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Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD
OBJECTIVE: To define the frequency, duration, and severity of intractable nausea, vomiting, or hiccups in aquaporin-4–immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and propose diagnostic criteria and a severity scale for area postrema syndrome (APS). METHODS: An...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Lippincott Williams & Wilkins
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6205685/ https://www.ncbi.nlm.nih.gov/pubmed/30258024 http://dx.doi.org/10.1212/WNL.0000000000006392 |
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author | Shosha, Eslam Dubey, Divyanshu Palace, Jacqueline Nakashima, Ichiro Jacob, Anu Fujihara, Kazuo Takahashi, Toshiyuki Whittam, Daniel Leite, Maria Isabel Misu, Tatsuro Yoshiki, Takai Messina, Silvia Elsone, Liene Majed, Masoud Flanagan, Eoin Gadoth, Avi Huebert, Carey Sagen, Jessica Greenberg, Benjamin M. Levy, Michael Banerjee, Aditya Weinshenker, Brian Pittock, Sean J. |
author_facet | Shosha, Eslam Dubey, Divyanshu Palace, Jacqueline Nakashima, Ichiro Jacob, Anu Fujihara, Kazuo Takahashi, Toshiyuki Whittam, Daniel Leite, Maria Isabel Misu, Tatsuro Yoshiki, Takai Messina, Silvia Elsone, Liene Majed, Masoud Flanagan, Eoin Gadoth, Avi Huebert, Carey Sagen, Jessica Greenberg, Benjamin M. Levy, Michael Banerjee, Aditya Weinshenker, Brian Pittock, Sean J. |
author_sort | Shosha, Eslam |
collection | PubMed |
description | OBJECTIVE: To define the frequency, duration, and severity of intractable nausea, vomiting, or hiccups in aquaporin-4–immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and propose diagnostic criteria and a severity scale for area postrema syndrome (APS). METHODS: An International NMOSD database was interrogated for frequency of APS. Patients with AQP4-IgG–positive NMOSD completed an APS symptom questionnaire. Nausea and vomiting severity was derived from the Pregnancy-Unique Quantification of Emesis and Nausea (PUQE) score. The diagnostic criteria, severity scale, and immunotherapy response was applied to a prospective validation cohort of patients from multiple centers. RESULTS: Analysis of an international database for AQP4-IgG–seropositive NMOSD (n = 430) revealed a high prevalence of isolated APS attacks (onset 7.1%–10.3%; subsequent 9.4%–14.5%) across continents. For 100 patients with 157 episodes of APS, nausea (n = 127, 81%) lasted for a median of 14 days (range 2–365), vomiting (113, 72%) with a median of 5 episodes/d (2–40) lasted 1–20 minutes, and hiccups (102, 65%) lasted a median of 14 days (2–365). Symptoms consistently and completely resolved following immunotherapy. Data were used to propose APS diagnostic criteria and repurpose PUQE score (hiccups severity grade based on symptom duration). The clinical utility was demonstrated in a prospective validation cohort. CONCLUSION: Isolated APS attacks are frequently encountered both at onset and during the NMOSD course. The diagnostic criteria proposed here will assist clinicians in recognizing APS. Diagnosis of an APS attack earlier than 48 hours is possible if a dorsal medulla lesion is detected. Accurate diagnosis and evaluation of APS attack severity will assist in outcome measurement in NMOSD clinical trials. |
format | Online Article Text |
id | pubmed-6205685 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-62056852018-11-13 Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD Shosha, Eslam Dubey, Divyanshu Palace, Jacqueline Nakashima, Ichiro Jacob, Anu Fujihara, Kazuo Takahashi, Toshiyuki Whittam, Daniel Leite, Maria Isabel Misu, Tatsuro Yoshiki, Takai Messina, Silvia Elsone, Liene Majed, Masoud Flanagan, Eoin Gadoth, Avi Huebert, Carey Sagen, Jessica Greenberg, Benjamin M. Levy, Michael Banerjee, Aditya Weinshenker, Brian Pittock, Sean J. Neurology Article OBJECTIVE: To define the frequency, duration, and severity of intractable nausea, vomiting, or hiccups in aquaporin-4–immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and propose diagnostic criteria and a severity scale for area postrema syndrome (APS). METHODS: An International NMOSD database was interrogated for frequency of APS. Patients with AQP4-IgG–positive NMOSD completed an APS symptom questionnaire. Nausea and vomiting severity was derived from the Pregnancy-Unique Quantification of Emesis and Nausea (PUQE) score. The diagnostic criteria, severity scale, and immunotherapy response was applied to a prospective validation cohort of patients from multiple centers. RESULTS: Analysis of an international database for AQP4-IgG–seropositive NMOSD (n = 430) revealed a high prevalence of isolated APS attacks (onset 7.1%–10.3%; subsequent 9.4%–14.5%) across continents. For 100 patients with 157 episodes of APS, nausea (n = 127, 81%) lasted for a median of 14 days (range 2–365), vomiting (113, 72%) with a median of 5 episodes/d (2–40) lasted 1–20 minutes, and hiccups (102, 65%) lasted a median of 14 days (2–365). Symptoms consistently and completely resolved following immunotherapy. Data were used to propose APS diagnostic criteria and repurpose PUQE score (hiccups severity grade based on symptom duration). The clinical utility was demonstrated in a prospective validation cohort. CONCLUSION: Isolated APS attacks are frequently encountered both at onset and during the NMOSD course. The diagnostic criteria proposed here will assist clinicians in recognizing APS. Diagnosis of an APS attack earlier than 48 hours is possible if a dorsal medulla lesion is detected. Accurate diagnosis and evaluation of APS attack severity will assist in outcome measurement in NMOSD clinical trials. Lippincott Williams & Wilkins 2018-10-23 /pmc/articles/PMC6205685/ /pubmed/30258024 http://dx.doi.org/10.1212/WNL.0000000000006392 Text en Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. |
spellingShingle | Article Shosha, Eslam Dubey, Divyanshu Palace, Jacqueline Nakashima, Ichiro Jacob, Anu Fujihara, Kazuo Takahashi, Toshiyuki Whittam, Daniel Leite, Maria Isabel Misu, Tatsuro Yoshiki, Takai Messina, Silvia Elsone, Liene Majed, Masoud Flanagan, Eoin Gadoth, Avi Huebert, Carey Sagen, Jessica Greenberg, Benjamin M. Levy, Michael Banerjee, Aditya Weinshenker, Brian Pittock, Sean J. Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD |
title | Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD |
title_full | Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD |
title_fullStr | Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD |
title_full_unstemmed | Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD |
title_short | Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD |
title_sort | area postrema syndrome: frequency, criteria, and severity in aqp4-igg–positive nmosd |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6205685/ https://www.ncbi.nlm.nih.gov/pubmed/30258024 http://dx.doi.org/10.1212/WNL.0000000000006392 |
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