An unusual presentation of thymoma: dysgeusia, ulcerative colitis, keratoconjunctivitis sicca, autoimmune retinopathy and myasthenia gravis

2022 ◽  
Vol 15 (1) ◽  
pp. e246861
Author(s):  
Priyanka Sanghi ◽  
Fion Bremner
Keyword(s):  
Ulcerative Colitis ◽  
Myasthenia Gravis ◽  
Keratoconjunctivitis Sicca ◽  
Case Reports ◽  
Gastrointestinal Symptoms ◽  
Myasthenic Crisis ◽  
Autoimmune Retinopathy ◽  
Dry Eyes ◽  
Eye Symptoms ◽  
Visual Field Constriction

A 41-year-old female presented with dysgeusia, dry eyes, nyctalopia with progressive visual field constriction (due to autoimmune retinopathy) and gastrointestinal symptoms (due to ulcerative colitis). She was subsequently admitted to intensive care with a myasthenic crisis, and CT of the thorax demonstrated a thymoma.Following thymectomy and adjuvant radiotherapy, she has remained in complete remission from her ulcerative colitis and myasthenia gravis, her retinopathy has stabilised and there has been no thymoma recurrence over a 10-year postoperative period. There was a brief relapse of her dysgeusia (causing weight loss) and dry eye symptoms 3 years after her surgery, which resolved 8 months later. While the association of thymomas with paraneoplastic syndromes (PNS) is well established, it is unusual to present with multiple PNS, and some of these have only been documented in sparse case reports to date. Thymectomy played a crucial role in improvement and stabilisation of her PNS.

scholarly journals Myasthenia Gravis following Low-Osmolality Iodinated Contrast Media

2014 ◽  
Vol 2014 ◽  
pp. 1-2 ◽  
Author(s):  
Luca Bonanni ◽  
Michele Dalla Vestra ◽  
Andrea Zancanaro ◽  
Fabio Presotto
Keyword(s):  
Contrast Media ◽  
Myasthenia Gravis ◽  
Case Reports ◽  
Orotracheal Intubation ◽  
Myasthenic Crisis ◽  
Iodinate Contrast Agent ◽  
Iodinated Contrast ◽  
History Of ◽  
Total Body ◽  
Progressive Dysphagia

We describe the case of 79-year-old man admitted to our general hospital for a 6-week history of progressive dysphagia to solids and liquids associated with weight loss. To reach a diagnosis a total body CT scan with low-osmolality iodinate contrast agent was performed. Two hours later the patient developed an acute respiratory failure requiring orotracheal intubation and mechanical ventilation. The laboratory and neurological tests allow formulating the diagnosis of myasthenia gravis. In literature, other three case reports have associated myasthenic crisis with exposure to low-osmolality contrast media. This suggests being careful in administering low-osmolality contrast media in myasthenic patients.

P29 Etanercept and IBD: can we identify at risk patients?

Rheumatology ◽  
2019 ◽  
Vol 58 (Supplement_4) ◽  
Author(s):  
Kathryn Shepherd ◽  
Nandita Pai ◽  
Daniella Adams ◽  
Nick Wilkinson ◽  
Vinay Shivamurthy
Keyword(s):  
Ulcerative Colitis ◽  
Treatment Option ◽  
Conflicts Of Interest ◽  
Case Reports ◽  
Gastrointestinal Symptoms ◽  
Patient Characteristics ◽  
Therapeutic Benefit ◽  
Line Treatment ◽  
Etanercept Treatment ◽  
The Uk

Poster presentation Tuesday 8 October Background We present four cases of patients on etanercept treatment for juvenile idiopathic arthritis (JIA) subsequently diagnosed with inflammatory bowel disease (IBD). We hypothesise that there may be identifiable characteristics of these patients which makes them more identifiable to the physician. Etanercept is a recombinant dimer of human tumour necrosis factor (TNF) receptor proteins bound to human IgG1. It is an effective second line treatment option in JIA for those who have failed, or are intolerant to, methotrexate, and an effective first line treatment option in those who have axial disease at diagnosis. Anti-TNF treatments are effective in IBD, however etanercept in particular does not have therapeutic benefit, and in one trial there was a non-significant trend towards worsening of disease. Methods Please refer to the Results section. Results Four cases, in a 15 year old male, 11 year old female, 10 year old female and 11 year old female, have been identified at our site. The time from etanercept initiation and diagnosis of IBD was between 2 weeks and 2 years 1 month. None of the cases had gastrointestinal symptoms at JIA diagnosis. All four were ANA negative, and in the two with information, both were HLA-B27 negative. Three out of four had hip arthritis involvement. There was a family history of IBD (ulcerative colitis) in only one of the cases. Aside from non-steroidal anti-inflammatory medications, there were no other immunomodulatory medications concurrently being taken in any of the cases while on etanercept. All four were anaemic (Hb < 120g/L) at diagnosis of IBD. Only one had raised ESR (43mm/hr) while all had normal CRP. Albumin was low in two (17g/L and 29g/L). Following gastroenterology review and endoscopy, two cases were diagnosed with Crohn’s disease and two with ulcerative colitis. Conclusion It remains unclear whether etanercept plays a role in the development of IBD in these patients, whether it unmasks subclinical disease, or whether there is no association and it is simply a coincidence. However, there continue to be case reports and series suggesting an association between the two. We hope to identify further cases similar to these from sources such as the UK Biologics Registry and via surveys from teams at paediatric rheumatology centres throughout the UK. Further to the identification of cases, a retrospective review of patient characteristics will be carried out. This may allow for further determination of identifiable characteristics for these patients, and therefore suggest a subgroup of JIA patients with a higher risk of developing IBD, in which case caution when considering etanercept treatment would be advised. Conflicts of Interest The authors declare no conflicts of interest.

scholarly journals Temporal association between SARS-CoV-2 and new-onset myasthenia gravis: is it causal or coincidental?

2021 ◽  
Vol 14 (7) ◽  
pp. e244146
Author(s):  
Y Muralidhar Reddy ◽  
Santhosh Kumar B ◽  
Syed Osman ◽  
Jagarlapudi M K Murthy
Keyword(s):  
Myasthenia Gravis ◽  
Case Reports ◽  
Male Gender ◽  
Temporal Association ◽  
Time Interval ◽  
Myasthenic Crisis ◽  
Ocular Symptoms ◽  
Antiacetylcholine Receptor Antibody ◽  
The One ◽  
New Onset

Several case reports of COVID-19 in patients with myasthenia gravis (MG) have been documented. However, new-onset autoimmune MG following COVID-19 has been reported very rarely. We report one such case here. A 65-year-old man presented to us with dysphagia 6 weeks following mild COVID-19. He was evaluated and diagnosed as antiacetylcholine receptor antibody (AchR) positive, non-thymomatous, generalised MG. He subsequently developed myasthenic crisis and improved after treatment with intravenous immunoglobulin, prednisolone and pyridostigmine. Systematic literature review showed eight more similar cases. Analysis of all cases including the one reported here showed these features: mean age 55.8 years, male gender (5), time interval between COVID-19 and MG (5–56 days), generalised (5), bulbar and/or ocular symptoms (4), anti-AchR antibodies (7) and antimuscle-specific kinase antibodies (2). All have improved with immunotherapy. Although, many hypothesis are proposed to explain causal relationship between the two, it could as well be sheer coincidence.

scholarly journals Ocular Rosacea: Treatment with Rifaximin, a Non-Systemic Antibiotic

2016 ◽  
Vol 1 (1) ◽  
Keyword(s):  
Gastrointestinal Symptoms ◽  
Bacterial Overgrowth ◽  
Small Intestinal Bacterial Overgrowth ◽  
Open Label ◽  
Dry Eyes ◽  
Corneal Ulcers ◽  
Ocular Manifestations ◽  
Mild Improvement ◽  
Ocular Rosacea ◽  
Eye Symptoms

Background: Rosacea is associated with Crohn’s disease, liver disease, chronic pancreatitis, achlorhydria, Helicobacter pylori and recently with idiopathic small intestinal bacterial overgrowth (SIBO). Two publications demonstrated that rifaximin, a non-absorbed, gut-directed antibiotic for SIBO led to improvement in facial rosacea. Ocular manifestations occur in up to 58% of rosacea patients and include dry eyes, foreign body sensation, photosensitivity, eyelid inflammation, neovascularization and corneal ulcers. Methods: Patients who had been diagnosed with ocular rosacea by four ophthalmologists were referred for SIBO testing using the lactulose breath test (LBT). All were refractory to rosacea therapy. An open-label, IRBapproved trial of rifaximin 550 mg/3x/day for 10-14 days was performed in LBT-positive subjects. Ten and twenty days after ending rifaximin, subjects were queried if their eye symptoms had marked, moderate or mild improvement or if they were unchanged. Results: Twenty four patients (21F/3M), mean age 59 with facial involvement in 4 were tested. The LBT was positive in 9/24 (38%). LBT-positive subjects had chronic gastrointestinal symptoms in 63% vs., 33% in LBTnegative subjects. Rifaximin was prescribed to 9 LBT-positive subjects. Insurance denied the prescription in one subject. One subject was lost was to follow up. Improvement in ocular rosacea symptoms was marked (4), moderate (1) and mild (2). Conclusions: Rifaximin therapy led to improvement in ocular rosacea in the setting of SIBO. Dysregulation of the innate immune system as a result of gastrointestinal inflammation could lead to an increase in systemic cytokines and microbial antigens/antibodies in the skin and eyelids leading to activation of rosacea.

scholarly journals The Lambert-Eaton Myasthenic Syndrome — an Overview

2018 ◽  
Vol 02 (01) ◽  
pp. E40-E45
Author(s):  
Siegfried Kohler ◽  
Andreas Meisel
Keyword(s):  
Myasthenia Gravis ◽  
Case Reports ◽  
Compound Muscle Action Potential ◽  
Small Cell Lung Carcinoma ◽  
Immunosuppressive Treatment ◽  
Myasthenic Crisis ◽  
Cell Lung Carcinoma ◽  
Myasthenic Syndrome ◽  
Exercise Induced

AbstractThe Lambert Eaton myasthenic syndrome (LEMS) has a prevalence of around 5/100 0000 and is around 10–20 times rarer than myasthenia gravis (MG). Although LEMS does have a number of similarities to MG, there are important differences. The syndrome is characterized by a mostly proximally localised exercise induced muscle weakness that can lead to respiratory failure often accompanied by autonomous dysfunction. Disease symptoms are caused by autoantibodies directed against P/Q type voltage gated calcium channels (VGCC) that are expressed in the presynaptic motoric nerve terminals. The diagnosis of LEMS is based on the detection of the pathogenic anti-VGCC antibodies as well as the observation of an increment of at least 60% in the electrophysiological examination of an affected muscle. An increment is defined by an increase of the at rest reduced compound muscle action potential (CMAP) either after voluntary maximal innervation or after high frequent (≥20 Hz) stimulation. In almost one third LEMS is of paraneoplastic origin. Therefore an intensive tumor screening is necessary after diagnosis.There are some differences in the clinical presentation between paraneoplastic (pLEMS) and the exclusively autoimmune (aiLEMS) form of LEMS. With respect to this the DELTA-P-Score and the detection of SOX1-antibody are important. The most frequent tumor associated with LEMS is small cell lung carcinoma (SCLC). Therapy is based on the initial distinction between paraneoplastic and autoimmune ethiology. pLEMS necessitates therapy of underlying neoplasia. Usually, aiLEMS- as well as pLEMS patients respond well to 3,4 diaminopyridine (3,4 DAP) often augmented by pyridostigmine. Similar to treatment of myasthenia gravis long-term immunosuppressive treatment is usually required to control symptoms effectively. Myasthenic crisis in LEMS can be controlled by intensive care and immunoglobulins, plasmaphereses or immunoadsorption. Based on case reports more specific immunomodulatory treatment approaches such as the B-cell depleting therapeutic antibody rituximab should be considered in therapy refractory courses of LEMS. Long-term prognosis of autoimmune LEMS with respect to clinical stabilization with (pharmacological) remission is good, although in around 75% of patients significant reductions in quality of life remain. Prognosis of tumor-associated LEMS is largely determined by the tumor and its effective therapy. Curative treatment of the tumour as well as complete remission of pLEMS are possible.

scholarly journals Takotsubo syndrome in patients with myasthenia gravis: a systematic review of previously reported cases

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Devarajan Rathish ◽  
Minuri Karalliyadda
Keyword(s):  
Systematic Review ◽  
Myasthenia Gravis ◽  
Case Reports ◽  
Rare Condition ◽  
Myasthenic Crisis ◽  
Takotsubo Syndrome ◽  
Clinical Presentations ◽  
Life Threatening ◽  
Care Guidelines ◽  
The Common

Abstract Background Myasthenia gravis associated takotsubo syndrome is a rare condition. This study aimed to explore its typical presentation, investigations and treatment through a systematic review of previously reported cases. Methods Databases and reference lists of the selected articles were searched for case reports on Myasthenia gravis associated takotsubo syndrome. CARE guidelines were used for the quality assessment of the selected articles. Results Sixteen cases were selected out of 580 search results. Western Pacific, American and European regions contributed to 88% of the cases. Females were most affected (81%). Features of both myasthenia gravis and takotsubo syndrome were the common clinical presentations. All cases had a myasthenic crisis. Half of the cases had no prior diagnosis of myasthenia gravis. Pyridostigmine and prednisolone were useful for myasthenia gravis while dobutamine was most commonly used for takotsubo syndrome. All cases survived except four (25%). Conclusions Myasthenia gravis associated takotsubo syndrome via a myasthenic crisis is rare but life-threatening. Therefore, predisposition due to emotional and physical triggers needs to be avoided for its prevention. The rare entity should be suspected even in patients without a prior diagnosis of Myasthenia gravis.

scholarly journals Absence of Association between Previous Mycoplasma pneumoniae Infection and Subsequent Myasthenia Gravis: A Nationwide Population-Based Matched Cohort Study

2021 ◽  
Vol 18 (14) ◽  
pp. 7677
Author(s):  
Kuan Chen ◽  
James Cheng-Chung Wei ◽  
Hei-Tung Yip ◽  
Mei-Chia Chou ◽  
Renin Chang
Keyword(s):  
Cohort Study ◽  
Myasthenia Gravis ◽  
Mycoplasma Pneumoniae ◽  
Pathogenic Bacteria ◽  
Case Reports ◽  
Statistical Significance ◽  
Subsequent Development ◽  
Population Based ◽  
Cox Proportional Hazards ◽  
The Impact

Mycoplasma pneumoniae (M. pneumoniae) is not only one of the most common pathogenic bacteria for respiratory infection but also a trigger for many autoimmune diseases. Its infection process shared many similarities with the pathogenesis of myasthenia gravis (MG) at cellular and cytokine levels. Recent case reports demonstrated patients present with MG after M. pneumoniae infection. However, no epidemiological studies ever looked into the association between the two. Our study aimed to investigate the relationship between M. pneumoniae infection and subsequent development of MG. In this population-based retrospective cohort study, the risk of MG was analyzed in patients who were newly diagnosed with M. pneumoniae infection between 2000 and 2013. A total of 2428 M. pneumoniae patients were included and matched with the non-M. pneumoniae control cohort at a 1:4 ratio by age, sex, and index date. Cox proportional hazards regression analysis was applied to analyze the risk of MG development after adjusting for sex, age, and comorbidities, with hazard ratios and 95% confidence intervals. The incidence rates of MG in the non-M. pneumoniae and M. pneumoniae cohorts were 0.96 and 1.97 per 10,000 person-years, respectively. Another case–control study of patients with MG (n = 515) was conducted to analyze the impact of M. pneumoniae on MG occurrence as a sensitivity analysis. The analysis yielded consistent absence of a link between M. pneumoniae and MG. Although previous studies have reported that M. pneumoniae infection and MG may share associated immunologic pathways, we found no statistical significance between M. pneumoniae infection and subsequent development of MG in this study.

scholarly journals Impact of 2′-Fucosyllactose on Gut Microbiota Composition in Adults with Chronic Gastrointestinal Conditions: Batch Culture Fermentation Model and Pilot Clinical Trial Findings

Nutrients ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 938
Author(s):  
Jennifer Joan Ryan ◽  
Andrea Monteagudo-Mera ◽  
Nikhat Contractor ◽  
Glenn R. Gibson
Keyword(s):  
Ulcerative Colitis ◽  
Batch Culture ◽  
Pilot Trial ◽  
Gastrointestinal Symptoms ◽  
Short Chain Fatty Acids ◽  
Open Label ◽  
Gastrointestinal Conditions ◽  
Fermentation Model ◽  
Quality Of Life Index

Intestinal dysbiosis has been described in patients with certain gastrointestinal conditions including irritable bowel syndrome (IBS) and ulcerative colitis. 2′-fucosyllactose (2′-FL), a prebiotic human milk oligosaccharide, is considered bifidogenic and butyrogenic. To assess prebiotic effects of 2′-FL, alone or in combination with probiotic strains (potential synbiotics), in vitro experiments were conducted on stool from healthy, IBS, and ulcerative colitis adult donors. In anaerobic batch culture fermenters, Bifidobacterium and Eubacterium rectale-Clostridium coccoides counts, and short-chain fatty acids (SCFAs) including butyrate increased during fermentation with 2′-FL and some of the 2′-FL/probiotic combinations. In a subsequent open-label pilot trial, the effect of a 2′-FL-containing nutritional formula was evaluated in twelve adults with IBS or ulcerative colitis. Gastrointestinal Quality of Life Index (GIQLI) total and gastrointestinal symptoms domain scores, stool counts of Bifidobacterium and Faecalibacterium prausnitzii, and stool SCFAs including butyrate, increased after six weeks of intervention. Consistent with documented effects of 2′-FL, the batch culture fermentation experiments demonstrated bifidogenic and butyrogenic effects of 2′-FL during fermentation with human stool samples. Consumption of the 2′-FL-containing nutritional formula by adults with IBS or ulcerative colitis was associated with improvements in intra- and extra-intestinal symptoms, and bifidogenic and butyrogenic effects.

scholarly journals Composite phaeochromocytomas—a systematic review of published literature

2021 ◽  
Author(s):  
K. Dhanasekar ◽  
V. Visakan ◽  
F. Tahir ◽  
S. P. Balasubramanian
Keyword(s):  
Case Reports ◽  
Descriptive Analysis ◽  
Management Strategies ◽  
Gastrointestinal Symptoms ◽  
Rare Condition ◽  
Epidemiological Data ◽  
Human Case ◽  
Rare Tumour ◽  
Palpable Mass

Abstract Introduction Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre’s records and presents a systematic literature review of composite phaeochromocytomas. Methods In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist. Results There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4–86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5–168) months. Conclusion Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.

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