Assuntos
Neuropatias Amiloides Familiares/complicações , Transtornos de Deglutição/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Humanos , Imunoglobulinas/sangue , Masculino , Pessoa de Meia-Idade , Pré-Albumina/metabolismo , Proteína Amiloide A Sérica/metabolismoRESUMO
Human T-cell lymphotropic virus (HTLV)-1-associated myelopathy (HAM) is well described. Clinical features are predominantly consistent with cord pathology, though imaging and autopsy studies also demonstrate brain inflammation. In general, this is subclinical; however, six cases have previously been reported of encephalopathy in HTLV-1-infected patients, without alternative identified aetiology. We describe three further cases of encephalitis in the UK HAM cohort (n = 142), whereas the annual incidence of acute encephalitis in the general population is 0.07-12.6 per 100,000. Clinical features included reduced consciousness, fever/hypothermia, headaches, seizures, and focal neurology. Investigation showed: raised CSF protein; pleocytosis; raised CSF:peripheral blood mononuclear cell HTLV-1 proviral load ratio; and MRI either normal or showing white matter changes in brain and cord. Four of the six previous case reports of encephalopathy in HTLV-infected patients also had HAM. Histopathology, reported in three, showed perivascular predominantly CD8+ lymphocytic infiltrates in the brain. One had cerebral demyelination, and all had cord demyelination. We have reviewed the existing six cases in the literature, together with our three new cases. In all seven with HAM, the spastic paraparesis deteriorated sub-acutely preceding encephalitis. Eight of the nine were female, and four of the seven treated with steroids improved. We propose that HTLV-associated encephalopathy may be part of the spectrum of HTLV-1-induced central nervous system disease.
Assuntos
Encefalopatias/etiologia , Encefalite/complicações , Infecções por HTLV-I/complicações , Adolescente , Adulto , Idoso , Encéfalo/diagnóstico por imagem , Encéfalo/virologia , Encefalopatias/diagnóstico por imagem , Encefalopatias/virologia , Encefalite/diagnóstico por imagem , Encefalite/virologia , Feminino , Vírus Linfotrópico T Tipo 1 Humano/patogenicidade , Humanos , Leucócitos Mononucleares/patologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Medula Espinal/diagnóstico por imagem , Medula Espinal/virologia , Linfócitos T/patologia , Carga ViralRESUMO
A myelodysplastic syndrome (MDS) was suspected in a middle-aged man who presented with neutropenia and macrocytosis. The correct non-neoplastic diagnosis was not made for 5 years. It is of crucial importance to exclude treatable causes of cytopenia and dysplasia when MDS is suspected.