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Introduction/Aims: Multidisciplinary care for patients with amyotrophic lateral sclerosis (ALS) is recommended in international guidelines, but reaches its limits when immobility increases. This pilot project addresses this gap by delivering home‐based, specialized, multiprofessional support to ALS patients who are not able to attend outpatient care. The study assessed the feasibility of this model of care and the satisfaction of both patients and caregivers. Methods: This was a longitudinal cohort study of patients with ALS and their caregivers in the surroundings of Munich, Germany. Patients were regularly visited at home by a multiprofessional team (neurologists/palliative care physicians, nurse, social worker, chaplain). Results: A total of 94 patients with ALS were included in the homecare project and 88 patients and 74 caregivers were enrolled in the accompanying study. The mean care duration was 221 days, enabling 61% of the 49 deceased patients to die at home. Notably, 20% of patients chose a way to hasten death. Patient satisfaction (ICECAP Supportive Care Measure [SCM]: 23.7/28, CollaboRATE: 10.6/12) and caregiver perception of the end‐of‐life phase (Caregiver Evaluation of the Quality of End‐Of‐Life Care [CEQUEL]: 24.9/26) were high. Discussion: This pilot project successfully implemented specialized, home‐based multidisciplinary care for ALS patients and caregivers, demonstrating both feasibility and high satisfaction. The program enabled a large proportion of patients to remain in their homes, reducing the need for hospital care. The multiprofessional approach, including neuropalliative, psychosocial and spiritual support provided comprehensive care that addressed needs of patients and caregivers. Further research is warranted to explore cost‐effectiveness. [ABSTRACT FROM AUTHOR]

Background: Muscle water T2 (T2w ) has been proposed as a biomarker to monitor disease activity and therapy effectiveness in patients with neuromuscular diseases (NMD). Multi-echo spin-echo (MESE) is known to be affected by fatty infiltration. A T2 -prepared 3D turbo spin echo (TSE) is an alternative method for T2 mapping, but has been only applied in healthy muscles.Purpose: To examine the performance of T2 -prepared 3D TSE in combination with spectral adiabatic inversion recovery (SPAIR) in measuring T2w in fatty infiltrated muscles based on simulations and in vivo measurements in thigh muscles of patients with NMD.Study Type: Prospective.Subjects: One healthy volunteer, 34 NMD patients.Field Strength/sequence: T2 -prepared stimulated echo acquisition mode (STEAM) magnetic resonance spectroscopy (MRS), SPAIR STEAM MRS, and SPAIR T2 -prepared STEAM MRS were performed in the subcutaneous fat of a healthy volunteer's thigh. T2 mapping based on SPAIR 2D MESE and SPAIR T2 -prepared 3D TSE was performed in the NMD patients' thigh region. Multi-TE STEAM MRS was performed for measuring a reference T2w at different thigh locations.Assessment: The behavior of the fat spectrum in the SPAIR T2 -prepared 3D TSE was simulated using Bloch simulations. The in vivo T2 results of the imaging methods were compared to the in vivo T2w MRS results.Statistical Tests: Pearson correlation coefficient with slope and intercept, relative error.Results: The simulated T2 for the SPAIR T2 -prepared 3D TSE sequence remained constant within a relative error of not more than 4% up to a fat fraction of 80%. In vivo T2 values of SPAIR T2 -prepared 3D TSE were in good agreement with the T2w values of STEAM MRS (R = 0.86; slope = 1.12; intercept = -1.41 ms). In vivo T2 values of SPAIR 2D MESE showed large deviations from the T2w values of STEAM MRS (R = 0.14; slope = 0.32; intercept = 38.83 ms).Data Conclusion: The proposed SPAIR T2 -prepared 3D TSE shows reduced sensitivity to fatty infiltration for T2w mapping in the thigh muscles of NMD patients.Level Of Evidence: 1 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2020;51:1727-1736. [ABSTRACT FROM AUTHOR]

Quantitative imaging techniques are emerging in the field of magnetic resonance imaging of neuromuscular diseases (NMD). T2 of water (T2w) is considered an important imaging marker to assess acute and chronic alterations of the muscle fibers, being generally interpreted as an indicator for "disease activity" in the muscle tissue. To validate the accuracy and robustness of quantitative imaging methods, 1H magnetic resonance spectroscopy (MRS) can be used as a gold standard. The purpose of the present work was to investigate T2w of remaining muscle tissue in regions of higher proton density fat fraction (PDFF) in 40 patients with defined NMD using multi‐TE single‐voxel 1H MRS. Patients underwent MR measurements on a 3 T system to perform a multi‐TE single‐voxel stimulated echo acquisition method (STEAM) MRS (TE = 11/15/20/25(/35) ms) in regions of healthy, edematous and fatty thigh muscle tissue. Muscle regions for MRS were selected based on T2‐weighted water and fat images of a two‐echo 2D Dixon TSE. MRS results were confined to regions with qualitatively defined remaining muscle tissue without edema and high fat content, based on visual grading of the imaging data. The results showed decreased T2w values with increasing PDFF with R2 = 0.45 (p < 10−3) (linear fit) and with R2 = 0.51 (exponential fit). The observed dependence of T2w on PDFF should be considered when using T2w as a marker in NMD imaging and when performing single‐voxel MRS for T2w in regions enclosing edematous, nonedematous and fatty infiltrated muscle tissue. [ABSTRACT FROM AUTHOR]

Objectives: Patients with progressive neurologic illness still lack access to quality palliative care services. Barriers to the comprehensive provision of neuropalliative care include gaps in palliative care education. To address this barrier, a novel international model of neuropalliative care education e-learning program was launched in 2022., Methods: This is a qualitative study on the self-reported learning outcomes and educational gains of participants of a neuropalliative care e-learning course., Results: Thematic analysis shows changes in the participants' perceptions of neuropalliative care and several specific educational gains. After attending the course, participants recognized neuropalliative care as a multiprofessional and interdisciplinary effort requiring more than medical knowledge and disease-specific treatment skills. They gained understanding of the complexity of prognosis in neurological diseases, as well as ethical concepts as the basis to approach difficult decisions. Valuing the needs of patients and caregivers, as well as honest and open communication were recognized as key components of the caring process. In particular, providing emotional support and building relationships to enhance the spiritual component of care were avidly discussed as essential nonmedical treatment options., Significance of Results: E-learning courses are helping to close the gaps in healthcare professionals' knowledge and skills about neuropalliative care.

Background: Muscle water T 2 (T 2w ) has been proposed as a biomarker to monitor disease activity and therapy effectiveness in patients with neuromuscular diseases (NMD). Multi-echo spin-echo (MESE) is known to be affected by fatty infiltration. A T 2 -prepared 3D turbo spin echo (TSE) is an alternative method for T 2 mapping, but has been only applied in healthy muscles., Purpose: To examine the performance of T 2 -prepared 3D TSE in combination with spectral adiabatic inversion recovery (SPAIR) in measuring T 2w in fatty infiltrated muscles based on simulations and in vivo measurements in thigh muscles of patients with NMD., Study Type: Prospective., Subjects: One healthy volunteer, 34 NMD patients., Field Strength/sequence: T 2 -prepared stimulated echo acquisition mode (STEAM) magnetic resonance spectroscopy (MRS), SPAIR STEAM MRS, and SPAIR T 2 -prepared STEAM MRS were performed in the subcutaneous fat of a healthy volunteer's thigh. T 2 mapping based on SPAIR 2D MESE and SPAIR T 2 -prepared 3D TSE was performed in the NMD patients' thigh region. Multi-TE STEAM MRS was performed for measuring a reference T 2w at different thigh locations., Assessment: The behavior of the fat spectrum in the SPAIR T 2 -prepared 3D TSE was simulated using Bloch simulations. The in vivo T 2 results of the imaging methods were compared to the in vivo T 2w MRS results., Statistical Tests: Pearson correlation coefficient with slope and intercept, relative error., Results: The simulated T 2 for the SPAIR T 2 -prepared 3D TSE sequence remained constant within a relative error of not more than 4% up to a fat fraction of 80%. In vivo T 2 values of SPAIR T 2 -prepared 3D TSE were in good agreement with the T 2w values of STEAM MRS (R = 0.86; slope = 1.12; intercept = -1.41 ms). In vivo T 2 values of SPAIR 2D MESE showed large deviations from the T 2w values of STEAM MRS (R = 0.14; slope = 0.32; intercept = 38.83 ms)., Data Conclusion: The proposed SPAIR T 2 -prepared 3D TSE shows reduced sensitivity to fatty infiltration for T 2w mapping in the thigh muscles of NMD patients., Level of Evidence: 1 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2020;51:1727-1736., (© 2019 The Authors. Journal of Magnetic Resonance Imaging published by Wiley Periodicals, Inc. on behalf of International Society for Magnetic Resonance in Medicine.)

Quantitative imaging techniques are emerging in the field of magnetic resonance imaging of neuromuscular diseases (NMD). T 2 of water (T 2w ) is considered an important imaging marker to assess acute and chronic alterations of the muscle fibers, being generally interpreted as an indicator for "disease activity" in the muscle tissue. To validate the accuracy and robustness of quantitative imaging methods, 1 H magnetic resonance spectroscopy (MRS) can be used as a gold standard. The purpose of the present work was to investigate T 2w of remaining muscle tissue in regions of higher proton density fat fraction (PDFF) in 40 patients with defined NMD using multi-TE single-voxel 1 H MRS. Patients underwent MR measurements on a 3 T system to perform a multi-TE single-voxel stimulated echo acquisition method (STEAM) MRS (TE = 11/15/20/25(/35) ms) in regions of healthy, edematous and fatty thigh muscle tissue. Muscle regions for MRS were selected based on T 2 -weighted water and fat images of a two-echo 2D Dixon TSE. MRS results were confined to regions with qualitatively defined remaining muscle tissue without edema and high fat content, based on visual grading of the imaging data. The results showed decreased T 2w values with increasing PDFF with R 2  = 0.45 (p < 10 -3 ) (linear fit) and with R 2  = 0.51 (exponential fit). The observed dependence of T 2w on PDFF should be considered when using T 2w as a marker in NMD imaging and when performing single-voxel MRS for T 2w in regions enclosing edematous, nonedematous and fatty infiltrated muscle tissue., (© 2019 John Wiley & Sons, Ltd.)