A RARE PRESENTATION OF DEVIC’S DISEASE A. Gaddam MD, S. Varakantam MD, A. Williams MD, B. Hubble, DO R. Kaval MD, S. Zuckerman MD, A. Dewitt MD, V. Tati MD. Department of Medicine, Baton Rouge General Medical Center, Baton Rouge, LA. INTRODUCTION: Neuromyelitis Optica (NMO, also known as Devic’s disease) and Neuromyelitis Optica Spectrum Disorder (NMOSD) are rare inflammatory disorders of the central nervous system characterized by severe, immune-mediated demyelination and axonal damage predominantly targeting optic nerves and spinal cord. The hallmark features include acute attacks of optic neuritis and acute myelitis. CASE: A 58-year-old women with hypertension and hypothyroidism presented with a two-day history of severe and rapidly progressive nausea and vomiting and associatedmid-thoracic upper back pain. Initial examandworkupwere unremarkable aside from an elevated protein gap. Serum protein electrophoresis revealed a polyclonal increase in gamma globulin with no evidence of monoclonal protein and HIV/hepatitis panel were negative. She experienced numbness and tingling in her right lower extremity, progressing to weakness of bilateral lower extremities, and urinary retention. Magnetic resonance imaging (MRI) of the head revealed increased signal in a periventricular distribution bilaterally and in the deep white matter of both cerebral hemispheres. MRI of the spine revealed extensive abnormal signal and diffuse enhancement of multiple spinal cord segments involving the lower cervical and upper thoracic cord extending to the T10 level, most significantly T2 to T5 levels. NMO was highly suspected, so high dose steroids were started with minimal response and ultimately required plasmapheresis. NMO-IgG (Aquaporin-4) antibody later confirmed the diagnosis. Neurologic symptoms persisted prompting initiation of Rituximab as the next guideline-directed therapy with gradual improvement. She was placed in a rehabilitation facility and at her nine month follow up, she remained stable with only minimal residual lower extremity weakness and no relapse. DISCUSSION: The real challenge is the recognition of NMO in settings unaccompanied by optic neuritis and acute myelitis. Diagnoses was confirmed by seropositivity for AQP4-IgG and presence of Area Postrema syndrome which is one of the six core clinical characteristics apart from Optic neuritis and Acute Myelitis. Longitudinally extensive spinal cord lesions are characteristic of NMO and differentiate it from multiple sclerosis. Early diagnosis and prompt initiation of therapy with intravenous glucocorticoids, plasmapheresis and Rituximab is essential for severe and unresponsive disease to prevent disabling effects of this rapidly progressive disorder. Long term immunosuppression with Rituximab should be tailored to the severity of attacks, disability, CD20 levels and to prevent relapses. Data is lacking regarding the duration of therapy to achieve remission, as well as in the maintenance of long term remission in those treated with Rituximab.
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