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Aims: Since its outbreak in 2020, the COVID-19 pandemic has directly caused the premature death of millions. However, indirect consequences, such as social restrictions, have affected a far greater number. We explored the association between the spread of COVID-19 and end-of-life circumstances in the infected and non-infected population in Sweden. Methods: In this descriptive, population-based, observational study, we primarily used data from the Swedish National Registry of Palliative Care, which covers about 60% of all deaths in Sweden. We explored the association between the spread of COVID-19 and place of death, people present at death and end-of-life symptoms using regression analyses. Results: The study included 190,291 individuals who died in any region of Sweden from 1 January 2019 to 30 June 2022, of which 10,646 were COVID-19 cases. Correlated to the temporal and geographical spread of COVID-19, there was a greater proportion of individuals dying without the presence of their next-of-kin, and consequently more people dying alone, both in those with and without COVID-19. There was a similar pattern of a greater proportion of deaths taking place in nursing homes and in the individual's own home. However, we did not find substantial associations to reported symptoms, such as anxiety or confusion. Conclusions: This study shows the profound effects of the COVID-19 pandemic on end-of-life circumstances in both the infected and non-infected population in Sweden. As we prepare for future pandemics, there is a need to develop strategies to minimise the impact on non-infected individuals. [ABSTRACT FROM AUTHOR]

Objective: Magnetic resonance imaging can detect neurodegenerative iron accumulation in the motor cortex, called the motor band sign. This study aims to evaluate its sensitivity/specificity and correlations to symptomatology, biomarkers, and clinical outcome in amyotrophic lateral sclerosis. Methods: This prospective study consecutively enrolled 114 persons with amyotrophic lateral sclerosis and 79 mimics referred to Karolinska University Hospital, and also 31 healthy controls. All underwent 3‐Tesla brain susceptibility‐weighted imaging. Three raters independently assessed motor cortex susceptibility with total and regional motor band scores. Survival was evaluated at a median of 34.2 months after the imaging. Results: The motor band sign identified amyotrophic lateral sclerosis with a sensitivity of 59.6% and a specificity of 91.1% versus mimics and 96.8% versus controls. Higher motor band scores were more common with genetic risk factors (p = 0.032), especially with C9orf72 mutation, and were associated with higher neurofilament light levels (std. β 0.22, p = 0.019). Regional scores correlated strongly with focal symptoms (medial region vs. gross motor dysfunction, std. β −0.64, p = 0.001; intermediate region vs. fine motor dysfunction, std. β −0.51, p = 0.031; lateral region vs. bulbar symptoms std. β −0.71, p < 0.001). There were no associations with cognition, progression rate, or survival. Interpretation: In a real‐life clinical setting, the motor band sign has high specificity but relatively low sensitivity for identifying amyotrophic lateral sclerosis. Associations with genetic risk factors, neurofilament levels and somatotopic correspondence to focal motor weakness suggest that the motor band sign could be a suitable biomarker for diagnostics and clinical trials in amyotrophic lateral sclerosis. [ABSTRACT FROM AUTHOR]

To describe the diagnostic and prognostic performance, and longitudinal trajectories, of potential biomarkers of neuroaxonal degeneration and neuroinflammation in amyotrophic lateral sclerosis (ALS). This case-control study included 192 incident ALS patients, 42 ALS mimics, 114 neurological controls, and 117 healthy controls from Stockholm, Sweden. Forty-four ALS patients provided repeated measurements. We assessed biomarkers of (1)neuroaxonal degeneration: neurofilament light (NfL) and phosphorylated neurofilament heavy (pNfH) in cerebrospinal fluid (CSF) and NfL in serum, and (2)neuroinflammation: chitotriosidase-1 (CHIT1) and monocyte chemoattractant protein 1 (MCP-1) in CSF. To evaluate diagnostic performance, we calculated the area under the curve (AUC). To estimate prognostic performance, we applied quantile regression and Cox regression. We used linear regression models with robust standard errors to assess temporal changes over time. Neurofilaments performed better at differentiating ALS patients from mimics (AUC: pNfH 0.92, CSF NfL 0.86, serum NfL 0.91) than neuroinflammatory biomarkers (AUC: CHIT1 0.71, MCP-1 0.56). Combining biomarkers did not improve diagnostic performance. Similarly, neurofilaments performed better than neuroinflammatory biomarkers at predicting functional decline and survival. The stratified analysis revealed differences according to the site of onset: in bulbar patients, neurofilaments and CHIT1 performed worse at predicting survival and correlations were lower between biomarkers. Finally, in bulbar patients, neurofilaments and CHIT1 increased longitudinally but were stable in spinal patients. Biomarkers of neuroaxonal degeneration displayed better diagnostic and prognostic value compared with neuroinflammatory biomarkers. However, in contrast to spinal patients, in bulbar patients neurofilaments and CHIT1 performed worse at predicting survival and seemed to increase over time. [ABSTRACT FROM AUTHOR]

Thanks to a more widespread knowledge of the disease, and improved diagnostic techniques, the clinical spectrum of cerebral amyloid angiopathy (CAA) is now broad. Sporadic CAA, hereditary CAA, CAA-related inflammation (CAA-ri) and iatrogenic CAA (iCAA) create a clinical and radiological continuum which is intriguing and only partially discovered. Despite being relatively rare, CAA-ri, an aggressive subtype of CAA with vascular inflammation, has gained growing attention also because of the therapeutic efficacy of anti-inflammatory and immunomodulating drugs. More recently, diagnostic criteria have been proposed for an unusual variant of CAA, probably related to an iatrogenic origin (iCAA), toward which there is mounting scientific interest. These atypical forms of CAA are still poorly known, and their recognition can be challenging and deserve to be pursued in specialized referral centres. The aim of this brief review is to focus current developments in the field of rare forms of CAA, its pathogenesis as well as clinical and biological features in order to increase awareness of these rare forms. [ABSTRACT FROM AUTHOR]

This retrospective cohort study aims to provide a comprehensive account of death in Swedish patients with ALS, including clinical status preceding death, the death setting, as well as symptoms. The study presents detailed information on a cohort of patients with ALS from Stockholm, Sweden, deceased in 2018–2020. In addition, selected information is presented on a larger complementary cohort of ALS patients from all regions of Sweden deceased in 2011–2020. Data were obtained from patient medical records, the Swedish Motor Neuron Disease Quality Registry, and the Swedish Quality Registry of Palliative Care. Ninety-three patients were included in the main cohort and 2224 patients in the complementary cohort. In the main cohort, there was a slow decline in weight and motor function during the 12 months preceding death. Most (93.4%) anticipated/prolonged deaths occurred in a palliative care unit, at home, or in an assisted living facility while 44.8% of precipitous deaths occurred in a hospital ward. Next of kin or health care staff were present at death for most patients (78.7%). In the final week of life, 41.1% experienced at least one symptom (either pain, anxiety, confusion, or dyspnea) that was only partially relieved or not at all. The majority of patients died in their own homes or at a palliative unit in the presence of next of kin and most symptoms were adequately managed. This paper might be used in educating patients, next of kin as well as health professionals, decreasing uncertainty surrounding the end of life. [ABSTRACT FROM AUTHOR]

Objective: To provide a detailed and differentiated description of the path to receiving the correct amyotrophic lateral sclerosis (ALS) diagnosis, including delay times, referrals, alternate diagnoses, and clinical progression. Methods: Medical records until the date of ALS diagnosis were reviewed and linked to the Swedish Motor Neuron Disease Quality Registry. Results: The study included 353 Stockholm ALS patients diagnosed in 2016–2021. Patients were divided into four groups: 117 (33.1%) with lower extremity (LE), 85 (24.1%) with upper extremity (UE), 136 (38.5%) with bulbar, and 15 (4.2%) with respiratory onset. The time from onset to diagnosis was 16.0 (9.4–27.5) months in LE, 12.9 (8.8–17.8) months in UE, 11.7 (7.4–16.0) months in bulbar, and 8.3 (4.7–15.6) months in respiratory onset. Patients with UE or LE onset were often referred to orthopedics or a spinal/hand surgery clinic (29.3% for LE and 41.8% for UE), while bulbar patients were more frequently referred to ENT (66.3%). For those with LE or UE onset, the most common alternate diagnosis was spinal/foraminal stenosis whereas myasthenia gravis and stroke were more common for bulbar onset patients. For the respiratory group, cardiopulmonary diagnoses predominated. The proportion of all patients in King's stage 3 or 4 increased from 11.3% to 46.1% from the initial health care visit to diagnosis. Conclusions: There was great variation in the path to ALS diagnosis according to the onset clinical phenotype. In all groups, the diagnostic delay and clinical progression was substantial. We identified subgroups where the delay was the longest and might be reduced. [ABSTRACT FROM AUTHOR]

Objective: This paper aims at examining the clinical characteristics of ischemic stroke patients with different levels of prestroke functional dependency, their long‐term outcome, and determinants of five‐year mortality. Materials and methods: We describe demographics, comorbidity, treatment, as well as long‐term mortality, and functional status of 5899 prestroke‐dependent ischemic stroke patients stratified by dependency level and compared to a concurrent cohort of 14 148 prestroke‐independent patients. The study was based on 2016 survey data from Riksstroke, the Swedish national stroke register, and patients were followed up at three months, 12 months, and either at three or five years. We used Cox regression for mortality predictor analysis and multiple imputation was performed to minimize bias from loss to follow‐up. Results: With increasing level of prestroke dependency, comorbidity burden was higher, drug prescription lower, and prognosis less favorable. At three years, the proportion that had died or deteriorated were 82.6%, 87.5%, and 86.3% in moderate, moderately severe, and severe dependency, respectively. In moderate dependency, prognosis was relatively favorable: Three‐month mortality was half of that seen in severe dependency (25.3% versus 49.6%). Differences in overall outcome between groups of varying prestroke functional dependency level were statistically significant (P <.05) at all follow‐up time points. Conclusions: There was great heterogeneity between groups of different level of prestroke dependency; those of moderate dependency had a relatively favorable prognosis. Patients of different prestroke level of dependency need to be addressed separately, and further research is needed characterizing this group and exploring management strategies. [ABSTRACT FROM AUTHOR]

Background: Informal caregivers provide a large amount of day-to-day assistance and are crucial for the ability of survivors to recover and adapt to life after stroke. Aim: The development of caregiver support programs is limited by lack of large long-term follow-up studies. We present a comprehensive study of Swedish stroke caregivers' life situation in relation to degree of functional dependency of the survivor. Patients and methods: In 2016, the Swedish Stroke Register, Riksstroke, conducted a long-term follow-up survey on caregivers to patients with stroke three and five years earlier. Items on psychological well-being were adapted from the 36-item short-form health survey and poor outcome was defined using the 36-item short-form health survey reference material. Survivor degree of dependency was indicated by the caregiver as independent, partially dependent, or completely dependent. Results: A total of 5063 community dwelling dyads were included: 56.5% of survivors were independent, 33.4% partially dependent, and 10.1% completely dependent. Caregiver life impact, need of support, and proportion of poor psychological well-being increased incrementally with survivor degree of dependency. In the completely dependent group where 41.1% of survivors could not be left unattended for more than 1 h, 23.7% of caregivers expressed unmet need of caregiver support; 51.4% reported poor psychological well-being compared to 19.3% in the independent group. Conclusion: The caregiver situation varies greatly with degree of survivor dependency which makes generalizations of caregiver needs difficult. Our results emphasize the need for integrating support aimed specifically at caregivers to survivors of stroke with a large degree of dependency. [ABSTRACT FROM AUTHOR]