Subject(s)
Child, Preschool , Humans , Male , Encephalomyelitis, Acute Disseminated/chemically induced , Influenza A Virus, H1N1 Subtype/immunology , Influenza Vaccines/adverse effects , Thalamic Nuclei , Encephalomyelitis, Acute Disseminated/pathology , Influenza, Human/prevention & control , Magnetic Resonance Imaging , Thalamic Nuclei/pathologyABSTRACT
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 percent) of our series were diagnosed as ADEM. The mean age onset was 35.23 years (range 12 to 77), 53.3 percent were male and follow-up range was 8.5 to 16 years. Two cases (13.3 percent) 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 percent) presented MRI with multiple large lesions. CSF was normal in 73.3 percent. The severe disability observed at EDSS onset improved in 86.66 percent 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.
Avaliamos as frequencia, características demográficas, clínicas e de associação genética dos alelos HLA DRB1*1501, DRB1*1503, DQA1*0102, DQB1*0602 e DPA1*0301 em pacientes com diagnóstico de encefalomielite aguda disseminada (ADEM) em população com doença desmielinizante do SNC. Quinze (8,4 por cento) pacientes de nossa série foram diagnosticados como ADEM. A média de idade foi 35,23 anos (variando entre 12 e 77), 53,3 por cento eram homens e o tempo de acompanhamento variou entre 8,5 e 16 anos. Dois casos (13,3 por cento) apresentaram infecção prévia, um apresentou processo desmielinizante para infeccioso e outro havia se submetido a vacinação para hepatite B quatro semanas antes. O EDSS variou entre 3,0 e 9,5. Oito pacientes (53,3 por cento) apresentaram grandes lesões na RM. O LCR foi normal em 73,3 por cento. A incapacidade grave quantificada pelo EDSS foi seguida de melhora importante em 86,6 por cento dos pacientes. A susceptibilidade genética na ADEM foi significativamente associada com os alelos HLA DQB1*0602, DRB1*1501 e DRB1*1503 (p<0,05) nos pacientes com quadro monofásico.
Subject(s)
Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Young Adult , Encephalomyelitis, Acute Disseminated/genetics , Gene Frequency/genetics , HLA-DP Antigens/genetics , HLA-DQ Antigens/genetics , HLA-DR Antigens/genetics , Case-Control Studies , Encephalomyelitis, Acute Disseminated/pathology , Genotype , Magnetic Resonance Imaging , Polymerase Chain Reaction , Severity of Illness Index , Young AdultABSTRACT
La encefalomielites aguda diseminada (EMAD) es una forma de encefalitis postinfecciosa, de etiología diversa, que suele manifestarse después de un cuadro infeccioso vírico o bacteriano o después de una inmunización (1-2-3-). Se presenta especialmente en la infancia. Objetivo: describir dos casos clínicos de encefalopatía aguda diseminada post viral en el Servicio de Pediatría del HCIPS. Materiales y método: Análisis retrospectivo de historias clínicas de dos pacientes con diagnóstico de encefalopatía aguda diseminada post viral, internados en el Servicio de Pediatría del HCIPS. Conclusión: En los dos casos estudiados se dió el patrón de evolución clínico e imageneológico descripto en la literatura para encefalopatía desmielinizante aguda. Se sugiere considerar este diagnóstico ante un niño con compromiso encefálico que se instala posterior a una enfermedad vírica. la RNM cerebral es el estudio por imagen recomendado para estos casos por ser el test radilógico mas sensible y específico. Sc
Subject(s)
Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/etiology , Encephalomyelitis, Acute Disseminated/pathologyABSTRACT
We report a 15 year-old female presenting with behavioral disturbances, headache, left hemiparesis and paresis of the vertical gaze. CAT scan and magnetic resonance showed an involvement of right thalamus, third ventricle and medial temporal lobe suggesting an encephalitis or lymphoma. 201Thalium SPECT suggested a lymphoma. A stereotaxic biopsy showed a subacute demyelinizing lesion, compatible with an acute disseminated encephalomyelitis. The patient was treated with Methylprednisolone with resolution of symptoms. She remains in good condition after one year of follow-up.
Subject(s)
Adolescent , Female , Humans , Encephalomyelitis, Acute Disseminated/pathology , Anti-Inflammatory Agents/therapeutic use , Biopsy, Needle/methods , Encephalomyelitis, Acute Disseminated/drug therapy , Methylprednisolone/therapeutic use , Thallium Radioisotopes , Tomography, Emission-Computed, Single-PhotonABSTRACT
Encefalomielitis postinfecciosa o encefalomielitis diseminada aguda, constituye una enfermedad que sigue a una variedad de infecciones virales y/o bacterianas. Su verdadera incidencia se desconoce, sin embargo en los Estados Unidos se reporta entre un diez al quince por ciento de todas las encefalitis virales. El número de casos disminuyó notablemente con la eliminación de vacunas a virus vivos. Clinicamente resulta imposible de diferenciar de las encefalitis, principalmente la herpética. La RNM con gadolinio es el método de diagnóstico de mas ayuda a la hora del diagnóstico diferencial, con las encefalitis virales, ya que es el único método que distingue claramente el compromiso de la sustancia blanca.