Acta Neurologica Scandinavica最新文献

Introduction: Early identification of stroke is important. The Face Arm Speech Test (FAST) and the modified National Institutes of Health Stroke Scale (mNIHSS) are two tools for stroke identification.

Aim: The aims of this study are to investigate (a) whether the use of the mNIHSS in an emergency medical service (EMS) setting improves stroke/transient ischaemic attack (TIA) identification compared with the FAST, (b) to what extent “code stroke” is activated, and (c) which neurologic deficits/symptoms affect stroke identification.

Methods: The method used is a retrospective pre–post-implementation study. EMS stroke identification was examined before and after the introduction of the mNIHSS, replacing the FAST, for EMS stroke screening. The FAST was replaced with the mNIHSS on 1 December 2022. Patients ≥ 18 years of age, diagnosed with stroke/TIA from 1 January 2021 to 31 May 2023 and under the care of the EMS, not more than 72 h before hospital care, were included. Data was manually extracted from EMS medical records regarding whether the FAST or mNIHSS was performed and if stroke/TIA was identified by the EMS personnel. The association between the applied stroke screening tool and EMS identification of stroke/TIA was then studied.

Results: A total of 1849 EMS missions with a hospital-confirmed diagnosis of stroke/TIA were included. The most common diagnosis was ischaemic stroke, 59.4%. Haemorrhagic stroke constituted 10.8%, and TIA 29.8%. Stroke/TIA was identified in 82.5% of cases. When the mNIHSS was used for stroke assessment, stroke/TIA was identified in 87.6% of cases. The corresponding figure for the FAST was 88.4%. For patients in whom neurological symptoms were unassessed, or a method for assessment other than the mNIHSS/FAST was applied, the identification rate for stroke/TIA was 41.6%. When a physician was consulted, “code stroke” was activated in 58.6% of all cases. The corresponding figure for the mNIHSS was 57.2% and for the FAST 59.9%.

Conclusions: The stroke identification rate does not appear to differ between the FAST and mNIHSS. The FAST and mNIHSS result in “code stroke” activation to an equal extent. Speech impairment and arm or leg paresis appear to improve EMS stroke identification. Conversely, impaired balance, convulsions, and vertigo/dizziness are associated with a lower identification rate. Both initial EMS suspicion of stroke and the subsequent application of a stroke scale appear to facilitate stroke identification.

Objectives: North Sea–Progressive Myoclonus Epilepsy (NS-PME) is a rare, childhood-onset disorder primarily characterized by movement disorders including progressive myoclonus and ataxia; subsequently accompanied by varying degrees of epilepsy. While motor symptoms are clearly defined, quality of life (QoL) has never been investigated in NS-PME. Additionally, nonmotor symptoms (NMS) including anxiety, depression, and cognitive problems are known to impact QoL and are increasingly being reported in other movement disorders. Hence, we examined the presence of anxiety and depression symptoms and the degree of QoL in NS-PME.

Materials and Methods: Data were collected from multiple cross-sectional, survey-based studies conducted between 2014 and 2020 at the University Medical Center Groningen (UMCG), the Netherlands. First, data on QoL, anxiety, and depression symptoms were collected from 11 persons with NS-PME. Second, to compare outcomes in NS-PME with controls (n = 21) and other hyperkinetic movement disorders (HMDs; n = 146), data from previously conducted studies in the UMCG were reused. The surveys used include the SF-36/RAND-36, the Beck Anxiety Inventory (BAI), and the Beck Depression Inventory (BDI).

Results: Symptoms of anxiety and depression were not present in the majority of persons with NS-PME. Compared to QoL scores of controls and other HMDs, persons with NS-PME scored significantly worse on physical functioning domains and, although nonsignificant, rendered the highest scores on emotional functioning domains.

Conclusion: Firstly, we did not find indications for significantly increased anxiety or depression symptoms in NS-PME. Secondly, our findings on QoL in NS-PME reveal the following: (1) a floor effect of the SF-36 questionnaire and (2) suggest a disability paradox in NS-PME, in which persons with NS-PME reported good emotional well-being, despite physical challenges. To explore the presence of a disability paradox further and generate more suitable QoL measures for movement disorders such as NS-PME, future studies should elaborate on personal perspectives, including the exploration of coping mechanisms.

Objectives: Mental fatigue is a common and debilitating symptom following an aneurysmal subarachnoid hemorrhage (aSAH). However, its long-term prevalence and consequences remain unclear. In this longitudinal pilot study, the Mental Fatigue Scale (MFS) was used to evaluate the prevalence, severity, and dynamics of mental fatigue for up to 5 years and to correlate patient demographics and early complications to the development of mental fatigue.

Method: Functional outcomes were scored using the Glasgow Outcome Scale-Extended (GOSE) during telephone interviews 1, 3, and 5 years after aSAH. The MFS questionnaires (maximum score 42, ≥ 10.5 points indicating mental fatigue) were subsequently sent by mail. Patient admission data and events during the acute phase were recorded.

Results: Of 64 included patients, 31 could be assessed at all time points. Mental fatigue (MFS score ≥ 10.5) was present in 58%, 48%, and 52% of the patients at 1, 3, and 5 years, respectively. A significant decrease in the total MFS score was observed between 1 and 5 years (p = 0.025). The proportion of patients experiencing severe mental fatigue halved from 1 to 3 years. The median (range) MFS scores were significantly higher for women (14.5, 0–29.5) than for men (3, 0–17.5) at 1 year (p = 0.043). Compared with patients experiencing loss of consciousness at ictus (LOCi), those without LOCi exhibited a progressive decline in the total MFS score (p = 0.003).

Conclusions: Although total MFS scores significantly improved, mental fatigue was a common and persistent symptom affecting half of the patients up to 5 years following aSAH. Mental fatigue was more prevalent in women than men; further, patients who experienced LOCi during the acute phase improved less over time. Our results highlight the importance of evaluating mental fatigue when assessing patient recovery and long-term outcomes.

Trial Registration: Clinical Trial NCT06239142

Background: Rapidly progressive dementia (RPD) is linked to several disorders. Reference centers for Creutzfeldt–Jakob disease have reported prion diseases as the most frequent cause of RPD. Alternatively, tertiary centers found high frequencies of nonprion diseases causing RPD, including a significant number of potentially treatable disorders. This study is aimed at describing the etiologies of RPD among inpatients from four tertiary centers in Florianópolis, Brazil.

Methods: We reviewed medical records of patients hospitalized from 2001 to 2020 under codes of the 10th revision of the International Classification of Diseases potentially related to RPD. Patients who fulfilled the criteria for RPD were selected. RPD was defined when (1) the criteria of the National Institute on Aging–Alzheimer’s Association for dementia were met and (2) the disease duration was 730 days or less. We revised all etiological diagnoses according to established clinical criteria.

Results: One hundred four patients with RPD were identified. The most frequent diagnostic groups were dementias of vascular origin (N = 27, 26.0%), nonprion degenerative diseases (N = 24, 23.0%), and mixed dementias (N = 9, 8.7%). All 11 patients with disease duration between 1 and 2 years had either dementia of vascular origin or nonprion degenerative dementia. Ten patients (9.6%)—diagnosed with autoimmune disorders, infectious disorders, neoplasia, or posterior reversible encephalopathy syndrome—showed improvement.

Conclusions: Dementias of vascular origin were the most frequent cause of RPD in our sample, which differs from what has been previously reported by the literature. Patients with dementia duration between 1 and 2 years seem to have disorders usually associated with slow progression, while all potentially reversible cases had a duration of 1 year or less. Thorough investigation of RPD is paramount for the identification of potentially reversible etiologies.

Background: Cognitive decline is an important factor affecting the health and well-being of older adults. Previous studies have shown that acrylamide (AA) caused neurological damage among occupationally exposed workers. However, the effect of AA on cognitive function in general older adults is unclear yet. Therefore, this cross-sectional study is aimed at examining the relationships between blood markers of AA and cognitive function in the general elderly population.

Methods: Four hundred sixty-seven older adults (230 men and 237 women) aged 60 years and older from the National Health and Nutrition Examination Survey cycles (2013–2014) were included in this study. Logistic regression models were applied to explore the relationships between blood markers of AA and cognitive function.

Results: After adjusting for all confounders, at the animal fluency test (AFT) dimension, the odds ratio (OR) for individuals in the highest tertile of hemoglobin (Hb) adducts of acrylamide (HbAA) and its metabolite glycidamide (hemoglobin adducts of glycidamide (HbGA)) were 0.251 (95% confidence interval (CI): 0.090, 0.699) and 0.354 (95% CI: 0.164, 0.761), respectively, compared with individuals in the lowest tertile, indicating that both HbAA and HbGA were negatively associated with the decline in cognitive function in dimension AFT. No significant associations were seen on other dimensions. Moreover, HbGA/HbAA had no association with any dimensions of cognitive decline.

Conclusion: Collectively, our results suggest that HbAA and HbGA are not positively associated with cognitive decline in the general elderly population and are negatively related to the AFT dimension of cognitive impairment.

Background: Randomised controlled trials (RCTs) for intravenous thrombolysis (IVT) in acute ischaemic stroke (AIS) historically excluded patients with pre-existing disability. This tradition echoes in clinical practice, despite evidence suggesting IVT improves chances of returning to prestroke functional status. We investigated the role of pre-existing symptomaticity by comparing IVT-treated versus IVT-withheld Modified Rankin Scale (mRS) 1 patients and mRS 0 versus mRS 1 IVT-treated patients.

Methods: The Virtual International Stroke Trials Archive (VISTA) has collated data from 38 AIS RCTs. Patients enrolled in trials whose investigative treatments reported no significant effect on outcomes or that were placebo-treated were eligible, yielding 3175 individual patient records. 2454 patients had prestroke mRS 0, and 721 had prestroke mRS 1. The negligible number of patients with prestroke mRS ≥ 2 prompted focus on prestroke mRS 1 patients. Propensity score and logistic regression were used to compare intracranial haemorrhage (ICH), neurological improvement, mortality, and functional outcome within 90 days.

Results: Of IVT-treated patients, mRS 1 subjects were female in higher proportion and had a more frequent history of stroke than mRS 0 subjects. Of mRS 1 patients, IVT-withheld subjects were older and had a more frequent history of stroke than IVT-treated subjects. No significant differences were found for National Institutes of Health Stroke Scale (NIHSS) severity and other baseline parameters. IVT-treated patients achieved significant neurological improvement more frequently than IVT-withheld patients (40.5% vs. 27.9%, p = 0.028; adjusted odds ratio (aOR) 1.53, 95% confidence interval (95% CI): 1.18–1.88), with no significant differences in mortality and favourable functional outcome. ICH was numerically higher among IVT-treated patients; however, this did not persist after matching and regression adjustment. Comparing IVT-treated mRS 0 and mRS 1 patients revealed no significant differences.

Conclusion: Our post hoc analysis of randomised data provides reassurance that IVT is safe and offers significant clinical benefit for AIS patients with pre-existing symptomaticity, as measured by the NIHSS. The lack of marked improvement on the mRS substantiates that the NIHSS is a key secondary outcome measure to capture recovery trajectories in studies concerned with reperfusion therapies.

Background: Cerebral venous thrombosis (CVT) is a rare cause of stroke with variable clinical presentation and challenging diagnosis. Computed tomography venography (CT-V) or magnetic resonance venography (MR-V) are recommended for detecting CVT, but their diagnostic performance is unclear. This systematic review examines the accuracy of CT-V and MR-V in diagnosing CVT.

Methods: We performed a systematic literature search up to 02 July 2023 to retrieve original articles studying the diagnostic accuracy of CT-V or MR-V in patients with suspected CVT. Studies with a reference standard comprising at least one other imaging method alone or in addition to clinical data were included. The quality of studies was assessed with the QUADAS-2 tool.

Results: We found eight studies (478 subjects) published between 1995 and 2022 that compared one or more diagnostic methods to a reference: CT-V (four studies, n = 95) and/or MR-V (three studies, n = 83) versus clinical and radiological consensus, CT-V versus MR-V (two studies, n = 292), or MR-V versus digital subtraction angiography (DSA) (one study, n = 52). They reported excellent diagnostic accuracy for both CT-V (sensitivity 100%, specificity 94%–100%) and MR-V (sensitivity 90%–100%, specificity 71%–100%) compared to clinical and radiological consensus, for CT-V compared to MR-V (sensitivity 96%–100%, specificity 99%–100%), and for MR-V compared to DSA (sensitivity 100%, specificity 71%). The heterogeneity of the studies allowed only an exploratory meta-analysis. The risk of bias in the use of the reference standard was high.

Conclusions: Our systematic review found a high accuracy of CT-V and MR-V for detecting CVT, suggesting that CT-V is a feasible option for centers with limited access to MR-V. However, the small sample sizes and variable reference methods impair conclusions drawn from the current literature.

Objectives: Migraine affects more than 80 million people in Western Europe. The present study evaluated disability and health-related quality of life (HRQoL) among patients with episodic migraine (EM) and chronic migraine (CM) who had failed multiple preventive treatments.

Materials and Methods: This study was an analysis of cross-sectional, web-based survey data from adult patients with migraine (EM and CM) from the United Kingdom, France, and Spain who had self-reported failure of two or more preventive treatments. Patient characteristics and patient-reported HRQoL, migraine-related disability, healthcare resource use (HCRU), and burden of migraine were evaluated by classification (CM and EM) and country (EM only).

Results: In this sample of 316 patients (United Kingdom, n = 106; Spain, n = 105; France, n = 105), 76 (24.1%) patients had CM, the mean (standard deviation [SD]) age was 39.5 (12.3) years, and 164 (51.9%) patients were female. Those patients with CM reported greater migraine-related disability based on Migraine Disability Assessment (MIDAS) scores versus those with EM (mean [SD]: 43.8 [44.7] vs. 23.2 [28.3]), as well as greater pain-related impact on daily activities and higher HCRU. Among patients with EM, MIDAS scores indicated disability was severe in Spain (mean [SD]: 31.6 [31.1]) and France (24.3 [31.1]) and moderate in the United Kingdom (13.8 [17.9]), while HRQoL was similar across countries. Regarding the burden of EM, higher levels of pain and symptom-related interference with many aspects of life, including occupational functioning, were reported in the United Kingdom and Spain versus France.

Conclusions: Migraine is associated with substantial disability and decreased HRQoL among patients who have failed previous preventive therapies. Although migraine burden varied by country, the results suggest high unmet needs in all countries. Appropriate treatment could reduce migraine-related burden and HCRU among patients with difficult-to-treat migraine.

This study is aimed at investigating brain morphometry differences between children with ASD and healthy controls (HCs) in Saudi Arabia and exploring the association between IQ levels and brain volumes within the ASD group. Participants (N = 29, 31.1% females and 68.9% males) ranging in age from 6 to 17 years were scanned by MRI at the King Faisal Specialist Hospital and Research Center (KFSHRC). Brain volumes were analyzed while correcting for age, sex, and total intracranial volume (TICV). The analysis revealed that individuals with ASD exhibited larger volumes compared to HCs in the left caudate (p < 0.001), right caudate (p < 0.001), total caudate (p < 0.001), and total hippocampus (p = 0.014). These findings provide evidence for anatomical brain abnormalities in individuals with ASD and highlight the heterogeneity of these differences across brain regions. Furthermore, the analysis revealed that higher IQ levels were negatively associated with the volumes of the left thalamus, left pallidum, left accumbens area, right thalamus, right hippocampus, and total thalamus but positively correlated with the third lateral ventricle volume in HCs, p < 0.05. These results suggest a meaningful relationship between cognitive abilities, as measured by IQ, and variations in brain volumes in HCs. The study significantly contributes to the understanding of the neurobiology of ASD in a Middle Eastern population and emphasizes the importance of considering cognitive functioning in relation to brain morphology in ASD research and clinical practice.

Insufficient evidence exists to assess the effect of titin antibodies on nonthymoma myasthenia gravis (MG) treatment and prognosis. We analyzed whether titin antibody-positive patients with nonthymoma MG (non-TMG) have a higher frequency of hospitalization than those who are titin antibody-negative. We retrospectively analyzed patient data from January 2017 to July 2022 obtained from an institutional databank. We compared the clinical characteristics, treatment regimens, and MG-related annual hospitalization rate between titin antibody-positive (Titin+ group) and titin antibody-negative (Titin− group) patients with non-TMG. Multivariate logistic regression was employed to examine the factors associated with multiple hospital admissions (≥ 2). Finally, 170 patients with non-TMG were included, of whom 67 (39.4%) were Titin+ and 103 (60.6%) were Titin−. Compared with the Titin− group, the Titin+ group exhibited a notably higher annual hospitalization rate (p = 0.011). Multivariate logistic regression analysis revealed that titin antibody positivity was significantly associated with multiple hospitalizations. The optimal cutoff value for titin antibody levels related to multiple hospitalizations was 3.085. In patients with non-TMG, titin antibodies were associated with more frequent hospitalizations. For these patients, especially those with titers ≥ 3.085, close monitoring of clinical symptom changes may reduce relapse.