No abstract

Human T-cell lymphotropic virus type I (HTLV-I) associated myelopathy/tropical spastic paraparesis (HAM/TSP) is the most common chronic myelopathy in Brazil. We present the case of a 53 year old man that fulfilled the diagnostic criteria for HAM/TSP but had at the magnetic resonance imaging (MRI) of the spinal cord evidences of syringohydromyelia at the C6-C7 and D2-D7 levels along with Chiari type I malformation. The clinical picture was more typical of HAM/TSP than of syringohydromyelia, which was probably asymptomatic. The present case clearly demonstrates that serology and neuroimaging should be always used together. We conclude that, specially in places where HTLV-I is endemic, every patient with a spastic paraparesis, even with a radiological picture suggestive of a structural spinal cord lesion, should have a screening test for HTLV-I. The clinical picture must dictate the final direction of the diagnosis.

As doenças por prions (proteínas, Isentas de ácido nucleic°, corn capacidade auto-replicathm) são um grupo de enfermidades cainicas, progressives e fatais do sistema nervoso, causadas por agente transmissivel com propriedades físicas, químicas e biológicas não convencionais. Essas doenças, ao contrário da maioria das enfermidades existentes, podem ser esporádicas, transmissíveis ou genéticas. As principals doenças humanas causadas por prlons ado o Kuru, a doença de Creutzfeldt-Jakob e suas variantes, a doença de GerstmannStráussler-Scheinker e a insônia familiar fatal. Neste artigo revilem-se os principals - e mais recentes - aspectos etiopatogênicos, aruitomopatológicos, clínicos e terapêuticos acerca deste Importante grupo de enfermidades neurodegenerativas.