Acta neurologica Belgica最新文献

Background: Patients with ischaemic stroke are increasingly encountered in neurological practice. Current guidelines recommend hospital admission for monitoring and coordination of appropriate aftercare, regardless of stroke severity. However, the clinical necessity of admitting all such patients remains uncertain. Some neurologists argue that selected low-risk patients can be discharged directly from the emergency department (ED). Given the ongoing debate and lack of high-quality evidence, we aimed to explore current practices and attitudes toward the management of patients with ischaemic stroke with minor neurological deficits in the Netherlands.

Methods: A national online survey was conducted to explore current variations in clinical practice regarding admission strategies for patients with ischaemic stroke. The survey included five clinical cases and was distributed to neurologists and neurology trainees. We assessed which patient and stroke characteristics influenced the decision to admit a patient.

Results: A total of 107 respondents completed the survey, comprising 72 neurologists (67%) and 36 neurology trainees (33%). Admission rates for ischaemic stroke with minor neurological deficits varied considerably across the five clinical vignettes, ranging from 21% to 89%. Willingness to randomise patients into a trial evaluating admission versus ED discharge was high, with rates between 77% and 94% across cases. The majority of respondents (89%) considered a risk threshold of up to 5% for secondary deterioration acceptable to justify direct discharge from the ED. Of these, 44% would accept a risk of 1-2%, while 45% would accept a risk of 3-5%. Only 10% indicated that all ischaemic stroke patients should be admitted regardless of risk.

Conclusion: Current guideline-driven practice in the Netherlands leads to hospital admission for nearly all patients with ischaemic stroke. However, our survey reveals substantial heterogeneity among neurologists and trainees regarding the willingness to consider direct ED discharge under specific conditions. If certain criteria are met, a majority would opt for ED discharge, with 89% accepting a risk of up to 5% for early neurological deterioration. These findings underscore the need for evidence-based admission strategies, potentially enabling safe ED discharge for selected patients and more efficient use of hospital resources.

Background: It is well known that scientific literature has a significant gender gap, as gender differences were not considered until very recently. Women were often excluded from studies, and even when included, the results are rarely analyzed separately by gender. This study aims to investigate gender-specific differences in patients with Myasthenia Gravis (MG) by segregating data from our cohort of patients in Antwerp (Belgium).

Methods: We analyzed the data of our previously published cohort of 163 patients with MG visited in the Antwerp University Hospital between 2019 and 2021, segregating the information by gender to observe any significant differences.

Results: The analysis revealed several notable gender-specific differences. Women experienced a delay in diagnosis of over one year more frequently than men. They also had dysarthria as presenting symptom more often than men. The MGFA scores at maximum severity were higher in women, who also reported more limitations due to the disease and required more treatments to control it. While some differences could be attributed to the younger onset of the disease in women, certain differences were independently influenced by gender.

Conclusions: Women experience more limitations due to MG than men and may face a more severe disease course. These differences should be taken into account when determining follow-up and treatment strategies. Additionally, these findings highlight the importance of segregating data by gender in scientific studies to better understand gender-specific differences in disease presentation and management.

Background: Idiopathic intracranial hypertension (IIH) is associated with well-known classical neuroimaging hallmarks, but not all patients exhibit these typical findings. We aimed to investigate novel imaging features that could aid in diagnosing IIH and to assess the relationships between these features and cerebrospinal fluid (CSF) opening pressures.

Methods: A total of 57 IIH patients (50 females, mean age 37 years) and 57 age- and sex-matched controls (mean age 34 years) were retrospectively evaluated. CSF opening pressures, brain MRI and MR venography findings were reviewed for typical IIH imaging features and novel basal cistern measurements, including Meckel cave dimensions, prepontine AP diameter, cerebellopontine cistern volume, mamillopontine distance, and pontomesencephalic/interpeduncular angles. Associations between imaging parameters and CSF opening pressure were analyzed.

Results: Empty sella was the most frequent typical imaging finding, present in nearly all patients with IIH. Compared to controls, IIH patients showed increased prepontine AP diameter and larger Meckel cave measurements, while mamillopontine distance was reduced. Higher CSF opening pressure was associated with optic nerve sheath enlargement and/or bilateral transverse sinus stenosis. Mamillopontine distance demonstrated the best diagnostic performance (AUC: 0.710), with 66.7% sensitivity and 70.2% specificity at a 6.35 mm cut-off.

Conclusions: In addition to classical imaging findings, Meckel cave, prepontine AP diameter, and mamillopontine distance measurements differ significantly in patients with IIH from controls. High CSF opening pressure is associated with optic nerve sheath enlargement and bilateral transverse sinus stenosis.

Introduction & objective: The Mini-Addenbrooke's Cognitive Examination (M-ACE) is an easy-to-administer and quick screening test that assesses four basic cognitive areas, namely orientation, verbal fluency, memory, and visuospatial abilities, out of 30 points. The objective of this study is to introduce M-ACE to the Turkish cognitive science literature and evaluate its applicability in Turkish society in comparison with the Mini-Mental State Examination (MMSE).

Materials & methods: A total of 90 native Turkish speakers aged 60 and over with at least 12 years of formal education, including 30 patients with mild cognitive impairment (MCI group), 30 patients with Alzheimer's disease (AD group), and 30 healty control subjects (control group), who applied to the neurology outpatient clinic between November 2020 and November 2021, were included in this prospective study. All participants were underwent MMSE and the Turkish version of M-ACE by experienced neurologists.

Results: The optimal M-ACE cut-off values were 24.5 for differentiating MCI from controls (90% sensitivity, 73% specificity), 23.5 for distinguishing AD from controls (97% sensitivity, 90% specificity), and 19.5 for differentiating MCI from AD (70% sensitivity, 80% specificity).

Conclusion: Turkish version of the M-ACE is a valid tool for assessing cognitive impairments in the elderly population.

A 21-year-old woman presented with a severe tremor and involuntary spasms in her hands. Her tremor started at the age of seven, and while it was initially mild and only in bilateral upper extremities, its severity increased over time. There is no consanguinity between her parents, and her early developmental milestones are consistent with her age. Her father had involuntary movements with similar semiology. The neurological examination revealed a mild intellectual disability, head titubation, postural and action tremors, and dystonia affecting the bilateral distal upper extremities. Brain magnetic resonance imaging and investigations for neurometabolic diseases were normal. Exome sequencing analysis revealed a heterozygous likely pathogenic variant of ANO3 gene ([NM_031418.4]:c.1943A>G p.[Asn648Ser]), and she was diagnosed with ANO3-related dystonia (DYT-ANO3). She was started on trihexyphenidyl, and at the follow-up, partial improvement in her involuntary movements was observed.DYT-ANO3 caused by pathogenic variants of ANO3 gene were first described in families affected by adult-onset tremulous craniocervical dystonia, and since then, the phenotypic spectrum has expanded significantly. The onset of clinical features may vary from the first months of life to adulthood, and cases may present with a wide range of clinical spectrum, from focal/segmental to generalized dystonia, and from isolated to tremulous or jerky dystonia. Cases with the same genotype may present with movement disorders of different characteristics, suggesting that there is no definitive genotype-phenotype correlation in DYT-ANO3 cases. Additionally, cases with nondystonic features, such as nondystonic tremor, myoclonus and/or neurodevelopmental delay have also been reported.

Background: Acquired brain injury (ABI) includes conditions such as cerebral stroke or traumatic brain injury and is a major cause of disability. People with ABI are one of the largest groups requiring rehabilitation. Rehabilitation prognosis is an important component for goal setting and life planning. The population average prognosis (overall prognosis) and its variation describes the contextual course of health outcomes. Here, we provide the overall rehabilitation prognosis of Danish patients with moderate to severe ABI who underwent comprehensive post-acute inpatient rehabilitation.

Methods: Routinely collected electronic health record data were extracted for adults with ABI, who were admitted to a Danish highly specialised rehabilitation facility between March 2011 and December 2022. Data were deterministically linked on an individual level where necessary. The Functional Independence Measure and Early Functional Ability scale were the rehabilitation outcomes estimated across demographic and clinical candidate predictors. In addition, the probability of a missing rehabilitation outcome assessment was investigated with logistic regression models.

Results: We included 6571 rehabilitation patients. Most patients were admitted due to ischaemic (48%) and haemorrhagic stroke (16%), or traumatic brain injury (12%); approx. 40% required total assistance on admission. Patients were discharged with a median (IQR) total, motor and cognitive FIM score of 106 (67-117), 80 (47-89), and 26 (19-30), respectively. Patients improved a median (IQR) of 20 (6-40) points on the FIM, or 0.4 (0.1-0.7) points per day of rehabilitation. Persistent limitations at discharge were observed for memory and problem solving. Functional improvements were similar across ABI types. The highest likelihood of a missing rehabilitation outcome assessments was observed in patients with moderate rehabilitation needs, short rehabilitation stays and long onset-admission intervals.

Conclusions: The present study provides estimates for the overall rehabilitation prognosis for patients with moderate to high rehabilitation needs, undergoing post-acute ABI rehabilitation in a highly specialised inpatient rehabilitation facility. In our large and diverse cohort, we provide estimates for overall rehabilitation outcomes, including probabilities for missing rehabilitation outcome assessments. The provided estimates may inform future prognosis research studies in similar populations or enable comparisons with other healthcare settings.

Objective: Glossopharyngeal neuralgia (GPN) is a rare craniofacial pain disorder causing paroxysmal throat and ear pain. For medically refractory cases, microvascular decompression (MVD) targets the underlying neurovascular compression and offers a potentially curative treatment. This systematic review and meta-analysis aimed to evaluate the efficacy, safety, and durability of MVD for GPN and identify predictors of outcome.

Methods: A comprehensive search of PubMed, Embase, Scopus, and Web of Science was conducted up to August 24, 2025, following PRISMA guidelines. Eligible studies included patients with clinically or radiologically confirmed GPN treated with MVD. Pooled estimates were calculated using random- or fixed-effects models, and heterogeneity, publication bias, and meta-regression analyses were performed.

Results: Twenty-five studies encompassing 1,009 patients were analyzed. The pooled rate of initial complete pain relief was 94.2% (95% CI: 90.2-97.3%), and long-term complete relief was 92.1% (95% CI: 86.9-96.2%). Pain recurrence occurred in 2.3% (95% CI: 0.5-4.8%), and permanent complications in 1.7% (95% CI: 0.2-4.2%). Meta-regression identified longer disease duration and prior MVD as negative predictors, while arterial compression and older age favored better outcomes.

Conclusion: MVD provides excellent and durable pain relief with minimal morbidity, confirming its role as the gold-standard treatment for classical GPN. Prospective, standardized studies are warranted to refine patient selection and optimize outcomes.

Background: Functional Cognitive Disorder (FCD) is defined by persistent subjective cognitive complaints that are inconsistent with objective performance on neuropsychological testing. One leading hypothesis suggests that FCD may involve a dissociation between different forms of metacognition, particularly between global and local self-monitoring mechanisms.

Objective: To quantitatively synthesize the evidence for global and local metacognitive performance in individuals with FCD through meta-analysis.

Methods: We conducted a systematic review and meta-analysis of studies measuring metacognitive performance in FCD, including comparisons with healthy controls and mild cognitive impairment (MCI). Global metacognition was assessed using self-appraisal metrics, while local metacognition was measured using trial-by-trial confidence accuracy tools such as meta-d'/d'. Effect sizes (Cohen's d) were calculated and pooled using random-effects models.

Results: Three studies were included in the global metacognition analysis, revealing a large pooled effect size indicating impaired global metacognition in FCD (Cohen's d = - 1.07). One study assessed local metacognition and showed no impairment (Cohen's d = 0.00). This supports the proposed dissociation, with preserved local but disrupted global self-monitoring.

Conclusion: Our findings provide meta-analytic evidence for a substantial deficit in global metacognitive insight in individuals with FCD, while local metacognitive abilities remain relatively intact. These results offer a mechanistic explanation for the discrepancy between subjective complaints and objective test performance in FCD. Interventions targeting global metacognitive appraisal may enhance clinical outcomes. Given the very small number of eligible studies, these findings should be interpreted cautiously and viewed as preliminary.