Central nervous system (CNS) attacks requiring ventilator support are rarely needed in patients with myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD) or aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD), according to study results published in Neurology.

While MOGAD and AQP4-NMOSD, 2 antibody mediated entities, are associated with distinct CNS demyelinating attacks, these attacks are rarely life threatening. Those of Black race are more likely to develop AQP4-NMOSD and worse disease outcomes. The objective of the current study was to assess the frequency, racial differences, neurologic phenotype, and outcome of attacks that required ventilator support in patients with MOGAD and AQP4-NMOSD.

The retrospective study included 279 patients with MOGAD and 503 patients with AQP4-NMOSD from Mayo Clinic, of whom 8 (2.9%) and 11 (2.2%) patients had attacks requiring respiratory support. Data on demographics, comorbidities, indication for respiratory support, radiological findings, treatments and outcomes, were collected.


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Patients with MOGAD that developed attacks requiring ventilatory support were younger (mean age, 31.5 years), compared with patients with AQP4-NMOSD with an attack requiring respiratory support (mean age, 43 years). The percentage of women was higher among patients with AQP4-NMOSD, compared with MOGAD (91% vs. 38%, respectively; P =.004).

The reasons for ventilation differed between MOGAD and AQP4-NMOSD, as the indication for ventilatory support with MOGAD was typically seizures or encephalitis (8 of 8 patients, 100%), while with AQP4-NMOSD it was mostly from severe quadriparesis with cervical myelitis (11 of 11 patients, 100%), and invasive rather than noninvasive ventilation predominated in both patients with MOGAD (100% vs. 0%, respectively) and AQP4-NMOSD (82% vs. 18%, respectively).

Median duration of respiratory support was 2 days (range, 1-7 days) for patients with MOGAD and 19 days (range, 6-330) for those with AQP4-NMOSD (P =.01). While all patients with MOGAD recovered and none required chronic tracheostomy for >6 months, 2 patients (18%) with AQP4-NMOSD died (P =.49) and 1 of the survivors required chronic tracheostomy.

In AQP4-NMOSD, those of Black race were more predisposed to require respiratory support (45% vs. 19%, respectively; P =.045).

The study had several limitations, including the retrospective design, potential referral bias, absence of antibody biomarkers for the entire study timeline, and missing data on pulmonary aspects.

“Ventilatory support is rarely required for MOGAD and AQP4-NMOSD attacks, and the indications differ. 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,” concluded the researchers.

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures. 

Reference

Zhao-Fleming HH, Valencia Sanchez C, Sechi E, et al. CNS demyelinating attacks requiring ventilatory support with myelin oligodendrocyte glycoprotein or aquaporin-4 antibodies. Neurology. Published online September 28, 2021. doi: 10.1212/WNL.0000000000012599