[Cases of tropical spastic paraparesis in Spain].
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A retrovirus, human T cell lymphotropic virus type 1 (HTLV-1), is an essential but not a sufficient aetiologic factor for tropical spastic paraparesis (TSP). Because some TSP patients have biological false positive tests for treponemal infections (BFP-STS), we used ELISA to study BFP-STS and anticardiolipin antibodies in 42 Jamaican TSP patients. The data indicate that in TSP anticardiolipin antibodies occur in about 26% of patients, are associated with biological false positive treponemal serology, are relatively restricted to the IgA isotype and may be induced by HTLV-1 or other nontreponemal infections.
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We report a 59-year-old woman with human T-cell lymphotrophic virus type-I (HTLV-I) associated myelopathy/tropical spastic paraparesis who showed high signal in the cervical and thoracic spinal cord on T2-weighted and contrast enhancement on T1-weighted images.
OBJECTIVE: To present the first documented case of human T-lymphotropic virus type I (HTLV-I) associated myelopathy/tropical spastic paraparesis in the Australian population. CLINICAL FEATURES: A 31-year-old Aboriginal man with an 18-month history of progressive weakness of the legs was found to have an upper motor neurone weakness of all limbs associated with sphincteric disturbance and impotence. HTLV-I antibodies were detected in his serum and no other cause for the patient's myelopathy could be found. INTERVENTION AND OUTCOME: He was counselled regarding HTLV-I associated myelopathy/tropical spastic paraparesis. CONCLUSION: This is the first description of HTLV-I associated myelopathy/tropical spastic paraparesis in an Australian. In cases of spinal cord disorder without evidence of compression we recommend serological testing for HTLV-I, especially in Aboriginal patients. Additionally, testing of blood donors for this retrovirus needs consideration.
Human T lymphotropic virus type I (HTLV-I) causes adult T cell leukemia/lymphoma and a chronic neurological disease called either tropical spastic paraparesis (TSP) or HTLV-I-associated myelopathy. The different outcomes of this infection could be due to both host and viral factors and it has been proposed that genetic differences could make some HTLV-I strains neurotropic. In this paper, we examined the pattern of tissue-specific expression determined by a long terminal repeat (LTR) obtained from a case of TSP. We constructed transgenic mice in which this LTR controlled the expression of the nlslacZ reporter gene. We observed that in three independent lines of transgenic mice, the reporter gene was expressed predominantly in the central nervous system (CNS), in choroid plexus, and in cells of the hippocampus and cerebellum. Our observations indicate the existence of CNS cells permissive for the expression of HTLV-I and which may be of importance in the pathogenesis of TSP.
An association between HTLV-1 infection and infective dermatitis (ID) a relapsing eczematous condition of Jamaican children, was reported in 1990. These patients are at risk of developing other known HTLV-1 related diseases. We have observed the development of HTLV-1 associated myelopathy/tropical spastic paraparesis in two patients, ages 14 and 35 years, who were diagnosed with ID at ages 2 and 10 years, respectively. Infective dermatitis of children serves as an early marker of HTLV-I infection and may predict later development of either the malignant outcome, adult T-cell leukaemia/lymphoma or the neurologic manifestation HAM/TSP among adult carriers of HTLV-1 infection.
HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) has been described in practically all regions of Brazil. The authors present eight clinically defined cases of HAM/TSP, as being the first reported in Sergipe (Northeastern Brazil). All of them were confirmed through ELISA in two examinations, although only two were confirmed by Western Blot. According to clinical/laboratorial investigation protocol, all patients presented involvement of the pyramidal tract with minimal sensory loss and sphincter alteration. The authors call the attention for the endemicity of HTLV-I in the region, whose prevalence amongst blood donors is significant (0.43%).
We report on a neuropathological analysis of two cases of TSP/HAM originating from Brazil. These two cases had, respectively, an evolution of 13 and 40 years. The main neuropathological findings consisted of spinal cord atrophy, mainly the lower thoracic cord, diffuse degeneration of the white and grey matter, rare foci of mononuclear and perivascular cuffs, and hyaline hardening of arteriolae. The supraspinal structures were normal, excepting for a slight gliosis in the cerebellum. An analysis on the long evolutive cases as described in the literature is outlined in this study.
During a field study in the Seychelles Islands, 19 patients with tropical spastic paraparesis (TSP) were evaluated electrophysiologically. Methods of assessment included motor and sensory nerve conduction studies, electromyography, and analysis of the somatosensory evoked potentials after stimulation of the tibial and median nerves. The results showed that the most prominent feature of the disease, a spastic paraparesis, is accompanied by subclinical involvement of spinal sensory pathways and a comparatively minor peripheral sensorimotor polyneuropathy.
The clinical spectrum of retroviruses is expanding rapidly. Human T-cell lymphotropic virus type I (HTLV-I) was the first retrovirus to be described, and its role had been established in adult T-cell leukemia/lymphoma and tropical spastic paraparesis. We report the case of a 35-year-old woman with HTLV-I and the unusual combination of cutaneous T-cell lymphoma, tropical spastic paraparesis, cerebral vasculitis, and protein S deficiency. We discuss the relationship of all her diseases to HTLV-I.
INTRODUCTION: Chronic idiopathic spastic paraparesis (CHISPA) have been associated to HTLV-I virus infection, and unknown environmental factors have been suggested to play a role in its pathogenesis. METHOD: Based in our previous studies we wanted to know if the lack of refrigerators and the mycotoxin contamination of food might have any relationship with the presence of CHISPA in patients living at southeastern Colombia. RESULTS: Interestingly only four out of 15 patients with CHISPA had some method of refrigeration, in, 14 out of these 15 patients at least a metabolite of Fusarium was detected. CONCLUSIONS: The fact that mycotoxins found in the urine samples of these patients are immunosuppressors and demyelinating allow us to suggest that these toxins could be the most important environmental cofactors leading to the susceptible people to the clinically established disease.
Ultrastructural studies on cell cultures derived from TSP/HAM and ATL patients, show the presence of large quantities of HTLV-I viral particles in extracellular spaces and budding at the cytoplasmic membrane. In addition, mature enveloped particles and images of endopinocytosis of virions are seen in the cytoplasm vacuoles suggesting the existence of a reinfection phenomenon. In this context, we decided to investigate some features of the replicative cycle, in particular the synthesis of unintegrated proviral forms. To increase the sensitivity of detection, we applied a procedure which combines the electrophoretic separation of closed circular forms and PCR amplification. By this procedure we produced evidence for the existence of supercoiled HTLV-I DNA in established cell lines from TSP/HAM and ATL and in patients peripheral blood mononuclear cells. These HTLV-I unintegrated proviral forms may play an important role in the physiopathology of HTLV-I associated diseases. Preliminary results of AZT/interferon treatment in ALT patients are largely superior to chemotherapy. The therapeutic effect of AZT, it known inhibitor of reverse transcriptase, may be through its inhibition of the synthesis of HTLV-I unintegrated proviral DNA.