CNS Demyelinating Attacks Requiring Ventilatory Support With Myelin Oligodendrocyte Glycoprotein or Aquaporin-4 Antibodies

Abstract

Background and Objective:Severe attacks of myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disorder (MOGAD) and aquaporin-4 (AQP4)-antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) may require ventilatory support but data on episodes is limited, particularly for MOGAD. We sought to compare the frequency, characteristics, and outcomes of MOGAD and AQP4-NMOSD attacks requiring ventilatory support.Methods:This retrospective descriptive study identified Mayo Clinic patients (1/1/1996-12/1/2020) with MOGAD or AQP4-NMOSD and an attack requiring non-invasive or invasive ventilation at Mayo Clinic or an outside facility by searching for relevant terms in their electronic medical record. Inclusion criteria were: 1) Attack-related requirement for non-invasive (BiPAP or CPAP) or invasive respiratory support (mechanical ventilation); 2) MOG or AQP4 antibody positivity with fulfillment of MOGAD and AQP4-NMOSD clinical diagnostic criteria, respectively; 3) Sufficient clinical details. We collected data on demographics, co-morbidities, indication for and duration of respiratory support, MRI findings, treatments, and outcomes. The race of those with attacks requiring respiratory support were compared to those without such attacks in MOGAD and AQP4-NMOSD.Results:Attacks requiring ventilatory support were similarly rare in MOGAD (8/279, 2.9%) and AQP4-NMOSD patients (11/503, 2.2%) (p=0.63). The age at attack (median years [range]) (MOGAD, 31.5[5-47] vs AQP4-NMOSD, 43[14-65]; p=0.01) and percentage of female sex (MOGAD, 3/8[38%] vs AQP4-NMOSD, 10/11[91%]; p=0.04) differed. The reasons for ventilation differed between MOGAD (inability to protect airway from seizure, encephalitis or encephalomyelitis with attacks of: acute disseminated encephalomyelitis, 5[62.5%]; or unilateral cortical encephalitis, 3[37.5%]) and AQP4-NMOSD (inability to protect airway from cervical myelitis, 9[82%]; rhombencephalitis, 1[9%]; or combinations of both, 1[9%]). Median ventilation duration for MOGAD was 2 days (range, 1-7) versus 19 days (range, 6-330) for AQP4-NMOSD (p=0.01). All MOGAD patients recovered, but 2/11 (18%) of AQP4-NMOSD died from the attack. For AQP4-NMOSD, Black race was over-represented with attacks requiring ventilatory support versus those without these episodes (5/11[45%] versus 88/457[19%]; p=0.045).Discussion:Ventilatory support is rarely required for MOGAD and AQP4-NMOSD attacks and the indications differ. When compared to MOGAD, these attacks in AQP4-NMOSD may have higher morbidity and mortality and those of Black race were more predisposed, which we suspect may relate to socially mediated health inequality.

COVID-19 susceptibility and outcomes among patients with neuromyelitis optica spectrum disorder (NMOSD): A systematic review and meta-analysis

“…Race and ethnicity have been suggested as contributing factors to COVID-19 outcomes ( Mathur et al, 2021 ; Sze et al, 2020 ). African Americans are at an increased risk of developing severe NMOSD relapse and mortality ( Mealy et al, 2018 ; Zhao-Fleming et al, 2021 ). However, no study has attempted to investigate the effect of race-ethnicity on infection outcome among NMOSD population.…”

“…Notwithstanding the aforementioned disadvantages, the efficacy of rituximab is higher than azathioprine and MMF ( Huang et al, 2019 ; Nikoo et al, 2017 ). Moreover, drug discontinuation could predispose NMOSD patients to potentially-life-threatening attacks ( Kim et al, 2021 ; Zhao-Fleming et al, 2021 ). Therefore, the administration of rituximab should be weighed against the risk of severe infection on a case-by-case basis.…”

“…Current data suggest as an explanation that race-ethnicity minority populations experience social and financial inequity and have inadequate access to healthcare services (Kabarriti et al, 2020;Ogedegbe et al, 2020) One of the greatest challenges physicians are facing during the pandemic is managing immunosuppressive therapies in patients with autoimmune diseases. NMOSD in most cases follows a relapsing course and may lead to neurological disability and morbidity as a consequence of cumulative sequelae of attacks, justifying early initiation of immunosuppressive treatment (Kim et al, 2021;Zhao-Fleming et al, 2021). Azathioprine and mycophenolate mofetil are two common immunosuppressive agents used for the treatment of NMOSD patients.…”

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management

“…Patients may present with encephalopathy, focal deficits referable to the brain, or have asymptomatic brain abnormalities in the context of other MOGAD manifestations (e.g., ON or myelitis). The severity of episodes can be such that patients require ventilatory support with cerebral attacks in up to 3% (69). Brain MRI usually shows multiple large T2-abnormalities variably affecting the supratentorial white matter, the cortex, and/or the deep gray nuclei; unilateral or bilateral thalamic and basal ganglia signal abnormalities are common (Figure 3) (54).…”

“…-Cerebral cortical encephalitis is a less common phenotype, also known as FLAMES (unilateral cortical FLAIRhyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures), characterized by encephalopathy, seizures, headache, marked CSF pleocytosis, and cortical hyperintensity on FLAIR images (82,83). The seizures may evolve into status epilepticus and require ventilatory support (69). The cortical hyperintensity is generally unilateral but can be bilateral and accompanied by leptomeningeal enhancement in the affected brain region (Figure 3).…”