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1/23. Recurrent encephalopathy and seizures in a US native with HTLV-I-associated myelopathy/tropical spastic paraparesis: a clinicopathologic study.

    A patient with progressive spastic paraparesis originally ascribed to multiple sclerosis developed recurrent encephalopathy and seizures. A diagnosis of HTLV-I-associated myelopathy/tropical spastic paraparesis was established prior to death. autopsy confirmed chronic inflammatory myelopathy and active inflammation in the white matter of the temporal lobes.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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2/23. High level of HTLV-I specific protein expression in a patient with adult T-cell leukemia, chronic progressive myelopathy and Kaposi's sarcoma.

    Analysis was made of serum anti-htlv-i antibodies, virus-specific proteins in peripheral blood lymphocytes (PBL) and proviruses in lymphocyte dna of a patient with adult T-cell leukemia (ATL), Kaposi's sarcoma, and chronic myelopathy. Using Western blot and PCR (with hiv-1 specific primers), it was shown that Kaposi's sarcoma was not linked to HIV infection. Western blot analysis of serum revealed antibodies against p19, p24 and Pr 53 of HTLV-I. Examination of proteins in fresh PBL by Western blot revealed a high level of HTLV-I specific protein expression. Southern blot analysis of the patient's dna revealed two different sites for HTLV-I provirus integration.
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ranking = 222.97845824591
keywords = chronic progressive
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3/23. Differential diagnosis of HTLV-I-associated myelopathy and multiple sclerosis in Iranian patients.

    Two Iranian patients with chronic progressive spastic paraparesis and urinary dysfunction were referred to our hospital with the presumptive diagnosis of multiple sclerosis (MS). Routine CSF analysis and magnetic resonance imaging of the two patients were only partially characteristic of MS. Testing for antibodies to human T-cell leukemia virus type I [HTLV-I] in serum using a radioimmune precipitation assay revealed antibodies to HTLV-I in both patients. The infection with HTLV-I was confirmed by polymerase chain reaction (PCR) and liquid hybridization analysis using primers to the tax/rex region and a corresponding probe, demonstrating proviral dna in peripheral blood mononuclear cells of both patients. On the basis of these findings demonstrating the presence of proviral HTLV-I dna in the two Iranian patients, the initial diagnosis of MS was corrected to that of HTLV-I-associated myelopathy (HAM). In contrast, several patients with definite MS (nine from germany, two from iran) with a relapsing and remitting form of the disease were tested for HTLV-I infection by enzyme-linked immunosorbent assay and PCR, which yielded negative results. However, the mother of one HAM patient was found to be infected with HTLV-I. To support an association between HTLV-I infection and CNS disease in the two HAM patients, we analyzed the production of specific IgG antibodies within the CNS based on a simple enzyme immunoassay for viral IgG antibodies in CSF and serum. In the two HAM patients there was significant intrathecal antibody production directed against HTLV-I, but this was not found in any of the samples from MS patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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ranking = 60.744614561478
keywords = chronic progressive, multiple sclerosis, sclerosis
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4/23. Progressive spastic myelopathy in a patient co-infected with hiv-1 and HTLV-II: autoantibodies to the human homologue of rig in blood and cerebrospinal fluid.

    OBJECTIVE: Human T-cell leukemia virus types I (HTLV-I) and II (HTLV-II) are closely related human retroviruses. HTLV-I has been implicated in a chronic progressive myelopathy, known as tropical spastic paraparesis (TSP) or HTLV-I-associated myelopathy (HAM). We sought to determine whether autoantibodies to brain antigens were present in the cerebrospinal fluid (CSF) of a patient with chronic progressive spastic myelopathy with evidence of both hiv-1 infection and HTLV-I/II seropositivity. DESIGN: A 54-year-old bisexual man with clinical features of HAM/TSP of over 20 years' duration was followed. methods: We applied discriminatory dna amplification (polymerase chain reaction) to distinguish HTLV-I from HTLV-II and to verify co-infection with hiv-1. The patient's CSF was used to screen a human brain cDNA expression library to identify antibodies directed against brain antigens. Autoreactive bacteriophage clones were isolated and sequenced. RESULTS: The patient was found to be co-infected with both hiv-1 and HTLV-II, but not with HTLV-I. HTLV-II proviral levels in the peripheral blood remained relatively constant, despite therapy with zidovudine. Prominent oligoclonal banding of immunoglobulins was present in the patient's CSF. A single repeatedly reactive cDNA clone was identified, by screening with CSF antibody, sequenced, and found to be the human homologue of the rat insulinoma gene, rig. CONCLUSIONS: HTLV-II infection may predispose to development of a HAM/TSP-like illness. Autoimmune mechanisms, such as autoantibody formation, may play a role in pathogenesis.
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ranking = 111.48922912296
keywords = chronic progressive
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5/23. HTLV-1-associated myelopathy/tropical spastic paraparesis accompanied with psoriasis.

    Two adult females developed HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and psoriasis. Both showed chronic progressive paraparesis and sharply demarcated erythematous scaling plaques on their extremities and trunk. One patient had polymyositis while in the other anti-thyroid antibodies, antinuclear antibodies and SS-A antibody, all autoantibodies, were positive. Both patients were treated by intramuscular injections of interferon-alpha for 2 to 4 weeks, resulting in amelioration of paraparesis. After the therapy psoriasis and polymyositis markedly improved in one patient without any additional therapy, while in the other simultaneous use of topical corticosteroids was effective. This is the first report to describe occurrences of psoriasis in HAM/TSP patients. Although there are several reports indicating interferon-alpha induces or exacerbates psoriasis, our experience suggests that psoriasis associated with HAM/TSP can be successfully managed even during interferon-alpha therapy.
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ranking = 55.744614561478
keywords = chronic progressive
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6/23. Human T-lymphotropic virus type I-associated myelopathy in patients with the acquired immunodeficiency syndrome.

    We describe two cases of serologically confirmed human T-lymphotropic virus type I (HTLV-I)-associated myelopathy involving North American men coinfected by the human immunodeficiency virus type 1. Our first patient suffered from a gradually progressive spastic paraparesis for 10 years prior to presenting with Kaposi's sarcoma, while our second patient developed subacutely progressive spastic paraparesis in the setting of full-blown acquired immunodeficiency syndrome. autopsy examination of the spinal cords from these two cases revealed widespread axonal loss and demyelination principally involving the lateral columns of case no. 1 and the lateral and anterior columns of case no. 2. Vascular sclerosis and hyalinization were prominent in both cases, but in neither was there a conspicuous inflammatory component. In case no. 2, HTLV-I mRNA was not detected by in situ hybridization, but HTLV-I proviral dna sequences were detected in this case by polymerase chain reaction. Neither case exhibited multinucleated cell (human immunodeficiency virus type 1) myelitis, vacuolar myelopathy, or evidence of HTLV-II infection by polymerase chain reaction assay.
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ranking = 0.054583808071457
keywords = sclerosis
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7/23. Differentiation of HAM/TSP from patients with multiple sclerosis infected with HTLV-I.

    OBJECTIVE: To better differentiate patients with human T lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) from patients with multiple sclerosis (MS) who are HTLV-I seropositive, we compared the htlv-i antibodies and HTLV-I proviral dna loads in CSF and peripheral blood mononuclear cells (PBMC). methods: Intrathecal synthesis of htlv-i antibodies and HTLV-I proviral dna loads in CSF and PBMC were measured and compared in 39 Brazilian patients: 17 HAM/TSP and 22 HTLV-I-seropositive non-HAM/TSP (7 with other neurologic diseases, 11 asymptomatic carriers, and 4 HTLV-I-seropositive patients with an MS-like phenotype). In addition, we followed immunologic and virologic markers in comparison to the clinical course (by Kurtzke Expanded Disability Status Scale) of seven patients (five with HAM/TSP and two with an MS-like phenotype) for a mean period of 16 (SD /- 5) months. RESULTS: The proviral load in CSF and PBMC was higher in HAM/TSP than in non-HAM/TSP patients, except in the two HTLV-I-seropositive patients with an MS-like phenotype that also fulfilled the criteria for HAM/TSP. Higher HTLV-I proviral dna load in CSF was associated with the higher proviral dna load in PBMC and lower intrathecal synthesis of htlv-i antibodies. These laboratory findings remained stable during follow-up. CONCLUSION: The high proviral load in peripheral blood mononuclear cells or in CSF or both may be a good marker of human T lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and can differentiate patients with HAM/TSP from patients with multiple sclerosis infected with HTLV-I.
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ranking = 6
keywords = multiple sclerosis, sclerosis
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8/23. autopsy report of HTLV-I-associated myelopathy presenting with ALS-like manifestations.

    We report an autopsy case of HTLV-I-associated myelopathy (HAM) showing clinical features indistinguishable from amyotrophic lateral sclerosis (ALS). A Japanese man developed bulbar palsy and generalized neurogenic muscular atrophy with symmetrical hyperreflexia at the age of 57 and died 4.5 years after the onset. He had an increased titer of anti-htlv-i antibodies in serum and CSF. At autopsy, the leptomeninges were thickened and infiltrated with inflammatory cells. The brain and spinal cord were atrophic. The pyramidal tracts and anterior horn cells of spinal cord were severely degenerated but degenerative changes were also found in the basal ganglia, thalamus, brainstem tegmentum, and the dorsal columns of spinal cord. In all degenerative areas, prominent infiltrations of inflammatory cells were found. The present case indicates that HAM may produce clinical features indistinguishable from ALS.
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ranking = 0.054583808071457
keywords = sclerosis
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9/23. Pathological and molecular biological features of a myelopathy associated with HTLV-1 infection.

    We report the pathological and molecular biological findings of human T-cell lymphotropic virus type 1 (HTLV-1) infection of the spinal cord in a patient with a chronic progressive myelopathy. light microscopy disclosed loss of myelin and axons, thickening of blood vessels and a lymphocytic cell infiltrate in the spinal cord especially at the cervical and thoracic levels. Electron microscopy confirmed the vascular appearance seen with light microscopy but virus particles were not observed. The HTLV-1 gag gene could be amplified (by polymerase chain reaction) from cervical spinal cord tissue while not from elsewhere in the neuroaxis. The presence of HTLV-1 genomic material in spinal cord tissue has not been previously reported.
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ranking = 55.744614561478
keywords = chronic progressive
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10/23. Low HTLV-1 seroprevalence in endemic tropical spastic paraparesis in ethiopia.

    Tropical spastic paraparesis (TSP), a chronic progressive myelopathy, occurs in ethiopia in epidemic form as neurolathyrism, while the endemic form has remained obscure. We describe the clinical features of 22 patients with TSP and the results of screening for HTLV-1 in these patients, 26 patients with other neurological disorders, 14 patients with leukaemia and 66 blood donors. The major manifestations in the patients with TSP were weakness and spasticity of the lower limbs with upper motor neurone signs and minimal sensory loss and bladder dysfunction. Two patients with TSP (9%), 2 patients with other neurological disorders (7.7%) and one patient with leukaemia and deafness were seropositive for HTLV-1. All the 66 blood donors were seronegative. Our results suggest that HTLV-1 may not play a major role in the pathogenesis of TSP in ethiopia.
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ranking = 55.744614561478
keywords = chronic progressive
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