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Modern Rheumatology Case Reports Dec 2023We present the case of a 75-year-old man diagnosed with myasthenia gravis (MG) based on lower leg weakness and ptosis for the past 2 months before admission to our...
We present the case of a 75-year-old man diagnosed with myasthenia gravis (MG) based on lower leg weakness and ptosis for the past 2 months before admission to our hospital. The patient was anti-acetylcholine receptor antibody-positive at admission. He was treated with pyridostigmine bromide and prednisolone, which improved the ptosis, but the lower leg muscle weakness remained. An additional lower leg magnetic resonance imaging examination suggested myositis. Inclusion body myositis (IBM) was diagnosed after a subsequent muscle biopsy. Although MG is often associated with inflammatory myopathy, IBM is rare. There is no effective treatment for IBM, but various treatment possibilities have recently been proposed. This case emphasises that myositis complications, including IBM, should be considered when elevated creatine kinase levels are observed and conventional treatments do not address chronic muscle weakness.
Topics: Male; Humans; Aged; Myositis, Inclusion Body; Myasthenia Gravis; Myositis; Muscle Weakness; Treatment Outcome
PubMed: 37210209
DOI: 10.1093/mrcr/rxad026 -
Journal of the American Association of... Aug 2023Congenital myasthenic syndrome (CMS) is a group of rare genetic disorders that mimics the symptoms of myasthenia gravis, but it is due to a genetic defect. We present a...
Congenital myasthenic syndrome (CMS) is a group of rare genetic disorders that mimics the symptoms of myasthenia gravis, but it is due to a genetic defect. We present a case of a male CMS patient, and the course of the disease through the years. The patient initially presented with generalized muscle weakness and difficulty swallowing. During the follow-up, he developed difficulty in chewing, bilateral external ophthalmoparesis with an almost full block of eye movements and bulbar syndrome. The case illustrates both the clinical heterogeneity and the progressive worsening of the symptoms of the disease over the years. The optimal treatment for CMS is based on the molecular defect and its localization in the neuromuscular junction. In our case, treatment with pyridostigmine resulted in good long-term control of symptoms. As a result of the patient's good compliance with treatment, he was not admitted to hospital because of respiratory distress. The lack of a unified protocol for the treatment of CMS highlights the need for a more personalized approach when dealing with patients with rare diseases.
Topics: Humans; Male; Mutation; Myasthenia Gravis; Myasthenic Syndromes, Congenital; Pyridostigmine Bromide; Adult; Treatment Outcome
PubMed: 37141567
DOI: 10.1097/JXX.0000000000000878 -
ANZ Journal of Surgery Sep 2023Chronic intestinal pseudo-obstruction (CIPO) may be a primary or secondary phenomenon and is often multifactorial. Treatment is largely directed at improving colonic... (Review)
Review
BACKGROUND
Chronic intestinal pseudo-obstruction (CIPO) may be a primary or secondary phenomenon and is often multifactorial. Treatment is largely directed at improving colonic motility. The use of cholinesterase inhibitors such as pyridostigmine has been hypothesized to increase acetylcholine in the bowel, improving symptoms and transit times.
METHODS
A systematic review of the use of pyridostigmine in CIPO was conducted using scientific and commercial search engines identifying scientific studies enrolling adult human subjects, published from 2000 to 2022 in the English language.
RESULTS
Four studies were identified including two randomized controlled trials (RCT) and two observational studies. The studies had heterogenous inclusion criteria, dosing regimens and reported outcomes. Two studies were identified as being at high risk of bias. All studies reported improved patient outcomes with use of pyridostigmine, and low rates (4.3%) of mild cholinergic side effects. No major side effects were reported.
CONCLUSION
The use of pyridostigmine in management of CIPO is biologically plausible due to its ability to increase colonic motility, and early studies on its role are uniformly suggestive of benefit with low side-effect profile. Four clinical studies have been conducted to date, with small sample sizes, heterogeneity and high risk of bias. Further high-quality studies are required to enable assessment of pyridostigmine's utility as an effective management strategy in CIPO.
Topics: Adult; Humans; Pyridostigmine Bromide; Gastrointestinal Motility; Intestinal Pseudo-Obstruction; Cholinesterase Inhibitors; Chronic Disease
PubMed: 37132128
DOI: 10.1111/ans.18478 -
International Ophthalmology Aug 2023Ocular myasthenia gravis (OMG) is an autoimmune disease which causes ptosis, diplopia, or both. It can be categorized as early or late onset, with differing presenting...
BACKGROUND
Ocular myasthenia gravis (OMG) is an autoimmune disease which causes ptosis, diplopia, or both. It can be categorized as early or late onset, with differing presenting characteristics and prognoses. Currently, there is limited information available to compare characteristics and outcomes in onset groups in Thailand.
OBJECTIVE
To describe and compare baseline characteristics and outcomes in OMG patients classified by onset groups and to investigate the factors associated with the disease, especially in terms of treatment responses classified according to the MGFA Post-Intervention Status (MGFA-PIS).
METHODS
OMG patients diagnosed between January 2014 and March 2021 at Rajavithi Hospital, Thailand, were categorized into 2 groups based on age of onset, and baseline characteristics were analyzed and compared. The treatment responses of each group in terms of time to achievement of minimal manifestations (MM) were analyzed.
RESULTS
Eighty-one patients (38 with early and 43 with late onset) were included, and the mean (SD) follow-up time was 35.85 months (17.25). There was no significant difference between the baseline characteristics of the two groups. A low dose of pyridostigmine was more commonly used in the early-onset group (p = 0.01), while the mean dose of corticosteroids was significantly lower in the late-onset patients (p < 0.001). We found that seropositivity of acetylcholine receptor antibody decreased the odds ratio of achievement of MM (OR 0.185, 95% CI 0.043-0.789, p = 0.023) and receiving a high dose of pyridostigmine (≥ 120 mg/day) increased the odds ratio of achieving it (OR 8.296, 95% CI 2.136-32.226, p = 0.002).
CONCLUSIONS
A higher dose of pyridostigmine may be necessary for achievement of favorable treatment response. AChRAb seropositivity is a predictor for unfavorable treatment response in Thai populations.
Topics: Humans; Pyridostigmine Bromide; Age of Onset; Retrospective Studies; Myasthenia Gravis; Receptors, Cholinergic
PubMed: 36879110
DOI: 10.1007/s10792-023-02676-4 -
Neuromuscular Disorders : NMD Aug 2023
Topics: Humans; DNA-Binding Proteins; Transcription Factors; Neuromuscular Junction; Epilepsy; Pyridostigmine Bromide
PubMed: 36631330
DOI: 10.1016/j.nmd.2022.11.001 -
Journal of Biomolecular Structure &... 2023Nerve agent poisoning is still a threat to civilization. Nerve agents function by binding with the enzyme acetylcholinesterase irreversibly. Accumulation of...
Nerve agent poisoning is still a threat to civilization. Nerve agents function by binding with the enzyme acetylcholinesterase irreversibly. Accumulation of acetylcholine in the synapse causes over-stimulation of muscarinic and nicotinic acetylcholinergic receptors. Thus miosis, glandular hyper secretion, bronchoconstriction, vomiting, diarrhea and bradycardia occurs (by M1-M5 receptors stimulation); whereas convulsion and seizures occur due to the nicotinic receptors. Atropine is a non-selective muscarinic antagonists but no nicotinic antagonists are known. Seizures are controlled by diazepam. Enzyme aging occurs without treatment which causes the enzyme resistant to oxime therapy. Though numerous wet-lab based works has carried out, however, recent time there is an over-growing trend to make comparative assessment of drugs and toxicants. Here we made a molecular docking based comparative assessments between nerve agents toxicity and efficacy of different drugs to prevent this toxicity. Our results suggest that VX is the most harmful organophosphate nerve agents and HI-6 is the best drug followed by Obidoxime and Pralidoxime to free acetylcholinestarase. Docking results correspond the data trend of different experiments for the assessment of severity of different nerve agents and/or effectiveness of different antidote drugs. Our study reinforces the utility of pretreatment of the enzyme with a carbamic acid derivative like Pyridostigmine bromide which inhibits the enzyme reversibly to a smaller extent and thus, prevent the enzyme from aging and the nerve agent binding.Communicated by Ramaswamy H. Sarma.
PubMed: 36152998
DOI: 10.1080/07391102.2022.2125904