HTLV II is a retrovirus endemic in some Amerindian tribes and spread worldwide with a high prevalence among intravenous drug abusers. It has three different genetic subtypes a, b, and d, defined mainly by the long terminal repeat (LTR) region. HTLV II has been associated with a neurodegenerative disease in few cases. We describe the first well-documented case in Brazil where the virus is endemic in isolated ethnic groups. The patient is a 55-year-old woman with a chronic and painful syndrome characterized by spastic paraparesis, hyperactive reflexes and spastic bladder. Somatosensory evoked potential indicates a thoracic spinal cord lesion. Computer tomography showed periventricular demyelination. Enzyme-linked immunosorbent assay was positive for HTLV I/II whereas the discriminatory Western blot was indeterminate. Molecular analysis of the Tax region revealed a HTLV II pattern that was also confirmed through sequencing the LTR region. Phylogenetic analysis of the LTR sequence shows an HTLV IIa subtype that clustered with the virus isolated from Kayapo Indians and Brazilian urban intravenous drug users. Indeterminate Western blots are frequently found using commercial kits, therefore we recommend that all cases in which a myelopathy is associated with an indeterminate serological result should be evaluated by PCR to determine the actual number of HTLV II associated myelopathy cases.

The frequency and importance of dysautonomia in human T-cell lymphotrophic virus type I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) have not been fully investigated. We describe the characteristics of dysautonomia in such patients in a case-control study. Our results indicate that autonomic disturbances are more frequent in HAM/TSP than has been previously suggested, with a predominance of sympathetic nervous system dysfunction. In some of these patients, the symptoms may be severe enough to warrant specific treatment.

SUMMARY -H T L V -I (Human T-lymphotropic virus type I ) associated myelopathy/tropical spastic paraparesis ( H A M / T S P ) is an immunomediated myelopathy induced by the H T L V -I .Some patients, specially those from Japan, seem to have a good response to steroid treatment. However, this has not been found in other regions of the world. High dose intravenous methylprednisolone has been used with success in patients with relapses of multiple sclerosis (MS), another autoimmune disease of the central nervous system. To test the effectiveness of methylprednisolone in patients with H A M / T S P , we devised an open trial in 23 patients. W e found a very limited benefit of this form of treatment in these patients. Only one patient, who had the shortest disease duration (five months) in the whole group, showed a sustained benefit. W e speculate that those patients with a shorter history, with presumably less demyelination and more inflammatory lesions, would show a better response to immunossupressive treatments. K E Y W O R D S : H T L V -I , tropical spastic paraparesis, methylprednisolone. Metilprednisolona endovenosa na mielopatia associada ao HTX.V-1/Paraparesia Espástica Tropical (MAH/PET) RESUMO -A mielopatia associada ao protovírus T-linfotrópico humano ( H T L V -I ) , também conhecida como paraparesia espástica tropical associada ao H T L V -I ( M A H / P E T ) , constituienfermidade imunomediada desencadeada pela infecção pelo H T L V -I . Nesta condição tem sido demonstrada, particularmente em pacientes japoneses, boa resposta clínica à terapêutica com cor ticos teróid es. Este efeito benéfico todavia não foi encontrado em todas as regiões do mundo. Pulsoterapia com metilprednisolona endovenosa tem sido utilizada com sucesso em pacientes com esclerose múltipla, outro exemplo de doença auto-imune do sistema nervoso central, especialmente durante as fases de exacerbação da doença. Objetivando testar a eficácia da pulsoterapia com metilprednisolona em pacientes com M A H / P E T , conduzimos estudo aberto em 23 doentes. Não constatamos efeito benéfico significativo desta forma de tratamento na maioria dos enfermos estudados. Apenas um dos pacientes, o qual exibia o menor tempo de duração de doença (cinco meses), obteve benefício a longo prazo. Acreditamos que tratamentos imunossupressivos devam ser de maior utilidade naqueles doentes com menor tempo de evolução, nos quais, possivelmente, há preponderância do processo inflamatório sobre o desmielinizante. P A L A