COVID-19 pandemic and the international classification of functioning in multiple system atrophy: a cross-sectional, nationwide survey in Japan |…

Study design

This study was part of a cross-sectional, nationwide, multipurpose, mail survey of Japanese PwMSA from October to December, 2020. The data reflect the effects of the early COVID-19 pandemic, corresponding to 8 to 9months after the global pandemic was declared. Prior to this study, we reported another study of aspects of social services in Japan23, and all 155 participants in the present study were also included in that analysis.

The study received approval from the ethics committee of the Faculty of Health Science of Juntendo University (Approval Number 20-012) and was performed in accordance with the Declaration of Helsinki. The study followed the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines and the American Association for Public Opinion Research (AAPOR) reporting guideline.

The survey was anonymous, and confidentiality of information was assured. Informed consent was obtained from all participants by providing a written explanation of the study and having them return the questionnaire form. Because it was anticipated that certain patients did not want their responses to be included in the study, their return of a letter of intent to refuse to respond was also accepted.

The participants of this study were respondents to a survey of all members of the Japanese spinocerebellar degeneration and MSA patient association (https://scdmsa.tokyo/). This is the largest nonprofit organization patient group in Japan and is composed of volunteers. However, the members may not be the patients themselves, because they may be individuals who agree with the purpose of the association. MSA was self-reported by the patients in this study, but the diagnosis of MSA in Japan is made by specialists according to the common diagnostic criteria specified by the Ministry of Health, Labour and Welfare for registration of designated intractable diseases. The criteria are based on the second consensus statement on the diagnosis of MSA12. It was unlikely that an individual belonging to a patient association of rare diseases would self-report without it being based the correct diagnosis. The exclusion criteria for this study were those who were not PwMSA, and those who refused to respond or had incomplete answers to all the questions related to COVID-19.

Questionnaires were mailed to all members using the address database used to send regular patient association mailings. The relevant questions consisted of original COVID-19-related items and background information items that did not reflect COVID-19. The primary outcome was the effect of the COVID-19 pandemic on ICF functioning. The domains of ICF functioning consisted of body function, activity, and participation, and these definitions were in accordance with the WHO22. The self-perception of ICF functioning during the pandemic was assessed through the patients global impression at the time of response. Participants were asked to rate the impact score on each ICF functioning associated with COVID-19 on a 7-point scale: strongly unaffected=3, moderately unaffected=2, slightly unaffected=1, undecided=0, slightly affected=+1, moderately affected=+2, and strongly affected=+3. Of these, responses 1 to3 were classified as Unaffected, and responses of +1 to+3 score were classified as Affected, with the latter responses defined as COVID-19-related decline of ICF functioning. Thus, if domains of ICF functioning (body function, activity, and participation) were affected, the terms impairment, activity limitation, and participation restriction were used, respectively. The infection status and behavioral effects of COVID-19 were also included in the survey.

Participant were asked about age, sex, disease type, disease duration, and dwelling place (home, long-term care facility, hospitalization), and the multiple system impairment questionnaire (MSIQ), patient health questionnaire-2 (PHQ-2), modified rankin scale (mRS) score, barthel index (BI), life-space assessment (LSA), and EuroQoL (EQ) were examined. Of these, the clinical assessment indices were based on self-reported responses of normal conditions before the COVID-19 pandemic. The MSIQ for comprehensive scoring of the severity of disease-related impairments and the PHQ-2 as a screening tool for depression reflect body function as a baseline. The mRS score was used as a simple indicator of independence level, and the BI was used for comprehensive scoring of basic activities of daily living (ADL) assessment, reflecting activity as a baseline. The LSA was used for comprehensive scoring of the extent of daily living space, and the EQ was used as a global assessment of health-related QOL, and they reflected participation as a baseline.

The MSIQ was developed specifically for this study and was scored on a self-report basis for 22 impairments that may occur in MSA (Online Resource 1). All impairments were described in writing to ensure specific understanding. It consists of a total of 22 items: ataxia, muscle weakness/atrophy, muscle rigidity, spasticity, balance disorder, postural abnormality, decreased endurance, fatigue, pain, numbness, sensory disturbance, tremor, involuntary movements, orthostatic hypotension, poor sleep, respiratory disturbance, speech/dysarthria, dysphagia, visual impairment, urinary impairment, voiding impairment, and cognitive impairment. Each impairment was scored as 0 (no impairment) to 3 (severe impairment). Thus, the maximum score of 66 is the most severe impairment, and a score of 0 is the complete absence of impairments.

The PHQ-2 for depression screening24 is a shortened version of the PHQ-925. It consists of two items with a score of 03 each, with 0 being normal and 6 being the most severe24. The PHQ-2 was used instead of the PHQ-9, which contains a motor-related item26.

The mRS was originally developed as an assessment grade for disability or dependence in the ADL of stroke patients. Today, it is widely used in patients with neurological diseases. The score was rated on a six-point scale from 0 (asymptomatic) to 5 (severe disability)27.

The BI evaluates the performance of basic ADL, such as feeding, personal hygiene, bathing, and dressing on a scale of 0100. A higher number reflects a greater ability to function independently and has the advantage of being applicable to self-assessment and direct administration28. To allow for self-assessment by mail, the content of previous studies was used29.

The LSA is a self-report measure to summarize the distance (five distance levels ranging from room to out of town) and frequency (five frequency levels ranging from not at all to every day) an individual travels in a given period of time. The results are calculated by the LSA score, which is 120 for the most active30.

The EQ is a comprehensive measure of health-related QOL that is used worldwide31. In this study, the official Japanese version of the EQ five-dimension five-level questionnaire (EQ-5D-5L) was used, and an index value was calculated, with 1 the highest and 0 the lowest32.

In summary, to clarify the effect of the COVID-19 pandemic on MSA, ICF functioning during the pandemic was defined as the main outcome, potential predictors of functioning decline were defined as MSIQ, PHQ-2, mRS, BI, LSA, and EQ, and the potential confounders were age, sex, disease duration, and dwelling place.

Descriptive statistics are presented for demographic variables and functional outcomes during the COVID-19 pandemic for PwMSA. Continuous variables are presented as means (standard deviation) and categorical variables as numbers (%). Spearmans product rate correlation coefficients were calculated for the associations between ICF functioning scores related to the COVID-19 pandemic. To compare the affected patients with the unaffected patients on ICF functioning, group comparisons were performed using Students t-test for numerical variables, the MannWhitney U test for ordinal variables, and the 2 test when appropriate for categorical variables. A score of 0 was excluded from the analysis in that domain. Among the data of selected patients, missing values were excluded only for that item.

Univariate logistic regression analyses were performed on the identified variables to assess the potential risk factors for affected functioning domains during the COVID-19 pandemic. The dependent variable was a dummy variable that was set to 0 for unaffected (3 to1) and 1 for affected (+1 to+3) for each functioning domain, and the independent variables were the MSIQ, PHQ-2, mRS, BI, LSA, and EQ. Similar analyses were performed for each domain of ICF functioning (impairment, activity limitation, and participation restriction). The associations between risk factors and outcomes are presented as odds ratios (ORs) and 95% confidence intervals (CIs). Finally, multivariate logistic regression analyses were performed to identify independent risk factors for affected function scores during the COVID-19 pandemic for each domain of ICF functioning. Age, sex, disease duration, and dwelling place were forced into the model as adjustment factors, and a stepwise variable increase method (likelihood ratio) was used with independent variables identified on univariate logistic regression analysis. ORs and 95% CIs are presented after adjustment for confounders, including age, sex, disease duration, and dwelling place. For multivariate analysis only, participants with even one missing value were excluded from the analysis, and only complete data were analyzed.

Data analysis was performed using SPSS statistical software version 27.0 (IBM Corp). The significance level was set at =0.05, and all tests were 2-tailed.

All procedures performed in the study were approved by the ethics committee of the Faculty of Health Science of Juntendo University (approval number 20-012).

Informed consent was obtained from all participants by providing a written explanation of the study and by their return of the questionnaire form.

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COVID-19 pandemic and the international classification of functioning in multiple system atrophy: a cross-sectional, nationwide survey in Japan |...