The Neuromuscular Disorder in Myasthenia Gravis

2015 ◽  
pp. 305-342
Author(s):  
J. E. Desmedt
Keyword(s):  
Myasthenia Gravis ◽  
Neuromuscular Disorder

Azathioprine-Induced Pancytopenia and Septic Complications: A Probable Cause of Death

2017 ◽  
Vol 31 (5) ◽  
pp. 510-513 ◽  
Author(s):  
Bijoy K. Panda ◽  
Siddhi Umarje ◽  
Arundhati Diwan
Keyword(s):  
Chronic Kidney Disease ◽  
Septic Shock ◽  
Myasthenia Gravis ◽  
Blood Count ◽  
Neuromuscular Disorder ◽  
Suspected Drug ◽  
Septic Complications ◽  
Clinical Investigations ◽  
Severe Pancytopenia ◽  
Relationship Of

Azathioprine, an immunosuppressant which is widely used in the management of the autoimmune neuromuscular disorder. Myasthenia gravis is known to cause myelotoxicity. A 55-year-old male recently diagnosed with myasthenia gravis and chronic kidney disease was put on azathioprine (100 mg/d) along with pyridostigmine and prednisolone. When the treatment was initiated, the hematological reports revealed normal levels of blood count. However, approximately within 3 weeks of continuing the prescribed drugs, the patient was readmitted for complaints of loose watery stools, weakness, and giddiness. Clinical investigations revealed severe pancytopenia, suspecting to be related to azathioprine. The suspected drug (azathioprine) was withdrawn, and the management for pancytopenia was initiated. However, on the second day of hospitalization, the patient underwent cardiac arrest and septic shock which lead to death. Adverse drug reaction assessment revealed a plausible and causal relationship of azathioprine with pancytopenia and other adverse effects seen in this patient.

scholarly journals Analysis of electrooculogram (EOG) signals in studying myasthenia gravis

2021 ◽  
Author(s):  
Timothy Liang
Keyword(s):  
Myasthenia Gravis ◽  
Time Domain ◽  
Early Stage ◽  
Screening Method ◽  
Neuromuscular Disorder ◽  
Severe Form ◽  
Linear Discriminant ◽  
Rise Rate ◽  
Common Precursor ◽  
Average Rise

Myasthenia Gravis (MG) is a neuromuscular disorder that induces muscle weakness and fatigue which can be fatal. A common precursor for severe form of MG is ocular MG. In this thesis, we explored signal processing methodologies for early stage detection of MG using electrooculogram (EOG) signals. An EOG signal database consisting of 62 control and 16 MG (mild to moderate) subjects were analyzed for eye movement characteristics and EOG signal morphologies using time domain and wavelet domain techniques. A linear discriminant analysis (LDA) based classifier was used to quantify the ability of features in separating MG from control samples. Average overall classification accuracy achieved by the proposed method for the best time domain feature (average rise rate) and best wavelet feature (scale band energy) was 82.5% (P<0.01, AUC=0.887) and 83.8% (P<0.01, AUC=0.893), respectively. The obtained results suggest EOG based analysis is a viable, non-invasive alternative MG screening method.

scholarly journals Zytux in Refractory Myasthenia Gravis: A Multicenter, Open-Labeled, Clinical Trial Study of Effectiveness and Safety of a Rituximab Biosimilar

2021 ◽  
Vol 12 ◽  
Author(s):  
Farzad Fatehi ◽  
Kamyar Moradi ◽  
Ali Asghar Okhovat ◽  
Ghazaleh Shojatalab ◽  
Behnaz Sedighi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Clinical Trial ◽  
Myasthenia Gravis ◽  
Clinical Status ◽  
Neuromuscular Disorder ◽  
Clinical Trial Registration ◽  
Prednisolone Dose ◽  
Immune Mediated ◽  
Post Infusion

Objectives: Myasthenia gravis (MG) is an immune-mediated neuromuscular disorder responsive to immunomodulatory treatments. 10–20% of MGs are not responsive to conventional first-line therapies. Here, we sought to investigate the efficacy and safety of rituximab therapy in the treatment of patients with refractory MG.Methods: In a 48-week, multicenter, open-labeled, prospective cohort setting, 34 participants with refractory MG were assigned to receive infusions of Zytux, which is a rituximab biosimilar, according to a validated protocol. Clinical, functional, and quality of life (QoL) measurements were recorded at baseline, and seven further visits using the Myasthenia Gravis Foundation of America (MGFA), Myasthenia Gravis Composite (MGC), Myasthenia Gravis Activities of Daily Living profile (MG-ADL), and Myasthenia Gravis Quality of Life (MGQoL-15) scales. Besides, the post-infusion side effects were systematically assessed throughout the study.Results: The correlation analysis performed by generalized estimating equations analysis represented a significant reduction of MGC, MG-ADL, and MGQoL-15 scores across the trial period. The subgroup analysis based on the patients' clinical status indicated a significant effect for the interaction between time and MGFA subtypes on MG-ADL score, MGC score, and pyridostigmine prednisolone dose, reflecting that the worse clinical condition was associated with a better response to rituximab. Finally, no serious adverse event was documented.Conclusions: Rituximab therapy could improve clinical, functional, and QoL in patients with refractory MG in a safe setting. Further investigations with larger sample size and a more extended follow-up period are warranted to confirm this finding.Clinical Trial Registration: The study was registered by the Iranian Registry of Clinical Trials (IRCT) (Code No: IRCT20150303021315N18).

scholarly journals Exploring Factors Influencing Complete Denture Management of Patient with Myasthenia Gravis

2020 ◽  
Vol 24 (2) ◽  
pp. 113-117
Author(s):  
Savvas Kamalakidis ◽  
Georgios Papaemmanouil ◽  
Vassiliki Anastassiadou
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Dental Treatment ◽  
Neuromuscular Disorder ◽  
Complete Denture ◽  
Treatment Protocols ◽  
Dental Management ◽  
Oral Symptoms ◽  
Natural Dentition ◽  
Neuromuscular Coordination

SummaryBackground/Aim: The purpose of this literature review and case report was to highlight the oral symptoms and the pathophysiology of Myasthenia Gravis (MG). MG is an autoimmune, antibody-mediated neuromuscular disorder. It is characterized by fluctuating fatigability and weakness affecting ocular, bulbar and (proximal) limb skeletal muscles.Case report: The case of an edentulous 72-year-old female patient with MG, and in need of removable prostheses was reported. The dental management of a complete denture wearer with MG required special attention and treatment protocols, as described in the present case report.Conclusions: Dental treatment, especially complete denture rehabilitation, of patients diagnosed with MG presents a challenge to the oral health care provider. Clinicians must be aware of the patients’ health status in order to preserve their natural dentition. Implant-supported overdentures must be the treatment of choice in cases of edentulism coupled with MG. In cases where a complete denture is the treatment option, the prosthesis should be placed in the zone of minimum conflict (neutral zone) for optimal neuromuscular coordination.

scholarly journals Incidence and clinical feature of Myasthenia gravis: A five-year data analysis in Ulaanbaatar, Mongolia

2021 ◽  
Vol 4 (3Suppl) ◽  
pp. 47-54
Author(s):  
Oyunaa Chimedregzen ◽  
Sarangerel Jambal ◽  
Munkhbayar Rentsenbat ◽  
Byambasuren Dagvajantsan
Keyword(s):  
Myasthenia Gravis ◽  
Age Of Onset ◽  
Disease Onset ◽  
Neuromuscular Disorder ◽  
Annual Incidence ◽  
Clinical Feature ◽  
Cross Sectional ◽  
Ocular Symptoms ◽  
Electrophysiological Tests ◽  
Review Period

Myasthenia gravis (MG) is a rare neuromuscular disorder. Till now, there are no studies on the prevalence and incidence of MG in Mongolia. The current study aimed to elucidate the incidence of MG in Ulaanbaatar, the age of onset, and the gender distribution of Mongolian patients with MG. We conducted a cross-sectional, hospital-based study involving MG patients (n=48) all around Ulaanbaatar from 1 January 2015 to 1 January 2020. The clinical diagnosis was assessed with the Myasthenia Gravis Foundation of America (MGFA) classification system. The disease severity was evaluated by using Osserman’s classification. The diagnosis was confirmed with serological and electrophysiological tests. Statistical analysis was performed using SPSS software. A total of 30 patients with MG were registered for the last five years in Ulaanbaatar. The average annual incidence of MG in Ulaanbaatar was 0.65 per 100,000 populations (95%CI 0.26-1.34), 0.60 in males (95%CI 0.25-1.28), and 0.69 in females (95%CI 0.33-1.46). The cumulative incidence in the study period was 3.2 per 100,000 populations. The ratio of males to females was 1:1,3. The median age for onset of MG was 33 years (ranging from 27 to 46 years); 43.3% of patients had ocular and 56.7% generalized symptoms at the disease onset. Only 23.3% of patients remained with purely ocular symptoms (Osserman I stage). The average incidence of MG between 2015 and 2020 was 6,5 per 1.000.000 population, and the annual incidence was relatively stable. Although ocular and generalized symptoms were observed each in about half of the cases, only one-fourth remained with pure ocular signs at the end of the review period.

Absence of Oligoclonal IgG Bands and Anti-Achr Antibodies in the Cerebrospinal Fluid of Patients with Myasthenia Gravis

1998 ◽  
Vol 11 (2) ◽  
pp. 87-90
Author(s):  
K. Kapinas ◽  
S. Tzartos ◽  
B. Kokkas ◽  
D. Divanoglou ◽  
M. Tsolaki ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Myasthenia Gravis ◽  
Human Skeletal Muscle ◽  
Neuromuscular Disorder ◽  
High Titre ◽  
Control Groups ◽  
Cns Involvement ◽  
Definite Multiple Sclerosis ◽  
Oligoclonal Igg ◽  
Oligoclonal Igg Bands

Myasthenia gravis (MG) is a neuromuscular disorder in which antibodies are directed against the nicotinic acetylcholine receptor (AChR) at the neuromuscular junction. Some investigators describe the existence of oligoclonal IgG bands and anti-AChR antibodies in the cerebrospinal fluid (CSF) of MG patients while other refuse it. This study was performed in 15 patients with clinical and electrophysiological diagnosis of MG. Oligoclonal IgG bands (OCB) and antibodies to the AChR from human skeletal muscle were determined in the serum and the CSF of the above MG patients. The last one was done in order to investigate any possible central nervous system (CNS) involvement. It was found that all the MG patients who had a high titre of anti- AChR antibodies in the serum (mean titre 29.2±24.3 nM, range 1.8 to 62 nM) did not present OCB and anti-AChR antibodies in their CSF. On the same time, in a group of 10 patients with a definite multiple sclerosis it was found that eight of them presented OCB in their CSF while the results were negative in another group of 10 patients without evidence of CNS disease. The last two groups served as control groups. Our findings are in accordance with the concept that MG is a pure neuromuscular disorder.

scholarly journals Perioperative Approach in a Patient with Myasthenia Gravis

2021 ◽  
Vol 9 (C) ◽  
pp. 136-139
Author(s):  
Saimir Kuci ◽  
Alfred Ibrahimi ◽  
Shaban Memeti ◽  
Marsela Goga ◽  
Selman Dumani ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Acetylcholine Receptors ◽  
Respiratory Muscles ◽  
Neuromuscular Disorder ◽  
Neuromuscular Blocking ◽  
Physiological Changes ◽  
Common Procedure ◽  
Anesthesia Management ◽  
Small Doses ◽  
Delayed Recovery

Background: Myasthenia gravis is an autoimmune neuromuscular disorder that causes the destruction and overall decrease in functional acetylcholine receptors at the neuromuscular junction. The resultant respiratory and cardiovascular implications are a primary cause of mortality; therefore, a complete and comprehensive understandings of this disorder is vital for the anesthesia provider. Anesthesia management in myasthenia gravis is a great challenge for all anesthesiologists. In this disease, even small doses of muscle relaxants could lead to delayed recovery for respiratory muscles. Case report: We present the case of a 38 years old woman (weight 87 kg) diagnosed with Myasthenia Gravis, which symptoms has worsened recently. The case demonstrates the anesthetic challenges involved, with a focus on the overall approach, pharmacologic considerations, physiological changes, and an emphasis on preoperative operative and post-operative optimization. Conclusion: Thymectomy is a common procedure performed in cases of myasthenia gravis (MG) with a thymoma or general MG that does not improve with medical therapy. During anesthesia the use of propofol or sevoflurane with opioids without the use of any neuromuscular blocking agents has been used with success.

scholarly journals Evidence-Based Practice in Rehabilitation of Myasthenia Gravis. A Systematic Review of the Literature

2020 ◽  
Vol 5 (4) ◽  
pp. 71
Author(s):  
Bruno Corrado ◽  
Benedetto Giardulli ◽  
Massimo Costa
Keyword(s):  
Myasthenia Gravis ◽  
Functional Outcomes ◽  
Balance Training ◽  
Neuromuscular Disorder ◽  
Final Phase ◽  
Exclusion Criteria ◽  
Scientific Databases ◽  
Respiratory Training ◽  
Meta Analyses

Myasthenia gravis is a rare neuromuscular disorder characterized by muscle weakness and fatigue. This review analyzes the most recent evidence regarding the effectiveness and safety of different rehabilitative approaches to the disease. The review was carried out in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 365 articles were found in the main scientific databases. Applying the inclusion/exclusion criteria, 11 studies were admitted to the final phase of the review. Three different rehabilitative approaches were identified: physical training, respiratory training, and balance training. All rehabilitative modalities contributed to enhancing functional outcomes, reducing fatigue, and improving quality of life, but currently none can be recommended over another for the lack of cross-comparative studies. The included studies showed methodological quality from low to fair. Despite the range of rehabilitative interventions available, there is a lack of high-quality evidence. However, this review suggests that a multidisciplinary rehabilitation approach should be recommended to people with myasthenia gravis, and above all, for those with mild to moderate symptomatology.

Voice Spectrography in the Evaluation of Myasthenia Gravis of the Larynx

1978 ◽  
Vol 87 (5) ◽  
pp. 722-728 ◽  
Author(s):  
Michael Rontal ◽  
Eugene Rontal ◽  
William Leuchter ◽  
Michael Rolnick
Keyword(s):  
Myasthenia Gravis ◽  
Cranial Nerves ◽  
Neuromuscular Disorder ◽  
Acetylcholine Esterase ◽  
Accurate Assessment ◽  
Striated Muscles ◽  
Specific Diagnosis ◽  
The Voice ◽  
Objective Recording

Myasthenia gravis is a neuromuscular disorder that affects striated muscles especially those innervated by the cranial nerves. The standard diagnostic regimen is to find a reversal of symptoms by acetylcholine esterase administration. The permanent and objective recording of this effect is the key to an accurate assessment of this test. The voice spectrograph is a noninvasive means of evaluating the voice. It has been used successfully to mate a clear and specific diagnosis of myasthenia gravis as it affects the larynx. The test can be used to distinguish myasthenia gravis from other functional, anatomical and neuromuscular laryngeal disorders.

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