Lower BAFF Levels in Myasthenic Patients Treated with Glucocorticoids.

B-cell activating factor (BAFF), a member of tumor necrosis factor family, activates B cells, promotes their survival and proliferation. BAFF is considered to have an influence on development of autoimmune diseases including myasthenia gravis (MG). We aimed to evaluate BAFF serum levels in MG patients, their potential connection with therapy and course of MG. Cross-sectional study. Two hundred eighteen adult patients with MG (67% women, age: 18-89 years, 82.6% AChR antibody seropositive (AChRAb(+)). Serum BAFF levels, their relationship with severity of clinical symptoms, therapy conducted, clinical and demographic features and other factors were analyzed. Patients with AChRAb(+) MG demonstrated significantly higher BAFF levels than MuSK-MG patients (831.2 ± 285.4 pg/ml vs. 745.6 ± 633.4 pg/ml, respectively; p = 0.030). Serum BAFF levels in women were significantly higher than in men (855.9 ± 302.5 vs. 756.6 ± 289.4, respectively; p = 0.017). Mean serum BAFF level was significantly decreased in patients who were ever treated with corticosteroids (CS) (770.4 ± 327.8 pg/ml vs. 891.3 ± 246.1 pg/ml, respectively; p = 0.001). Thymoma-MG patients demonstrated significantly lower BAFF levels (671.2 ± 244.9 vs. 833.5 ± 302.4, respectively; p = 0.044). Thymectomized patients did not differ in BAFF levels from the MG patients who had not undergone thymectomy. In multiple linear regression model, recent CS therapy and male sex were found to be independent predictors of lower BAFF levels. Serum BAFF level is decreased in patients treated with CS, which may suggest inhibiting influence of CS on BAFF-a potential mechanism contributing to the effectiveness of such therapy.

Determinants of Quality of Life in Myasthenia Gravis Patients.

Background: Although approximately half of myasthenia gravis (MG) patents achieve remission, for the remaining group MG is often a life-long disease. Better understanding of the determinants of Quality of Life (QoL) in MG is needed to optimize treatment goals in chronic cases. Materials and Methods: We performed a single center cross-sectional study in 339 MG adult patients (64.9% women), with ocular or generalized disease. SF-36 and a structured questionnaire was administered, including information on previous and current MG severity, medications, comorbidities, education, occupation and BMI of the patient. Mean disease duration was 7.5 + 9.3 years. Current age was 51.6 + 18.3 years, 55% had Early-Onset (<50 years) MG. Results: There were no statistically significant differences in mean SF-36 subscores between women and men. Worse MGFA class was related to lower QoL in physical (PCS) and mental (MCS) subscore (p = 0.000 for both). Patients with MGFA I-II class had significantly better QoL in physical and mental subscores than patients with more severe MG (p < 0.005). Late-onset MG patients had worse QoL than EOMG in physical score domain PCS (p = 0.049). Overweight and obese patients had lower PCS (p = 0.002) and MCS (p = 0.038) than patients with normal BMI. University education was related to statistically higher PCS (p = 0.015) and MCS (p = 0.006). QoL in currently employed was better in PCS and MCS (p = 0.000), with white collar workers reporting higher PCS (p = 0.049) than the remaining group. Patients living with family evaluated their MCS (p = 0.015) better than living alone. Moderate physical activity (twice a week) improved PCS (p = 0.045). Conclusion: Our study confirmed that greater severity of symptoms, age, age of onset but also BMI, type of work, education status and physical activity affect QoL in MG.

Serum interleukin 15 levels in patients with seropositive myasthenia gravis do not correlate with disease severity.

AIM: To assess interleukin 15 (IL-15) serum levels in patients with seropositive myasthenia gravis (MG); searching for potential relationship between IL-15 levels and clinical features such as gender, age at onset, clinical presentation or treatment received. BACKGROUND: IL-15 plays pivotal role in T-cell dependent autoimmunity. Increased IL-15 serum levels have been reported in several autoimmune diseases including MG patients from Japan. PATIENTS AND METHODS: Sera of 42 seropositive MG patients (66.7% women), mean age 50.6±23.7 years) have been tested by ELISA for IL-15 levels. RESULTS: There were no statistically significant differences between IL-15 serum levels in MG patients in comparison with controls as well as between subgroups of MG patients (early vs. late onset and thymoma MG). Mean/median IL-15 serum levels were similar in MG patients treated with corticosteroids (CS) and CS naïve. Outliers (very high values) were seen only in untreated generalized MG patients. CONCLUSIONS: Serum interleukin 15 levels in patients with seropositive myasthenia gravis do not correlate with disease severity.

Prevalence and impact of autoimmune thyroid disease on myasthenia gravis course.

OBJECTIVES: Autoimmune thyroid diseases (ATDs) frequently accompany myasthenia gravis (MG) and may influence its course. We aimed to determine the association and impact of ATD with early- (<50 years), late-onset MG, or thymoma-MG. MATERIALS AND METHODS: Prevalence of ATD was measured in a cross-sectional study of 343 consecutive patients with MG (236 F, 107 M) aged 4-89 years; 83.8% were seropositive, in 2.9%, anti-MuSK antibodies were detected. Concentrations of antithyroid peroxidase antibodies, antithyroglobulin antibodies, antithyrotropin receptor antibodies, and TSH level were measured in all patients. MG clinical course, treatment received, and treatment results were evaluated. RESULTS: Autoimmune thyroid diseases were diagnosed in 92 (26.8%) of MG patients including 4.4% with Graves (GD), 9% with Hashimoto thyroiditis (HT), and 13.4% with antithyroid antibodies only. GD patients had ocular symptoms more often than patients with antithyroid antibodies or HT (p = .008). ATD prevalence was comparable in MG with early and late onset, while non-ATDs were more frequent in thymoma-MG (p = .049). Immunosuppressive therapy was less frequently needed in the patients with MG and ATD, indirectly indicating milder MG course (p = .005). Risk of myasthenic crisis and the results of treatment did not differ between patients with and without ATD. CONCLUSIONS: Autoimmune thyroid diseases are frequently accompanied by early-and late-onset MG, while thymoma-MG is related to higher risk of non-ATD. Myasthenia coexisting with ATD follows milder course than MG alone.

[A case of the Lambert-Eaton syndrome of non-neoplastic origin. Ten-year follow-up]. / Przypadek zespolu Lamberta-Eatona pochodzenia nienowotworowego. Obserwacja 10-letnia.

A case is presented of a 34-year-old man who developed weakness of the proximal muscles of the extremities, particularly lower, slight myalgia, and vegetative symptoms (dryness in the mouth). Those symptoms progressed within a month. On examination weakness of the muscles of the extremities was found as well as weak tendon reflexes, slight atrophy of muscles of the arms and thighs and apokamnosis. Edrophonium test was slightly positive. Electrostimulation revealed changes typical of the Lambert-Eaton syndrome: low amplitude of the compound muscle action potential on single stimulus, decreasing amplitude of the subsequent responses to 3 Hz stimulation, marked facilitation on 30 Hz stimulation. Neoplastic etiology was excluded by chest X-ray and CT, as well as by bronchoscopy, abdominal and prostatic USG, and thyroid USG and scintigraphy. Antibodies to AChR were not found in the serum. The titre of the antibodies against voltage-gated calcium channels was highly positive which was decisive in the diagnosing of the Lambert-Eaton syndrome. The patient was treated with pyridostigmine, corticosteroids, cyclophosphamide and immunoglobulins. Ten years of follow-up have fully confirmed the diagnosis of a non-neoplastic Lambert-Eaton syndrome.

Transcatheter closure of patent foramen ovale in patients with cryptogenic stroke.

BACKGROUND: Paradoxical embolism due to the presence of patent foramen ovale (PFO) is a well-established possible mechanism of ischaemic stroke of unknown origin. Mechanical sealing of the interatrial septum seems to be the most effective method for the prevention of stroke recurrences. AIM: To assess prospectively the short- and mid-term results of transcatheter closure of PFO in consecutive patients with a history of cryptogenic ischaemic stroke. METHODS: Between March 1999 and December 2002, thirty two patients with PFO (15 males, age from 19 to 55 years, mean 41 years) with a history of documented ischaemic stroke of unknown origin underwent transcatheter closure of PFO using an Amplatzer occluder. All procedures were performed under general anaesthesia and with transesophageal echocardiographic guidance. RESULTS: In all patients the procedure was effective and no complications were observed. During the follow-up period of a mean of 25.9 months (>12 months in 22 patients), no new neurological events were recorded. Control transesophageal echocardiography was performed in 28 patients mean 22.3 months after the procedure and confirmed the correct positioning of the occluder. A significant (>30 bubbles of contrast) residual shunt was detected in two patients. One patient developed episodes of paroxysmal supraventricular tachycardia which were effectively cured by radiofrequency ablation. CONCLUSIONS: Transcatheter closure of PFO is safe, effective and devoid of side effects connected with extracorporeal circulation. This procedure may become the treatment of choice in patients with the highest risk of recurrent ischaemic stroke.