1. Present a pair of MOGAD cases occurring in conjunction with hematological disease
2. Review literature and phenotypic spectrum of MOGAD
3. Outline a promising future area of MOGAD pathogenicity research
MOGAD most commonly presents with acute disseminated encephalomyelitis (ADEM), optic neuritis, or myelitis. ADEM is more common in pediatric patients, and a second MOGAD peak occurs in young adults. We hereby present two unique clinical presentations of MOGAD occurring with profound hematological comorbidities, in patients over the age of 60. These cases are interpreted within the framework of an institutional cohort (n=77) and help expand the current understanding of the spectrum of clinical phenotypes and natural histories seen in MOG antibody disease.
Case presentation, chart review, literature review
The first patient is a 61-year-old woman with history of allogeneic HSCT transplant who presents with right>left bilateral loss of vision. Workup for infectious, paraneoplastic, and vascular etiologies returned negative. Brain MRI demonstrated edematous right optic nerve and enhancing DWI+ lesions in the occipital lobe and brainstem, associated with MOG Antibody seropositivity. Her optic neuritis was steroid sensitive but required maintenance IVIG given disease progression. The second patient is a 62-year-old man with history of oculoauriculovertebral syndrome and MOG Antibody disease with recurrent optic neuritis, on maintenance Rituximab, who presents with recurrent visual loss, oral ulcers, and profound neutropenia found to have MOG Antibody titer >1:1000. Bone marrow biopsy was concerning for primary autoimmune etiology for neutropenia. Given refractory disease, patient was switched to IVIG maintenance therapy with efficacy.
These cases demonstrate clinical MOGAD in the setting of hematologic/oncologic diagnoses in the 60+ age group. Such phenotypes add to an expanding spectrum of MOG disease presentations. Future research is needed to elucidate potential MOG pathogenicity or reactivity in the setting of post-transplant immune reconstitution and autoimmune cytopenias to guide treatment.