Anterior horn cell degeneration in polymyositis associated with human T lymphotropic virus Type-1 in patients from Barbados

Anterior horn cell degeneration has only ocassionally been noted in patients with tropical spastic paraparesis associated with human T lymphotropic virus type-1 (HTLV-1) infection. We report on three adult patients with HTLV-1-associated polymyositis who had clinical evidence of anterior horn cell degeneration. One patient had moderate proximal weakness and muscle wasting in all four limbs, while two had mild upper limb weakness with more profound proximal weakness and wasting in the lower limbs. In all three patients, elctromyographic findings were compatible with motor unit loss and muscle biopsies showed mononuclear inflammatory cell infiltration; muscle cell biopsies in two patients showed features of denervation. Immunoglobulin G (IgG) antibodies to HTLV-1 were detected by enzyme-linked immunosorbent assay (ELISA) and confirmed by Western immunoblot in serum and cerobrospinal fluid in all three patients. In two, cell cultures were established from peripheral blood lymphocytes and HTLV-1 antigen was identified by immunofluorescence and the ELISA antigen-capture technique using an anti-p19 HTLV-1 mouse monoclonal antibody. The three cases illustrate the variety of neuromuscular disease, other than spastic paraparesis, that may occur in HTLV-1 infection. In some cases of HTLV-1-associated polymyositis, anterior horn cell degeneration may make a significant contribution to the muscle atrophy observed. (AU)

Anterior horn cell degeneration in HTLV-1 associated polymyositis in patients from Barbados - abstract

Anterior horn cell degeneration has only been noted occasionally in patients with tropical spastic paraparesis associated with human T-lymphotropic virus type I infection (HTLV-I). We report on three adult patients with HTLV-I associated polymyositis who had evidence of anterior horn cell degeneration. One patient had moderate proximal weakness in all 4 limbs, while 2 had mild upper limb weakness and profound proximal weakness in the lower limbs. Electromyographic findings indicated motor unit loss. Muscle biopsies in 2 patients showed features of denervation, as well as mono-nuclear inflammatory cell infiltration. HTLV-1 IgG antibodies were detected by enzyme-linked immunosorbent assay, and confirmed by Western-immunoblot, in serum and cerebrospinal fluid in all 3 patients. In two, cell cultures were established from peripheral blood lymphocytes and HTLV-1 antigen was identified by immunofluorescence and the ELISA antigen capture technique, using an anti-p 19 HTLV-1 mouse monoclonal antibody. These 3 cases illustrate the variety of neuromuscular disease, other than spastic paraparesis, that may occur in HTLV-1 infection. Anterior horn cell degeneration may coexist with HTLV-1 associated polymyositis and may make a significant contribution to the muscle atrophy observed in these cases (AU)