Progressive multifocal leukoencephalopathy in patients with sarcoidosis. 2014

Yvan Jamilloux, and Antoine Néel, and Marie Lecouffe-Desprets, and Anne Fèvre, and Sebastien Kerever, and Benoit Guillon, and Diane Bouvry, and Loig Varron, and Cécile Redares, and Stéphane Dominique, and Mareille Roux, and Catherine Chapelon-Abric, and Dominique Valeyre, and François Ducray, and Claire Bernard, and Christiane Broussolle, and Mohamed Hamidou, and Pascal Sève
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.

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.

UI MeSH Term Description Entries
D007968 Leukoencephalopathy, Progressive Multifocal An opportunistic viral infection of the central nervous system associated with conditions that impair cell-mediated immunity (e.g., ACQUIRED IMMUNODEFICIENCY SYNDROME and other IMMUNOLOGIC DEFICIENCY SYNDROMES; HEMATOLOGIC NEOPLASMS; IMMUNOSUPPRESSION; and COLLAGEN DISEASES). The causative organism is JC Polyomavirus (JC VIRUS) which primarily affects oligodendrocytes, resulting in multiple areas of demyelination. Clinical manifestations include DEMENTIA; ATAXIA; visual disturbances; and other focal neurologic deficits, generally progressing to a vegetative state within 6 months. (From Joynt, Clinical Neurology, 1996, Ch26, pp36-7) Encephalitis, JC Polyomavirus,Progressive Multifocal Leukoencephalopathy,JC Polyomavirus Encephalopathy,Encephalopathies, JC Polyomavirus,Encephalopathy, JC Polyomavirus,JC Polyomavirus Encephalitis,Leukoencephalopathies, Progressive Multifocal,Multifocal Leukoencephalopathies, Progressive,Multifocal Leukoencephalopathy, Progressive,Progressive Multifocal Leukoencephalopathies
D008297 Male Males
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000305 Adrenal Cortex Hormones HORMONES produced by the ADRENAL CORTEX, including both steroid and peptide hormones. The major hormones produced are HYDROCORTISONE and ALDOSTERONE. Adrenal Cortex Hormone,Corticoid,Corticoids,Corticosteroid,Corticosteroids,Cortex Hormone, Adrenal,Hormone, Adrenal Cortex,Hormones, Adrenal Cortex
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000998 Antiviral Agents Agents used in the prophylaxis or therapy of VIRUS DISEASES. Some of the ways they may act include preventing viral replication by inhibiting viral DNA polymerase; binding to specific cell-surface receptors and inhibiting viral penetration or uncoating; inhibiting viral protein synthesis; or blocking late stages of virus assembly. Antiviral,Antiviral Agent,Antiviral Drug,Antivirals,Antiviral Drugs,Agent, Antiviral,Agents, Antiviral,Drug, Antiviral,Drugs, Antiviral
D012307 Risk Factors An aspect of personal behavior or lifestyle, environmental exposure, inborn or inherited characteristic, which, based on epidemiological evidence, is known to be associated with a health-related condition considered important to prevent. Health Correlates,Risk Factor Scores,Risk Scores,Social Risk Factors,Population at Risk,Populations at Risk,Correlates, Health,Factor, Risk,Factor, Social Risk,Factors, Social Risk,Risk Factor,Risk Factor Score,Risk Factor, Social,Risk Factors, Social,Risk Score,Score, Risk,Score, Risk Factor,Social Risk Factor
D012507 Sarcoidosis An idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis. It usually invades the lungs with fibrosis and may also involve lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands. Besnier-Boeck Disease,Boeck's Sarcoid,Besnier-Boeck-Schaumann Syndrome,Boeck Disease,Boeck's Disease,Schaumann Disease,Schaumann Syndrome,Schaumann's Syndrome,Besnier Boeck Disease,Besnier Boeck Schaumann Syndrome,Boeck Sarcoid,Boecks Disease,Boecks Sarcoid,Disease, Schaumann,Sarcoid, Boeck's,Sarcoidoses,Schaumann's Syndromes,Syndrome, Besnier-Boeck-Schaumann,Syndrome, Schaumann,Syndrome, Schaumann's

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