title: Fulminant central nervous system demyelination associated with interferon-alpha therapy and hepatitis C virus infection.
abstract: Hepatitis C virus (HCV) infection is common in the general population and may coincide with disease in the central and peripheral nervous system. Interferon-alpha (IFN-alpha) is used as treatment for HCV infection. The therapeutic benefit is assumed to result from activation of natural killer cells and CD8+ T cells. Despite its beneficial effects, it has been associated with a number of autoimmune disorders, such as chronic inflammatory demyelinating polyneuropathy and multiple sclerosis. Several clinical reports including magnetic resonance imaging exist, but neuropathological confirmation of MS associated with IFN-alpha therapy and HCV infection is lacking. We report a case of a female patient with chronic HCV infection who developed ;acute MS'-like demyelinating disease after IFN-alpha administration, with extensive lesions throughout brain and thoracic spinal cord. The patient died after a disease duration of 6 months. Brain autopsy revealed Baló-like demyelinating plaques with positive HCV sequences within florid lesions. The development of fulminant demyelinating disease after administration of IFN-alpha suggests that autoimmune mechanisms such as T cell mediated tissue damage might be initiated or aggravated by IFN-alpha therapy. Additionally, the presence of HCV RNA within the demyelinated lesion indicates a possible role in triggering or propagating disease.
authors.affiliation: Institute of Neurology, Medical University of Vienna, Austria.
authors.forename: R
authors.initials: R
authors.lastname: Höftberger
authors.forename: F
authors.initials: F
authors.lastname: Garzuly
authors.forename: H P
authors.initials: HP
authors.lastname: Dienes
authors.forename: J
authors.initials: J
authors.lastname: Grubits
authors.forename: B
authors.initials: B
authors.lastname: Rohonyi
authors.forename: G
authors.initials: G
authors.lastname: Fischer
authors.forename: Z
authors.initials: Z
authors.lastname: Hanzely
authors.forename: H
authors.initials: H
authors.lastname: Lassmann
authors.forename: H
authors.initials: H
authors.lastname: Budka
mesh_terms: D000208:Acute Disease; D000328:Adult; D000998:Antiviral Agents / Q000009:adverse effects*; D001706:Biopsy; D003711:Demyelinating Diseases / Q000139:chemically induced* / Q000473:pathology; D005260:Female; D016174:Hepacivirus / Q000235:genetics / Q000302:isolation & purification*; D019698:Hepatitis C, Chronic / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D016898:Interferon-alpha / Q000009:adverse effects*; D012367:RNA, Viral / Q000302:isolation & purification; D012720:Severity of Illness Index
chemical_list: D000998:Antiviral Agents; D016898:Interferon-alpha; D012367:RNA, Viral