CLINICAL AND NEUROPATHOLOGICAL STUDY OF 6 PATIENTS WITH SPASTIC PARAPARESIS ASSOCIATED WITH HTLV-I - AN AXOMYELINIC DEGENERATION OF THE CENTRAL-NERVOUS-SYSTEM

Between 1990 to 1994, 6 TSP/HAM patients, 3 women and 3 men with an av erage age of 57.1 years (39 to 76 years old), who died in the Salvador Hospital were submitted to postmortem examination. The mean time of p araparesis was 7 years (3 to 17 years), and 2 patients had pseudobulba r signs. Three cases had macroscopic atrophy of the spinal cord. Histo logically, all cases had lesions in the pyramidal tracts and 4 cases s howed somatotopic lesions of the Goll's tracts which followed a ''dyin g back'' ascendant and descendant distribution, respectively. In 2 cas es, both of which had intellectual impairment, demyelination of the su bcortical and parathalamic areas was observed without U fiber involvem ent. Abnormal vessels with gross thickening of the adventitia, many of them with lymphocytic cuffs, were seen everywhere, especially in the spinal cord, brain stem, midbrain and meninges, but no relation betwee n these findings and the parenchymal lesions was observed. Also, in th e cases with posterior column involvement, neuronal changes and prolif eration of satellite cells in the dorsal ganglia were found. All cases showed histological sialoadenitis and none had inflammatory muscle ch anges. We conclude that the lesions affected the neuraxis in a systemi c axial fashion as in degenerative diseases, and did not seem to be se condary to vascular or inflammatory abnormalities.