Chen, John J.

Background: Acute disseminated encephalomyelitis (ADEM) is a demyelinating syndrome observed in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) but there are limited data on application of this definition to its cerebral attacks or in adults with this disease. Methods: We analyzed patients from the Mayo Clinic MOGAD cohort ( n = 263) with cerebral attacks. We assessed whether children (<18 years-old) and adults (⩾18 years-old) fulfilled the 2013 ADEM definition. Results: We identified 89 patients (children, 34; adults, 55) and 55/89 (62%) were of female sex. Acute magnetic resonance imaging (MRI) abnormalities included: poorly demarcated T2 lesions (white matter, 85 (96%); deep gray matter, 41 (46%); T1-hypointense lesions (42 (47%)). Forty-two patients did not fulfill the ADEM definition due to: monofocal syndrome, 9; or no encephalopathy, 33. The remaining 47 patients (53%: children, 27/34 (79%); adults, 20/55 (36%)) fulfilled the ADEM definition, and 14 had preceding attacks, and 34 had subsequent attacks relative to ADEM episode. The features in children and adults with ADEM were broadly comparable. Conclusion: Applying the ADEM definition to MOGAD cerebral attacks highlighted that an update of the 2013 ADEM definition would be beneficial and could explicitly state its application to both adults and children and allow it to be applied to attacks at any time during the disease course.

Seronegative neuromyelitis optica spectrum disorder (NMOSD) is a diagnostic challenge. This study aimed to characterize misapplication of the 2015 international criteria for seronegative NMOSD. We reviewed 35 consecutive seronegative NMOSD referrals to a tertiary center. Two (6%) met formal criteria, and 2 (6%) were clinically suspected of NMOSD without fulfilling radiologic supportive criteria. Alternative diagnoses were common including multiple sclerosis (37%), optic neuritis (14%), functional neurologic disorder (11%), and other (38%). Immunosuppression with rituximab was used in 3 of 4 patients with functional neurologic disorder. Misapplication of the 2015 criteria is common, and alternative explanations in seronegative NMOSD patients should be strongly considered.

Background Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and multiple sclerosis (MS) have both overlapping and distinct MRI lesion features, which vary with imaging timing. This study identified distinguishing MRI characteristics using paired MRIs at clinical attack and remission. Methods We retrospectively identified Mayo Clinic patients with MOGAD and MS that: (1) fulfilled respective diagnostic criteria; (2) had paired attack (≤30 days) and remission MRI scans (≥12 months) without interval attacks. MRIs were compared between groups for key features. Results We included 43 patients with MOGAD (median age 31 years (range, 3–67); 63% female) and 49 patients with MS (median age 39 years (range, 17–65); 65% female). Resolution of at least one T2-lesion differentiated MOGAD from MS (sensitivity, (95% CI 77% to 100%), specificity, (95% CI 86% to 99%); Youden’s index (YI)=0.90). Resolution of at least two T2-lesions indicated MOGAD (sensitivity 62% (95% CI 41% to 79%); specificity, 100% (95% CI 94% to 100%); YI=0.62). MOGAD patients were more likely to have normal MRI scans at follow-up compared with MS (brain 14/44 (32%) vs 0/60 (0%), p<0.001; spine 21/27 (78%) vs 7/36 (19%), p<0.001). In addition, the presence of T1-hypointense, ovoid periventricular T2, and enhancing lesions were more common in MS versus MOGAD at attack and remission and in the spine, longitudinally extensive T2 lesions were more common in MOGAD attacks (8/27 (30%)). Conclusion Paired MRI at attack and remission revealed distinctive characteristics of MOGAD and MS, with greater diagnostic value at remission driven by the discriminating power of T2-lesion resolution. In MOGAD patients with initial parenchymal involvement, a 1-year follow-up MRI may aid diagnosis and serve as a new baseline.

This international multicenter study seeks to determine the frequency and characteristics of meningitis in myelin oligodendrocyte glycoprotein antibody (MOGAD)–associated disease and highlight its pathology.

Increased intracranial pressure (ICP) is increasingly recognized in myelin oligodendrocyte glycoprotein antibody-associated disease but has not been systematically explored in other demyelinating conditions. This cross-sectional study examines the features of raised ICP in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS). Lumbar puncture opening pressure (OP) done within 30 days of an attack was identified in 41 NMOSD patients and 37 MS patients. Raised OP was present in 7 (17.1%) NMOSD patients and 4 (10.8%) MS patients who were largely asymptomatic, partially explained by obesity, and did not require ICP lowering medications beyond acute treatment with corticosteroids.

Abstract Introduction Lung involvement is common in ANCA-associated vasculitis (AAV), presenting in various forms: necrotizing granulomatous inflammation, tracheobronchial inflammation, pulmonary capillaritis and diffuse alveolar hemorrhage (DAH), interstitial lung disease (ILD), and asthma.1 Radiologic manifestations are diverse, often complicating diagnosis based on imaging alone. Typical patterns include lung nodules, cavitation, pulmonary hemorrhage, and peripheral consolidation; however, cystic-like lesions are rarely seen.2 We present a case of AAV with bilateral cystic-like lung lesions. Case presentation A 54-year-old male with childhood asthma initially presented to an outside hospital due to progressive exertional dyspnea for 6 years and dysphonia for 1 year. Initial computer tomography (CT) revealed pulmonary cysts (Figure 1), and a CT-guided biopsy of a right upper lobe cystic lesion revealed intra-alveolar hemorrhage, though pathology was non-diagnostic. He received a diagnosis of congenital cystic lung disease and prescribed Trelegy and fluticasone inhalers. He was instructed to take oral prednisone with taper whenever dyspnea recurs for presumed asthma exacerbation. His exertional dyspnea indeed respond to prednisone but not to inhalers, and it would recur a few weeks later, and his dysphonia persisted. He came to our facility for second opinion given being told lung transplantation is the only ultimate treatment in his case. Additional history revealed chronic ear infection, bilateral rupturesd tympanic membrane, chronic rhinosinusitis, and recurrent nasolacrimal duct infection. He had been on oral prednisone for 4 weeks and remained on 10mg daily. Physical exam noted inspiratory wheezing. Further workups were notable for normal eospinophils, normal renal function, positive C-ANCA and PR3, normal pulmonary function test. High-resolution CT deomnstrated numerous areas of parenchymal lucency with circumferential surrounding groundglass opacity (GGO). At this point, the diagnosis of PR3-AAV is made. Patient is subsequently referred to rheumatology for further management. Discussion The diagnosis of AAV relies on clinical, serologic, radiologic, and pathologic data. The atypical presentation may delay diagnosis, especially in cases limited to ear, nose, and throat involvement and the radiological findings are of low specificity.3 While cystic lesions in AAV-related interstitial pneumonia are documented4, this case lacked a defined cyst wall. The central lucency may present partially destroyed lung parenchyma due to GPA-related necrosis (emphysema or pneumatocele) with surrounding areas of blood products deposited from prior peri-lesional hemorrhage. Clinicians should consider a broad differentials when encounter a cystic-like lesion with thorough history-taking being essential.