RESUMEN
Neuromyelitis optica (NMO) is an inflammatory demyelinating disease with a poor prognosis that is characterized by inflammatory optic neuritis and myelitis. Although it is commonly misdiagnosed as multiple sclerosis (MS), distinguishing NMO from MS is important, as therapeutic approaches approved for MS are ineffective in patients with NMO. The aquaporin-4 (AQP4) antibody is a pathogenic and diagnostic biomarker for NMO. We report an AQP4 antibody-positive 9-year-old female with intractable hiccups and nausea (IHN). Brain imaging revealed lesions in the brainstem, thalami, and hypothalamus. Nevertheless, she had no clinical or radiological signs referable to the optic nerve or spinal cord. We propose that in patients with characteristic IHN associated lesions involving the brainstem or hypothalamus, measurement of AQP4 antibody should be considered for selectivity of treatment, even if the patient has no optic nerve or spinal cord lesions.
Asunto(s)
Acuaporina 4/inmunología , Autoanticuerpos/inmunología , Encéfalo/patología , Hipo/inmunología , Náusea/inmunología , Autoanticuerpos/sangre , Autoantígenos/inmunología , Niño , Femenino , Humanos , Mielitis/patología , Nervio Óptico/patología , Neuritis Óptica/patología , Médula Espinal/patologíaRESUMEN
We report for the first time the single photon emission computed tomography (SPECT) findings of a patient with clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) associated with Kawasaki disease, which showed hypoperfusion of the bilateral cingulate gyri, thalamus, basal ganglia, brainstem, and cortex of the frontal lobes. These findings indicate that the pathogenesis of MERS is based on cerebral hypoperfusion due to vasculitis or cerebrovascular dehydration.
Asunto(s)
Cuerpo Calloso/diagnóstico por imagen , Encefalitis/diagnóstico por imagen , Síndrome Mucocutáneo Linfonodular/diagnóstico por imagen , Circulación Cerebrovascular/fisiología , Niño , Encefalitis/etiología , Femenino , Humanos , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/diagnóstico , Tomografía Computarizada de Emisión de Fotón ÚnicoRESUMEN
BACKGROUND: Dengue virus (DV) infection is one of the most important mosquito-borne diseases in the tropics. Recently, the severe forms, dengue hemorrhagic fever (DHF) and dengue shock syndrome (DSS), have become the leading cause of death among children in Southern Vietnam. Protective and/or pathogenic T cell immunity is supposed to be important in the pathogenesis of DHF and DSS. METHODOLOGY/PRINCIPAL FINDINGS: To identify HLA alleles controlling T cell immunity against dengue virus (DV), we performed a hospital-based case control study at Children's Hospital No.2, Ho Chi Minh City (HCMC), and Vinh Long Province Hospital (VL) in Southern Vietnam from 2002 to 2005. A total of 211 and 418 patients with DHF and DSS, respectively, diagnosed according to the World Health Organization (WHO) criteria, were analyzed for their characteristic HLA-A, -B and -DRB1 alleles. Four hundred fifty healthy children (250 from HCMC and 200 from VL) of the same Kinh ethnicity were also analyzed as population background. In HLA class I, frequency of the HLA-A*24 showed increased tendency in both DHF and DSS patients, which reproduced a previous study. The frequency of A*24 with histidine at codon 70 (A*2402/03/10), based on main anchor binding site specificity analysis in DSS and DHF patients, was significantly higher than that in the population background groups (HCMC 02-03 DSS: OR = 1.89, P = 0.008, DHF: OR = 1.75, P = 0.033; VL 02-03 DSS: OR = 1.70, P = 0.03, DHF: OR = 1.46, P = 0.38; VL 04-05 DSS: OR = 2.09, P = 0.0075, DHF: OR = 2.02, P = 0.038). In HLA class II, the HLA-DRB1*0901 frequency was significantly decreased in secondary infection of DSS in VL 04-05 (OR = 0.35, P = 0.0025, Pc = 0.03). Moreover, the frequency of HLA-DRB1*0901 in particular was significantly decreased in DSS when compared with DHF in DEN-2 infection (P = 0.02). CONCLUSION: This study improves our understanding of the risk of HLA-class I for severe outcome of DV infection in the light of peptide anchor binding site and provides novel evidence that HLA-class II may control disease severity (DHF to DSS) in DV infection.