Acta Neurologica Scandinavica最新文献

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.

Background: Dysfunctions of the hypothalamic-pituitary-adrenal (HPA) axis can trigger multiple sclerosis (MS) symptoms. Mood disorders comorbid with MS are implicated in the HPA axis activation in most people with MS (pwMS). This study purposed to examine salivary cortisol (SC) levels and their association with mood disorders in pwMS.

Methods: Forty-three men as pwMS and sixteen men as healthy controls (HC) were included in this study. pwMS and HC completed the Beck Depression Inventory (BDI), 36-Item Short Form Survey (SF-36), Fatigue Severity Scale (FSS), Depression Anxiety and Stress Scale, and Hospital Anxiety and Depression Scale questionnaires. SC levels were also measured in pwMS and HC. A linear regression model was used to analyze the relationship between the Expanded Disability Status Scale (EDSS) and mood disorders and SC levels. Pearson’s or Spearman’s tests examined the correlation between SC levels and mood disorders.

Results: The SC level, anxiety, depression, fatigue, and stress were significantly higher in pwMS than in HC (p < 0.05). pwMS also showed significant correlations between SC levels and role physical (r = 0.3, p < 0.05) and emotional (r = 0.34, p < 0.05) in SF-36. Among SF-36 domains, only general health (β = −0.72, p < 0.05) and physical functioning (β = −1.61, p < 0.05) were negatively associated with disease duration. No correlation was found between SC levels and anxiety, depression, and fatigue (p > 0.05).

Conclusions: The pwMS showed mild to moderate depression, anxiety, and fatigue. Only general health and physical functioning as aspects of quality of life were related to disease duration, while SC levels, mood disorders, and other quality-of-life domains were not associated with clinical characteristics. Also, this study failed to find a significant role for SC as a valuable marker in approaching mood disorders in pwMS.

Background: Rituximab (RTX) and eculizumab (ECU) are treatment options for refractory myasthenia gravis (MG), but comparative clinical data derived from real-world experience are limited. Here, we describe the baseline characteristics, treatment, and safety outcomes of patients with antiacetylcholine receptor antibody-positive (AChR+) generalized myasthenia gravis (gMG) treated with ECU and/or RTX in our clinic.

Methods: Patients with refractory AChR+ gMG who received ECU or/and RTX treatment for more than 1 year at the Department of Neurology, Istanbul Faculty of Medicine were included in this observational study. After obtaining written patient consent, data were collected retrospectively from medical records.

Results: Twelve patients treated with ECU and 25 patients treated with RTX were included in the analysis. Groups were comparable with regard to demographic and clinical characteristics, including age at onset of MG, disease duration, and history of thymoma. ECU was associated with significantly better outcomes compared with RTX, as measured by decreases in the mean MG activities of daily living score at 1 (p = 0.024), 3 (p < 0.001), 6 (p < 0.001), and 12 (p < 0.001) months of treatment; steroid-sparing effect after 1 year of treatment (decrease in mean [standard deviation] daily prednisolone dose of −21.8 mg [13.5] vs. −6.6 mg [9.4] with RTX; p < 0.001); and need for rescue treatment and number of myasthenic crisis episodes during treatment (p < 0.001). No new safety signals were observed with either treatment.

Conclusion: Our data provide real-world evidence supporting ECU over RTX to treat patients with refractory AChR+ gMG.

Background: This study was aimed at analyzing cognitive function and quantitative electroencephalogram (qEEG) in patients with isolated REM sleep behavior disorder (iRBD) based on the presence of depression and at evaluating the impact of depression on phenoconversion to neurodegenerative diseases.

Methods: Individuals diagnosed with iRBD via polysomnography were included. Based on the presence of depression, patients were categorized into two groups. Neuropsychological tests and qEEG were conducted following the diagnosis of iRBD, and outcomes were compared between the two groups. Patients were regularly followed to monitor their phenoconversion status. Cox regression analysis was performed to assess the hazard ratio associated with depression.

Results: Ninety iRBD patients (70% males) were included, with a median age of 66.3 years. Depression was identified in 26 (28.9%) of these patients. The depressed group showed significantly poorer performance only in color reading subtest of Stroop (p = 0.029) compared to the nondepressed group, showing reduced processing speed. In qEEG, relative gamma power (p = 0.034) and high gamma power (p = 0.020) in the parietal region were significantly higher in the depressed group than in the nondepressed group. Depression was associated with a hazard ratio of 3.32 for the risk of phenoconversion to neurodegenerative diseases in iRBD patients (p = 0.011).

Conclusion: Depressive symptoms in iRBD patients should be closely monitored as they could aggravate cognitive dysfunction and increase the risk of phenoconversion to neurodegenerative diseases.

Objective: Work phobic anxiety can occur as an additional problem in any somatic illness and is often associated with work capacity limitations and sick leave. This study investigates work-related capacity limitations in patients with and without work phobic anxiety who are undergoing neurological rehabilitation. It was conducted in the rehabilitation facility Brandenburgklinik Berlin-Brandenburg in Germany.

Methods: Work phobic anxiety was assessed with the Workplace Phobia Scale (WPS). The response rate was 69.51%. Capacity limitations (Mini-ICF-APP) were compared between 19 patients with neurological conditions and work phobic anxiety and 209 patients with neurological conditions without work phobic anxiety. Work participation restrictions were examined and compared using the self- and observer rated Index for Measuring Participation (IMET, IMEP-O). The work ability assessments regarding the patients’ last occupation as well as the general labor market were conducted by the treating physicians as part of their medical reports and compared between the subgroups. The work ability was rated as less than 3 h, 3 to less than 6 h, or 6 h or more a day for more than 6 months, with the latter being an indication for prognostic work ability and potential reintegration into the labor market. This is a common classification in sociomedical assessments in Germany.

Results: Independent t-tests showed that patients with work phobic anxiety were significantly more limited in their planning and structuring of tasks (t[20.104] = 2.310, p = 0.032, d = 0.68), flexibility (t[217] = 3.586, p < 0.001, d = 0.86), assertiveness (t[19.613] = 2.151, p = 0.044, d = 0.70), group integration (t[19.534] = 2.274, p = 0.034, d = 0.76), and mobility capacities (t[16.616] = 2.198, p = 0.042, d = 0.76) and significantly more restricted in participating in work compared to patients without work phobic anxiety (IMEP-O: t[23.549] = 2.298, p = 0.031, d = 0.40; IMET: t[27.191] = 4.581, p < 0.001, d = 0.78). Chi-squared tests revealed no significant differences in the physicians’ work ability assessments between patients with and without work phobic anxiety.

Conclusions: While work phobic anxiety seems to not be decisive concerning early retirement assessments, it is associated with greater capacity limitations and work participation restrictions. The results highlight the need for identifying work phobic anxiety and associated capacity limitations in clinical practice.

Background: The assessment of serum neurofilament light chain (sNfL) is increasingly significant in the field of neurology. In multiple sclerosis (MS), it proves valuable as a marker for monitoring disease activity and treatment response.

Objective: To compare the Simoa and Lumipulse platforms for measuring sNfL and to establish age-specific reference ranges within a substantial cohort of individuals diagnosed with MS.

Methods: Two hundred sixty-one sNfL measurements from a cohort of 229 MS patients were analyzed with Simoa and Lumipulse. Reference ranges for sNfL were established for three age groups (18–39 years, 40–59 years, and > 60 years) selecting data from 166 patients with stable relapsing–remitting MS.

Results: While sNfL levels correlated between assays, Lumipulse exhibited values higher than Simoa. Passing–Bablok’s analysis confirmed linearity between the two datasets, and the Bland–Altman comparison further supported the agreement between the methods. Analyzing reference ranges for sNfL across three age groups in stable RRMS patients revealed significant differences between the groups on each of the platforms. Although the values followed the same trend, each technology utilized distinct reference ranges.

Conclusion: Simoa and Lumipulse platforms proved equally effective in monitoring patients with MS. The enhanced accessibility of the Lumipulse platform facilitates the expansion of research on sNfL as a biomarker for monitoring MS, thus offering promising opportunities for broader accessibility and advancement in this field.

Purpose: Visual impairment (VI) is still underdiagnosed in patients with acquired brain injury despite the large impact on daily life activities and rehabilitation. The aim of this study was to explore the current practice of identification of VI, management plans, referrals, and experienced barriers in Norwegian hospital settings.

Materials and Methods: An online survey was sent out to leaders (n = 62) in hospitals treating patients with acquired brain injury. Data from 22 items covering routines, interdisciplinary collaboration, assessment tools, protocols, barriers, referral practice, and background information were collected.

Results: Respondents (n = 108) comprised various professions from mainly acute or subacute settings. Visual field deficits, oculomotor dysfunction, and neglect were most commonly but not routinely assessed (53%–57%). Besides medical examination, patient interviews, and observations rather than standardized tests or questionnaires (82% vs.25%) were used. Few workplaces seemed to have interdisciplinary vision teams (13%). Barriers were patient characteristics such as cognitive and language impairment and limited resources. Below half (45%) felt competent in assessing VI or had attended courses on the topic (44%).

Conclusions: The study highlights the need to implement standardized assessment, improve interdisciplinary collaboration, and facilitate training courses to enable hospital staff to assess VI after brain injury. These steps could contribute to improved assessment and management of VI and contribute to overcome the indicated barriers leading to better patient care and outcome.

Multiple sclerosis (MS) is a demyelinating disease of the central nervous system, which is a primary cause of neurological disability in young and middle-aged individuals in economically developed nations. Although MS is more prevalent and of ever-increasing incidence in developed countries, patients with MS in developing nations confront unique challenges marked by significant economic and social burdens due to their limited access to the advanced diagnostic and treatment resources that are available in developed regions. Timely diagnosis is paramount for effective intervention in MS cases. Regrettably, there is limited availability of neuroimaging evaluations in low- and middle-income nations, prompting the need to obtain alternative diagnostic approaches. In this article, we provide an overview of the worldwide epidemiology of MS, delving into associated risk factors, and examining serological biomarkers as alternatives to imaging. It is, therefore, imperative that strategies for the early diagnosis and treatment of MS are implemented in low- and middle-income countries. This will lead to improved prognoses and quality of life, with a particular focus on enhancing outcomes.