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Background: Cognitive impairment is observed in up to 50% of patients with amyotrophic lateral sclerosis (ALS). The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) is an ALS-specific multi-domain screening tool. Few studies have examined the relationship between ECAS scores and [ 18 F]fluorodeoxyglucose positron emission tomography ([ 18 F]FDG-PET) findings. Objective: To assess the relationship between ECAS scores and glucose metabolism patterns on [ 18 F]FDG -PET images in ALS. Methods: We collected [ 18 F]FDG-PET images from 65 patients with ALS and 39 healthy controls. ECAS scores were collected on all patients and we calculated the correlation to [ 18 F]FDG-PET in order to investigate the potential links between cognition and glucose metabolism. Results: We observed hypometabolism in the frontal cortex, insula, and limbic system, together with hypermetabolism in the cerebellum in patients with ALS compared to controls. A lower ECAS total score was associated with lower glucose metabolism in the right orbitofrontal gyrus and higher glucose metabolism in lateral occipital, medial occipital, and cerebellar regions, among patients with ALS. Similar results, although less widespread, were observed in the analyses of ECAS ALS-specific scores. Conclusions: The metabolic patterns in [ 18 F]FDG -PET show that changes in the glucose metabolism of corresponding areas are related to cognitive dysfunction in ALS, and can be detected using the ECAS.

Introduction: Brain calcifications are frequent findings on imaging. In a small proportion of cases, these calcifications are associated with pathogenic gene variants, hence termed primary familial brain calcification (PFBC). The clinical penetrance is incomplete and phenotypic variability is substantial. This paper aims to characterize a Swedish PFBC cohort including 25 patients: 20 from seven families and five sporadic cases., Methods: Longitudinal clinical assessment and CT imaging were conducted, abnormalities were assessed using the total calcification score (TCS). Genetic analyses, including a panel of six known PFBC genes, were performed in all index and sporadic cases. Additionally, three patients carrying a novel pathogenic copy number variant in SLC20A2 had their cerebrospinal fluid phosphate (CSF-Pi) levels measured., Results: Among the 25 patients, the majority (76%) displayed varying symptoms during the initial assessment including motor (60%), psychiatric (40%), and/or cognitive abnormalities (24%). Clinical progression was observed in most patients (78.6%), but there was no significant difference in calcification between the first and second scans, with mean scores of 27.3 and 32.8, respectively. In three families and two sporadic cases, pathogenic genetic variants were identified, including a novel finding, in the SLC20A2 gene. In the three tested patients, the CSF-Pi levels were normal., Conclusions: This report demonstrates the variable expressivity seen in PFBC and includes a novel pathogenic variant in the SLC20A2 gene. In four families and three sporadic cases, no pathogenic variants were found, suggesting that new PFBC genes remain to be discovered., Competing Interests: Declaration of competing interest The authors declare that there are no conflicts of interest relevant to this work., (Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)

Background: Motor neuron diseases (MND), with amyotrophic lateral sclerosis constituting most cases, are rare conditions of unknown etiology. There have been reports of an increase in incidence during the latter half of the twentieth century in various Western countries, including Sweden. This study provides updated data on the incidence of MND in Sweden during the last 20 years., Methods: Data was obtained from the Swedish National Patient Register on individuals diagnosed with MND from 2002 to 2021 and analysed in relation to group level data for the entire Swedish population. Incidence rates were calculated and presented in relation to year, age, sex, and region., Results: In the early 2000s, there was a crude incidence rate of 3.5-3.7 per 100,000 person-years, which then increased to 4.0-4.6 from 2008 onward. Age standardization to the starting year (2002) partially mitigated this increase. The incidence rate was greater among men compared to women and was highest within the age range of 70 to 84 years. There were indications of a higher incidence rate in the northernmost parts of the country, although the difference was not statistically significant., Conclusions: The incidence rate of MND in Sweden now seems to have surpassed 4 cases per 100,000 person-years. This is higher when compared to both other European countries and previous Swedish studies. It remains to be determined if this increase reflects an actual increasing incidence of MND in Sweden or is due to other factors such as better registry coverage., (© 2024. The Author(s).)

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., (© 2024 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

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., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To describe the diagnostic and prognostic performance, and longitudinal trajectories, of potential biomarkers of neuroaxonal degeneration and neuroinflammation in amyotrophic lateral sclerosis (ALS)., Methods: 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., Results: 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., Conclusions: 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.

Brain fluid dynamics remains poorly understood with central issues unresolved. In this study, we first review the literature regarding points of controversy, then pilot study if conventional MRI techniques can assess brain fluid outflow pathways and explore potential associations with small vessel disease (SVD). We assessed 19 subjects participating in the Mild Stroke Study 3 who had FLAIR imaging before and 20-30 minutes after intravenous Gadolinium (Gd)-based contrast. Signal intensity (SI) change was assessed semi-quantitatively by placing regions of interest, and qualitatively by a visual scoring system, along dorsal and basal fluid outflow routes. Following i.v. Gd, SI increased substantially along the anterior, middle, and posterior superior sagittal sinus (SSS) (82%, 104%, and 119%, respectively), at basal areas (cribriform plate, 67%; jugular foramina, 72%), and in narrow channels surrounding superficial cortical veins separated from surrounding cerebrospinal fluid (CSF) (96%) (all p < 0.001). The SI increase was associated with higher intraparenchymal perivascular spaces (PVS) scores (Std. Beta 0.71, p = 0.01). Our findings suggests that interstitial fluid drainage is visible on conventional MRI and drains from brain parenchyma via cortical perivenous spaces to dural meningeal lymphatics along the SSS remaining separate from the CSF. An association with parenchymal PVS requires further research, now feasible in humans., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease, and the time from symptom onset to diagnosis remains long. With the advent of disease-modifying treatments, the need to identify and diagnose ALS in a timely fashion has never been greater., Methods: We reviewed the literature to define the severity of ALS diagnostic delay, the various factors that contribute to this delay (including patient and physician factors), and the role that site of symptom onset plays in a patient's diagnostic journey., Results: Diagnostic delay is influenced by general practitioners' lack of recognition of ALS due to disease rarity and heterogenous presentations. As a result, patients are referred to non-neurologists, have unnecessary diagnostic testing, and may ultimately be misdiagnosed. Patient factors include their illness behavior-which impacts diagnostic delay-and their site of symptom onset. Limb-onset patients have the greatest diagnostic delay because they are frequently misdiagnosed with degenerative spine disease or peripheral neuropathy., Conclusion: Prompt ALS diagnosis results in more effective clinical management, with earlier access to disease-modifying therapies, multidisciplinary care, and, if desired, clinical trial involvement. Due to lack of commercially available ALS biomarkers, alternative strategies to identify and triage patients who likely have ALS must be employed. Several diagnostic tools have been developed to encourage general practitioners to consider ALS and make an urgent referral to ALS specialists, bypassing unnecessary referrals to non-neurologists and unnecessary diagnostic workup., (© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Objective: Amyotrophic lateral sclerosis (ALS) is a clinically heterogenous disease, typically presenting with focal motor weakness that eventually generalizes. Weather there is a correlation between focal motor weakness and metabolic alterations in specific areas of the brain has not been thoroughly explored. This study aims to systematically investigate this by using fluorodeoxyglucose-positron emission tomography (FDG-PET), including longitudinal imaging. Methods: This observational imaging study included 131 ALS patients diagnosed and examined with FDG-PET at the ALS Clinical Research Center at the Karolinska University Hospital in Stockholm, Sweden. Thirteen ALS patients had a second scan and were analyzed longitudinally. The findings were compared to 39 healthy controls examined at the University Medical Center of Gröningen, the Netherlands. Results: There was a general pattern of brain metabolic alterations consistent with previously reported findings in ALS, namely hypometabolism in frontal regions and hypermetabolism in posterior regions. A higher symptom burden was associated with increased hypometabolism and decreased hypermetabolism. However, there was no clear correlation between focal motor weakness and specific metabolic alterations, neither when analyzing focal motor weakness with concomitant upper motor neuron signs or when including all focal motor weakness. Longitudinal FDG-PET imaging showed inconsistent results with little correlation between progression of motor weakness and metabolic alterations. Conclusion: Our results support the disease model of ALS as a diffuse process since no clear correlation was seen between focal motor weakness and specific metabolic alterations. However, there is need for further research on a larger number of patients, particularly including longitudinal imaging.

Objective: Cognitive and behavioral impairment is observed in up to 50% of patients with amyotrophic lateral sclerosis (ALS). The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) is a 5-domain screening tool customized for quick cognitive screening in patients with ALS. Although the ECAS is available in Swedish at the Karolinska University Hospital (SK-ECAS), it has not yet been validated in Sweden stressing the need to assess validity and reliability of the SK-ECAS Version A., Methods: The study included 176 patients with ALS or other motor neuron disease diagnosed between September 2017 and October 2021 at the Karolinska ALS Clinical Research Center in Stockholm, Sweden, and 35 age-matched healthy control subjects. SK-ECAS was validated against the Montreal Cognitive Assessment (MoCA) and optimal cutoffs, receiver operating characteristic (ROC) curve and area under the curve (AUC) were calculated., Results: We identified an optimal cutoff of 108 for the SK-ECAS total score and 82 for the SK-ECAS ALS-specific score to detect cognitive impairment. The SK-ECAS showed good performance in indicating abnormal cognition with an AUC of 0.73 for SK-ECAS ALS-specific score and 0.77 for SK-ECAS total score. There was good internal consistency with a Cronbach's alpha of 0.79., Conclusions: This study demonstrates good validity and reliability indices for SK-ECAS Version A for the detection of cognitive impairment in newly diagnosed ALS patients.

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Storti, Gabriel, Sennfält, Canavero, Rifino, Gatti and Bersano.)

Objective: Systemic capillary leak syndrome (SCLS) is a rare condition associated with episodes of hypotension, hemoconcentration, hypoalbuminemia, and rhabdomyolysis. We describe a middle-aged man presenting with several distinct SCLS-like episodes, the last being fatal. In addition, in the year before the final event, he developed rapid cognitive decline with contrast-enhancing lesions on MRI and highly elevated neurofilament light protein levels in CSF., Methods: Data and imaging were obtained from patient medical records., Results: At the time, the SCLS-like episodes were interpreted as myositis secondary to viral infection. A thorough workup for other causes, including genetic testing, was negative. As for the rapid cognitive decline, despite an extensive workup for infectious and inflammatory causes, no definitive diagnosis was made. Whole genome sequencing however identified a C9orf72 hexanucleotide expansion., Discussion: The C9orf72 expansion is associated with frontotemporal dementia and amyotrophic lateral sclerosis but has also been shown to increase susceptibility to neuroinflammation. Recent findings also suggest C9orf72 to exert functions in the immune system, in particular regulation of type I interferon responses, in turn shown to be associated with SCLS. This case suggests a possible link between SCLS, cerebral inflammation, dysregulated type I interferon signaling, and expansions in C9orf72 ., Competing Interests: The authors report no relevant disclosures. Go to Neurology.org/NG for full disclosures., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Objectives: 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., Methods: 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., Results: 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., Conclusion: 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.

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.

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., (© 2020 The Authors. Acta Neurologica Scandinavica published by John Wiley & Sons Ltd.)

Introduction: While acute treatment and secondary prevention in stroke have undergone major improvements, hospital readmission after index stroke remains high. However, there are few reports on long-term readmission patterns., Patients and Methods: For this prospective observational study, data on demographics, functional status and living conditions were obtained from the Swedish Stroke Register (Riksstroke). Data on comorbidity and hospital readmissions up to five years post-index stroke were obtained from the Swedish National Patient Register. Patients were grouped based on number of readmissions: low (0-1) intermediate (2-4), high (5-9) or very high (≥10)., Results: Of the 10,092 patients included, 43.7% had been readmitted within 12 months and 74.0% within 5 years. There was an average of three readmissions per individual during the five-year interval. A small group of patients with a high-comorbidity burden accounted for the majority of readmissions: approximately 20% of patients accounted for 60% of readmissions, and 5% of patients accounted for 25%. Circulatory conditions were the most common cause followed by infectious disease, stroke, trauma and diseases of the nervous system other than stroke. The proportion of readmissions due to stroke decreased sharply in the first six months., Conclusion: A small number of patients with a high degree of comorbidity accounted for the majority of hospital readmissions after index stroke. Our results highlight the need for further development of strategies to support high-risk comorbid stroke patients in the community setting. Further research describing characteristics and healthcare utilisation patterns in this group is warranted., (© European Stroke Organisation 2020.)

Purpose: Comorbidity in stroke is common, but comprehensive reports are sparse. We describe prevalence of comorbidity and the prognostic impact on mortality and functional outcome in a large national ischemic stroke cohort., Methods: We used outcome data from a long-term follow-up survey conducted in 2016 by the Swedish Stroke Register (Riksstroke). Those included in the study were 11 775 pre-stroke functionally independent patients with first-ever ischemic stroke followed up at three months and 12 months (all patients), and three years (2013 cohort) or five years (2011 cohort). Pre-stroke comorbidity data for 16 chronic conditions were obtained from the Swedish National Patient Register, the Swedish Prescribed Drugs Register and the Riksstroke register. Individuals were grouped according to number of conditions: none (0), low (1), moderate (2-3) or high (≥4). Co-occurrence was analysed using hierarchical clustering, and multivariable analyses were used to estimate the prognostic significance of individual conditions., Results: The proportion of patients without comorbidity was 24.8%; 31.8% had low comorbidity; 33.5% had moderate comorbidity and 9.9% had high comorbidity. At 12 months, the proportion of poor outcome (dead or dependent: mRS ≥3) was 24.8% (no comorbidity), 34.7% (low), 45.2% (moderate) and 59.4% (high). At five years, these proportions were 37.7%, 50.3%, 64.3%, and 81.7%, respectively. There was clustering of cardiovascular conditions and substantial negative effects of dementia, kidney, and heart failure., Conclusion: Comorbidity is common and has a strong impact on mortality and functional outcome. Our results highlight the need for health systems to shift focus to a comprehensive approach in stroke care that includes multimorbidity as a key component., (© European Stroke Organisation 2019.)

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.

Background and Purpose- Longitudinal long-term prognostic data after stroke based on large cohorts are sparse. We report recent survival and functional outcome data on ischemic stroke (IS) and intracerebral hemorrhage (ICH) for up to 5 years poststroke from the Swedish Stroke Register (Riksstroke). Methods- Beyond Riksstroke's regular follow-up surveys at 3 and 12 months, additional surveys were conducted in 2016 on 2 one-year cohorts with stroke 3 and 5 years earlier. Functional dependency was defined as modified Rankin Scale ≥3. Mortality data of the original cohorts were obtained from the Swedish Causes of Death Register. Multiple imputation was used to estimate functional status in nonresponders. Results- The study included 22 929 patients, 87.5% with IS and 12.5% with ICH. Loss to follow-up in the 4 surveys was 12.8% to 21.2%. Thirty-day mortality was higher for ICH than for IS (30.7% versus 11.1%; P <0.01), whereas for 30-day survivors, 5-year mortality did not differ significantly ( P =0.858). Functional outcome was less favorable for ICH at all follow-up points. At 5 years, poor outcome (death or dependency) was 79% in ICH and 70.6% in IS (including imputed data; P <0.01). Favorable outcome was less common with increasing age and in patients with prestroke functional dependency. Conclusions- Despite advances in stroke care, long-term prognosis remains a cause for concern. At 5 years after stroke over 2 in 3 patients with IS, and over 3 in 4 patients with ICH, were dead or dependent. We present robust long-term prognostic data to serve as a reference for further development of healthcare and research in stroke.