title: Acute disseminated encephalomyelitis: clinical features, HLA DRB1*1501, HLA DRB1*1503, HLA DQA1*0102, HLA DQB1*0602, and HLA DPA1*0301 allelic association study.
abstract: We evaluated the frequency, demographic, clinical, disability evolution and genetic association of HLA DRB1*1501, DRB1*1503, DQA1*0102, DQB1*0602 and DPA1*0301 alleles in patients diagnosed as acute disseminated encephalomyelitis (ADEM) among a population of CNS demyelinating diseases. Fifteen patients (8.4%) of our series were diagnosed as ADEM. The mean age onset was 35.23 years (range 12 to 77), 53.3% were male and follow-up range was 8.5 to 16 years. Two cases (13.3%) had a preceding infection before neurological symptoms, one presented a parainfectious demyelinating, and one case had been submitted to hepatitis B vaccination four weeks before the clinical onset. The EDSS range was 3.0 to 9.5. Eight patients (53.3%) presented MRI with multiple large lesions. CSF was normal in 73.3%. The severe disability observed at EDSS onset improved in 86.66% patients. The genetic susceptibility for ADEM was significantly associated with the HLA DQB1*0602, DRB1*1501 and DRB1*1503 alleles (<0.05) in monophasic ADEM.
authors.affiliation: Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil. sonizavleon@globo.com
authors.forename: Soniza Vieira
authors.initials: SV
authors.lastname: Alves-Leon
authors.forename: Maria Lucia
authors.initials: ML
authors.lastname: Veluttini-Pimentel
authors.forename: Maria Emmerick
authors.initials: ME
authors.lastname: Gouveia
authors.forename: Fabíola Rachid
authors.initials: FR
authors.lastname: Malfetano
authors.forename: Emerson L
authors.initials: EL
authors.lastname: Gaspareto
authors.forename: Marcos P
authors.initials: MP
authors.lastname: Alvarenga
authors.forename: Izabel
authors.initials: I
authors.lastname: Frugulhetti
authors.forename: Thereza
authors.initials: T
authors.lastname: Quirico-Santos
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002648:Child; D004673:Encephalomyelitis, Acute Disseminated / Q000235:genetics* / Q000473:pathology; D005260:Female; D005787:Gene Frequency / Q000235:genetics*; D005838:Genotype; D006682:HLA-DP Antigens / Q000235:genetics*; D059867:HLA-DP alpha-Chains; D006683:HLA-DQ Antigens / Q000235:genetics*; D059848:HLA-DQ alpha-Chains; D059866:HLA-DQ beta-Chains; D006684:HLA-DR Antigens / Q000235:genetics*; D059811:HLA-DRB1 Chains; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D012720:Severity of Illness Index; D055815:Young Adult
chemical_list: D006682:HLA-DP Antigens; D059867:HLA-DP alpha-Chains; C071172:HLA-DPA1 antigen; D006683:HLA-DQ Antigens; D059848:HLA-DQ alpha-Chains; D059866:HLA-DQ beta-Chains; C069050:HLA-DQA1 antigen; C069051:HLA-DQB1 antigen; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains