title: Progressive multifocal leukoencephalopathy in patients with sarcoidosis.
abstract: OBJECTIVE
  To describe characteristics, risk factors, and treatment outcome of progressive multifocal leukoencephalopathy (PML) complicating sarcoidosis.
  
  
  METHODS
  A retrospective chart and literature review was performed. Patients were identified through records from physicians of the Groupe Sarcoïdose Francophone. Each case was compared with 3 controls.
  
  
  RESULTS
  Ten cases were found (8 men). The median age at sarcoidosis diagnosis was 34.9 (±6) years. PML and sarcoidosis were diagnosed concomitantly in 2 cases, while sarcoidosis was previously known in 8 cases, including 7 cases treated with steroids (mean time between sarcoidosis diagnosis and PML was 114 [±99] months). The mean CD4 cell count was 215 (±139)/mm(3). Neurosarcoidosis was thought to be the problem in 8 cases and treatment was intensified, delaying PML diagnosis by 4.5 (±3.9) months. Eight patients received PML-specific treatment. On the whole, 6 patients died of PML within a mean time of 8 (±4.3) months. Patients with PML were significantly younger than controls. When combining our 10 patients with another 20 from the literature, we found that 17 patients (57%) died from sarcoidosis-associated PML; thus, the fatality rate was 57%.
  
  
  CONCLUSIONS
  PML during sarcoidosis is often misdiagnosed. It is not associated with severe CD4 lymphocytopenia. Fatality rate is high in comparison with PML associated with other conditions. Interrupting immunosuppression remains the mainstay of treatment.
authors.affiliation: From the Service de Médecine Interne (Y.J., L.V., C. Bernard, C. Broussolle, P.S.), Hôpital de la Croix-Rousse, Lyon; Service de Médecine Interne (A.N., M.L.-D., M.H.) and Service de Neurologie (B.G.), CHU Nantes; Service d'Endocrinologie (A.F.), Diabète et Nutrition, CHU Reims; Biostatistic and Medical Information Department (S.K.), UMR 717 INSERM, Saint Louis University Hospital, AP-HP, University of Paris VII Denis Diderot, Paris; Assistance Publique-Hôpitaux de Paris (D.B., D.V.), Hôpital Avicenne et Université Paris 13, Sorbonne Paris Cité, Bobigny; Service de Médecine Interne et Gériatrie (C.R.), CH Belfort-Montbéliard, Belfort; Service de Pneumologie (S.D.), CHU Nicolle, Rouen; Service de Médecine Interne (M.R.), CH Oudot, Bourgoin-Jallieu; Department of Internal Medicine II (C.C.-A.), CHU Pitié Salpêtrière, Université Pierre et Marie Curie, Paris; and Service de Neuro-oncologie (F.D.), Hôpital Neurologique, Lyon, France.
authors.forename: Yvan
authors.initials: Y
authors.lastname: Jamilloux
authors.forename: Antoine
authors.initials: A
authors.lastname: Néel
authors.forename: Marie
authors.initials: M
authors.lastname: Lecouffe-Desprets
authors.forename: Anne
authors.initials: A
authors.lastname: Fèvre
authors.forename: Sebastien
authors.initials: S
authors.lastname: Kerever
authors.forename: Benoit
authors.initials: B
authors.lastname: Guillon
authors.forename: Diane
authors.initials: D
authors.lastname: Bouvry
authors.forename: Loig
authors.initials: L
authors.lastname: Varron
authors.forename: Cécile
authors.initials: C
authors.lastname: Redares
authors.forename: Stéphane
authors.initials: S
authors.lastname: Dominique
authors.forename: Mareille
authors.initials: M
authors.lastname: Roux
authors.forename: Catherine
authors.initials: C
authors.lastname: Chapelon-Abric
authors.forename: Dominique
authors.initials: D
authors.lastname: Valeyre
authors.forename: François
authors.initials: F
authors.lastname: Ducray
authors.forename: Claire
authors.initials: C
authors.lastname: Bernard
authors.forename: Christiane
authors.initials: C
authors.lastname: Broussolle
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.forename: Pascal
authors.initials: P
authors.lastname: Sève
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000998:Antiviral Agents / Q000627:therapeutic use; D001921:Brain / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D012307:Risk Factors; D012507:Sarcoidosis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000998:Antiviral Agents