scholarly journals Myasthenia Crisis Vs Cholinergic Crisis: Challenges in Crisis Management Without Plasmapheresis or Intravenous Immunoglobulin (IVIG)

Author(s):  
Lila Tri Harjana ◽  
Hardiono Hardiono

Introduction: Myasthenia gravis (MG) is an acquired autoimmune disorder clinically characterized by skeletal muscle weakness & fatigability on exertion with prevalence as high as 2–7 in 10,000 and women are affected more frequently than men (~3:2). Over 12-16% of generalized MG patients experience crisis once in their lifetime. A serious complication of myasthenia gravis is respiratory failure. This may be secondary to an exacerbation of myasthenia (myasthenia crisis) or to treatment with excess doses of a cholinesterase inhibitor (cholinergic crisis). Case Report: Thirty-two years old woman refereed from a private hospital to ED for further treatment with myasthenia in crisis, after nine days of treatment in the previous ICU. Patient already in intubation with mechanical ventilation and history of the treatment of a high dose of multiple anticholinesterase drugs and steroids without plasmapheresis or immunoglobulin intravenous. During admission, diarrhea was present, with no sign of GI infection. On the third day of admission, the patient performed a Spontaneous Breathing Trial and was a success then extubated. Then two day after extubation, the patient falls to respiratory failure and need mechanical ventilation. Anticholinesterase test was performed, and it shows no improvement in clinical signs, and diagnose as Cholinergic Crisis. After re-adjustment of anticholinesterase drug with a lower dose, clinically, the respiratory condition improved, and on the 10th day of admission, the patient was succeed extubated. At 12nd days of ICU admission, patient discharge from ICU. Discussion: Myasthenia and Cholinergic Crisis is a severe and life-threatening condition characterized by generalized muscle weakness with a respiratory compromise that requires ventilatory support. Respiratory failure may be present in the cholinergic crisis without cholinergic symptoms (miosis, diarrhea, urinary incontinence, bradycardia, emesis, lacrimation, or salivation). The most important management aspect of Myasthenia patients in crisis is the recognition and treatment of myasthenia vs cholinergic crisis.

Author(s):  
Lila Tri Harjana ◽  
Hardiono Hardiono

Introduction: Myasthenia gravis (MG) is an acquired autoimmune disorder clinically characterized by skeletal muscle weakness & fatigability on exertion with prevalence as high as 2–7 in 10,000 and women are affected more frequently than men (~3:2). Over 12-16% of generalized MG patients experience crisis once in their lifetime. A serious complication of myasthenia gravis is respiratory failure. This may be secondary to an exacerbation of myasthenia (myasthenia crisis) or to treatment with excess doses of a cholinesterase inhibitor (cholinergic crisis). Case Report: Thirty-two years old woman refereed from a private hospital to ED for further treatment with myasthenia in crisis, after nine days of treatment in the previous ICU. Patient already in intubation with mechanical ventilation and history of the treatment of a high dose of multiple anticholinesterase drugs and steroids without plasmapheresis or immunoglobulin intravenous. During admission, diarrhea was present, with no sign of GI infection. On the third day of admission, the patient performed a Spontaneous Breathing Trial and was a success then extubated. Then two day after extubation, the patient falls to respiratory failure and need mechanical ventilation. Anticholinesterase test was performed, and it shows no improvement in clinical signs, and diagnose as Cholinergic Crisis. After re-adjustment of anticholinesterase drug with a lower dose, clinically, the respiratory condition improved, and on the 10th day of admission, the patient was succeed extubated. At 12nd days of ICU admission, patient discharge from ICU. Discussion: Myasthenia and Cholinergic Crisis is a severe and life-threatening condition characterized by generalized muscle weakness with a respiratory compromise that requires ventilatory support. Respiratory failure may be present in the cholinergic crisis without cholinergic symptoms (miosis, diarrhea, urinary incontinence, bradycardia, emesis, lacrimation, or salivation). The most important management aspect of Myasthenia patients in crisis is the recognition and treatment of myasthenia vs cholinergic crisis.


2020 ◽  
Author(s):  
Cyrus Vahdatpour ◽  
Alexander Pichler ◽  
Harold I Palevsky ◽  
Michael J Kallan ◽  
Namrata B Patel ◽  
...  

Abstract Background Interstitial lung disease (ILD) patients requiring invasive mechanical ventilation (IMV) for acute respiratory failure (ARF) are known to have a poor prognosis. Few studies have investigated determinants of outcomes and the utility of trialing non-invasive positive pressure ventilation (NIPPV) prior to IMV to see if there are any effect(s) on mortality or morbidity. Methods We designed a retrospective study using patients at four different intensive care units within one health care system. Our primary objective was to determine if there are differences in outcomes for in-hospital and one-year mortality between patients who undergo NIPPV prior to IMV and those who receive only IMV. A secondary objective was to identify potential determinants of outcomes. Results Of 54 ILD patients with ARF treated with IMV, 20 (37.0%) survived to hospital discharge and 10 (18.5%) were alive at one-year. There was no significant mortality difference between patients trialed on NIPPV prior to IMV and those receiving only IMV. Several key determinants of outcomes were identified with higher mortality, including: higher ventilatory support, idiopathic pulmonary fibrosis (IPF) subtype, high dose steroids, use of vasopressors, supraventricular tachycardias (SVTs), and higher body mass index. Conclusions Considering that patients trialed on NIPPV prior to IMV was associated with no mortality disadvantage to patients treated with only IMV, trialing patients on NIPPV may identify responders and avoid complications associated with IMV. Increased ventilator support, need of vasopressors, SVTs, and high dose steroids reflect higher mortality and palliative care involvement should be considered as early as possible if lung transplant is not an option.


2020 ◽  
Vol 14 (1) ◽  
pp. 67-77
Author(s):  
Cyrus A. Vahdatpour ◽  
Alexander Pichler ◽  
Harold I. Palevsky ◽  
Michael J. Kallan ◽  
Namrata B. Patel ◽  
...  

Background: Interstitial Lung Disease [ILD] patients requiring Invasive Mechanical Ventilation [IMV] for Acute Respiratory Failure [ARF] are known to have a poor prognosis. Few studies have investigated determinants of outcomes and the utility of trialing Non-Invasive Positive Pressure Ventilation [NIPPV] prior to IMV to see if there are any effect[s] on mortality or morbidity. Methods: A retrospective study was designed using patients at four different intensive care units within one health care system. The primary objective was to determine if there are differences in outcomes for in-hospital and one-year mortality between patients who undergo NIPPV prior to IMV and those who receive only IMV. A secondary objective was to identify potential determinants of outcomes. Results: Out of 54 ILD patients with ARF treated with IMV, 20 (37.0%) survived until hospital discharge and 10 (18.5%) were alive at one-year. There was no significant mortality difference between patients trialed on NIPPV prior to IMV and those receiving only IMV. Several key determinants of outcomes were identified with higher mortality, including higher ventilatory support, idiopathic pulmonary fibrosis (IPF) subtype, high dose steroids, use of vasopressors, supraventricular tachycardias (SVTs), and higher body mass index. Conclusion: Considering that patients trialed on NIPPV prior to IMV were associated with no mortality disadvantage to patients treated with only IMV, trialing patients on NIPPV may identify responders and avoid complications associated with IMV. Increased ventilator support, need of vasopressors, SVTs, and high dose steroids reflect higher mortality and palliative care involvement should be considered as early as possible if a lung transplant is not an option.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
José A. Fernández ◽  
Antonio Fernández-Valiñas ◽  
Daniel Hernández ◽  
Joel Orozco ◽  
Antonio Lugo

Myasthenia gravis is an autoimmune disease characterized by muscle weakness. Subjects with antibodies against acetylcholine usually have greater ocular symptoms, lower bulbar weakness, and fewer respiratory complications, compared to individuals with anti-MuSK antibodies. The presence of positivity to both types of antibodies in the same patient is uncommon, and the clinical behavior of these individuals is uncertain. A myasthenic crisis is characterized by respiratory and bulbar muscle weakness, causing acute respiratory failure which requires mechanical ventilatory support. We present the case of a 73-year-old man with a medical history of myasthenia gravis and positive antibody titers against acetylcholine and anti-MuSK, who sought for medical assessment because of respiratory tract infection symptoms, dysphagia, and generalized weakness. Initially, no respiratory distress was found. After 24 hours the patient showed respiratory deterioration and neurological impairment. Endotracheal intubation was rejected, so ventilatory support with noninvasive ventilation was started. The patient was supported by intense respiratory therapy, and infusion of immunoglobulin was initiated. The individual responded favorably, improving his general condition. Weaning from noninvasive mechanical ventilation was possible after six days. Our case illustrates that noninvasive ventilation, properly supported by intense respiratory therapy, can be a great option to avoid intubation in the myasthenic patient.


2016 ◽  
Vol 7 (6) ◽  
pp. 100-102
Author(s):  
Sandeep Lahiry ◽  
Dipak Bhowmik

Myasthenia gravis (MG) is a relatively rare autoimmune disorder in which antibodies form against acetylcholine (Ach) nicotinic postsynaptic receptors at the neuromuscular junction of skeletal muscles. The usual initial complaint is a muscle weakness but can also present as respiratory failure, as in our case. MG is treated with medications such as Acetylcholine-esterase (AChE) inhibitors or immunosuppresants and, in selected cases, thymectomy.Here, we present a case of 32 year old women with MG, who presented with an unexplained respiratory failure. She was stabilized with mechanical ventilation and subsequently treated with immunoglobins and thymectomy.Asian Journal of Medical Sciences Vol.7(5) 2016 100-102


2020 ◽  
Author(s):  
Cyrus A. Vahdatpour ◽  
Alexander Pichler ◽  
Harold I Palevsky ◽  
Michael J Kallan ◽  
Namrata B Patel ◽  
...  

Abstract Background Interstitial lung disease (ILD) patients requiring invasive mechanical ventilation (IMV) for acute respiratory failure (ARF) are known to have a poor prognosis. Few studies have investigated determinants of outcomes and the utility of trialing non-invasive positive pressure ventilation (NIPPV) prior to IMV to see if there are any effect(s) on mortality or morbidity.Methods We designed a retrospective study using patients at four different intensive care units within one health care system. Our primary objective was to determine if there are differences in outcomes for in-hospital and one-year mortality between patients who undergo NIPPV prior to IMV and those who receive only IMV. A secondary objective was to identify potential determinants of outcomes.Results Of 54 ILD patients with ARF treated with IMV, 20 (37.0%) survived to hospital discharge and 10 (18.5%) were alive at one-year. There was no significant mortality difference between patients trialed on NIPPV prior to IMV and those receiving only IMV. Several key determinants of outcomes were identified with higher mortality, including: higher ventilatory support, idiopathic pulmonary fibrosis (IPF) subtype, high dose steroids, use of vasopressors, supraventricular tachycardias (SVTs), and higher body mass index.Conclusions Considering that patients trialed on NIPPV prior to IMV was associated with no mortality disadvantage to patients treated with only IMV, trialing patients on NIPPV may identify responders and avoid complications associated with IMV. Increased ventilator support, need of vasopressors, SVTs, and high dose steroids reflect higher mortality and palliative care involvement should be considered as early as possible if lung transplant is not an option.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S153-S154
Author(s):  
E Conner ◽  
D Troxclair ◽  
H Khokhar ◽  
W Beversdorf

Abstract Introduction/Objective Acute interstitial pneumonia (AIP) is a rare disease clinically characterized by rapidly progressing respiratory failure in individuals with no history of respiratory illness or other inciting factors. While most often diagnosed in middle-aged adults, it may present in any age group. Initial presentation is described as influenza- like, and respiratory failure requiring ventilatory support often progresses within weeks to months. Prognosis is poor, with an estimated mortality rate approaching 80% without treatment. Methods We present the case of a 44-year-old male nonsmoker with no significant medical history, who presented in 2018 with 1.5 months of dyspnea and headache initially diagnosed as atypical pneumonia. Chest imaging revealed bilateral opacities; however, microbial workup revealed no evidence of infectious etiology. Autoimmune serology studies were likewise unrevealing. Despite aggressive supportive and medical management, he deteriorated to respiratory failure and succumbed. Results At autopsy, the lungs were symmetrically congested and edematous (combined weight 2,340 g) but free of evident consolidation or discrete lesions. Microscopic examination revealed diffuse alveolar damage with extensive hyaline membrane formation, interstitial edema, and fibroblastic proliferation. The vasculature was severely congested, and the alveoli contained hemorrhage and scattered macrophages. No fungal or mycobacterial elements were identified by staining. Based on the histologic features and clinical context, the diagnosis of AIP was made. Conclusion AIP is a rare, aggressive, and diagnostically challenging disease that includes a broad range of both clinical and histologic differentials. Timely recognition and intervention with aggressive respiratory support and high- dose glucocorticoids are the mainstays of clinical management. The diagnostic role of histology is significant, but hinges on early clinical consideration of AIP as disease progression may later preclude the biopsy procedure. We share this case to raise awareness of this rapidly progressive and diagnostically troubling interstitial lung disease while emphasizing the importance of clinicopathologic correlation.


2018 ◽  
Author(s):  
Pauline K. Park ◽  
Nicole L Werner ◽  
Carl Haas

Invasive and noninvasive ventilation are important tools in the clinician’s armamentarium for managing acute respiratory failure. Although these modalities do not treat the underlying disease, they can provide the necessary oxygenation and ventilatory support until the causal pathology resolves. Care must be taken as even appropriate application can cause harm. Knowledge of pulmonary mechanics, appreciation of the basic machine settings, and an understanding of how common and advanced modes function allows the clinician to optimally tailor support to the patient while limiting iatrogenic injury. This second chapter reviews indications for mechanical ventilation, routine management, troubleshooting, and liberation from mechanical ventilation This review contains 6 figures, 7 tables and 60 references Keywords: Mechanical ventilation, lung protective ventilation, sedation, ventilator-induced lung injury, liberation from mechanical ventilation 


2018 ◽  
Author(s):  
Adrian A. Maung ◽  
Lewis J Kaplan

In this chapter, we complete the discussion of mechanical ventilation by examining approaches to mechanical ventilation for different patient populations and how to assess whether a patient is ready for liberation from mechanical ventilation. Each of the three chapters is intended to build on the preceding one and therefore establishes a functional unit with regard to mechanical ventilation, whether it is provided in an invasive or a noninvasive fashion.  This review contains 1 Figure, 1 Table and 31 references Key Words: acute respiratory failure, ARDS, mechanical ventilation liberation, spontaneous breathing trial, tracheostomy 


2015 ◽  
Vol 3 (3) ◽  
pp. 122-125
Author(s):  
Mona Sharma

Post trans-sternal thymectomy, the patient may develop respiratory failure, usually due to myasthenic crisis or cholinergic crisis. These crises may prolong mechanical ventilation and impede on-table extubation. We report a case of respiratory failure due to unilateral phrenic nerve palsy in a case of myasthenia gravis. A 35 year old female, known case of myasthenia gravis was posted for trans-sternal thymectomy. Anaesthesia was induced with Propofol, Fentanyl and low dose of Atracurium. Thymoma was incasing right subclavian vein and was adherent with the pericardium, Masaoka Grade III. Blood loss was approximately 200ml. Immediate post operative period showed low tidal volume, tachypnea and tachycardia in spite of train of four ratio >0.9. Patient was kept intubated with suspicion of myasthenic crisis and the dose of Pyridostigmine was increased, but this lead to increased cholinergic symptoms. Pain was addressed with thoracic epidural analgesia. Chest radiograph done later in the evening showed elevated right hemi diaphragm, thereby, confirming the diagnosis of phrenic nerve palsy.   Difficult dissection in this case has resulted in right phrenic nerve palsy. Phrenic nerve palsy has to be considered as a cause of respiratory failure post sternotomy thymectomy, especially in case of Masaoka grade III and IV.DOI: http://dx.doi.org/10.3126/jkmc.v3i3.12249Journal of Kathmandu Medical CollegeVol. 3, No. 3, Issue 9, Jul.-Sep., 2014, Page: 122-125


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