Jung-Ah Lim1,2, Soon-Tae Lee1, Jangsup Moon1,3, Jin-Sun Jun1,4, Tae-Joon Kim1,5, Yong-Won Shin1, Suhailah Abdullah6, Jung-Ick Byun1,7, Jun-Sang Sunwoo1,8, Keun Tae Kim9, Tae-Won Yang10, Woo-Jin Lee1, Hye-Jin Moon11, Dong Wook Kim12, Byung Chan Lim13, Yong Won Cho9, Tae-Ho Yang14, Hee Jin Kim15,16, Young-Soo Kim17, Yong Seo Koo18, Byeongsu Park19, Keun-Hwa Jung1, Manho Kim1,20, Kyung-Il Park1,21, Ki-Young Jung1, Kon Chu1, Sang Kun Lee1. 1. Department of Neurology, Seoul National University Hospital, Seoul, South Korea. 2. Department of Neurology, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, Seoul, South Korea. 3. Department of Neurosurgery, Seoul National University Hospital, Seoul, South Korea. 4. Department of Neurology, School of Medicine, Kyungpook National University, Kyungpook National University Chilgok Hospital, Daegu, South Korea. 5. Department of Neurology, Ajou University School of Medicine, Ajou University Medical Center, Suwon, South Korea. 6. Division of Neurology, Department of Medicine, University Malaya Medical Center, Kuala Lumpur, Malaysia. 7. Department of Neurology, Kyung Hee University Hospital at Gangdong, Seoul, South Korea. 8. Department of Neurology, Soonchunhyang University School of Medicine, Seoul, South Korea. 9. Department of Neurology, Keimyung University Dongsan Medical Center, School of Medicine, Daegu, South Korea. 10. Department of Neurology, Gyeongsang National University Changwon Hospital, Gyeongsang National University School of Medicine, Changwon, South Korea. 11. Department of Neurology, Soonchunhyang University Bucheon Hospital, Bucheon, South Korea. 12. Department of Neurology, Konkuk University School of Medicine, Seoul, South Korea. 13. Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea. 14. Department of Neurology, Chonbuk National University Hospital, Jeonju, South Korea. 15. Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea. 16. Neuroscience Center, Samsung Medical Center, Seoul, South Korea. 17. Department of Neurology and Institute of Health Science, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Seoul, South Korea. 18. Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Ulsan, South Korea. 19. Department of Neurology, Ulsan University Hospital, Ulsan University College of Medicine, Ulsan, South Korea. 20. Center for Neuroscience and Protein Metabolism, Seoul National University College of Medicine, Seoul, South Korea. 21. Department of Neurology, Seoul National University Hospital Healthcare System Gangnam Center, Seoul, South Korea.
Abstract
OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.
OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.
Authors: Gregory S Day; Melanie Y Yarbrough; Peter Körtvelyessy; Harald Prüss; Robert C Bucelli; Marvin J Fritzler; Warren Mason; David F Tang-Wai; Claude Steriade; Julien Hébert; Rachel L Henson; Elizabeth M Herries; Jack H Ladenson; A Sebastian Lopez-Chiriboga; Neill R Graff-Radford; John C Morris; Anne Fagan Journal: Neurology Date: 2021-04-01 Impact factor: 9.910
Authors: Thomas Seifert-Held; Katharina Eberhard; Christian Lechner; Stefan Macher; Harald Hegen; Tobias Moser; Gregor Brecl Jacob; Gertraud Puttinger; Raffi Topakian; Michael Guger; Emrah Kacar; Lea Zoche; Desiree De Simoni; Andreas Seiser; Stefan Oberndorfer; Christoph Baumgartner; Walter Struhal; Friedrich Zimprich; Johann Sellner; Florian Deisenhammer; Christian Enzinger; Markus Reindl; Helmut Rauschka; Thomas Berger; Romana Höftberger Journal: Front Immunol Date: 2021-05-21 Impact factor: 7.561