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)

HTLV-1 polymyositis

The case is described of an HTLV-1 seropositive Jamaican woman who presented with signs and symptoms of polymyositis and myelopathy. A muscle biopsy showed features of myositis with a mononuclear inflammatory infiltrate, variation in fibre size and evidence of regeneration. Immunocytochemistry showed the mononuclear cells were composed of macrophages and T-lymphocytes suggesting a cell-mediated response. Multiplex PCR demonstrated the presence of the HTLV-I tax gene within the muscle. (AU)

Human T cell lymphotropic virus (HTLV-1): cutaneous manifestations

Skin manifestations are a common feature of HTLV-1 associated disorders and of HTLV-1 infection itself. These include the lymphomatous skin infiltrates in adult T-cell lymphoma/leukaemia, most commonly manifesting as persistent, generalised papules, nodules and plaques with later ulceration, acquired ichthyosis and xeroderma in HAM/TSP, infective dermatitis of children, dermatomyositis, crusted (Norwegian) scabies, psoriasiform rashes which may precede one of the more serious disease associations, and possibly also seborrhoeic dermatitis. Disorders typically associated with immunosuppression such as disseminated herpes zoster, and ulcerative non-healing herpes simplex may also be seen occasionally both in ATK as well as in other wise asymptomatic HTLV-1 infection (AU)

The echocardiographic features of polymyositis - abstract

Two dimensional echocardiography with doppler studies were performed in 30 patients at the UWI with the diagnosis of dermatomyositis/polymyositis established by the usual clinical and investigative criteria. This was done in order to elucidate the echocardiographic features of this condition which had not been clearly defined before. There were 25 females and 5 males, age range 28-65 years (mean - 48 years), with the majority of patients (70 percent) being in the 41 - 70 year age group. No valvular abnormalities were observed, and left ventricular internal dimension in systole LVIDs-1.9-3.7 cm, mean-2.8cm) and contractility (ejection fraction - range 43 - 89 percent, mean - 69 percent) were normal. No regional wall motion abnormalities were observed. Only 3 patients (10 percent) had evidence of global reduction in systolic function. There was hypertrophy of the interventricular septum in 9 patients (30 percent), and in the left ventricular posterior wall in 6 patients (20 percent). The dimensions of the left atrium (2.6-3.7 cm; mean - 3.2 cm), aorta (2.5-3.6 cm; mean 3.2 cm) and right ventricle (range 1.6-2.5 cm; mean - 2.1 cm) were normal. Doppler studies did not reveal any significant valvular regurgitation, and pulmonary artery velocities documented normal mean pulmonary artery pressures (mean 18 mm Hg) in all patients. Left ventricular diastolic function as indicated by E/A ratios (0.55-1.39;mean-1.23) from mitral valve inflow studies was normal. Conclusion: Echocardiographic doppler studies in patients with dermatomyositis/polymyositis were largely normal apart from minor degrees of left ventricular hypertrophy in a minority of patients (AU)

Identification of human T-cell leukemia/lymphoma virus type I antibodies, DNA, and protein in patients with polymyositis

OBJECTIVE: To investigate a possible association between human T cell leukemia/lymphoma virus type I (HTLV-I) and polymyositis (PM). METHODS: Sera and muscle biopsy samples from 9 Jamaican PM patients were compared with specimens from American HTLV-I positive PM patients and normal controls. Sera were evaluated for HTLV antibodies by enzyme-linked immunosorbent assay and Western blot. The biopsy samples were analyzed for HTLV-I/II DNA by polymerase chain reaction and were also immunohistochemically stained for HTLV gp46 envelope protein. RESULTS: Seven of the 8 Jamaican PM patients from whom sera were available were HTLV-I seropositive. The muscle biopsies of all 9 Jamaican patients demonstrated severe lymphocytic infiltration, cellular degeneration, myofiber atrophy, and fibrosis. Each muscle biopsy specimen contained HTLV-I DNA. Two of 6 samples demonstrated intense staining for HTLV-I gp46 in many of the invading mononuclear cells and weak staining for HTLV-I gp46 in many of the other specimens were weakly positive for gp46 in rare mononuclear cells. All controls specimens were negative for the presence of HTLV-I DNA and protein. CONClUSION: HTLV-I is associated with an inflammatory muscle disease characterized by direct invasion of the affected muscle by HTLV-I-infected mononuclear cells.(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)

Seroprevalence of HTLV-1 in chronic patients in Jamaica

A high seropositivity rate of human T cell lymphotropic virus type 1 (HTLV-1) infection was found in Jamaican patients with chronic diseases. However, except for tropical spastic paraparesis, polymositis, adult T cell leukaemia/lymphoma and polyneuropathies of undetermined cause, HTLV-1 seropositivity rates in chronic disease patients were not significantly different from that found in healthy Jamaicans. These results indicate that there is no increased risk of HTLV-1 infection or HTLV-1 associated disease in patients with chronic disease compared to the general Jamaican population. The association of unclassified polyneuropathies with HTLV-1 reported herein is a novel one which requires further studies to elucidate its nature.(AU)