title: Progressive tumefactive inflammatory central nervous system demyelinating disease in an acquired immunodeficiency syndrome patient treated with highly active antiretroviral therapy.
abstract: We report here a case of progressive tumefactive inflammatory central nervous system (CNS) demyelinating disease in a human immunodeficiency virus (HIV)-seropositive patient treated with highly active antiretroviral therapy (HAART). Biopsy revealed diffuse macrophage and perivascular T-lymphocytic infiltrates with severe demyelination and relative axonal sparing. The disease progressed in a centrifugal fashion, to involve bihemispheric cerebral white matter, with subsequent central necrotic changes and atrophy. Treatment with HAART was discontinued, and inflammatory disease was treated with subcutaneous interferon (IFN)beta-1a. Massive brain edema was controlled with courses of intravenous corticosteroids. Following fulminant monophasic disease, the patient stabilized with no evidence of disease progression over long-term follow up. We propose that immune response reconstituted by HAART can unmask an autoimmune response in susceptible individuals, analogous to the enhanced immune response to the preexisting acquired immunodeficiency syndrome (AIDS) opportunistic infections. Therapeutic options are considered.
authors.affiliation: Department of Neurology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA.
authors.forename: Eric
authors.initials: E
authors.lastname: Lindzen
authors.forename: Valerie
authors.initials: V
authors.lastname: Jewells
authors.forename: Thomas
authors.initials: T
authors.lastname: Bouldin
authors.forename: Danielle
authors.initials: D
authors.lastname: Speer
authors.forename: Walter
authors.initials: W
authors.lastname: Royal
authors.forename: Silva
authors.initials: S
authors.lastname: Markovic-Plese
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000188:drug therapy*; D000276:Adjuvants, Immunologic / Q000008:administration & dosage / Q000627:therapeutic use; D000328:Adult; D044966:Anti-Retroviral Agents / Q000009:adverse effects* / Q000627:therapeutic use; D023241:Antiretroviral Therapy, Highly Active; D003711:Demyelinating Diseases / Q000175:diagnosis* / Q000209:etiology*; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006679:HIV Seropositivity / Q000188:drug therapy*; D006801:Humans; D054019:Immune Reconstitution Inflammatory Syndrome / Q000188:drug therapy / Q000209:etiology*; D007275:Injections, Intravenous; D007279:Injections, Subcutaneous; D000068556:Interferon beta-1a; D016899:Interferon-beta / Q000008:administration & dosage / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging
chemical_list: D000276:Adjuvants, Immunologic; D044966:Anti-Retroviral Agents; D005938:Glucocorticoids; D016899:Interferon-beta; D000068556:Interferon beta-1a