title: Clinical and Radiological Characterization of Progressive Multifocal Leukoencephalopathy in HIV-Infected Patients: A Retrospective Analysis and Review of the Literature.
abstract: BACKGROUND
  Progressive multifocal leukoencephalopathy is a demyelinating disease of the central nervous system caused by John Cunningham virus, mostly associated with immunodeficiency conditions, such as the human immunodeficiency virus infection. Progressive multifocal leukoencephalopathy can have multiple clinical features and usually presents a typical lesion pattern on brain magnetic resonance imaging. Its course may be rapidly progressive, although immunological responsiveness can be associated with an improved prognosis.
  
  
  METHODS
  We performed a retrospective analysis of the clinical and radiological data from patients admitted in our institution between January 2005 and April 2014 with the diagnosis of definitive progressive multifocal leukoencephalopathy (ICD10:A81.2) in the setting of human immunodeficiency virus infection.
  
  
  RESULTS
  Twenty-one patients were included in our study, mostly men (n = 20, 95.2%). Mean age at diagnosis was 39 years. Motor deficits were the most common clinical finding. John Cunningham virus-DNA was detected in the cerebral spinal fluid in 20 patients (95.2%). Brain imaging studies most commonly disclosed bilateral supratentorial, asymmetric lesions. Four (19%) patients developed immune reconstitution inflammatory syndrome in the follow-up. Therapeutic approach included initiation and continuation/optimization of antiretroviral therapy, with adjunctive therapy with corticosteroids in four patients. Seventeen (81%) patients died during the study period; median survival time following progressive multifocal leukoencephalopathy diagnosis was 3 months (range 1 - 13).
  
  
  CONCLUSIONS
  The results of our study are in accordance with the data previously published on progressive multifocal leukoencephalopathy in human immunodeficiency virus patients. Progressive multifocal leukoencephalopathy is predominantly associated with severe immunosuppression, particularly in patients who are not under anti-retroviral therapy, and usually presents with motor and cognitive symptoms and signs. A typical bilateral asymmetric pattern in conventional magnetic resonance imaging is present in the majority of the patients. There is no specific therapy for progressive multifocal leukoencephalopathy and it is usually fatal, although outcomes can improve with highly active anti-retroviral therapy. Immune reconstitution inflammatory syndrome is also an important complication related with progressive multifocal leukoencephalopathy, usually associated with anti-retroviral therapy. Progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome presents with different imaging characteristics from progressive multifocal leukoencephalopathy and treatment with steroids can improve survival.
  
  
  CONCLUSIONS
  The mortality rate and long-term neurological morbidity associated with progressive multifocal leukoencephalopathy are quite high. These data should increase clinician awareness to the occurrence of progressive multifocal leukoencephalopathy among human immunodeficiency virus patients and highlight the important role of magnetic resonance imaging, as early diagnosis may beassociated with better outcome.
authors.affiliation: Neuroradiology Department. Centro Hospitalar São João. Porto. Portugal.
authors.forename: Luís
authors.initials: L
authors.lastname: Augusto
authors.affiliation: Infectious Diseases Department. Centro Hospitalar São João. Porto. Portugal.
authors.forename: Nélia
authors.initials: N
authors.lastname: Neves
authors.affiliation: Neuroradiology Department. Centro Hospitalar São João. Porto. Portugal.
authors.forename: Carina
authors.initials: C
authors.lastname: Reis
authors.affiliation: Infectious Diseases Department. Centro Hospitalar São João. Porto. Portugal.
authors.forename: Cândida
authors.initials: C
authors.lastname: Abreu
authors.affiliation: Infectious Diseases Department. Centro Hospitalar São João. Porto. Portugal. Infectious Diseases Department. Faculty of Medicine. University of Porto. Porto. Portugal.
authors.forename: António
authors.initials: A
authors.lastname: Sarmento
mesh_terms: D000328:Adult; D005260:Female; D015658:HIV Infections / Q000150:complications*; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging; D012189:Retrospective Studies; D055815:Young Adult