A 59-Year-Old Woman With Progressive Shortness of Breath, Intermittent Fevers, and Restrictive Lung Disease.

CASE PRESENTATION: A 59-year-old woman sought treatment for 5 weeks of progressive exercise intolerance. At the time of presentation, dyspnea limited her ability to speak in complete sentences. She also reported new orthopnea. Her respiratory symptoms improved with rest and while standing. She endorsed associated intermittent low-grade fevers, cough productive of scant clear sputum, lower extremity swelling, bloating, weight loss, and reduced appetite. She had undergone two recent admissions with similar symptoms to other hospitals, during which she was treated empirically for community-acquired pneumonia and discharged after workups for infectious disease were unrevealing. She had a history notable for systemic lupus erythematosus (SLE) diagnosed in 2006, complicated by lupus nephritis in 2009. Most recently, her SLE had been quiescent while she was taking hydroxychloroquine (400 mg daily) and mycophenolate mofetil (MMF; 1 g twice daily). She reported baseline mild dyspnea with exertion since she received a diagnosis of SLE, but her symptoms had not previously affected her activities of daily living. The patient did not smoke, drink alcohol, or use recreational drugs, and her family history was unremarkable.

Seizures and Encephalitis in Myelin Oligodendrocyte Glycoprotein IgG Disease vs Aquaporin 4 IgG Disease.

Importance: Antibodies to myelin oligodendrocyte glycoprotein IgG (MOG-IgG) are increasingly detected in patients with non-multiple sclerosis-related demyelination, some of whom manifest a neuromyelitis optica (NMO) phenotype. Cortical involvement, encephalopathy, and seizures are rare in aquaporin 4 antibody (AQP4-IgG)-related NMO in the white European population. However, the authors encountered several patients with seizures associated with MOG-IgG disease. Objective: To compare incidence of seizures and encephalitis-like presentation, or both between AQP4-IgG-positive and MOG-IgG-positive patients. Design, Setting, and Participants: Retrospective case series of all patients who were seropositive for MOG-IgG (n = 34) and the last 100 patients with AQP4-IgG disease (NMO spectrum disorder) seen in the NMO service between January 2013 and December 2016, and analysis was completed January 4, 2017. All patients were seen in a tertiary neurological center, The Walton Centre NHS Foundation Trust in Liverpool, England. Main Outcomes and Measures: The difference in seizure frequency between the AQP4-IgG-positive and MOG-IgG-positive patient groups was determined. Results: Thirty-four patients with MOG-IgG disease (20 female) with a median age at analysis of 30.5 years (interquartile range [IQR], 15-69 years), and 100 AQP4-IgG-positive patients (86 female) with a median age at analysis of 54 years (IQR, 12-91 years) were studied. Most patients were of white race. Five of the 34 patients with MOG-IgG (14.7%) had seizures compared with 1 patient with AQP4-IgG (2-sided P < .008, Fisher test). On magnetic resonance imaging, all 5 MOG-IgG-positive patients had inflammatory cortical brain lesions associated with the seizures. In 3 of the 5 MOG-IgG-positive patients, seizures occurred as part of the index event. Four of the 5 presented with encephalopathy and seizures, and disease relapsed in all 5 patients. Four of these patients were receiving immunosuppressant medication at last follow-up, and 3 continued to take antiepileptic medication. In contrast, the only AQP4-IgG-positive patient with seizures had a diagnosis of complex partial epilepsy preceding the onset of NMO by several years and experienced no encephalitic illness; her magnetic resonance imaging results demonstrated no cortical, subcortical, or basal ganglia involvement. Conclusions and Relevance: Patients with MOG-IgG-associated disease were more likely to have seizures and encephalitis-like presentation than patients with AQP4-IgG-associated disease.