Diagnostic yields and clinical features of ocular myasthenia gravis.

ABSTRACT: To investigate clinical features and diagnosis process of ocular myasthenia gravis (OMG) in ophthalmology department.A total of 36 patients with ptosis or diplopia who had follow-up for at least 3 months between March 2016 and December 2019 were included in this study. Clinical symptoms of patients and the test results were analyzed. According to the positivity of serologic test, these patients were divided into 2 groups (confirmed OMG and possible OMG with relief of symptoms after antimyasthenic treatment) for comparison.Ptosis was present in 12 (33.33%) patients, diplopia was present in 14 (38.89%) patients, and both ptosis and diplopia were present in 10 (27.78%) patients. Acetylcholine receptor auto-antibody (AchR Ab) was positive in 14 (38.89%) of 36 patients and ice test was positive in 15 (71.43%) of 21 patients with ptosis. Unequivocal response to pyridostigmine was observed in 31 (86.11%) patients. For seropositive cases, AchR Ab titer was significantly higher in the group with 2 clinical symptoms than that in the 1 clinical symptom (P = .011).This study presents the usefulness and diagnostic validity of antimyasthenic treatment for OMG, especially seronegative OMG, with detailed symptom analysis.

Clinical relevance of thyroid-stimulating immunoglobulins in graves' ophthalmopathy.

PURPOSE: Thyroid-stimulating immunoglobulins (TSIs) likely mediate Graves' ophthalmopathy (GO). The clinical relevance of these functional autoantibodies was assessed in GO. DESIGN: Cross-sectional trial. PARTICIPANTS: A total of 108 untreated patients with GO. METHODS: Thyroid-stimulating immunoglobulins, assessed with a novel bioassay, bind to the thyrotropin receptor (TSHR) and transmit signals for cyclic adenosine monophosphate (cAMP)-dependent activation of luciferase gene expression. The cAMP/cAMP response element-binding protein/cAMP-regulatory element complex induces luciferase that is quantified after cell lysis. The TSI levels were correlated with activity and severity of GO and compared with a TSHR binding inhibitory immunoglobulin (TBII) assay. MAIN OUTCOME MEASURES: Thyroid-stimulating immunoglobulins, activity and severity of GO, diplopia, and TBII. RESULTS: Thyroid-stimulating immunoglobulins were detected in 106 of 108 patients (98%) with GO. All 53 hyperthyroid patients were TSI positive versus 47 patients (89%) who were TBII positive. All 69 patients with active GO were TSI positive, whereas only 58 of 69 patients (84%) were TBII positive. Thyroid-stimulating immunoglobulins correlated with the activity (r=0.83, P < 0.001) and severity (r=0.81, P < 0.001) of GO. All 59 patients with GO with diplopia were TSI positive, and 50 of 59 patients (85%) were TBII positive. Among patients with moderate-to-severe and mild GO, 75 of 75 (100%) and 31 of 33 (94%) were TSI positive compared with TBII positivity in 63 of 75 (84%) and 24 of 33 (73%), respectively. The TSI levels were higher in moderate-to-severe versus mild GO (489%±137% vs. 251%±100%, P < 0.001). Chemosis and GO activity predicted TSI levels alone (P < 0.001, multivariable analysis). The TSI levels were higher in patients with chemosis (527%±131%) than in patients without chemosis (313%±127%, P < 0.001). CONCLUSIONS: Thyroid-stimulating immunoglobulins show more significant association with clinical features of GO than TBII and may be regarded as functional biomarkers for GO. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Visual symptoms associated with the presence of a lupus anticoagulant.

The lupus anticoagulant is an acquired serum immunoglobulin that prolongs several coagulation parameters, most notably the partial thromboplastin time (PTT). Most commonly, this condition is found in association with systemic lupus erythematosus (SLE) but may be seen with other collagen-vascular diseases and in otherwise healthy individuals. Despite the laboratory tests suggesting impaired coagulation, clinically the lupus anticoagulant has been associated with thrombosis. The authors present five patients with the lupus anticoagulant who came to medical attention because of branch retinal artery occlusion, ischemic optic neuropathy, transient visual loss, transient diplopia, or vertebrobasilar insufficiency. Eleven previously reported patients with the lupus anticoagulant and disturbed vision are also reviewed with additional findings of retinal venous occlusive disease and homonymous visual field loss. The relationship of these findings to retinopathy in SLE is discussed. Patients with the lupus anticoagulant (with or without SLE) may develop disturbances in vision due to thrombosis from a hypercoagulable state. We recommended obtaining a PTT, VDRL, and the more sensitive anticardiolipin antibody in patients with unexplained visual symptoms.