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Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center

INTRODUCTION: There is scarce literature regarding the clinical course, comorbidities and long-term outcomes after myasthenic crisis (MC). The natural history of myasthenia gravis (MG) in this subset remains uncertain. METHODS: The study included a cohort admitted with MC (2007–2017) in a tertiary c...

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Autores principales: Sivadasan, Ajith, Alexander, Mathew, Aaron, Sanjith, Mathew, Vivek, Nair, Shalini, Muthusamy, Karthik, Prabhakar, AT, Benjamin, Rohit N., Shaikh, Atif, Rynjah, Gideon
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6839309/
https://www.ncbi.nlm.nih.gov/pubmed/31736571
http://dx.doi.org/10.4103/aian.AIAN_197_19
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author Sivadasan, Ajith
Alexander, Mathew
Aaron, Sanjith
Mathew, Vivek
Nair, Shalini
Muthusamy, Karthik
Prabhakar, AT
Benjamin, Rohit N.
Shaikh, Atif
Rynjah, Gideon
author_facet Sivadasan, Ajith
Alexander, Mathew
Aaron, Sanjith
Mathew, Vivek
Nair, Shalini
Muthusamy, Karthik
Prabhakar, AT
Benjamin, Rohit N.
Shaikh, Atif
Rynjah, Gideon
author_sort Sivadasan, Ajith
collection PubMed
description INTRODUCTION: There is scarce literature regarding the clinical course, comorbidities and long-term outcomes after myasthenic crisis (MC). The natural history of myasthenia gravis (MG) in this subset remains uncertain. METHODS: The study included a cohort admitted with MC (2007–2017) in a tertiary care hospital. The comorbidities, outcomes after discharge, and prognostic factors were analyzed. RESULTS: Sixty-two patients (89 episodes of MC) were included. Demographic data was comparable between the early- (<50 years) and late-onset (≥50 years) groups. Comorbidities included stress cardiomyopathy (14.5%), arrhythmias (6.4%), neuropathy (17.7%), pancytopenia (12.9%), encephalopathy (11.2%), neuromyotonia (4.8%), myelopathy (3.2%), and myositis (3.2%). Pulmonary embolism (P < 0.008), dysautonomia (P < 0.002), sepsis (P < 0.008), neuropathy (P < 0.002), and phrenic dysfunction (P < 0.016) were associated with prolonged ventilation. Majority of the patients (42, 67.7%) had a favorable outcome (disease status) as defined by remission/minimal manifestations at the time of last follow-up (median 36 months, IQR 15–66). Persistent bulbar weakness (P < 0.001), neuropsychiatric illness (P < 0.001), and comorbidities (P < 0.017) were associated with refractory MG. Eighteen patients (29%) had recurrent crisis. Eleven patients succumbed in the cohort. The main predictors of mortality were tumor progression (P < 0.001) and cardiac illness (P < 0.004). DISCUSSION: A comprehensive treatment approach in MC will translate to good short- and long-term outcomes. The main cornerstones of therapy will include (1) Identification of refractory MG with the implementation of phenotype-based therapy; (2) Addressing comorbidities including cardiac autonomic neuropathy, bulbar weakness, phrenic dysfunction; and (3) Meticulous tumor surveillance.
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spelling pubmed-68393092019-11-15 Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center Sivadasan, Ajith Alexander, Mathew Aaron, Sanjith Mathew, Vivek Nair, Shalini Muthusamy, Karthik Prabhakar, AT Benjamin, Rohit N. Shaikh, Atif Rynjah, Gideon Ann Indian Acad Neurol Original Article INTRODUCTION: There is scarce literature regarding the clinical course, comorbidities and long-term outcomes after myasthenic crisis (MC). The natural history of myasthenia gravis (MG) in this subset remains uncertain. METHODS: The study included a cohort admitted with MC (2007–2017) in a tertiary care hospital. The comorbidities, outcomes after discharge, and prognostic factors were analyzed. RESULTS: Sixty-two patients (89 episodes of MC) were included. Demographic data was comparable between the early- (<50 years) and late-onset (≥50 years) groups. Comorbidities included stress cardiomyopathy (14.5%), arrhythmias (6.4%), neuropathy (17.7%), pancytopenia (12.9%), encephalopathy (11.2%), neuromyotonia (4.8%), myelopathy (3.2%), and myositis (3.2%). Pulmonary embolism (P < 0.008), dysautonomia (P < 0.002), sepsis (P < 0.008), neuropathy (P < 0.002), and phrenic dysfunction (P < 0.016) were associated with prolonged ventilation. Majority of the patients (42, 67.7%) had a favorable outcome (disease status) as defined by remission/minimal manifestations at the time of last follow-up (median 36 months, IQR 15–66). Persistent bulbar weakness (P < 0.001), neuropsychiatric illness (P < 0.001), and comorbidities (P < 0.017) were associated with refractory MG. Eighteen patients (29%) had recurrent crisis. Eleven patients succumbed in the cohort. The main predictors of mortality were tumor progression (P < 0.001) and cardiac illness (P < 0.004). DISCUSSION: A comprehensive treatment approach in MC will translate to good short- and long-term outcomes. The main cornerstones of therapy will include (1) Identification of refractory MG with the implementation of phenotype-based therapy; (2) Addressing comorbidities including cardiac autonomic neuropathy, bulbar weakness, phrenic dysfunction; and (3) Meticulous tumor surveillance. Wolters Kluwer - Medknow 2019 2019-10-25 /pmc/articles/PMC6839309/ /pubmed/31736571 http://dx.doi.org/10.4103/aian.AIAN_197_19 Text en Copyright: © 2006 - 2019 Annals of Indian Academy of Neurology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Original Article
Sivadasan, Ajith
Alexander, Mathew
Aaron, Sanjith
Mathew, Vivek
Nair, Shalini
Muthusamy, Karthik
Prabhakar, AT
Benjamin, Rohit N.
Shaikh, Atif
Rynjah, Gideon
Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center
title Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center
title_full Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center
title_fullStr Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center
title_full_unstemmed Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center
title_short Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center
title_sort comorbidities and long-term outcomes in a cohort with myasthenic crisis: experiences from a tertiary care center
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6839309/
https://www.ncbi.nlm.nih.gov/pubmed/31736571
http://dx.doi.org/10.4103/aian.AIAN_197_19
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