Acta Neurologica Scandinavica最新文献

Background. Stroke incidence and mortality have drastically decreased in high-income countries in the past twenty years. In this study, we provide updated estimates on mortality, recurrent stroke, and functional dependency among patients with first-ever ischemic stroke and assess predictors associated with poor outcomes with a focus on age, vascular factors, stroke severity, function, and comorbidity burden. Methods. MIDNOR STROKE is a multicenter prospective longitudinal study including patients with first-ever ischemic stroke admitted to stroke units in Central Norway during 2015-2017. Data on survival, stroke recurrence, and functional dependency were collected during hospital stay and follow-up. Multivariable Cox proportional hazard models and logistic regression models were used to analyze predictors of mortality, stroke recurrence, and functional dependency. Results. A total of 794 participants were included in the study. After a year, 7.6% of the participants had died, 5.8% had a recurrent stroke, and 13.6% experienced functional deterioration to dependency. Multivariable analysis revealed that age (HR: 1.07, 96% CI: 1.03, 1.10), stroke severity (HR: 1.10, 95% CI: 1.07, 1.13), comorbidity burden (low: HR: 4.05, 95% CI: 1.48, 11.10; moderate: HR: 5.44, 95% CI: 2.06, 14.40; and high: 7.72, 95% CI: 2.85, 21.00), and coronary artery disease (HR: 2.40, 95% CI: 1.32, 4.38) predicted all-cause death. Statin therapy predicted improved survival (HR: 0.39, 95% CI: 0.21, 0.75). High age (HR: 1.09, 95% CI: 1.05, 1.14) and increased stroke severity (OR: 1.26, 95% CI: 1.17, 1.38) predicted elevated risk of functional dependency at one year. Conclusions. In this study, we have demonstrated that 1-year survival following first-ever ischemic stroke was high compared to previous reports and that statin therapy predicted improved survival. The risk of recurrent stroke after one year was found to be low compared to previous studies. Approximately 14% of stroke survivors who were initially functionally independent experienced deterioration to functional dependency. In addition to older age and stroke severity, increased comorbidity burden and a history of coronary artery disease predicted poor stroke prognosis. Interventions aimed at reducing stroke severity may improve patient outcomes. Furthermore, prevention efforts targeting conditions such as CAD and reducing overall comorbidity burden in stroke patients may favorably improve survival. This trial is registered with NCT03962127.

Background. Up to 80% of people with MS experience worsening of their condition upon an increase in body temperature. Therefore, various options of cooling are being evaluated to help improve physical performance in people with MS. Most previous studies used active cooling methods. Our aim was to study the effect of simple device providing passive cooling. Methods. A randomized crossover study was conducted in 21 thermosensitive people with mild to moderate disability. Subjects were tested immediately before and after intervention (experimental or sham cooling). The assessment included timed 25-foot walk test, the 2- and 6-minute walk test, nine-hole peg test, and symbol digit modalities test. Results. A significant improvement was found in the experimental group in timed 25-foot walk test (p = 0.011) and in nine-hole peg test for dominant hand (p = 0.033). No significant improvement was found in the control group (sham cooling). Conclusions. Wearing cooling cap can improve short-term functional performance (walking and fine motor skills) in thermosensitive people with MS. This passive cooling method can be considered as a symptomatic treatment for some people with MS. This trial is registered with ISRCTN56350227.

Background. Parkinson’s disease (PD) is a common neurodegenerative disorder characterized by bradykinesia, resting tremor, and muscle rigidity. Visual disturbances have been also described among non-motor features. Objective. We aimed to investigate the structural and vascular changes in the retinal and choroidal vascular networks, and to assess any relationship with motor and non-motor symptoms (NMS) in PD patients. Methods. Ganglion cell complex (GCC), retinal nerve fiber layer (RNFL), and subfoveal choroidal thickness (SFCT) were examined using spectral domain-optical coherence tomography (SD-OCT). The vessel density (VD) of retinal and choriocapillary vascular networks in macular area and the foveal avascular zone (FAZ) area were evaluated by OCT angiography (OCTA). All patients underwent clinical evaluation using motor section of the Unified PD Rating Scale (UPDRS-III) and the Hoehn and Yahr (HY) scale. Results. A total of 48 eyes from 24 PD patients and 50 eyes from 25 controls were assessed. At SD-OCT, GCC and RNFL were more significantly thin in patients compared to controls. At OCTA exam, PD subjects showed lower values in VD of superficial capillary plexus (SCP) and radial peripapillary capillary plexus in comparison to controls, whereas FAZ area resulted in a significant increase in the patient group. We found a negative correlation between the age at onset and VD of SCP, and between HY score and RNFL thickness and FAZ. UPDRS-III score was negatively correlated with VD of deep capillary plexus. Discussion. The impairment of retinal structure and microvasculature seems to correlate with disease severity and progression in PD. Retinal anomalies can be considered as non-motor manifestations that could occur already in the early stage of the disease.

Background and Aims. Gait impairment is a common manifestation of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). However, clinicians lack an effective monitoring tool, as no gait test has been validated for CIDP. The aim of this study was to determine the usefulness of three tests in monitoring the clinical course of patients with CIDP: Timed Up and Go (TUG), 10-Meter Walk Test (10MWT), and 30-Second Chair Stand (30SCS). Methods. This is a prospective, single-center observational study. We included newly diagnosed CIDP patients starting treatment or relapsed CIDP patients requiring new treatment. We monitored the clinical course using CIDP-validated clinical scales and correlated changes in clinical status with the results of the gait tests. A ROC curve was developed, and we chose the cut-off point on each scale with the best specificity and sensitivity to detect change in clinical status. Results. A total of 20 patients have been recruited. The 3 tests show a statistical correlation with objective clinical improvement. In patients who have showed clinical improvement during the follow-up examination, a mean reduction of 4.8 seconds in TUG and 2.6 in 10MWT and a gain of 3 repetitions in 30SCS have been observed. The optimal cut-off points for each test were TUG ≤ 1 seconds, 10MWT ≤ 1 seconds, and 30SCS ≥ 1 repetition. The TUG test has the highest sensitivity (82.6%), and the 30SCS test has the highest specificity (100%) for detecting clinical improvement. Conclusions. The study found that the TUG and 30SCS tests could become effective tools for monitoring treatment response in CIDP patients.

Background. Sleep disturbances are commonly reported, although underestimated complaints from people with multiple sclerosis (MS). The aim of the study was to analyze the frequency and type of sleep disturbances in MS patients and to evaluate their relationships with demographics and clinical data. Methods. The study group consisted of 178 patients with relapsing-remitting MS: 130 females and 48 males. Clinical measures (disease duration, disability level in Expanded Disability Status Scale (EDSS), and treatment) were acquired from medical records. The questionnaire was applied, containing questions about sleep disturbances, somatic complaints, perception of fatigue, depression, anxiety, and problems at work and in social/family life. Athens Insomnia Scale (AIS) and Karolinska Sleepiness Scale (KSS) were performed to quantify sleep problems and Hamilton Depression Rating Scale (HDRS) and Addenbrooke’s Cognitive Examination (Mini-ACE) to assess level of depression and cognitive performance. Electroencephalography was recorded to identify electrophysiological indices of sleep. Results. 109 patients (61%) reported sleep disturbances, most frequently insomnia, snoring, and parasomnias. This subgroup had significantly higher scores in AIS (p < 0.0001) and KSS (p = 0.010) and slightly higher EDSS score (p = 0.048) and more often complained of fatigue (71% vs. 53%, p = 0.0148), involuntary limb movement (42% vs. 25, p = 0.0170), and breathing disturbances (10% vs. 0%). There was a significant correlation between the results of AIS and HDRS (Rs = 0.715, p < 0.05). Conclusion. Sleep disturbances, predominantly insomnia, are reported by more than a half of the patients with relapsing-remitting MS. Significant associations were found between sleep problems and MS-related clinical symptoms and psychosocial issues.

Background. Potassium and sodium are inextricably linked to the maintenance of cell potential and electrolyte homeostasis. Few studies have examined their joint relationship with outcomes after stroke. The objective of this study is to ascertain whether combined low nadir serum sodium and potassium levels are correlated with an unfavorable outcome in ischemic stroke. Methods. Retrospective cohort study of 2,920 consecutive patients with first-ever ischemic stroke. Serum sodium and potassium levels were measured at 10 consecutive time points over 10 days poststroke. The Youden Index was performed to identify patients with low nadir sodium (<140 mmol/L) and potassium (<3.65 mmol/L) levels, defined as a NaK score of 2. Multivariable logistic regression and Cox proportional hazard analysis were used to evaluate the independent relationship of sodium and potassium levels with clinical outcomes at three months. Results. A total of 2,920 patients with ischemic stroke were analyzed (mean age 62.14 ± 14.13 years; 60.19% male), of whom 740 (25.3%) with both low nadir sodium and potassium levels had a quintuple 3-month case fatality compared to other patients (10.6% vs. 2.1%). Multivariable analyses identified NaK = 2 as an independent predictor of 3-month death (adjusted odds ratio (OR) 2.23; 95% confidence interval (CI) 1.17-4.53; p = 0.019) and an unfavorable shift in the distribution of scores on the modified Rankin scale (adjusted OR 1.51, 95% CI 1.12-2.04; p = 0.007). Conclusions. Low sodium and potassium levels are common after ischemic stroke and are independent predictors of subsequent death.

Purpose. This retrospective study is aimed at investigating the clinical characteristics of autoimmune encephalitis (AE) and long-term prognosis of patients who initially present with seizures as well as risk factors for enduring predisposition to seizures in AE. Methods. From January 1, 2013, to October 31, 2021, a total of 343 AE patients from a single center diagnosed with autoimmune encephalitis (AE) were enrolled in this study, including 198 antibody-positive AE and 145 antibody-negative but probable AE. According to initial symptoms, AE patients were divided into two groups: onset with seizure group and onset with nonseizure group. The clinical characteristics were retrospectively reviewed. Patients were clinically evaluated at onset and at 6, 12, and 24 months of follow-up. Modified Rankin Scale (MRS) score, Clinical Assessment Scale in Autoimmune Encephalitis (CASE) score, and seizure-related information were assessed. Results. In AE, patients with seizures as the first presentation were younger, with a median-onset age of 28 years old. Compared with other types of antibody-positive AE, anti-GABABR AE more frequently began with seizures, while anti-CASPR2, anti-AMPAR, and anti-DPPX encephalitis usually began with symptoms other than seizures. The most common type of initial seizures in AE was focal to bilateral seizure (67.6%), with a significant prevalence in antibody-positive AE (P = 0.001). In addition, compared with nonseizure group, patients with seizures as an initial presentation had higher MRS and CASE scores at 24 months of follow-up. Older age at onset and focal nonmotor seizure type were independent risk factors for an enduring predisposition to seizures in AE patients. Conclusion. The younger and anti-GABABR-positive AE patients are more prone to onset with seizures. AE patients who initially presented with seizures had worse long-term neurological recovery. Onset age and seizure type should be highly appreciated when formulating the strategy for therapy at post-AE status.

Background. Although patients can present with progressive pseudobulbar palsy due to neurodegenerative diseases, detection of the precise location of radiological abnormalities can be difficult. ¹⁸F-THK5351 was initially developed as a tau positron emission tomography (PET) tracer. Later, it was found to sensitively detect astrogliosis associated with neurodegeneration. Therefore, it has been used in diagnosis of various diseases. However, its utility in progressive pseudobulbar palsy was unknown. Methods. ¹⁸F-THK5351 PET results of two patients presenting with progressive pseudobulbar palsy are reported. Results. Patient 1 was a 77-year-old man with a two-year history, and Patient 2 was a 61-year-old woman with a 1-year history. Both patients presented with gradually progressive spastic dysarthria, suggesting pseudobulbar palsy without clinical lower motor neuron signs. Facial asymmetry was detected in both patients, while left-dominant pyramidal signs in the extremities were detected only in Patient 2. Brain magnetic resonance imaging did not show signal abnormality explaining pseudobulbar palsy. However, ¹⁸F-THK5351 PET clearly visualized bilateral increased uptake in limited areas of the posterior portion of the precentral gyrus, corresponding to the midportion of the primary motor cortex. Laterality of increased ¹⁸F-THK5351 uptake corresponded to the symptom laterality and was higher on the left and right side in patients 1 and 2, respectively. After one year, Patient 1 was unable to vocalize and could only produce grunts; concomitant apraxia of speech was suspected. Conclusions. ¹⁸F-THK5351 PET is a useful method to detect bilateral primary motor cortex involvement in patients presenting with progressive pseudobulbar palsy, likely by imaging astrogliosis.

Objective. Nocturnal seizures are usually underestimated and represent a major problem in adult patients with epilepsy. Our aim was to study the effectiveness of perampanel for the treatment of nocturnal seizures in adult patients with epilepsy. Methods. Observational study of a prospectively acquired sample of adult patients with focal and generalized epilepsy in which perampanel was started from January to October 2021 in a specialized epilepsy unit in a tertiary hospital. Demographic and clinical characteristics were recorded. All patients completed a follow-up period of at least 3 months. Seizure frequency during the 6-month period before the patient started treatment was obtained from medical records. Retention and responder rates (considered as a nocturnal seizure frequency reduction of ≥50%) and improvement of subjective sleep disturbances were analyzed as outcome measures. Results. Forty-eight patients were included (mean age 39.8 ± 17.4; 60.4% men), and 38 of them had a 6-month follow-up. Focal epilepsy was the most common diagnosis (81.3%), and most patients had a structural etiology (56.3%). Thirty-four (70.8%) patients had drug-resistant epilepsy. The mean nocturnal seizure frequency per month at baseline was 13.2 ± 35.9. Fifteen (31.3%) patients had subjective sleep disturbances at baseline, of which insomnia was the most frequent complaint (16.7%). Perampanel was started at a median dose of 4 mg/day (range = 2-14). At 3-month follow-up, the retention rate was 74.6%; 64.6% were considered responders (54.2% were seizure-free). Monthly nocturnal seizures decreased significantly at 3 months (8.2 ± 26.7 vs. 13.2 ± 35.9 seizures/month; p = 0.044) and 6 months (5.3 ± 18.2 vs. 13.2 ± 35.9 seizures/month; p = 0.006). Subjective sleep disturbances improved at 3-month follow-up (10.4% vs. 31.3%; p = 0.002) and 6-month follow-up (10.5% vs. 31.3%; p = 0.022). Significance. Perampanel can be a suitable treatment option in adult patients with both focal and generalized epilepsy with nocturnal seizures and can reduce the presence of sleep complaints.

Background. Acute sensorineural hearing loss (SNHL) is a rare development in the central nervous system (CNS) demyelinating diseases such as aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods. We retrospectively reviewed consecutive patients with seropositive NMOSD or MOGAD in the CNS Inflammatory and Demyelinating Disease Registry at Samsung Medical Center from January 2015 to December 2020. After the medical chart review, the demographic data and the results of brain magnetic resonance imaging (MRI) and audiometry of patients with hearing loss were collected. Results. Five patients (NMOSD, n = 3; MOGAD, n = 2) were diagnosed with SNHL, two developed SNHL before the first core clinical symptom, and another two patients who underwent brain MRI at the timing of hearing loss showed lesions. Only three patients received high-dose steroids; however, hearing loss did not improve in any patients. Conclusion. SNHL was observed in a small number of patients with seropositive NMOSD and MOGAD; however, it could be underrecognized. Further large cohort prospective studies are helpful to elucidate the clinical implication of SNHL in NMOSD and MOGAD.