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Subacute progression of HTLV-1 associated myelopathy/tropical spastic paraparesis in Peru: a report of 20 cases

Background

The course of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is most frequently insidious. However, the disease evolves rapidly in a subgroup of patients. The purpose of this study is to better characterize subacute HAM/TSP.

Methods

We describe a series of cases with subacute progression of HAM/TSP from the HTLV-1 cohort at the Institute of Tropical Medicine Alexander von Humboldt in Lima. The diagnosis of HAM/TSP was based on WHO criteria. We used the IPEC (Insituto de Pesquisa Clinica Evandro Chagas) disability scale and the EDSS (Expanded Disability Status Scale) to evaluate HAM/TSP progression. Subacute progression was defined as the inability to walk unaided within two years after onset of lower limb symptoms. The HTLV-1 provirus load (PVL) was determined by SYBR-Green-based real-time quantitative PCR, using human endogenous retrovirus-3 as an internal reference. The PVL was expressed as the number of HTLV-1 copies per 10,000 peripheral blood mononuclear cells.

Results

In 2005–2008, 164 patients with HAM/TSP underwent neurological evaluation. Twenty subjects (12%) presented subacute progression of HAM/TSP. The mean age at disease onset was 45 years (standard deviation (SD) 16; range 12–67); 9/20 (45%) were men. Among 144 subjects with slow progression, the mean age at disease onset was 42 (SD: 15; T-test P = 0.4); and 27 were men (19%; fisher's exact test: P = 0.02). Among those with subacute progression, the time to maximum disability ranged from 1 week to 24 months (mean: 10 months). Symptoms at disease onset were lumbar pain and weakness (15/20), sensory (3/20) and urinary symptoms (2/20). The mean EDSS score was 6.7 (range: 5–8.5) and the mean IPEC score was 20.25 (range: 15–29). After the initial, rapidly progressive phase, 9/20 patients were wheelchair-bound, 7/20 needed two walking-sticks, and 4/20 presented variable remission. Thoracic spinal cord magnetic resonance imaging (MRI) was performed in 14/20. In four subjects, there was hyperintensity in T2 with gadolinium enhancement; in one subject there was atrophy. The remaining MRIs were normal. Cyto-biochemical evaluation of cerebrospinal fluid was done in five subjects, no abnormalities were found. PVL results were available in 7 subjects with subacute progression (mean: 3135; SD: 1482), and 54 subjects with slow progression (mean: 3337; SD: 1896; T-test: P = 0.8).

Conclusion

By contrast with other reports, subacute HAM/TSP progression was associated with male sex, but not with provirus load in this setting. The outcome of subacute HAM/TSP is variable, and remission occurs occasionally.

Author information

Correspondence to Martin Tipismana.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Keywords

  • Disease Onset
  • Expand Disability Status Scale
  • Slow Progression
  • Provirus Load
  • Neurological Evaluation