Treatment Response Variation in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease presenting as Acute Disseminated Encephalomyelitis: Two Case Illustrations. (P13-5.029)

Abstract

<h3>Objective:</h3> Present two fairly distinct presentations of Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) <h3>Background:</h3> MOGAD is an inflammatory demyelinating disease of the central nervous system. MOG is a highly immunogenic part of the outermost lamellae of the myelin sheath. MOGAD incidence is 3.4 per one million person-years, three times higher in children versus adults, with equal gender distribution. <h3>Design/Methods:</h3> Data collected primarily through chart review <h3>Results:</h3> We present an 18-year-old female with recent resolved upper respiratory infection who presented with fever, headache, and left hemiparesis, with rapid progression to quadriparesis, urinary retention, and encephalopathy. The hospital course involved autonomic dysfunction, prolonged intubation requiring tracheostomy and gastrostomy. Cerebrospinal fluid (CSF) showed pleocytosis, normal biochemical parameters with a positive MOG titer (1:40). Magnetic resonance imaging (MRI) showed extensive cervicothoracic T2 hyperintensity and brain multifocal T2 hyperintensities. Treatment included high dose steroids and Intravenous Immunoglobulin (IVIG) with recovery to baseline mentation and antigravity limb movements by discharge over two weeks. A 22-year-old male presented with progressive lower extremity paresthesia and weakness over six weeks. CSF demonstrated pleocytosis, normal glucose and elevated protein, oligoclonal bands and MOG antibody. MRI revealed multiple subcortical T2-hyperintense lesions and enhancing mid-cervical and lower thoracic lesions. Treatment with intravenous corticosteroids resulted in minor improvement with discharge on steroid taper and azathioprine. Patient’s disease progressed with a fluctuating course requiring two readmissions with bilateral upper extremity weakness, right optic neuritis, urinary sphincteric dysfunction with neuro-radiologic worsening. Treatment through multiple admissions included intravenous steroids, IVIG, plasmapheresis, Mycophenolate mofetil, and Rituximab with minimal improvement, symptom recurrence and progression of multifocal lesions. <h3>Conclusions:</h3> MOGAD has variable presentations like optic neuritis, transverse myelitis, or acute disseminated encephalomyelitis (ADEM), with or without encephalopathy. We present two rare cases of ADEM admitted within weeks of each other but distinct in severity and treatment responses, without any identifiable factors affecting clinical outcomes. <b>Disclosure:</b> Dr. Banerjee has nothing to disclose. Dr. Cabral Frade has nothing to disclose. Dr. Lobaina has nothing to disclose. Dr. Brown has nothing to disclose. Dr. Narvaez Caicedo has nothing to disclose. Dr. Kamal Mamoud Elnaeem has nothing to disclose. Sandeep Bhatt has nothing to disclose. Dr. Wu has nothing to disclose. Dr. Bhardwaj has nothing to disclose. Dr. Dabi has nothing to disclose.