| 1. |
- Ashizawa, T., et al.
(författare)
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Consensus-based care recommendations for adults with myotonic dystrophy type 1
- 2018
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Ingår i: Neurology-Clinical Practice. - : Ovid Technologies (Wolters Kluwer Health). - 2163-0402 .- 2163-0933. ; 8:6, s. 507-520
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Forskningsöversikt (refereegranskat)abstract
- Purpose of review Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects between 1 in 3,000 and 8,000 individuals globally. No evidence-based guideline exists to inform the care of these patients, and most do not have access to multidisciplinary care centers staffed by experienced professionals, creating a clinical care deficit. Recent findings The Myotonic Dystrophy Foundation (MDF) recruited 66 international clinicians experienced in DM1 patient care to develop consensus-based care recommendations. MDF created a 2-step methodology for the project using elements of the Single Text Procedure and the Nominal Group Technique. The process generated a 4-page Quick Reference Guide and a comprehensive, 55-page document that provides clinical care recommendations for 19 discrete body systems and/or care considerations. The resulting recommendations are intended to help standardize and elevate care for this patient population and reduce variability in clinical trial and study environments.
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| 2. |
- Calcagno, A., et al.
(författare)
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Alzheimer Dementia in People Living With HIV
- 2021
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Ingår i: Neurology-Clinical Practice. - : Ovid Technologies (Wolters Kluwer Health). - 2163-0402 .- 2163-0933. ; 11:5
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Tidskriftsartikel (refereegranskat)abstract
- Objective Given the aging of people living with HIV (PLWH) and the high prevalence of HIV-associated neurocognitive disorders, we aimed at describing the clinical, instrumental, and CSF features of PLWH diagnosed with Alzheimer dementia (AD). Methods The databases of 3 large Italian outpatient clinics taking care of more than 9,000 PLWH were searched for the diagnosis of AD. After obtaining patients' or their next of kin's consent for publication, anonymous data were collected in an excel spreadsheet and described. Routinely collected CSF biomarkers and radiologic imaging results were recorded whether available. Results Four patients were included in this case series who were diagnosed with AD aged between 60 and 74 years. All participants were on highly active antiretroviral therapy and showed nondetectable serum HIV RNA. Memory impairment was the most prominent cognitive feature. The diagnosis was obtained considering the exclusion of other potential causes, MRI and fluorodeoxyglucose-PET features, and, in (in 2/4), CSF AD biomarkers levels. In 1 patient, longitudinal CSF tau/p-tau increased, and beta-amyloid(1-42) decreased over time despite antiretroviral therapy containing nucleotide reverse transcriptase inhibitors. Conclusions In older PLWH cognitive symptoms may represent the onset of AD: a multidisciplinary team may be needed for reaching a likely in vivo diagnosis.
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| 3. |
- Darehed, David, et al.
(författare)
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Death and ADL dependency after scoring zero on the NIHSS
- 2023
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Ingår i: Neurology. - : Wolters Kluwer. - 2163-0402 .- 2163-0933. ; 13:5
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Tidskriftsartikel (refereegranskat)abstract
- Background and Objectives: Of all strokes, mild strokes (defined as 5 points or less on the National Institutes of Health Stroke Scale [NIHSS]) are in the majority. However, up to one-third of patients with mild strokes still exhibit significant deficits 3 months after the stroke. Studies on the presumably mildest strokes, defined by zero points on the NIHSS (0-NIHSS) at admission, are scarce. Hence, we aimed to study patient characteristics and outcomes among patients with 0-NIHSS strokes.Methods: Our retrospective registry-based study included a total of 6,491 adult patients with stroke admitted to 3 different stroke units in Gothenburg, Sweden, from November 2014 to June 2019. Our main outcome was a composite measure including death and activities of daily living (ADL) dependency 3 months after the stroke. Analyses of patient characteristics were followed by adjusted analyses including multiple confounders.Results: In total, 5,945 patients had data on NIHSS at admission, of whom 1,412 (24%) presented with a 0-NIHSS stroke. Among these, the median age was 72 years, 600 (42%) were female, and 86 (6%) had a hemorrhagic stroke. Among previously ADL-independent patients, 65 (6%) were either dead or ADL-dependent 3 months after the stroke. Prestroke physical inactivity (OR 2.48, 95% CI 1.40–4.38) and age (OR 1.05 per gained year, 95% CI 1.02–1.08) significantly increased the risk of death and ADL dependency.Discussion: One of 17 patients has either died or become ADL-dependent 3 months after a 0-NIHSS stroke, stressing that these strokes are not always benign. Older and physically inactive patients are at greater risk of an adverse outcome.
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| 4. |
- Strupp, Michael, et al.
(författare)
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Worldwide survey on laboratory testing of vestibular function
- 2020
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Ingår i: Neurology. - 2163-0402 .- 2163-0933. ; 10:5, s. 379-387
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Tidskriftsartikel (refereegranskat)abstract
- Background The function of the peripheral vestibular system can nowadays be quantified. The video head impulse test (vHIT) and caloric irrigation are used for the semicircular canals, cervical vestibular evoked myogenic potentials (cVEMP) for the sacculus, and ocular vestibular evoked myogenic potentials (oVEMP) for the utriculus. Because there is no agreement on normal and pathologic values, we performed a worldwide survey.Methods A web-based standardized survey questionnaire was used to collect data on “reference values” and “cutoff” values. Thirty-eight centers from all continents (except Africa) replied.Results “Reference values”: vHIT: mean for the vestibulo-ocular reflex gain of the left horizontal canal 0.91 (range: 0.7–1.01) and of the left horizontal canal 0.92 (0.7–1.05); side difference 0.15 (0.25–0.3). Caloric irrigation: mean peak slow phase velocity of caloric-induced nystagmus for warm (44°C) water 18.65°/s (12–30°/s); cold (30°C) water 18.21°/s (10–25°/s). cVEMP: P13-N23 amplitude mean for the lower limit 28.67 μV (16–50 μV); upper limit 200 μV (50–350 μV). “Cutoff values”: vHIT: side difference 0.26 (0.1–0.4), bilateral vestibulopathy <0.61 (0.3–0.8); unilateral vestibulopathy (UVP) <0.68 (0.4–0.8). Caloric irrigation pathologic side difference mean 25.93% (17.7%–40%) or 12°/sec (5–30°/s); side difference UVP 26.73% (20%–40%) or 29.8°/s (5–100°/s). cVEMP: P13/N23 amplitude mean lower cutoff 32.5 μV (15–50 μV), mean upper cutoff 125 μV (50–200 μV), asymmetry 36.08 μV (20–50 μV).Conclusion This worldwide survey showed a large variability in terms of reference and pathologic cutoff values in the 38 centers included. Therefore, standardization of how to achieve these values and agreement on which values should be used is highly warranted to guarantee a high quality of vestibular testing and interpretation of clinical and scientific results.
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| 5. |
- Darehed, David, et al.
(författare)
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Death and ADL Dependency After Scoring Zero on the NIHSS: A Swedish Retrospective Registry-Based Study.
- 2023
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Ingår i: Neurology. Clinical practice. - 2163-0402. ; 13:5
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Tidskriftsartikel (refereegranskat)abstract
- Of all strokes, mild strokes (defined as 5 points or less on the National Institutes of Health Stroke Scale [NIHSS]) are in the majority. However, up to one-third of patients with mild strokes still exhibit significant deficits 3 months after the stroke. Studies on the presumably mildest strokes, defined by zero points on the NIHSS (0-NIHSS) at admission, are scarce. Hence, we aimed to study patient characteristics and outcomes among patients with 0-NIHSS strokes.Our retrospective registry-based study included a total of 6,491 adult patients with stroke admitted to 3 different stroke units in Gothenburg, Sweden, from November 2014 to June 2019. Our main outcome was a composite measure including death and activities of daily living (ADL) dependency 3 months after the stroke. Analyses of patient characteristics were followed by adjusted analyses including multiple confounders.In total, 5,945 patients had data on NIHSS at admission, of whom 1,412 (24%) presented with a 0-NIHSS stroke. Among these, the median age was 72 years, 600 (42%) were female, and 86 (6%) had a hemorrhagic stroke. Among previously ADL-independent patients, 65 (6%) were either dead or ADL-dependent 3 months after the stroke. Prestroke physical inactivity (OR 2.48, 95% CI 1.40-4.38) and age (OR 1.05 per gained year, 95% CI 1.02-1.08) significantly increased the risk of death and ADL dependency.One of 17 patients has either died or become ADL-dependent 3 months after a 0-NIHSS stroke, stressing that these strokes are not always benign. Older and physically inactive patients are at greater risk of an adverse outcome.
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| 6. |
- De Havenon, Adam, et al.
(författare)
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Variability of the Modified Rankin Scale Score between Day 90 and 1 Year after Ischemic Stroke
- 2021
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Ingår i: Neurology: Clinical Practice. - 2163-0402. ; 11:3, s. 239-244
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Tidskriftsartikel (refereegranskat)abstract
- Objective Studies indicate that the functional outcome evolves in the year after ischemic stroke onset. However, the traditional outcome measure in stroke trials is the modified Rankin Scale (mRS) at 90 days from onset. To determine mRS fluctuations in the first year after stroke, we examined data from 3 major stroke trials.MethodsIn a secondary analysis, we evaluated intrapatient mRS between 90 days and 1 year from stroke onset, the mRS shift (ΔmRS = 1 year-day 90), and the trials' primary outcome at day 90 and 1 yearResultsWe included 624 patients from the National Institute of Neurological Disorders and Stroke rt-PA Stroke Study, 587 from Albumin Treatment for Acute Ischaemic Stroke, and 611 from Interventional Management of Stroke III, for which the proportion of patients with a ΔmRS change between day 90 and 1 year was 36.5%, 41.7%, and 36.0%. However, the trials' primary outcomes did not differ at 1 year vs 90 days. Similar findings were seen in a second cohort where we pooled the trials and excluded patients with recurrent stroke or death during the follow-up. In those 1,314 patients, 544 (41.4%) had a ΔmRS change, of which 379 (28.9%) had improvement and 165 (12.5%) had worsening, apart from death.ConclusionWe describe the patient-level spectrum of mRS change from day 90 to 1 year after ischemic stroke in 3 high-quality randomized trials. The patient-level shifts consisted of a sufficiently counterbalanced number of mRS improvements and declines, which masked clinical evolution occurring in over one-third of patients. These results may have important implications, both for clinical trial design and outcome adjudication in stroke research and duration of rehabilitative therapy.
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| 11. |
- Zhang, Jiasi Vicky, et al.
(författare)
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Neurofilament Light Chain Related to Longitudinal Decline in Frontotemporal Lobar Degeneration.
- 2021
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Ingår i: Neurology. Clinical practice. - 2163-0402. ; 11:2, s. 105-116
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Tidskriftsartikel (refereegranskat)abstract
- Accurate diagnosis and prognosis of frontotemporal lobar degeneration (FTLD) during life is an urgent concern in the context of emerging disease-modifying treatment trials. Few CSF markers have been validated longitudinally in patients with known pathology, and we hypothesized that CSF neurofilament light chain (NfL) would be associated with longitudinal cognitive decline in patients with known FTLD-TAR DNA binding protein ~43kD (TDP) pathology.This case-control study evaluated CSF NfL, total tau, phosphorylated tau, and β-amyloid1-42 in patients with known FTLD-tau or FTLD-TDP pathology (n = 50) and healthy controls (n = 65) and an extended cohort of clinically diagnosed patients with likely FTLD-tau or FTLD-TDP (n = 148). Regression analyses related CSF analytes to longitudinal cognitive decline (follow-up ∼1 year), controlling for demographic variables and core AD CSF analytes.In FTLD-TDP with known pathology, CSF NfL is significantly elevated compared with controls and significantly associated with longitudinal decline on specific executive and language measures, after controlling for age, disease duration, and core AD CSF analytes. Similar findings are found in the extended cohort, also including clinically identified likely FTLD-TDP. Although CSF NfL is elevated in FTLD-tau compared with controls, the association between NfL and longitudinal cognitive decline is limited to executive measures.CSF NfL is associated with longitudinal clinical decline in relevant cognitive domains in patients with FTLD-TDP after controlling for demographic factors and core AD CSF analytes and may also be related to longitudinal decline in executive functioning in FTLD-tau.
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