CHSU Discovery

Neuromyelitis Optica Spectrum Disorders (NMOSD) aka’s Devic Disease or Neuromyelitis Optica (NMO) is a rare disease. It has 10x high predilection to female than male, ages 32-41, primarily African American and Afro-Caribbean with diagnosing criteria that must include the core clinical symptoms plus positive AQP4-IgG Ab, and exclusion of another diagnosis, including Multiple Sclerosis (MS) and Myelin Oligodendrocyte Glycoprotein Ab Disease (MOGAD). Brain MRI is often normal or nonspecific, but if present, there will be cloud enhancement changes in the area postrema, peri-third/fourth ventricle, splenium, diffuse corpus callosum, and a pencil-thin ependymal. CSF usually lymphocytic but can be neutrophilic or eosinophil. Typically relapsing and usually without secondary progression. It is also attack-related accumulation of disability. Owing to its rarity, the patient scenario has involved multi-disciplinary specialties, including Family/Internal Medicine, Infectious Disease, Neurology, Neuro-oncology, Nephrology, PT/OT, Pharmacy, Social Services, and Case Management teams. The MRI revealed trans-ependymal edema, and abnormal signal from mid and upper thoracic spinal cord. The CSF was positive for AQP-4 Ab, and the brain biopsy was suggestive of demyelinating disease in R temp lobe. The initial running diagnosis was bacterial vs viral vs fungal meningitis, and then the appropriate treatment was initiated once NMOSD diagnosis was established. The patient was placed on methylprednisolone IV 1 g qOD x5d; PLEX qOD x5; eculizumab IV 900 mg weekly x4w, followed by 1200 mg x 1week, & then 1200 mg q2weekly, and has recovered well. He was later transferred to Hanford Hospital for further studies, and elevated level of care.