Immunoablative therapy followed by autologous hematopoietic stem cell transplantation as the first-line disease-modifying therapy in patients with multiple sclerosis.

INTRODUCTION: Immunoablative therapy followed by autologous hematopoietic stem cell transplantation (AHSCT) is one of the possible disease-modifying therapies (DMTs) for patients with multiple sclerosis (MS). In this case series, we would like to present six patients with MS, who underwent AHSCT as the first-line DMT. CASE REPORTS: Six MS patients with a rapid progression of disability with or without relapses underwent AHSCT as the first-line DMT at the University Hospital Ostrava between 2018 and 2021. The conditioning regimens for AHSCT used were a medium-intensity regime BEAM (Carmustine, Etoposid, Cytarabin, Melphalan) and low-intensity regime based on Cyclophosphamide. Four out of six patients showed some disability progression after AHSCT, so the rapid progression of MS was just slowed down by AHSCT. One patient developed activity on magnetic resonance imaging three months after AHSCT, and two experienced mild relapses during the follow-up period. None of our patients developed grade 4 non-hematological toxicity; all infections were mild. In one patient, an allergic reaction probably to dimethyl sulfoxide was observed. CONCLUSION: Our case series of 6 patients shows that AHSCT is a promising therapeutic approach to slow down the rapid progression of clinical disability in MS patients with a good safety profile.

The Attitudes of Progressive Neurological Disease Patients and Their Family Members to End of Life Care: A Cross-Sectional Study.

The objective was to identify the attitudes of progressive neurological disease (PND) patients and their family members regarding end-of-life care, and their worries about dying. The sample included 327 participants. The Attitudes of Patients with PND to End-of-Life Care questionnaire was used to collect the data. Statistically significant differences in the assessment of attitudes towards end-of-life care between patients and family members were identified (p < 0.001). Family members more frequently favored patients being kept alive at any cost; patients more commonly wished to have their end of life under control. Respondents most frequently deferred to doctors when it came to decisions on treatment to keep patients alive. However, both patients and family members wanted patients to be able to decide on their treatment by leaving a written record of their previously stated wishes. The demands of patients and their families regarding end-of-life care should be documented in individual care plans.

Factors Influencing Health-Related Quality of Life of Patients with Multiple Sclerosis and Their Caregivers.

BACKGROUND: Only a few studies have been done focusing on the quality of life (QoL) of patients with multiple sclerosis (MS) as well as their family members. The aim of our research was to determine the factors that influence the QoL of MS patients in advanced stage of disease and their caregivers. METHODS: The sample of the cross-sectional study included 153 patients with MS and 74 caregivers. QoL was measured using the PNDQoL questionnaire (Progressive Neurological Diseases Quality of Life), and the severity of illness was assessed through the following scales: EDSS (Expanded Disability Status scale), PPS (Palliative Performance Scale), and ADL (Activity Daily Living). RESULTS: The following predictors of the global QoL of the MS patients were identified - age, EDSS, symptom burden, daily care, emotional functioning, and spiritual_nonreligion functioning (R2 = 0.569; F = 32.900; p < 0.001). The following predictors of the global QoL of caregivers were identified - age, emotional functioning, spiritual_nonreligion functioning, patient's QoL, and feeling of care (R2 = 0.431; F = 18.690; p < 0.001). CONCLUSION: Appropriate intervention should be directed particularly at older patients and caregivers who have faced the disease for longer time and at patients without any partner. Supporting the emotional and social well-being and mitigating the burden caused by symptoms of the patients as well as caregivers can improve the QoL of both groups.

Cerebrospinal fluid oligoclonal IgM test in routine practice: Comparison with quantitative assessment of intrathecal IgM synthesis.

BACKGROUND: Intrathecal IgM synthesis demonstrated either as cerebrospinal fluid (CSF)-restricted oligoclonal (o-) IgM bands or calculated using various formulas has been linked to more aggressive multiple sclerosis (MS) course. However, the proportion of MS patients showing intrathecal IgM synthesis varies largely between studies. We aimed to explore the relation between different formulas and results of o-IgM, and to assess the frequency of o-IgM bands in an unselected series of samples. METHODS: 432 samples were analyzed for o-IgM, o-IgG and quantitative measures of IgM and IgG synthesis. IgM index and formulas of Reiber, Auer and Öhman were compared to the result of the o-IgM test. RESULTS: At the cut-off commonly used, the non-linear formulas for intrathecal synthesis were specific (>94%) but rather insensitive (<40% even at a cut-off of 4 CSF-restricted bands) compared to o-IgM. No significant difference was noted in the performance of different formulas. At a cut-off of 4 bands, 61% of MS patients, but none of the controls were positive for o-IgM. CONCLUSIONS: Formulas for intrathecal IgM synthesis are insensitive compared to o-IgM. We propose to evaluate samples with 2 or 3 extra-CSF IgM bands as borderline and only samples with 4 or more as definitely positive.