Neuromyelitis Optica In The Setting Of Human Immunodeficiency Virus Infection

Background: Neuromyelitis optica (NMO) is a severe inflammatory demyelinating disease, distinct from multiple sclerosis (MS), which preferentially targets optic nerves and the spinal cord. Acute viral and bacterial infections have been reported to precede the onset of NMO; however, little is known about the disease course within the context of human immunodeficiency virus (HIV) infection.

Objectives: We describe two cases of HIV infected patients who developed NMO. Here, we report our experience with maintenance immunotherapy in patients with combined NMO and HIV infection.

Methods: Retrospective clinical data and magnetic resonance imaging (MRI) studies were reviewed on two HIV infected patients who developed NMO. The two patients were followed up prospectively for three years with regular neurology clinic visits.

Results: Two African American patients, a 32 year old male (case 1) and a 49 year old female (case 2) with known HIV infections receiving antiretroviral treatment, developed NMO. Initially, our cohort presented with monocular blindness. Five to nine months later, they presented with evidence of acute transverse myelitis manifested by severe paraparesis. Both of these patients had CD4+T-cell count of >350/µL and HIV viral load <3000 RNA copies/mL throughout the study period. Cerebrospinal fluid (CSF) analysis revealed neutrophilic pleocytosis (12-700cells/µL), elevated protein (70-900mg/dL), and normal glucose concentrations. Case 2 tested positive for aquaporin 4 immunoglobulin G (AQP4-IgG) antibody in the serum. Oligoclonal bands of IgG were detected in the CSF of case 1. In both cases, initial MRI of the brain showed uniform contrast enhancement of the optic nerve unilaterally. When these patients developed paraparesis, spine MRI revealed extensive spinal cord T2-weighted lesions spanning >3 contiguous vertebral segments. Patients were treated with high dose pulsed intravenous methylprednisolone followed by standard sessions of plasmapheresis both at the initial presentation and during disease relapses. For maintenance therapy, case 1 received a total of 2 g rituximab IV divided into 2 monthly infusions while the second case received mycophenolate mofetil 500mg orally once daily. Over the three year follow-up period, case 2 had three disease relapses while the first case developed a progressive disease. Currently, both patients are wheelchair-bound due to severe paraparesis. Case 2 has become functionally blind.

Conclusions :NMO can occur in the course of HIV infection and poses an ongoing therapeutic challenge. This report highlights the rapidly disabling nature of NMO when compared to typical relapsing and remitting MS, especially in immunocompromised individuals. Further epidemiological and clinical studies are needed to demonstrate etiologic association between HIV and NMO.