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Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis

Background: Vertical transmission of HTLV-1 could lead to the early development of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). This significantly affects quality of life and increases morbimortality. Objective: To describe the epidemiological and clinical characteristics of...

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Autores principales: Schwalb, Alvaro, Pérez-Muto, Valeria, Cachay, Rodrigo, Tipismana, Martín, Álvarez, Carolina, Mejía, Fernando, González-Lagos, Elsa, Gotuzzo, Eduardo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7350296/
https://www.ncbi.nlm.nih.gov/pubmed/32517313
http://dx.doi.org/10.3390/pathogens9060450
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author Schwalb, Alvaro
Pérez-Muto, Valeria
Cachay, Rodrigo
Tipismana, Martín
Álvarez, Carolina
Mejía, Fernando
González-Lagos, Elsa
Gotuzzo, Eduardo
author_facet Schwalb, Alvaro
Pérez-Muto, Valeria
Cachay, Rodrigo
Tipismana, Martín
Álvarez, Carolina
Mejía, Fernando
González-Lagos, Elsa
Gotuzzo, Eduardo
author_sort Schwalb, Alvaro
collection PubMed
description Background: Vertical transmission of HTLV-1 could lead to the early development of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). This significantly affects quality of life and increases morbimortality. Objective: To describe the epidemiological and clinical characteristics of patients with early-onset HAM/TSP, defined as disease onset before 20 years of age. Methods: This is a retrospective study from an HTLV-1 clinical cohort between 1989 and 2019. We searched for medical records of patients with (1) diagnosis of HTLV-1 infection using two ELISA and/or one Western blot, (2) clinical diagnosis of HAM/TSP by neurological assessment, and (3) HAM/TSP symptom-onset before 20 years of age. Results: A total of 38 cases were identified in the cohort; 25 were female (66%). The median age of onset was 14 years old. 31 (82%) cases had HTLV-1 testing done among family members; 22 out of 25 tested mothers (88%) were HTLV-1 positive. Most patients (27/34) were breastfed for more than one year. Disease progression measured through EDSS and IPEC-1 showed an upward trend towards worsening spasticity with 18 patients (47%) eventually requiring mobility aids. Conclusions: Cases of early-onset HAM/TSP are not of rare occurrence, which translates into many more years of dependency, the use of mobility aids, and increased overall morbidity.
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spelling pubmed-73502962020-07-15 Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis Schwalb, Alvaro Pérez-Muto, Valeria Cachay, Rodrigo Tipismana, Martín Álvarez, Carolina Mejía, Fernando González-Lagos, Elsa Gotuzzo, Eduardo Pathogens Article Background: Vertical transmission of HTLV-1 could lead to the early development of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). This significantly affects quality of life and increases morbimortality. Objective: To describe the epidemiological and clinical characteristics of patients with early-onset HAM/TSP, defined as disease onset before 20 years of age. Methods: This is a retrospective study from an HTLV-1 clinical cohort between 1989 and 2019. We searched for medical records of patients with (1) diagnosis of HTLV-1 infection using two ELISA and/or one Western blot, (2) clinical diagnosis of HAM/TSP by neurological assessment, and (3) HAM/TSP symptom-onset before 20 years of age. Results: A total of 38 cases were identified in the cohort; 25 were female (66%). The median age of onset was 14 years old. 31 (82%) cases had HTLV-1 testing done among family members; 22 out of 25 tested mothers (88%) were HTLV-1 positive. Most patients (27/34) were breastfed for more than one year. Disease progression measured through EDSS and IPEC-1 showed an upward trend towards worsening spasticity with 18 patients (47%) eventually requiring mobility aids. Conclusions: Cases of early-onset HAM/TSP are not of rare occurrence, which translates into many more years of dependency, the use of mobility aids, and increased overall morbidity. MDPI 2020-06-07 /pmc/articles/PMC7350296/ /pubmed/32517313 http://dx.doi.org/10.3390/pathogens9060450 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Schwalb, Alvaro
Pérez-Muto, Valeria
Cachay, Rodrigo
Tipismana, Martín
Álvarez, Carolina
Mejía, Fernando
González-Lagos, Elsa
Gotuzzo, Eduardo
Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis
title Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis
title_full Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis
title_fullStr Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis
title_full_unstemmed Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis
title_short Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis
title_sort early-onset htlv-1-associated myelopathy/tropical spastic paraparesis
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7350296/
https://www.ncbi.nlm.nih.gov/pubmed/32517313
http://dx.doi.org/10.3390/pathogens9060450
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