Receptor Antibody Titers Research Articles

The Role of Clinicopathological Factors on the TSH Receptor Antibody (TRAb) Titers in Patients with Thyroid Papillary Cancer and Associated Subclinical Hyperthyroidism

The Role of Clinicopathological Factors on the TSH Receptor Antibody (TRAb) Titers in Patients with Thyroid Papillary Cancer and Associated Subclinical Hyperthyroidism

Changes in antibody titers and clinical course in myasthenia gravis retrospective study

Myasthenia gravis (MG) is a rare, long‐term autoimmune disorder that causes muscle weakness; the condition typically has phases when it remits and phases when it relapses. Even though treatment response with regards to immunosuppressive therapies in MG patients is classically monitored by clinical scores, identification of a serological biomarker that monitors treatment response could be helpful. Here, the authors explore the correlation between acetylcholine receptor (AChR) antibody titers with changes in clinical severity in patients with MG and discuss the implications for clinical practice.

Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease

Autoantibodies to the insulin receptor are rare and typically cause severe insulin resistance and hyperglycemia, a condition termed type B insulin resistance. Uncommonly, antibodies to the insulin receptor can cause hypoglycemia. We present the case of a woman who developed recurrent severe hypoglycemia and myopathy, was found to have insulin receptor autoantibodies and mixed connective tissue disease, and had resolution of hypoglycemia with immunosuppression. A 55-year-old woman with a history of obesity, hypertension, and prior hemorrhagic stroke presented with recurrent severe hypoglycemia. A diagnostic fast resulted in hypoinsulinemic hypoketotic hypoglycemia. Adrenal function was intact. Progressive myopathy had developed simultaneously with her hypoglycemia, and rheumatologic evaluation revealed mixed connective tissue disease. The plasma acylcarnitine profile was normal, extensive oncologic evaluation including insulin-like growth factor 2 measurement was unrevealing, and anti-insulin antibody testing was negative. Ultimately, anti-insulin receptor antibodies were found to be present. The patient was treated with glucocorticoids and rituximab. Eight weeks after initiation of immunosuppression, the insulin receptor antibody titer had decreased and hypoglycemia had resolved. Eight months after diagnosis, the patient remained free of severe hypoglycemia despite tapering of glucocorticoids to a near-physiologic dose. Though antibodies to the insulin receptor typically cause severe insulin resistance, this patient had no evidence of insulin resistance and instead presented with recurrent severe hypoglycemia, which responded to glucocorticoids and rituximab. The diagnosis of insulin receptor antibody–mediated hypoglycemia is rare but should be considered in patients with systemic autoimmune disease, including mixed connective tissue disease, in the appropriate clinical context.

Feline acquired thymoma‐associated myasthenia gravis managed with surgery and therapeutic plasma exchange

AbstractA 3‐year‐old cat with thymoma‐associated myasthenia gravis underwent thymectomy. Skeletal muscle weakness deteriorated after surgery, and the cat became non‐ambulatory tetraparetic. A higher dose of pyridostigmine failed to improve the weakness. Therapeutic plasma exchange was performed twice, 4 and 8 weeks after surgery, with continued pyridostigmine treatment. Improvement in clinical signs was noted after each therapeutic plasma exchange. Improvement was short‐lasting after the first and long‐lasting after the second session. The dose of pyridostigmine was reduced 4 and 21 months after thymectomy without relapse. Thirty‐two months after thymectomy, the cat remained in clinical remission with continued pyridostigmine treatment, but serum anti‐acetylcholine receptor antibody titre remained elevated (2.0 nmol/L). This is the first report of therapeutic plasma exchange in a cat with acquired thymoma‐associated myasthenia gravis. Therapeutic plasma exchange may be considered an additional immune‐modulating option to medical treatment in cats with acquired myasthenia gravis.

Relationship between Low and High Anti-acetylcholine Receptor Antibody Titers and Clinical Severity in Myasthenia Gravis.

To determine the frequency of low and high anti-AChR (acetylcholine receptor) antibody titers and to evaluate their relationship with clinical severity in myasthenia gravis. Cross-sectional, observational study. Department of Neurology, King Edward Medical University/Mayo Hospital, Lahore from April 2017 to March 2018. Fifty-six seropositive patients, aged between 18-75 years, were included. A blood sample was obtained from each patient to assess for the anti-AChR antibody titers by enzyme-linked immunosorbent assay (ELISA) technique and classified as low (0.4-<50 nmol/L) and high AChR antibody titers (>50 nmol/L). Clinical severity was graded according to the Osserman's classification. Out of 56 patients, 51.79% (n=29) were males and 48.21% (n=27) were females, and mean age was 32.73 +8.48 years. Mean anti-AChR antibody titer was found 40.45 + 13.54; 60.71% (n=34) had low and 39.29% (n=22) had high titers. Upon grading the severity, 1.79% (n=1) had grade I, 25% (n=14) had grade IIa, 26.79% (n=15) had grade IIb, 37.5% (n=21) had grade III, and 8.93% (n=5) had grade IV. These grades were significantly associated with high/low titers of anti-AChR antibody (p<0.001) but no significant association was found with age and gender (p=0.39 and 0.19 respectively). Serum concentration of anti-AChR antibodies has significant association with the clinical severity in myasthenia gravis. Key Words: Anti-acetylcholine receptor antibody, Myasthenia gravis, Neuromuscular junction diseases.

The clinical utility of neostigmine administration in the diagnosis of acquired myasthenia gravis.

To assess the clinical utility of neostigmine methylsulfate administration in the diagnosis of suspected acquired myasthenia gravis (MG) in dogs and cats. Retrospective study (2017-2019). Five university teaching hospitals and 2 private referral hospitals. Twenty-two dogs and 3 cats. Criteria for inclusion were clinical signs consistent with acquired MG, performance of a neostigmine challenge and acetylcholine receptor antibody titers. None. The route of neostigmine administration was recorded. Response to neostigmine challenge was determined via sequential evaluation of muscle strength and ambulation following administration of neostigmine methylsulfate. Response to neostigmine challenge was compared to acetylcholine receptor antibody titers, which were used as the biochemical gold standard in this study. Sixteen out of 22 dogs were diagnosed with acquired MG. Thirteen of 16 had a strong positive response to neostigmine challenge whereas 3 of 16 had no response. Two out of 3 dogs with polymyositis also had a strong positive response to neostigmine challenge. Weak positive results were seen with intracranial neoplasia (n=1) and a dog with dilated cardiomyopathy and coxofemoral joint disease (n=1). One cat was diagnosed with acquired MG and had a positive response to neostigmine challenge. Two cats had no response to neostigmine challenge and were diagnosed with alternate conditions. Two cats were premedicated with glycopyrrolate, one of which had a mild adverse response to neostigmine challenge (sialorrhea and mild transient tremors). Three out of 22 dogs had minimal adverse effects (sialorrhea and 1 dog with muscle tremors). The neostigmine challenge appears to be safe and viable alternative to the previously utilized edrophonium challenge, particularly when weak positive responses are considered negative for acquired MG. Polymyositis cases may have a false positive response to neostigmine challenge.

Anti-PLA2R1 Antibodies as Prognostic Biomarker in Membranous Nephropathy

IntroductionPersonalized treatment for patients with membranous nephropathy requires accurate prediction of the disease course at an early stage. In this study, we evaluated the value of baseline anti–phospholipase A2 receptor (PLA2R1) antibody titer as a prognostic biomarker in patients with PLA2R1-associated membranous nephropathy.MethodsIn this cohort study, we included 168 patients (118 men, 50 women) referred to our nephrology center between February 1995 and November 2016. Mean age was 52 ± 13 years. There were 156 patients with new-onset disease and 12 patients with a relapse (n = 10) or recent use of immunosuppressive therapy (n = 2). We measured anti-PLA2R1 titer at baseline and analyzed progression to severe disease (30% increase of serum creatinine or start of immunosuppressive therapy) as a primary study endpoint over 60 months.ResultsThere was a clear association between anti-PLA2R1 antibody titer and severity of the nephrotic syndrome. In univariate analysis, anti-PLA2R1 antibody titer was also associated with disease progression. However, in Cox proportional hazard models that included proteinuria and serum creatinine, anti-PLA2R1 antibody titer was no longer associated with clinical outcome. Results were similar when limiting the analysis to the patients with new-onset disease.ConclusionOur study questions the relevance of single measurement of anti-PLA2R1 antibodies at baseline as a prognostic biomarker in membranous nephropathy. Future studies are needed to determine the possible role of sequential measurements of anti-PLA2R1 antibodies as a prognostic biomarker of disease progression.

Association of Other Autoimmune Diseases With Thyroid Eye Disease.

BackgroundThyroid eye disease (TED) is a potentially disfiguring and sight-threatening autoimmune (AI) orbitopathy, affecting up to 400,000 people in the UK. There are no accurate early predictors of TED severity. Although polyautoimmunity has been shown to affect AI disease severity, its influence on TED severity has never been investigated. The prevalence of polyautoimmunity among TED patients is also unclear, with discordant results reported in the literature. This study evaluates the prevalence of non-thyroid/“other” AI (OAI) conditions in an ethnically diverse TED cohort and assesses how polyautoimmunity affects TED severity and activity.MethodsA retrospective study of patients presenting to multidisciplinary TED clinics across three North-West London hospitals between 2011 and 2019. Data collected included: 1) demographics; 2) OAI conditions and management; 3) endocrine management of thyroid dysfunction; 4) details of TED and clinical activity score at presentation.ResultsTwo hundred and sixty-seven patients with a median age of 46 (35–54) years were included, 79.4% were female and 55% were Black, Asian and minority ethnic (BAME). Thirty-seven patients (13.9%) had OAI conditions, with rheumatoid arthritis (3.7%), vitiligo (3.0%) and psoriasis (3.0%) among the most prevalent. Of patients with OAI conditions, 43.2% (16/37) required immunosuppression prior to TED onset. Non-immunosuppressed patients with OAI conditions had a significantly higher clinical activity score at presentation than TED-only and previously immunosuppressed patients (p=0.02). No significant differences were observed in thyroid receptor antibody titers between these groups.ConclusionsThis study finds a 13.9% prevalence of OAI conditions among TED patients. Patients with OAI conditions overall have a tendency for more severe and significantly more clinically active TED than those without OAI conditions. Larger, prospective studies are warranted to further evaluate polyautoimmunity as an early predictor of TED severity.

Risk factors for developing post-thymectomy myasthenia gravis in patients with thymoma.

Thymectomy is required for the treatment of thymoma-associated myasthenia gravis (MG). However, MG may develop only after thymectomy, a condition known as post-thymectomy MG. This study aimed to investigate the risk factors for post-thymectomy MG in patients with thymoma. We retrospectively identified 235 patients with thymoma who underwent thymectomy at a single hospital from January 2008 to December 2017: 44 with preoperatively diagnosed MG were excluded, leaving 191 patients in the final analysis. Univariable survival analyses using Cox proportional hazards regression model and Kaplan-Meier estimate were conducted to identify risk factors for post-thymectomy MG. Post-thymectomy MG developed in 4.2% (8/191) of the patients with thymoma between 18 days and 108 mo after surgery. Hazard ratios (HRs) of pre- and postoperative anti-acetylcholine receptor antibody (AChR-Ab) titers were 2.267 (P = .002) and 1.506 (P < .001), respectively. Patients with extended thymectomy had a low chance of post-thymectomy MG (HR 0.035, P = .007). Larger thymoma (HR, 1.359; P = .005) and type A or AB thymoma according to World Health Organization histological classification (HR, 11.92; P = .021) were associated with higher chances of post-thymectomy MG. Within the subgroup of preoperatively AChR-Ab seropositive patients, post-thymectomy MG developed in 22.2% (6/27). Pre- and postoperative AChR-Ab levels should be measured in patients with thymoma. A large thymoma and partial thymectomy appear to be associated with a higher probability of post-thymectomy MG.

Serum level of soluble urokinase plasminogen activator receptor (suPAR) as a disease severity marker of myasthenia gravis: a pilot study.

Myasthenia gravis (MG) is an autoantibody-mediated inflammatory disease of the neuromuscular junction. Biomarkers indicating disease activity in MG are warranted. Recently, the soluble urokinase plasminogen activator receptor (suPAR) has been reported to be associated with inflammation, tissue damage, disease activity and prognosis in various diseases, including autoimmune diseases. In this study, serum suPAR levels were measured in 40patients with anti-acetylcholine receptor antibody-positive MG and 30controls, and their correlations with clinical variables and severity scale scores were investigated. We identified that serum suPAR levels significantly correlated with MG activities of daily living scale (Spearman's ρ=0·45; P=0·004) and MG Foundation of America classification (Spearman's ρ=0·37; P=0·02) at serum sampling, but not with anti-acetylcholine receptor antibody titers. In conclusion, serum suPAR levels can be a candidate for a novel biomarker of disease activity in anti-acetylcholine receptor antibody-positive MG.

Radioiodine Therapy-Induced Conversion of Toxic Adenoma to Graves’ Disease

We present a 50-year-old female who was evaluated for the symptoms of thyrotoxicosis. She had low thyroid stimulating hormone (TSH) 0.02 with normal free thyroxine (FT4) 1.00 (0.61-1.76 ng/dL) and normal total triiodothyronine (TT3) 1.0 (0.60-2.20 ng/mL) levels. Her thyrotropin receptor antibody (TRAbs) and thyroid peroxidase antibody (TPOAb) titers were negative. Thyroid ultrasound revealed an ill-defined, heterogeneous, 1.8 cm x 0.8 cm x 0.7 cm nodule in the left lower lobe. 123-radioiodine (RAI) thyroid scan revealed 38.5% uptake, which was concentrated in the lower left thyroid lobe, a finding consistent with a solitary toxic adenoma of the thyroid.The patient became clinically and biochemically euthyroid on methimazole (MMI). She then underwent 131-RAI therapy with 12 mCi, which cured her hyperthyroidism with normalization of TSH levels for four months. She then developed overt thyrotoxicosis with low TSH of 0.02, elevated TT3 of 3.2, and normal FT4 of 0.91. Repeat TRAbs and TPOAb were elevated along with diffusely increased uptake on the I-123 RAI thyroid uptake scan, consistent with Graves’ disease (GD). The patient was then placed on MMI again to bridge to definitive treatment with total thyroidectomy. Our case is a rare case where the patient with solitary toxic adenoma with negative TPOAb serology developed GD following I-131 RAI treatment.

抗构象表位桥粒芯蛋白抗体和乙酰胆碱受体抗体滴度与寻常型天疱疮病情活动度的相关性研究

抗构象表位桥粒芯蛋白抗体和乙酰胆碱受体抗体滴度与寻常型天疱疮病情活动度的相关性研究

Pharmacodynamic Response to Anti-thyroid Drugs in Graves' Hyperthyroidism.

Objective: Graves' disease is the commonest cause of hyperthyroidism in populations with sufficient dietary iodine intake. Anti-thyroid drugs (ATD) are often used as the initial treatment for Graves' hyperthyroidism, however there is a paucity of data relating the dose of ATD therapy to the effect on thyroid hormone levels, increasing the risk of both over- and under-treatment. We aimed to determine the pharmacodynamic response to the ATD carbimazole.Design: Retrospective cohort study.Methods: Participants were patients (n = 441) diagnosed with Graves' disease at Imperial College Healthcare NHS Trust between 2009 and 2018. The main outcome measure was change in thyroid hormone levels in response to ATD.Results: Baseline thyroid hormone levels were positively associated with TSH receptor antibody titres (P < 0.0001). Baseline free triiodothyronine (fT3) were linearly related to free thyroxine (fT4) levels in the hyperthyroid state (fT3 = fT4*0.97–11), and fell proportionately with carbimazole. The percentage falls in fT4 and fT3 per day were associated with carbimazole dose (P < 0.0001). The magnitude of fall in thyroid hormones after the same dose of carbimazole was lower during follow up than at the initiation visit. The fall in thyroid hormone levels approximated to a linear response if assessed at least 3 weeks after commencement of carbimazole. Following withdrawal of antithyroid drug treatment, the risk of relapse was greater in patients with higher initial fT4, initial TSH receptor antibody titre, males, smokers, and British Caucasian ethnicity.Conclusion: We identify a dose-response relationship for fall in thyroid hormones in response to carbimazole to aid in the selection of dose for Graves' hyperthyroidism.

Subxiphoid completion thymectomy for refractory non-thymomatous myasthenia gravis.

BackgroundCompletion thymectomy may be performed in patients with non-thymomatous refractory myasthenia gravis (MG) to allow a complete and definitive clearance from residual thymic tissue located in the mediastinum or in lower neck. Hereby we present our short- and long-term results of completion thymectomy using subxiphoid video-assisted thoracoscopy.MethodsBetween July 2010 and December 2017, 15 consecutive patients with refractory non-thymomatous myasthenia, 8 women and 7 men with a median age of 44 [interquartile range (IQR) 38.5–53.5] years, underwent video-thoracoscopic completion thymectomy through a subxiphoid approach.ResultsPositron emission tomography (PET) showed mildly avid areas [standardized uptake value (SUV) more than or equal to 1.8] in 11 instances. Median operative time was 106 (IQR, 77–141) minutes. No operative deaths nor major morbidity occurred. Mean 1-day postoperative Visual Analogue Scale value was 2.53±0.63. Median hospital stay was 2 (IQR, 1–3.5) days. A significant decrease of the anti-acetylcholine receptor antibodies was observed after 1 month [median percentage changes −67% (IQR, −39% to −83%)]. Median follow-up was 45 (IQR, 21–58) months. At the most recent follow-up complete stable remission was achieved in 5 patients. Another 9 patients had significant improvement in bulbar and limb function, requiring lower doses of corticosteroids and anticholinesterase drugs. Only one patient remained clinically stable albeit drug doses were reduced. One-month postoperative drop of anti-acetylcholine receptor antibodies was significantly correlated with complete stable remission (P=0.002).ConclusionsThis initial experience confirms that removal of ectopic and residual thymus through a subxiphoid approach can reduce anti-acetylcholine receptor antibody titer correlating to good outcome of refractory MG.

SAT-398 LOW DOSE OF RITUXIMAB IS CLINICALLY AND COST EFFECTIVE IN PRIMARY MEMBRANOUS NEPHROPATHY

B cells are thought to be involved in the pathogenesis of Primary Membranous nephropathy(IMN) and therapies targeting B cells may have a role. Though cyclophosphamide based therapies were traditionally used, targeting B cells with Rituximab and thereby depleting antibody production has been suggested as an alternative and possibly more effective therapy1. Most of the studies have extrapolated the dose used in treating B cell lymphoproliferative diseases in a four weekly dose of 375mg/m2. There have even been suggestions that mega doses may be more effective by preventing epitope spreading2. At the same time, clinical efficacy with a single dose has also been reported3. We studied the role of a single low dose of 100mg Rituximab in decreasing CD19 B cells, proteinuria, renal functions and antiphospholipid 2 receptor antibody titres (Anti PLA2R) in IMN. A single centre, prospective observational study was conducted at a tertiary care centre in South India over a six month period. Biopsy proven cases of IMN with proteinuria >8g/day and eGFR >30ml/min/m2 who did not receive previous therapy with cyclophosphamide or calcineurin inhibitors received Inj. Rituximab 100 mg as monotherapy after informed consent along with Angiotensin 11 receptor blockers, statins and diuretics. Dose of antihypertensive drugs were targeted to keep blood pressure <140/90 mmHg. CD 19 counts were measured at baseline, at 4 weeks,12 weeks and 24weeks following Rituximab. Anti PLA2R titres were also measured at baseline and if elevated, periodically. Other baseline parameters like serum albumin, total cholesterol and proteinuria were also recorded at baseline and at intervals mentioned above. Five patients including 3 males with a mean age of 55+/-2 years were studied. Mean serum creatinine at baseline was 1.3+/-0.6 mg/dl. Baseline 24 hour proteinuria was 10.2+/-1.8g.Four patients were PLA2R negative and one was positive. Following 100mg of rituximab, CD19 counts were suppressed in all patients at 1 month. Four patients attained partial remission by 3 months and were in complete remission by 6months. One patient had transient decrease in proteinuria and Anti PLA2R titres but relapsed by 2nd month and thereafter failed to respond. At the end of 24 weeks significant reduction in proteinuria along with normal serum albumin and total cholesterol were observed in 4 patients. Among those who responded, proteinuria at the end of 24 weeks came down to an average of 1.08+/-0.5g/day(p<0.005). There were a limited number of minor side-effects. Though initial cluster of differentiation 19 (CD19)+ B cell were depleted in all patients at 4 weeks(count <1%), repopulation of CD19+ B cells occurred in 4 patients by 3 months. Clinical remission could be achieved at the end of 24 weeks in 4 of our patients. Lack of response in one patient despite initial fall in PLA2R titres and CD19 count could be explained due to PLA2R epitope spreading. A single dose of 100mg Rituximab was sufficient to suppress the CD 19 count to less than 1% at 4 weeks. Targetting further doses depending on whether repopulation of CD19 Bcells occur and Anti PLA2R titres become elevated could limit the cumulative dose of rituximab, be cost effective and decrease toxicity. These observations may need to be confirmed by larger, preferably multicentre trials.

Factors associated with severity of orbitopathy in patients with Graves' disease.

PURPOSE:Graves' orbitopathy (GO) is one of the most frequent orbital disorders and is the most common cause of proptosis in adults. GO is described as chronic inflammation of orbital and periorbital tissues. This study aimed to evaluate the risk factors for severe GO in patients with Graves' disease (GD).MATERIALS AND METHODS:This was a prospective cross-sectional study done on 52 newly diagnosed patients of GD with GO documented by thyroid 99mTechnetium-pertechnetate scan at our center between May 2016 and May 2019. All patients underwent a comprehensive ophthalmological examination and laboratory and hormonal analysis. Clinical Activity Score (CAS) and severity were estimated as per the European Group on Graves Orbitopathy. Thirty-four patients with mild GO were compared with 18 patients with moderate-to-severe GO (severe) for baseline risk parameters.RESULTS:Majority of the patients had mild orbitopathy (34 [65.4%]) followed by moderate to severe (18 [34.6%]). CAS was active in 13.5% of the study group. There was a statistically significant male preponderance in severe GO. Current smoking increased the risk of severe GO (P = 0.003). Duration of GD symptoms at presentation was statistically significantly longer in severe GO patients than mild GO (P = 0.004). Thyrotropin receptor antibody (TRAb) titer significantly increased in severe GO group (6.2 ± 2.4 IU/L) when compared to mild GO (3.2 ± 1.6 IU/L) (P < 0.001). TRAb positivity was similar between groups. Braley's sign, i.e., the differential intraocular pressure (IOP) of >6 mmHg, was statistically significantly higher in severe GO (P < 0.001). Male gender, current smoking, TRAb >2 upper limit of normal (ULN), and differential IOP >6 mmHg were found to be associated with severe GO.CONCLUSION:Approximately 35% of the patients with GO have severe disease, with a higher risk in men. This study identified male gender, current smoking, TRAb >2 ULN, and differential IOP >6 mmHg to be associated with severe GO.

C3, C5a and anti-acetylcholine receptor antibody as severity biomarkers in myasthenia gravis.

Background:Although the pathogenesis of myasthenia gravis (MG) is well known, prognostic markers are not yet available. We assessed the utility of anti-acetylcholine receptor (AChR) antibody (AChR-ab) titer and concentration of C3, C4, and C5a as potential severity biomarkers in MG.Methods:Levels of C3, C4, C5a, and AChR-ab were measured in 60 AChR-ab-positive patients with MG. Their relationship with clinical severity was analyzed using the activities of daily living (ADL) and MG composite (MGC) scales.Results:AChR-ab titer correlated with severity of MG according to ADL (p = 0.002) and MGC scales (p = 0.001). When patients were classified according to disease duration, a statistically significant correlation between AChR-ab titer and clinical severity was only found in the subgroup of patients with fewer than 5 years from symptoms onset. C5a levels showed a positive correlation with MG severity according to the ADL scale (p = 0.041; τb = 0.18), although C5a levels were not different from the control group.Discussion:AChR-ab titers and C5a levels could potentially be considered markers of severity in patients with MG.

Disease Severity Assessment and Short-Term Outcome in Patients with Myasthenia Gravis.

Background:Myasthenia gravis (MG) is an autoimmune disorder with a chronic fluctuating course. The outcome measures encapsulate disease severity, functional impact at diagnosis, and objective evaluation of clinical benefit from therapeutic interventions.Aims and Objective:To assess the disease severity, correlation between various outcome measures, and to evaluate the short-term outcome at 3 months and 6 months in a cohort of MG patients.Materials and Methods:Quantitative myasthenia gravis (QMG) score, myasthenia gravis composite (MGC) score, and myasthenia gravis quality of life-15 (MG-QoL-15) score were applied to 54 patients at first visit, 3 months and 6 months follow-up.Results:Mean quality of life-15 (QoL-15) score at base line was 15.241. Mean QMG and MGC scores at baseline were 14.63 ± 8.37 and 15.87 ± 9.14, respectively. QMG score showed a strong positive correlation with both MGC and MG-QoL-15 scores. QMG and MGC scores showed a moderate correlation with acetylcholine receptor antibody (AChR Ab) titers. Mean QMG at follow-up was 9.95 ± 5.49 at 3 months and 6.74 ± 4.74 at 6 months. Mean MGC at follow-up was 10.75 ± 5.58 at 3 months and 6.51 ± 4.36 at 6 months.Conclusion:The combination of physician-evaluated and patient-reported outcome measures provided a more discerning picture of patient status and response to treatment. Incorporating MG outcome measures into clinical practice would aid in modulating therapies.

Risk Factors for the Exacerbation of Myasthenic Symptoms After Surgical Therapy for Myasthenia Gravis and Thymoma

Extended thymectomy is employed for patients with myasthenia gravis (MG) and/or thymoma with elevated serum antiacetylcholine receptor antibody (AchR) titers. However, MG symptoms occasionally worsen in post-thymectomy patients. We explored the risk factors for exacerbation of MG symptoms after surgical therapy for patients with MG and/or thymoma with an elevated AchR titer. We retrospectively analyzed 90 patients suffering from MG and/or thymoma with an elevated AchR titer who underwent thymectomy in our institute. Patients were classified into Improved, Unchanged, and Exacerbated groups by assessing their postoperative myasthenic symptoms, amount of medication, and incidence of myasthenic crisis. Risk factors for postoperative exacerbation of myasthenic symptoms were assessed by comparing the Exacerbated with the Improved and Unchanged groups. Of the 90 patients, 29 were classified into the Improved group, 47 into the Unchanged group, and 14 into the Exacerbated group. The presence of thymoma and Masaoka stage were significantly different between the Exacerbated and Improved/Unchanged groups. Although preoperative AchR titers did not significantly differ among the groups, the perioperative AchR titers in the Exacerbated group were significantly higher than those in the other groups (P = 0.003). A multiple logistic regression analysis with stepwise forward selection showed that advanced-stage thymoma was a risk factor for postoperative exacerbation of myasthenic symptoms (P = 0.007). Patients with advanced-stage thymoma have a relative risk for exacerbation of myasthenic symptoms after surgical therapy.

Surgical treatment of an oesophageal achalasia in a small breed dog

A 6-year-old, male Yorkshire Terrier dog presented with persistent regurgitation and severe weight loss. Based on the clinical signs, physical and other various diagnostic examinations, including fluoroscopy, were performed. Myasthenia gravis was ruled out through a serum acetylcholine receptor antibody titre measurement and a negative response to neostigmine bromide (0.02 mg/kg) within 4 hours of injection. The dog was diagnosed with idiopathic oesophageal achalasia. As a treatment, a modified Heller’s oesophagomyotomy was performed, and the dog recovered well with no signs of recurrence for 18 months.