Reliability and consistency of the Japanese version of the Primary Lateral Sclerosis Functional Rating Scale

Reliability and consistency of the Japanese version of the Primary Lateral Sclerosis Functional Rating Scale | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Reliability and consistency of the Japanese version of the Primary Lateral Sclerosis Functional Rating Scale Masaru Yanagihashi, Takehisa Hirayama, Mari Shibukawa, Junpei Nagasawa, and 12 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4295513/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 13 Aug, 2024 Read the published version in BMC Neurology → Version 1 posted 10 You are reading this latest preprint version Abstract Background Primary lateral sclerosis (PLS) is an extremely rare condition; therefore, to date no clinical studies have been conducted. The Primary Lateral Sclerosis Functional Rating Scale (PLSFRS) was developed in the United States of America. The PLSFRS is a crucial assessment scale for international collaborative research and future clinical trials for PLS. It is useful for evaluating medical conditions through face-to-face assessments and telephone interviews such as when a face-to-face assessment is not possible due to disasters or the burden of hospital visits. This study assessed the reliability and consistency of in-person and telephone interviews using the Japanese version of the PLSFRS. Methods We enrolled 19 Japanese patients who met the specific criteria for inclusion at the six collaborating institutions. The PLSFRS assessments were performed by two evaluators at defined time points and analyzed for intra-rater and inter-rater reliability and consistency between the in-person and telephone interviews. Results The Japanese version of the PLSFRS was developed by a specialized company and translator, and modified to consider the Japanese lifestyle through a consensus among motor neuron specialists. The quadratic-weighted kappa coefficients for the intra-rater and the inter-rater agreement were substantial (intra-rater: 0.691-1.000, inter-rater: 0.634-1.000). Moreover, the intraclass correlation coefficient for the PLSFRS total score was 0.997 (95% confidence interval, 0.992–0.999). Conclusions This study provides results regarding the Japanese version of the PLSFRS intra-rater and inter-rater reliability and consistency between in-person and telephone interviews. primary lateral sclerosis PLSFRS Japanese version face-to-face assessments Figures Figure 1 Background Primary lateral sclerosis (PLS) is a progressive neurodegenerative disease in which upper motor neurons are selectively damaged. PLS is rare, occurring in less than 5% of motor neuron diseases [ 1 , 2 ] ; therefore to date, no therapeutic agents or clinical trials have been evaluated. Since there have been no indicators to evaluate PLS, in 2020 a PLS Functional Rating Scale (PLSFRS) was developed in the United States of America, and the results were consistent with intra-rater, inter-rater, face-to-face visits, and telephone test-retest reliability during 1 year [ 3 ] . The PLSFRS is an essential tool for future international collaborative studies and clinical trials for PLS. Our study evaluated the reliability of the Japanese version of the PLSFRS and assessed the consistency between face-to-face evaluations and telephone interviews. Methods Institutional review board and volunteer participation The study protocol was approved by the Ethics Committee of Toho University Faculty of Medicine, Tokyo, Japan (A21013, M20282), and other participating institutions (4058, rinfu 21 − 008, TS R03-017, ippan2021-318, 2021 − 0101). Written informed consent was obtained from all the patients. The PLSFRS was translated into Japanese by a specialized company and translator (2-14-10 Sotokanda Chiyoda-ku Tokyo 101 − 0021 Crimson Interactive Japan Co., ltd.), and it was further modified to account for the Japanese lifestyle based on consensus among the motor neuron specialists. Nineteen patients were enrolled in the study. Protocol and inclusion and exclusion criteria The study and evaluation protocols are presented in Table 1 . Face-to-face examinations and telephone interviews were conducted at intervals of at least 24 hours but no more than 7 days. All evaluators for this study were board-certified neurologists and were trained appropriately before the evaluation. The inclusion and exclusion criteria are listed in Table 2 . The patients were encouraged to state their condition, and the caregivers were advised not to make their interpretations. Participants were excluded if they withdraw consent, died, or were deemed inappropriate to continue in the study. Statistical analyses We conducted statistical analyses of the PLSFRS to assess intra-rater and inter-rater reliability for each item of the PLSFRS and the consistency of the PLSFRS total score between a face-to-face examination and telephone interview using Microsoft Excel version 2210 (Microsoft Corp., Redmond, WA, USA) and IBM SPSS Statistics version 29.0 (IBM Corp., Armonk, NY, USA). Intra-rater reliability was assessed between the baseline and 8 weeks after baseline examination. Inter-rater reliability was assessed between the baseline and 4 weeks after baseline examination. The consistency between a face-to-face examination and telephone interview was assessed 4 weeks after the baseline examination. Results Demographics Nineteen patients were enrolled at baseline. The average age of the participants at baseline was 68.2 (42–85) years. The sample included eight men (42.1%) and 11 women (57.9%). The median disease duration was 72 (12–264) months. Dropouts Four participants were excluded from the study. Two patients had aspiration pneumonia during the evaluation period; however this was not considered an adverse event associated with the evaluation. The evaluation could not be completed for the other two patients because they could not consult the doctor within the evaluation period. Intra-rater and inter-rater reliability The quadratic-weighted kappa coefficients for intra-rater and inter-rater agreement were substantial (intra-rater; 0.691-1.000, inter-rater; 0.634-1.000) (Fig. 1 ). Consistency The intraclass correlation coefficient for the PLSFRS total score between face-to-face examination and telephone interview was 0.997 (95% confidence interval, 0.992–0.999). Discussion PLS is a rare motor neuron disease, and to date, no clinical trials have been conducted. To develop therapeutic drugs in the future, it will be necessary to work globally, which includes collecting cases; therefore, creating a Japanese version of the PLSFRS is essential. Previously, the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) alone evaluated the intra-rater and inter-rater reliabilities. The usefulness of administering the ALSFRS-R over the telephone was subsequently evaluated [ 4 , 5 ] . Telephone interviews minimized the burden on participants and caregivers by reducing the number of visits required to the study sites. Four consultations, including three visits in 8 weeks, may have increased the burden; however, no adverse events were associated with the visits for this evaluation. The availability of quick and reliable measures that can be administered over the telephone is indispensable for conducting this type of research. Thus, in this study, the consistency of the telephone interviews was ensured from the outset. Repeated evaluator training is crucial for maintaining reliability of the scale. When creating this scale, we considered the Japanese lifestyle and, added instructions to prevent confusion during the evaluation. The Japanese version of the PLSFRS is provided as a supplement, and the original instructions were referenced and added to the table for the evaluators’ convenience. The present study has several limitations. The current diagnostic criteria require the absence of clinical and electrophysiological lower motor neuron involvement for at least 4 years after symptom onset before diagnosing PLS [ 6 ] . Although the participants in this study were recruited according to the criteria, some patients were in the early stage of disease onset and may have been diagnosed with upper motor neuron-dominant amyotrophic lateral sclerosis. Moreover, we implemented less stringent electromyography criteria to enroll more participants and accommodate a wider spectrum of patients with PLS. This approach can be an ambivalent issue because the participants who developed pure upper motor disease for less than 4 years could not receive a definite diagnosis and may have been classified as having predominantly upper motor neuron dominant amyotrophic lateral sclerosis [ 7 ] . The concern with the PLSFRS was that, since there were two additional score tiers compared to the ALSFRS-R, except for orthopnea and respiratory insufficiency, the evaluator and the participant can get confused about the subtleties. We initially believed that evaluations by phone or in person would be difficult; however, the results showed no significant differences. Therefore, the Japanese version of the PLSFRS may be a valuable indicator for assessing medical conditions, even when face-to-face assessment is impossible due to disasters or hospital visit burdens. Conclusions This study provides the results for the Japanese version of the PLSFRS intra-rater and inter-rater reliability and an evaluation of the consistency between in-person and telephone interviews. Similar to the ALSFRS-R for amyotrophic lateral sclerosis, we believe that the Japanese version of the PLSFRS will be widely used not only by neurologists but also by internists and medical staff other than doctors and is a well-validated tool for PLS researchers to conduct future clinical trials. Abbreviations ALSFRS-R Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised PLS primary lateral sclerosis PLSFRS PLS Functional Rating Scale Declarations Ethics approval and consent to participate The study protocol was approved by the Ethics Committee of Toho University Faculty of Medicine, Tokyo, Japan (A21013, M20282), and other participating institutions (4058, rinfu 21-008, TS R03-017, ippan2021-318, 2021-0101). Consent for publication Not applicable Availability of data and materials The data that support the findings of this study are available from the corresponding author upon reasonable request. Competing interests The authors declare that they have no competing interests. Funding This work was partly supported by Grants-in-Aid from the Research Committee of CNS Degenerative Diseases, Research on Policy Planning and Evaluation for Rare and Intractable Diseases, Health, Labour and Welfare Sciences Research Grants, and the Ministry of Health, Labour and Welfare, Japan (20FC1049). Authors’ contributions MY: Conceptualization; Data Curation; Formal Analysis; Investigation; Methodology; Project Administration; Resources; Validation; Visualization; Writing – Original Draft Preparation; and Writing – Review and Editing. TH: Data Curation; Investigation; and Resources. MS: Project Administration. JN: Project Administration. KF: Conceptualization; Investigation; Methodology; and Resources. YI: Conceptualization; Investigation; Methodology; and Resources. MM: Conceptualization; Investigation; Methodology; and Resources. KB: Conceptualization; Investigation; Methodology; and Resources. KT: Conceptualization; Investigation; Methodology; and Resources. KK: Conceptualization; and Methodology. NA: Conceptualization; Methodology; Project Administration; and Supervision. YI: Conceptualization; and Methodology. MK: Conceptualization; and Methodology. YM: Conceptualization; Data Curation; Formal Analysis; and Methodology. HM: Conceptualization; and Methodology. OK: Conceptualization; Data Curation; Funding acquisition; Methodology; Project Administration; and Supervision. All authors read and approved the final manuscript. Acknowledgements The authors are grateful to all the participants and their families. We also thank Mrs. Yurika Okuma and Mrs. Tamayo Yamashita, executive assistants to Department of Neurology, Toho University Faculty of Medicine, who supported this article. Moreover, we would like to thank Editage (www.editage.com) for English language editing. Previous presentations These data were presented at the 64 th Annual Meeting of Japanese Society of Neurology, Chiba, Japan, the 41 st Annual Meeting of Japanese Society of Neurotherapy, Tokyo, Japan, Neurodegenerative Disease Workshop in 2020, 2023, Tokyo, Japan. References Statland JM, Barohn RJ, Dimachkie MM, Floeter MK, Mitsumoto H. Primary lateral sclerosis. Neurologic Clin. 2015;33:749–60. 10.1016/j.ncl.2015.07.007 . Gordon PH, Cheng B, Katz IB, Pinto M, Hays AP, Mitsumoto H, et al. The natural history of primary lateral sclerosis. Neurology. 2006;66:647–53. 10.1212/01.wnl.0000200962.94777.71 . Mitsumoto H, Chiuzan C, Gilmore M, Zhang Y, Simmons Z, Paganoni S, et al. Primary lateral sclerosis (PLS) functional rating scale: PLS-specific clinimetric scale. Muscle Nerve. 2020;61:163–72. 10.1002/mus.26765 . Kasarskis EJ, Dempsey-Hall L, Thompson MM, Luu LC, Mendiondo M, Kryscio R. Rating the severity of ALS by caregivers over the telephone using the ALSFRS-R. Amyotroph Lateral Scler Other Motor Neuron Disord. 2005;6:50–4. 10.1080/14660820510027107 . Kaufmann P, Levy G, Montes J, Buchsbaum R, Barsdorf AI, Battista V, et al. Excellent inter-rater, intra-rater, and telephone-administered reliability of the ALSFRS-R in a multicenter clinical trial. Amyotroph Lateral Scler. 2007;8:42–6. 10.1080/17482960600888156 . Turner MR, Barohn RJ, Corcia P, Fink JK, Harms MB, Kiernan MC, et al. Primary lateral sclerosis: consensus diagnostic criteria. J Neurol Neurosurg Psychiatry. 2020;91:373–77. 10.1136/jnnp-2019-322541 . Gordon PH, Miller RG, Moore DH. ALSFRS-R. Amyotroph Lateral Scler Other Motor Neuron Disord. 2004;5: 90–3. 10.1080/17434470410019906 . Tables Table 1. Study protocol P † , primary evaluator; PLSFRS, Primary Lateral Sclerosis Functional Rating Scale; S ‡ , secondary evaluator. Time Baseline 4 weeks later 8 weeks later Way to evaluate Face-to-Face Face-to-Face Telephone Face-to-Face Eligibility Assessment P † Consent/Enrollment P PLSFRS Evaluation P S ‡ S P Table 2. Inclusion and exclusion criteria † Upper motor neuron dysfunction included spasticity and associated weakness, pathological hyperreflexia that is Hoffman’s sign and bilateral extensor toe responses, pseudobulbar affect. ‡ Normal needle electromyography or the absence of active lower motor neuron degeneration that minimally increased insertional activity and positive sharp waves or fibrillation potentials in extremity muscles. Inclusion Criteria Exclusion Criteria Japanese who are 20 years of age or older. No family history or consanguinity. No cognitive decline or hearing loss. Unexplained upper motor neuron disorders † in at least two of the three regions of the brain, upper limbs, and lower limbs regardless of the disease duration. Normal needle electromyography ‡ , or no evidence of active lower motor neuron disorders, Able to sign a consent form (if unable to sign due to muscle weakness in the upper extremities, or unable to respond due to dysarthria, writing on behalf of the patient was acceptable). Cognitive decline or hearing loss, including proxy. If consent to this research was not obtained. Other cases the researcher determined inappropriate, such as the patient being unable to express intentions accurately. Additional Declarations No competing interests reported. Supplementary Files SupplementPrimaryLateralSclerosisFunctionalRatingScalePLSFRS.pdf Cite Share Download PDF Status: Published Journal Publication published 13 Aug, 2024 Read the published version in BMC Neurology → Version 1 posted Editorial decision: Revision requested 03 Jun, 2024 Reviews received at journal 02 Jun, 2024 Reviewers agreed at journal 01 Jun, 2024 Reviews received at journal 24 May, 2024 Reviewers agreed at journal 03 May, 2024 Reviewers invited by journal 25 Apr, 2024 Editor invited by journal 25 Apr, 2024 Submission checks completed at journal 22 Apr, 2024 Editor assigned by journal 22 Apr, 2024 First submitted to journal 19 Apr, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4295513","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":295574429,"identity":"4ba547a8-c761-4996-93c4-f37cc6e8d97c","order_by":0,"name":"Masaru Yanagihashi","email":"","orcid":"","institution":"Toho University Faculty of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Masaru","middleName":"","lastName":"Yanagihashi","suffix":""},{"id":295574430,"identity":"715d4cf4-9506-410f-a9a0-d32d829fdbb2","order_by":1,"name":"Takehisa Hirayama","email":"","orcid":"","institution":"Toho University Faculty of 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Scale","fulltext":[{"header":"Background","content":"\u003cp\u003ePrimary lateral sclerosis (PLS) is a progressive neurodegenerative disease in which upper motor neurons are selectively damaged. PLS is rare, occurring in less than 5% of motor neuron diseases \u003csup\u003e[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]\u003c/sup\u003e; therefore to date, no therapeutic agents or clinical trials have been evaluated. Since there have been no indicators to evaluate PLS, in 2020 a PLS Functional Rating Scale (PLSFRS) was developed in the United States of America, and the results were consistent with intra-rater, inter-rater, face-to-face visits, and telephone test-retest reliability during 1 year \u003csup\u003e[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]\u003c/sup\u003e. The PLSFRS is an essential tool for future international collaborative studies and clinical trials for PLS. Our study evaluated the reliability of the Japanese version of the PLSFRS and assessed the consistency between face-to-face evaluations and telephone interviews.\u003c/p\u003e"},{"header":"Methods","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\n \u003ch2\u003eInstitutional review board and volunteer participation\u003c/h2\u003e\n \u003cp\u003eThe study protocol was approved by the Ethics Committee of Toho University Faculty of Medicine, Tokyo, Japan (A21013, M20282), and other participating institutions (4058, rinfu 21\u0026thinsp;\u0026minus;\u0026thinsp;008, TS R03-017, ippan2021-318, 2021\u0026thinsp;\u0026minus;\u0026thinsp;0101). Written informed consent was obtained from all the patients. The PLSFRS was translated into Japanese by a specialized company and translator (2-14-10 Sotokanda Chiyoda-ku Tokyo 101\u0026thinsp;\u0026minus;\u0026thinsp;0021 Crimson Interactive Japan Co., ltd.), and it was further modified to account for the Japanese lifestyle based on consensus among the motor neuron specialists. Nineteen patients were enrolled in the study.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec4\" class=\"Section2\"\u003e\n \u003ch2\u003eProtocol and inclusion and exclusion criteria\u003c/h2\u003e\n \u003cp\u003eThe study and evaluation protocols are presented in Table \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e. Face-to-face examinations and telephone interviews were conducted at intervals of at least 24 hours but no more than 7 days. All evaluators for this study were board-certified neurologists and were trained appropriately before the evaluation. The inclusion and exclusion criteria are listed in Table \u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e. The patients were encouraged to state their condition, and the caregivers were advised not to make their interpretations. Participants were excluded if they withdraw consent, died, or were deemed inappropriate to continue in the study.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec5\" class=\"Section2\"\u003e\n \u003ch2\u003eStatistical analyses\u003c/h2\u003e\n \u003cp\u003eWe conducted statistical analyses of the PLSFRS to assess intra-rater and inter-rater reliability for each item of the PLSFRS and the consistency of the PLSFRS total score between a face-to-face examination and telephone interview using Microsoft Excel version 2210 (Microsoft Corp., Redmond, WA, USA) and IBM SPSS Statistics version 29.0 (IBM Corp., Armonk, NY, USA). Intra-rater reliability was assessed between the baseline and 8 weeks after baseline examination. Inter-rater reliability was assessed between the baseline and 4 weeks after baseline examination. The consistency between a face-to-face examination and telephone interview was assessed 4 weeks after the baseline examination.\u003c/p\u003e\n\u003c/div\u003e"},{"header":"Results","content":"\u003cdiv id=\"Sec7\" class=\"Section2\"\u003e \u003ch2\u003eDemographics\u003c/h2\u003e \u003cp\u003eNineteen patients were enrolled at baseline. The average age of the participants at baseline was 68.2 (42\u0026ndash;85) years. The sample included eight men (42.1%) and 11 women (57.9%). The median disease duration was 72 (12\u0026ndash;264) months.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec8\" class=\"Section2\"\u003e \u003ch2\u003eDropouts\u003c/h2\u003e \u003cp\u003eFour participants were excluded from the study. Two patients had aspiration pneumonia during the evaluation period; however this was not considered an adverse event associated with the evaluation. The evaluation could not be completed for the other two patients because they could not consult the doctor within the evaluation period.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec9\" class=\"Section2\"\u003e \u003ch2\u003eIntra-rater and inter-rater reliability\u003c/h2\u003e \u003cp\u003eThe quadratic-weighted kappa coefficients for intra-rater and inter-rater agreement were substantial (intra-rater; 0.691-1.000, inter-rater; 0.634-1.000) (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec10\" class=\"Section2\"\u003e \u003ch2\u003eConsistency\u003c/h2\u003e \u003cp\u003eThe intraclass correlation coefficient for the PLSFRS total score between face-to-face examination and telephone interview was 0.997 (95% confidence interval, 0.992\u0026ndash;0.999).\u003c/p\u003e \u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003ePLS is a rare motor neuron disease, and to date, no clinical trials have been conducted. To develop therapeutic drugs in the future, it will be necessary to work globally, which includes collecting cases; therefore, creating a Japanese version of the PLSFRS is essential. Previously, the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) alone evaluated the intra-rater and inter-rater reliabilities. The usefulness of administering the ALSFRS-R over the telephone was subsequently evaluated \u003csup\u003e[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]\u003c/sup\u003e. Telephone interviews minimized the burden on participants and caregivers by reducing the number of visits required to the study sites. Four consultations, including three visits in 8 weeks, may have increased the burden; however, no adverse events were associated with the visits for this evaluation. The availability of quick and reliable measures that can be administered over the telephone is indispensable for conducting this type of research. Thus, in this study, the consistency of the telephone interviews was ensured from the outset. Repeated evaluator training is crucial for maintaining reliability of the scale. When creating this scale, we considered the Japanese lifestyle and, added instructions to prevent confusion during the evaluation. The Japanese version of the PLSFRS is provided as a supplement, and the original instructions were referenced and added to the table for the evaluators\u0026rsquo; convenience.\u003c/p\u003e \u003cp\u003eThe present study has several limitations. The current diagnostic criteria require the absence of clinical and electrophysiological lower motor neuron involvement for at least 4 years after symptom onset before diagnosing PLS \u003csup\u003e[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]\u003c/sup\u003e. Although the participants in this study were recruited according to the criteria, some patients were in the early stage of disease onset and may have been diagnosed with upper motor neuron-dominant amyotrophic lateral sclerosis. Moreover, we implemented less stringent electromyography criteria to enroll more participants and accommodate a wider spectrum of patients with PLS. This approach can be an ambivalent issue because the participants who developed pure upper motor disease for less than 4 years could not receive a definite diagnosis and may have been classified as having predominantly upper motor neuron dominant amyotrophic lateral sclerosis \u003csup\u003e[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]\u003c/sup\u003e. The concern with the PLSFRS was that, since there were two additional score tiers compared to the ALSFRS-R, except for orthopnea and respiratory insufficiency, the evaluator and the participant can get confused about the subtleties. We initially believed that evaluations by phone or in person would be difficult; however, the results showed no significant differences. Therefore, the Japanese version of the PLSFRS may be a valuable indicator for assessing medical conditions, even when face-to-face assessment is impossible due to disasters or hospital visit burdens.\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003eThis study provides the results for the Japanese version of the PLSFRS intra-rater and inter-rater reliability and an evaluation of the consistency between in-person and telephone interviews. Similar to the ALSFRS-R for amyotrophic lateral sclerosis, we believe that the Japanese version of the PLSFRS will be widely used not only by neurologists but also by internists and medical staff other than doctors and is a well-validated tool for PLS researchers to conduct future clinical trials.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eALSFRS-R\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eAmyotrophic Lateral Sclerosis Functional Rating Scale-Revised\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003ePLS\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eprimary lateral sclerosis\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003ePLSFRS\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003ePLS Functional Rating Scale\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe study protocol was approved by the Ethics Committee of Toho University\u0026nbsp;Faculty\u0026nbsp;of Medicine,\u0026nbsp;Tokyo, Japan\u0026nbsp;(A21013, M20282), and other participating institutions (4058, rinfu 21-008, TS R03-017, ippan2021-318, 2021-0101).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe data that support the findings of this study are available from the\u0026nbsp;corresponding\u0026nbsp;author upon reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no\u0026nbsp;competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis work was partly supported by Grants-in-Aid from\u0026nbsp;the Research Committee of CNS Degenerative Diseases, Research on Policy Planning and Evaluation for Rare and Intractable Diseases, Health, Labour and Welfare Sciences Research Grants, and the Ministry of Health, Labour and Welfare, Japan (20FC1049).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eMY:\u0026nbsp;Conceptualization; Data Curation; Formal Analysis; Investigation; Methodology; Project Administration; Resources; Validation; Visualization; Writing \u0026ndash; Original Draft Preparation; and Writing \u0026ndash; Review and Editing.\u0026nbsp;TH:\u0026nbsp;Data Curation; Investigation; and Resources.\u0026nbsp;MS:\u0026nbsp;Project Administration.\u0026nbsp;JN:\u0026nbsp;Project Administration.\u0026nbsp;KF:\u0026nbsp;Conceptualization; Investigation; Methodology; and Resources.\u0026nbsp;YI:\u0026nbsp;Conceptualization; Investigation; Methodology; and Resources.\u0026nbsp;MM: Conceptualization; Investigation; Methodology; and Resources.\u0026nbsp;KB:\u0026nbsp;Conceptualization; Investigation; Methodology; and Resources.\u0026nbsp;KT: Conceptualization; Investigation; Methodology; and Resources.\u0026nbsp;KK:\u0026nbsp;Conceptualization; and Methodology.\u0026nbsp;NA:\u0026nbsp;Conceptualization; Methodology; Project Administration; and Supervision.\u0026nbsp;YI:\u0026nbsp;Conceptualization; and Methodology.\u0026nbsp;MK:\u0026nbsp;Conceptualization; and Methodology.\u0026nbsp;YM:\u0026nbsp;Conceptualization; Data Curation; Formal Analysis; and Methodology.\u0026nbsp;HM:\u0026nbsp;Conceptualization; and Methodology.\u0026nbsp;OK:\u0026nbsp;Conceptualization; Data Curation; Funding acquisition; Methodology; Project Administration; and Supervision. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors are grateful to all the participants and their families. We also thank Mrs. Yurika Okuma and Mrs. Tamayo Yamashita, executive assistants to Department of Neurology, Toho University Faculty of Medicine, who supported this article. Moreover, we would like to thank Editage (www.editage.com) for English language editing.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePrevious presentations\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThese data were presented at the 64\u003csup\u003eth\u003c/sup\u003e Annual Meeting of Japanese Society of Neurology, Chiba, Japan, the 41\u003csup\u003est\u003c/sup\u003e Annual Meeting of Japanese Society of Neurotherapy, Tokyo, Japan, Neurodegenerative Disease Workshop in 2020, 2023, Tokyo, Japan.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eStatland JM, Barohn RJ, Dimachkie MM, Floeter MK, Mitsumoto H. Primary lateral sclerosis. Neurologic Clin. 2015;33:749\u0026ndash;60. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.ncl.2015.07.007\u003c/span\u003e\u003cspan address=\"10.1016/j.ncl.2015.07.007\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eGordon PH, Cheng B, Katz IB, Pinto M, Hays AP, Mitsumoto H, et al. The natural history of primary lateral sclerosis. Neurology. 2006;66:647\u0026ndash;53. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1212/01.wnl.0000200962.94777.71\u003c/span\u003e\u003cspan address=\"10.1212/01.wnl.0000200962.94777.71\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMitsumoto H, Chiuzan C, Gilmore M, Zhang Y, Simmons Z, Paganoni S, et al. Primary lateral sclerosis (PLS) functional rating scale: PLS-specific clinimetric scale. Muscle Nerve. 2020;61:163\u0026ndash;72. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1002/mus.26765\u003c/span\u003e\u003cspan address=\"10.1002/mus.26765\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKasarskis EJ, Dempsey-Hall L, Thompson MM, Luu LC, Mendiondo M, Kryscio R. Rating the severity of ALS by caregivers over the telephone using the ALSFRS-R. Amyotroph Lateral Scler Other Motor Neuron Disord. 2005;6:50\u0026ndash;4. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1080/14660820510027107\u003c/span\u003e\u003cspan address=\"10.1080/14660820510027107\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKaufmann P, Levy G, Montes J, Buchsbaum R, Barsdorf AI, Battista V, et al. Excellent inter-rater, intra-rater, and telephone-administered reliability of the ALSFRS-R in a multicenter clinical trial. Amyotroph Lateral Scler. 2007;8:42\u0026ndash;6. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1080/17482960600888156\u003c/span\u003e\u003cspan address=\"10.1080/17482960600888156\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eTurner MR, Barohn RJ, Corcia P, Fink JK, Harms MB, Kiernan MC, et al. Primary lateral sclerosis: consensus diagnostic criteria. J Neurol Neurosurg Psychiatry. 2020;91:373\u0026ndash;77. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1136/jnnp-2019-322541\u003c/span\u003e\u003cspan address=\"10.1136/jnnp-2019-322541\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eGordon PH, Miller RG, Moore DH. ALSFRS-R. Amyotroph Lateral Scler Other Motor Neuron Disord. 2004;5: 90\u0026ndash;3. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1080/17434470410019906\u003c/span\u003e\u003cspan address=\"10.1080/17434470410019906\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"},{"header":"Tables","content":"\u003cp\u003e\u003cstrong\u003eTable 1.\u0026nbsp;\u003c/strong\u003eStudy protocol\u003c/p\u003e\n\u003cp\u003eP\u003csup\u003e\u0026dagger;\u003c/sup\u003e, primary evaluator; PLSFRS, Primary Lateral Sclerosis Functional Rating Scale; S\u003csup\u003e\u0026Dagger;\u003c/sup\u003e, secondary evaluator.\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd width=\"28.268551236749115%\" valign=\"top\"\u003e\n \u003cp\u003eTime\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eBaseline\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"34.98233215547703%\" colspan=\"2\" valign=\"top\"\u003e\n \u003cp\u003e4 weeks later\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003e8 weeks later\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"28.268551236749115%\" valign=\"top\"\u003e\n \u003cp\u003eWay to evaluate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eFace-to-Face\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eFace-to-Face\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"16.607773851590107%\" valign=\"top\"\u003e\n \u003cp\u003eTelephone\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eFace-to-Face\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"28.268551236749115%\" valign=\"top\"\u003e\n \u003cp\u003eEligibility Assessment\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eP\u003csup\u003e\u0026dagger;\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"16.607773851590107%\" valign=\"top\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"28.268551236749115%\" valign=\"top\"\u003e\n \u003cp\u003eConsent/Enrollment\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eP\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"16.607773851590107%\" valign=\"top\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"28.268551236749115%\" valign=\"top\"\u003e\n \u003cp\u003ePLSFRS Evaluation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eP\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eS\u003csup\u003e\u0026Dagger;\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"16.607773851590107%\" valign=\"top\"\u003e\n \u003cp\u003eS\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"18.374558303886925%\" valign=\"top\"\u003e\n \u003cp\u003eP\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cstrong\u003eTable 2.\u0026nbsp;\u003c/strong\u003eInclusion and exclusion criteria\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003csup\u003e\u0026dagger;\u003c/sup\u003eUpper motor neuron dysfunction included spasticity and associated weakness, pathological hyperreflexia that is Hoffman\u0026rsquo;s\u0026nbsp;sign and bilateral extensor toe responses, pseudobulbar affect.\u003c/p\u003e\n\u003cp\u003e\u003csup\u003e\u0026Dagger;\u003c/sup\u003eNormal needle electromyography or the absence of active lower motor neuron degeneration that minimally increased insertional activity and positive sharp waves or fibrillation potentials in extremity muscles.\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd width=\"50%\" valign=\"top\"\u003e\n \u003cp\u003eInclusion Criteria\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"50%\" valign=\"top\"\u003e\n \u003cp\u003eExclusion Criteria\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50%\" valign=\"top\"\u003e\n \u003cul\u003e\n \u003cli\u003eJapanese who are 20 years of age or older.\u003c/li\u003e\n \u003cli\u003eNo family history or consanguinity.\u003c/li\u003e\n \u003cli\u003eNo cognitive decline or hearing loss.\u003c/li\u003e\n \u003cli\u003eUnexplained upper motor neuron disorders\u003csup\u003e\u0026dagger;\u003c/sup\u003e in at least two of the three regions of the brain, upper limbs, and lower limbs regardless of the disease duration.\u003c/li\u003e\n \u003cli\u003eNormal needle electromyography\u003csup\u003e\u0026Dagger;\u003c/sup\u003e, or no evidence of active lower motor neuron disorders,\u003c/li\u003e\n \u003cli\u003eAble to sign a consent form (if unable to sign due to muscle weakness in the upper extremities, or unable to respond due to dysarthria, writing on behalf of the patient was acceptable).\u003c/li\u003e\n \u003c/ul\u003e\n \u003c/td\u003e\n \u003ctd width=\"50%\" valign=\"top\"\u003e\n \u003cul\u003e\n \u003cli\u003eCognitive decline or hearing loss, including proxy.\u003c/li\u003e\n \u003cli\u003eIf consent to this research was not obtained.\u003c/li\u003e\n \u003cli\u003eOther cases the researcher determined inappropriate, such as the patient being unable to express intentions accurately.\u003c/li\u003e\n \u003c/ul\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-neurology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"nurl","sideBox":"Learn more about [BMC Neurology](http://bmcneurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/nurl","title":"BMC Neurology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"primary lateral sclerosis, PLSFRS Japanese version, face-to-face assessments","lastPublishedDoi":"10.21203/rs.3.rs-4295513/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4295513/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003ePrimary lateral sclerosis (PLS) is an extremely rare condition; therefore, to date no clinical studies have been conducted. The Primary Lateral Sclerosis Functional Rating Scale (PLSFRS) was developed in the United States of America. The PLSFRS is a crucial assessment scale for international collaborative research and future clinical trials for PLS. It is useful for evaluating medical conditions through face-to-face assessments and telephone interviews such as when a face-to-face assessment is not possible due to disasters or the burden of hospital visits. This study assessed the reliability and consistency of in-person and telephone interviews using the Japanese version of the PLSFRS.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e \u003cp\u003eWe enrolled 19 Japanese patients who met the specific criteria for inclusion at the six collaborating institutions. The PLSFRS assessments were performed by two evaluators at defined time points and analyzed for intra-rater and inter-rater reliability and consistency between the in-person and telephone interviews.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e \u003cp\u003eThe Japanese version of the PLSFRS was developed by a specialized company and translator, and modified to consider the Japanese lifestyle through a consensus among motor neuron specialists. The quadratic-weighted kappa coefficients for the intra-rater and the inter-rater agreement were substantial (intra-rater: 0.691-1.000, inter-rater: 0.634-1.000). Moreover, the intraclass correlation coefficient for the PLSFRS total score was 0.997 (95% confidence interval, 0.992\u0026ndash;0.999).\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eThis study provides results regarding the Japanese version of the PLSFRS intra-rater and inter-rater reliability and consistency between in-person and telephone interviews.\u003c/p\u003e","manuscriptTitle":"Reliability and consistency of the Japanese version of the Primary Lateral Sclerosis Functional Rating Scale","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-04-30 19:36:00","doi":"10.21203/rs.3.rs-4295513/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-06-03T14:21:56+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-06-02T11:17:04+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"177649606546536626558262996272664900980","date":"2024-06-01T21:48:00+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-05-24T21:34:48+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"203937671547969600670517552236396827077","date":"2024-05-03T08:19:50+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-04-25T14:17:36+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2024-04-25T09:54:29+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-04-23T02:09:28+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-04-23T02:09:28+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Neurology","date":"2024-04-20T02:22:05+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-neurology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"nurl","sideBox":"Learn more about [BMC Neurology](http://bmcneurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/nurl","title":"BMC Neurology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"a98e5387-6193-4ea5-85d6-1001f21658b4","owner":[],"postedDate":"April 30th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-08-22T19:32:44+00:00","versionOfRecord":{"articleIdentity":"rs-4295513","link":"https://doi.org/10.1186/s12883-024-03729-6","journal":{"identity":"bmc-neurology","isVorOnly":false,"title":"BMC Neurology"},"publishedOn":"2024-08-13 15:58:16","publishedOnDateReadable":"August 13th, 2024"},"versionCreatedAt":"2024-04-30 19:36:00","video":"","vorDoi":"10.1186/s12883-024-03729-6","vorDoiUrl":"https://doi.org/10.1186/s12883-024-03729-6","workflowStages":[]},"version":"v1","identity":"rs-4295513","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4295513","identity":"rs-4295513","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}