Area postrema syndrome

Frequency, criteria, and severity in AQP4-IgG-positive NMOSD

Eslam Shosha, Divyanshu Dubey, Jacqueline Palace, Ichiro Nakashima, Anu Jacob, Kazuo Fujihara, Toshiyuki Takahashi, Daniel Whittam, Maria Isabel Leite, Tatsuro Misu, Takai Yoshiki, Silvia Messina, Liene Elsone, Masoud Majed, Eoin Flanagan, Avi Gadoth, Carey Huebert, Jessica Sagen, Benjamin M. Greenberg, Michael Levy & 3 others Aditya Banerjee, Brian Weinshenker, Sean J. Pittock

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)e1642-e1651
JournalNeurology
Volume91
Issue number17
DOIs
StatePublished - Oct 23 2018

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Aquaporin 4
Neuromyelitis Optica
Area Postrema
Immunoglobulin G
Nausea
Vomiting
Hiccup
Immunotherapy
Databases
Pregnancy
Clinical Trials

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Shosha, E., Dubey, D., Palace, J., Nakashima, I., Jacob, A., Fujihara, K., ... Pittock, S. J. (2018). Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG-positive NMOSD. Neurology, 91(17), e1642-e1651. https://doi.org/10.1212/WNL.0000000000006392

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.

In: Neurology, Vol. 91, No. 17, 23.10.2018, p. e1642-e1651.

Research output: Contribution to journalArticle

Shosha, E, Dubey, D, Palace, J, Nakashima, I, Jacob, A, Fujihara, K, Takahashi, T, Whittam, D, Leite, MI, Misu, T, Yoshiki, T, Messina, S, Elsone, L, Majed, M, Flanagan, E, Gadoth, A, Huebert, C, Sagen, J, Greenberg, BM, Levy, M, Banerjee, A, Weinshenker, B & Pittock, SJ 2018, 'Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG-positive NMOSD', Neurology, vol. 91, no. 17, pp. e1642-e1651. https://doi.org/10.1212/WNL.0000000000006392
Shosha E, Dubey D, Palace J, Nakashima I, Jacob A, Fujihara K et al. Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG-positive NMOSD. Neurology. 2018 Oct 23;91(17):e1642-e1651. https://doi.org/10.1212/WNL.0000000000006392
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. In: Neurology. 2018 ; Vol. 91, No. 17. pp. e1642-e1651.
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T2 - Frequency, criteria, and severity in AQP4-IgG-positive NMOSD

AU - Shosha, Eslam

AU - Dubey, Divyanshu

AU - Palace, Jacqueline

AU - Nakashima, Ichiro

AU - Jacob, Anu

AU - Fujihara, Kazuo

AU - Takahashi, Toshiyuki

AU - Whittam, Daniel

AU - Leite, Maria Isabel

AU - Misu, Tatsuro

AU - Yoshiki, Takai

AU - Messina, Silvia

AU - Elsone, Liene

AU - Majed, Masoud

AU - Flanagan, Eoin

AU - Gadoth, Avi

AU - Huebert, Carey

AU - Sagen, Jessica

AU - Greenberg, Benjamin M.

AU - Levy, Michael

AU - Banerjee, Aditya

AU - Weinshenker, Brian

AU - Pittock, Sean J.

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N2 - 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.

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