores, MG-activity of daily living (MG-ADL) scores, anti-MuSK antibody titers, blood routine, and serum biochemicals.

Results: After 4 weeks of tacrolimus treatment, there was a significant improvement in prednisone dose, QMG, and MG-ADL scores, which continued to improve over 1 year. In addition, clinical grade of modified Osserman scale was improved in all patients, 16 (88.9%) of whom were asymptomatic at the last visit. More importantly, the mean titers of anti-MuSK antibody were significantly decreased from 0.777 ± 0.381 to 0.283 ± 0.178 nmol/L after a median of 1.4 years of tacrolimus treatment in 9 patients with MuSK-MG (P = 0.015). All patients achieved minimal manifestations status (MMS) after tacrolimus treatment (range, 4-32 weeks). Subsequently, seven patients (38.9%) underwent a taper of tacrolimus dosage. However, four patients (57.1%) experienced an exacerbation. Adverse events occurred in 2 patients (11.1%), all of which were mild and resolved after the tacrolimus dose was adjusted or discontinued.

Conclusion: Our results suggest that tacrolimus may be an effective and safe steroid-sparing treatment for patients with MuSK-MG. However, tacrolimus should be carefully tapered to avoid disease exacerbation.

Background: Central nervous system symptoms, such as cognitive dysfunction, have been reported in Hereditary Transthyretin Amyloidosis (ATTRv). However, there is a lack of neuroimaging studies investigating structural alterations in the brain related to cognition in ATTRv amyloidosis. This study aimed to investigate cognition and cortical morphology in a cohort of ATTRv patients.

Methods: 29 ATTRv patients and 26 healthy controls completed neuropsychological assessment. 21 of these patients underwent 3T brain MRI, and 23 healthy subjects constituted the control group for MRI. Cortical measures of volume, thickness, fractional anisotropy (FA), and mean diffusivity (MD) were obtained for both groups. Correlation analyses between brain and cognitive measurements were performed.

Results: Patients displayed worse performance than controls in executive functions, verbal and visual memory, visuospatial domains, and language tests. Our study indicated cortical thinning in ATTRv patients in the temporal, occipital, frontal, and parietal areas. The inferior temporal gyrus correlated with verbal memory. Insula and, pars opercularis correlated with both verbal memory and executive function.

Conclusions: Cortical thickness in the inferior temporal gyrus, pars opercularis, and insula were linked to memory and executive function. We observed no correlations between cortical volume measures and cognition. Further investigations are imperative to confirm these findings across different populations.

Background: Human herpesvirus (HHV)-6 encephalitis secondary to chimeric antigen receptor (CAR)-T cell therapyis relatively rare in clinical practice and needs to be differentiated from immune effector cell-associatedneurotoxicity syndrome (ICANS).

Methods: We retrospectively reported a case of HHV-6 encephalitis secondary to CAR-T cell therapy.

Results: A male patient from China with diffuse large B-cell lymphoma underwent chimeric CAR-T cell therapy anddeveloped a generalized rash on the 8^th day, followed by cognitive changes, memory loss, and disorientation onthe 14^th day after CAR-T cell therapy. Initially, ICANS was suspected. A lumbar puncture was performed on the 18^th day. The cerebrospinal fluid (CSF) analysis revealed slightly elevated protein levels and a high presence of HHV-6B sequences by mNGS. Brain MRI showed bilateral hippocampal abnormalities. The patient was ultimatelydiagnosed with HHV-6 encephalitis and treated with ganciclovir and dexamethasone. After one week of treatment,follow-up CSF analysis showed a reduction in HHV-6B sequences. The patient was discharged with improvedmemory and orientation.

Conclusion: HHV-6 encephalitis secondary to CAR-T cell therapy may be easily confused with ICANS. Timely andaggressive diagnostic procedures, such as mNGS of CSF and cranial imaging, along with prompt antiviral therapy,are crucial for improving patient outcomes.

Objectives: In March 2020, the World Health Organization declared the coronavirus-related disease SARS-CoV-2 infection pandemic. Italy was one of the most affected countries and managed the emergency also by a health care reorganization.

Methods: The Education and Career Development Task Force of the Young Epilepsy Section-Italy (YES-I) designed a survey to assess the impact of the pandemic on the training and work of young epileptologists (< 40 years).

Results: Fifty-three responses were collected: 45.3% were resident, 9.4% PhD students and the remainder specialists. Clinical activity changed for most (83%) during the pandemic. Educational activity at epilepsy centers was reduced for 35.8% of the survey participants, while 30.2% of research projects involving patients participation were stopped to switch mainly to COVID-19-related research. For 73.6% of survey participants, attending online courses and congresses was easier in terms of cost and organization, although for 50.9% the level of training was lower in quality. In contrast, 58.5% rated the webinars organized by YES-I very educational. Less than 50% of the clinicians used telemedicine in the pandemic period and continue to use it. Despite several positive aspects of virtual medicine, a small number (32.1%) of our interviewees were satisfied from telemedicine and few of them (30.2%) reported that it led to improvement of clinical practice.

Conclusions: Our survey showed that the pandemic has had a negative impact on training, research and clinical activity in the epilepsy field; moreover, it underlined the critical aspects of virtual communication methods in order to improve its use for the future.