Distribución geográfica y tipo de infección del virus linfotrópico T humano en pacientes peruanos 2019-2021

En el presente estudio describimos y caracterizamos la distribución geográfica de los casos positivos confirmados a HTLV-1 y 2 de pacientes peruanos con diagnóstico presuntivo entre 2019 y 2021. De un total de 555 muestras positivas confirmadas, 546 (98,4%) fueron HTLV-1 y 9 (1,6%) HTLV-2. Además, 22 de 24 departamentos del Perú presentaron casos de HTLV-1, siendo los principales motivos de solicitud de confirmación diagnóstica: aspirante a donar sangre con prueba de tamizaje reactivo, sospecha de leucemia/linfoma y paraparesia espástica tropical. Los resultados reflejan que la identificación de los puntos críticos constituye una brecha persistente respecto al diagnóstico, siendo cruciales para reducir el número de nuevos casos en Perú.

In the present study we describe and characterize the geographic distribution of HTLV-1 and 2 positive cases from Peruvian patients with presumptive diagnosis 2019 - 2021. Of a total of 555 confirmed positive samples, 546 (98.4%) were HTLV-1 and 9 (1.6%) HTLV-2. In addition, 22 of 24 departments of Peru presented cases of HTLV-1. The main reasons for requesting a confirmatory diagnosis being: aspiring to donate blood with a reactive screening test, suspicion of leukemia/ lymphoma and tropical spastic paraparesis. The results reflect that the identification of critical points constitutes a persistent gap regarding the diagnosis, being crucial to reduce the number of new cases in Peru.

Impaired flexibility in patients with tropical spastic paraparesis/HTLV-associated myelopathy: evaluation via pendulum fleximeter / Flexibilidade prejudicada em pacientes com paraparesia espástica tropical/mielopatia associada ao HTLV: avaliação via flexímetro pendular

Abstract Background Flexibility is crucial to the harmonious execution of joint movements. While skeletal muscle dysfunction in patients with HTLV-1 can interfere with mobility, it is unclear whether these patients experience reduced flexibility. Objective To evaluate the differences in flexibility between HTLV-1-infected individuals with and without myelopathy compared with uninfected controls. We also investigated whether age, sex, body mass index (BMI), physical activity level, or lower back pain influence flexibility in HTLV-1-infected individuals. Methods The sample consisted of 56 adults, of which 15 did not have HTLV-1, 15 had HTLV-1 without myelopathy, and 26 had TSP/HAM. Their flexibility was assessed using the sit-and-reach test and a pendulum fleximeter. Results No differences in flexibility were observed between the groups with and without myelopathy and controls without HTLV-1 infection using the sit-and-reach test. The pendulum fleximeter results of individuals with TSP/HAM presented the lowest flexibility among the groups with respect to trunk flexion, hip flexion and extension, knee flexion, and ankle dorsiflexion, even after adjusting for age, sex, BMI, level of physical activity, and lower back pain using multiple linear regression models. Additionally, HTLV-1-infected individuals without myelopathy demonstrated reduced flexibility in movements: knee flexion, dorsiflexion, and ankle plantar flexion. Conclusions Individuals with TSP/HAM demonstrated reduced flexibility in most of the movements evaluated by the pendulum fleximeter. Additionally, HTLV-1-infected individuals without myelopathy demonstrated reduced knee and ankle flexibility, potentially representing a marker of myelopathic development.

Resumo Antecedentes A flexibilidade é fundamental para a execução harmoniosa dos movimentos articulares. Embora a disfunção do músculo esquelético em pacientes com HTLV-1 possa interferir na mobilidade, não está claro se esses pacientes apresentam flexibilidade reduzida. Objetivo Avaliar as diferenças de flexibilidade entre os indivíduos infectados com e sem mielopatia e o grupo controle sem infecção HTLV-1. Também investigamos se idade, sexo, índice de massa corporal (IMC), nível de atividade física ou dor lombar influenciam a flexibilidade em indivíduos infectados pelo HTLV-1. Métodos A amostra foi composta por 56 adultos, dos quais 15 não possuíam HTLV-1, 15 possuíam HTLV-1 sem mielopatia e 26 possuíam TSP/HAM. A flexibilidade foi avaliada por meio do teste de sentar e alcançar e do flexímetro de pêndulo. Resultados Não foram observadas diferenças na flexibilidade entre os grupos com e sem mielopatia no teste de sentar e alcançar. Os resultados do flexímetro pendular dos indivíduos com TSP/HAM apresentaram a menor flexibilidade entre os grupos em relação à flexão do tronco, flexão e extensão do quadril, flexão do joelho e dorsiflexão do tornozelo, mesmo após ajuste para idade, sexo, IMC, nível de atividade física e dor lombar usando modelos de regressão múltipla linear. Além disso, os indivíduos infectados pelo HTLV-1 sem mielopatia demonstraram redução da flexibilidade nos movimentos de flexão do joelho, dorsiflexão e flexão plantar do tornozelo. Conclusão Indivíduos com TSP/HAM demonstraram redução da flexibilidade na maioria dos movimentos avaliados pelo flexímetro pendular. Além disso, indivíduos infectados pelo HTLV-1 sem mielopatia demonstraram redução da flexibilidade do joelho e tornozelo, representando potencialmente um marcador de desenvolvimento mielopático.

Percepção subjetiva de esforço e recuperação da fadiga pós-sessão fisioterapêutica em pacientes com paraparesia espástica tropical / Subjective effort perception and fatigue recovery after physical therapy session in patients with tropical spastic paraparesis

Introdução: Vírus Linfotrópico de Células T Humana do Tipo-1 (HTLV-1) é um retrovírus que afeta os linfócitos T humano e desencadeia inflamação na medula, levando à paraparesia espástica tropical/mielopatia associada ao HTLV-1 (PET/MAH) com prejuízos funcionais. Tais disfunções podem influenciar nos efeitos da fisioterapia, gerando diferentes níveis de fadiga. Objetivo: Avaliar a percepção subjetiva de esforço e de recuperação de indivíduos com PET/MAH após sessão única de fisioterapia. Métodos: Incluíram-se 12 participantes sintomáticos para PET/MAH, de ambos os sexos, que foram submetidos uma vez ao protocolo fisioterapêutico. Os instrumentos avaliativos foram: Escala Modificada de Borg, Escala de Percepção Subjetiva de Recuperação (1º, 5º e 10º minuto após a sessão) e Escala de Incapacidade Neurológica do Instituto de Pesquisa Clínica Evandro Chagas ­ 2 (EIPEC). Resultados: Encontrou-se correlação moderada entre idade e taxa de percepção subjetiva de recuperação ao 1º (r = - 0,4923) e 5º (r = - 0,4913) minuto e entre índice do EIPEC-2 e taxa de percepção subjetiva de recuperação ao 1º (r = 0,3592) e 5º (r = - 0,3772) minuto. Conclusão: Indivíduos deste estudo com maior idade e grau de incapacidade neurológica têm menor percepção subjetiva de recuperação. (AU)

Introduction: Human T-Cell Lymphotropic Virus Type-1 (HTLV-1) is a retrovirus that affects human T lymphocytes and triggers inflammation in the spinal cord, leading to tropical spastic paraparesis / HTLV-1 associated myelopathy (TSP/HAM) with functional impairments. Such dysfunctions can influence the effects of physical therapy, generating different levels of fatigue. Objective: To evaluate the subjective perception of effort and recovery of individuals with TSP/HAM after a single session of physical therapy. Methods: 12 symptomatic participants for TSP/HAM, of both sexes, who were submitted once to a physiotherapeutic protocol were included. The evaluative instruments were: Modified Borg Scale, Scale of Subjective Perception of Recovery (1st, 5th and 10th minute after the session) and Scale of Neurological Disability at the Evandro Chagas Clinical Research Institute - 2 (EIPEC). Results: A moderate correlation was found between age and subjective perception rate of recovery at the 1st (r = - 0.4923) and 5th (r = - 0.4913) minute and between the EIPEC-2 index and the subjective perception rate of recovery at the 1st (r = 0.3592) and 5th (r = - 0.3772) minute. Conclusion: Individuals in this study with older age and degree of neurological disability have less subjective perception of recovery. (AU)

Co-expression of HBZ, TAX and FOXp3 and HTLV-1-associated myelopathy/tropical spastic paraparesis development in HTLV-1-infected individuals: A systematic review

Human T-cell lymphotropic virus type 1 (HTLV-1) is associated with the development of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). It has been reported that the HTLV-1 proteins (specifically TAX and HBZ) can modulate FOXp3, resulting in an immune imbalance that can favor the progression of HAM/TSP. This review aims to summarize the literature in order to clarify the relationship between the expression of HTLV-1 mRNAs and/or viral proteins (TAX and HBZ) with the expression of mRNA and/or protein FOXp3 and their correlation with HAM/TSP development. This systematic review was conducted according to the recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analysis. The search strategy was performed on the Medical Literature Analysis and Retrieval System Online and Latin American and Caribbean Literature in Health Sciences Platform using subject descriptors. After screening, six articles were included in this review. The studies suggested that TAX and HBZ have a directly proportional correlation with FOXp3 in individuals with HAM/TSP, which also presented an increased expression of FOXp3 compared to asymptomatic controls and/or healthy donors. This systematic review indicates that TAX and HBZ can interact with FOXp3 and that interaction may influence HAM/TSP development.

Therapeutic effects of virtual reality video gaming on functional mobility, balance, and gait speed in individuals with tropical spastic paraparesis: A randomized crossover clinical trial

Abstract INTRODUCTION: Individuals with human T-cell lymphotropic virus 1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) experience sensorimotor alterations, which can affect functional performance. Virtual reality (VR) videogaming is a therapeutic option, though there is scarce evidence for its use in this population. We aimed to investigate the therapeutic effects of a VR video game on functional mobility, balance, and gait speed in individuals with HAM/TSP. METHODS: We conducted a blinded, crossover clinical trial comprising 29 individuals with HAM/TSP and randomized them into two groups: (1) early therapy: rehabilitative protocol started immediately after the initial evaluation and (2) late therapy: rehabilitative protocol started 10 weeks later. We assessed all participants for balance using the Berg Balance Scale (BBS) scores, functional mobility using the Timed Up and Go (TUG) test, and gait speed using video camera and CvMob software. Differences were considered significant if p<0.05. RESULTS: The early therapy group individuals presented with higher BBS scores (p=0.415), less TUG times (p=0.290), and greater gait speed (p=0.296) than the late therapy group individuals. CONCLUSIONS: VR videogaming is a useful option for rehabilitative therapy in individuals with HAM/TSP; it positively affects balance, functional mobility, and gait speed.

Infección por virus HTLV-1/2 confirmada por serología y detección de provirus en pacientes ecuatorianos de paraparesis espástica tropical / HTLV-1/2 virus infection confirmed by serology and provirus DNA detection in tropical spastic paraparesis Ecuadorian patients

Introduccion: La infección con el virus linfotrópico de células T humano (HTLV) de tipo 1 y 2 es endémica en América del Sur. Existen muy pocos reportes clínicos de este retrovirus en pacientes de Ecuador y nunca confirmaron la presencia del virus con el aislamiento o la detección de su ADN. Esta infección se encuentra desatendida por las autoridades de salud pública, sin que existan estudios epidemiológicos de prevalencia a nivel del país. Objetivos: Este estudio tiene como objetivo la detección de infección por HTLV -1/2 en individuos sintomáticos de paraparesis espástica tropical (TSP) utilizando por primera vez en Ecuador diagnóstico serológico y la detección del provirus por biología molecular. Diseño del estudio. Se tomaron muestras de nueve pacientes con un diagnóstico de mielopatía y sospechoso de TSP, que fueron analizadas para la detección del virus HTLV-1/2 usando tres metodologías: ELISA comercial, ensayo de inmunofluorescencia indirecta (IF) y PCR anidada. Resultados: Cinco de los 9 (55.5%) pacientes fueron positivos tanto para la prueba de ELISA como para IF y PCR anidada. Conclusión: La alta prevalencia de infección por HTLV-1/2 entre individuos sintomáticos de mielopatía muestra la endemicidad de este retrovirus en Ecuador, la asociación de HTLV-1/2 con TSP y la necesidad de implementar estrategias de control y prevención para evitar la diseminación de esta enfermedad infecciosa desatendida.

Background: Infection with the Human T-cell lymphotropic virus (HTLV) type 1 and 2 is endemic in South America. There are only a few clinical reports of this retrovirus in patients in Ecuador, without employing virus-isolation methods or viral DNA detection. The infection is usually neglected in health care settings and by public health authorities and no country wide prevalence data are available. Objectives: This study aims to detect the HTLV-1/2 infection in symptomatic individuals for tropical spastic paraparesis (TSP) using for the first time in Ecuador both immunological and molecular biology methodologies. Study Design: Blood samples of 9 symptomatic individuals with myelopathy were analyzed for the detection of the HTLV-1/2 virus with three methods: ELISA, an indirect immunofluorescence (IF) assay and a nested-PCR. Results: Five out of 9 (55.5%) myelopathy patients were positive for ELISA, IF and nested- HTLV-1/2 PCR. Conclusion. The high prevalence of HTLV-1/2 infection among myelopathy patients shows the endemicity of this retrovirus in Ecuador, the association of HTLV-1/2 infection with TSP and the need of the implementation of control and prevention interventions to overcome the further dissemination of this neglected infectious disease.

The effect of home exercise on the posture and mobility of people with HAM/TSP: a randomized clinical trial / Efeitos de exercícios domiciliares sobre a postura e mobilidade de pessoas com HAM/TSP: ensaio clínico randomizado

Abstract Background: Physical therapy has positive results in people with tropical spastic paraparesis (TSP). However, mobility and distance from rehabilitation centers limit the participation in outpatient programs. Objective: To evaluate the impact of a home exercise program on the posture and functional mobility of people with TSP. Methods: A randomized controlled trial comparing three groups of people who performed guided exercises from a guidebook for six months: supervised (SG), unsupervised (WG), and control (CG). Primary outcomes: postural angles (SAPO®) and functional mobility (TUG). Secondary outcomes: gait parameters (CVMob®). Results: The protocol described in the guidebook improved postural angles and functional mobility. There were also positive gait parameter effects (p<0.05). SG presented better responses than WG did, but both were preferable to CG. Conclusion: Home exercises oriented by a guidebook may benefit posture, functional mobility and gait parameters in people with TSP, and physiotherapist supervision can ensure better results.

Resumo Introdução: A fisioterapia apresenta resultados positivos em pessoas com paraparesia espástica tropical (PET). Entretanto, a dificuldade de locomoção e a distância dos centros de reabilitação limitam a participação em programas ambulatoriais. Objetivo: Avaliar o impacto de um programa de exercícios domiciliares na postura e mobilidade funcional de pessoas com PET. Métodos: Um ensaio clínico randomizado comparou três grupos de pessoas que realizaram exercícios guiados por cartilha: com supervisão (GS), sem supervisão (GN) e controle (GC) durante seis meses. Desfechos primários: ângulos posturais (SAPO®) e mobilidade funcional (TUG). Desfechos secundários: parâmetros da marcha (CVMob®). Resultados: O protocolo descrito na cartilha melhorou os ângulos posturais e a mobilidade funcional. Os resultados também foram positivos para os parâmetros da marcha (p<0,05). O GS apresentou melhores respostas que o GN, porém ambos foram preferíveis ao GC. Conclusão: Exercícios domiciliares orientados por cartilha podem ser úteis para beneficiar a postura, mobilidade funcional e parâmetros de marcha em pessoas com PET, e a supervisão do fisioterapeuta possibilita garantir melhores resultados.

Balance assessment in HTLV-1 associated myelopathy or tropical spastic paraparesis

Abstract INTRODUCTION: A good rating of the device in people with HTLV-1 in this population is essential for accuracy in prescribing data (walking). Thus, this study aimed to analyze the counterpart assessment methods that are best suited to patients with human T-cell lymphotropic virus (HTLV)-1 associated myelopathy or tropical spastic paraparesis (HAM/TSP). METHODS: This cross-sectional study related stabilometric and kinematic variables of postural oscillations with Berg's balance scale (BBS) and Timed Up and Go (TUG) in subjects with HAM/TSP compared to asymptomatic subjects. To assess the posterior and lateral postural projection, baropodometry and the Footwork® system was used, and the CVMob system was applied to kinematic parameters. The means comparison tests and correlations were applied with an alpha of 5%. RESULTS: Thirty-nine subjects (predominantly female) made up the sample. There was an increase in barodopometric oscillations, in the total oscillation area (p = 0.004), in the anteroposterior oscillation in the left (p = 0.015) and right views (p = 0.036), and in the lateral oscillation (p = 0.039) in the HAM/TSP group. Moderate correlations were found between oscillation baropodometry and the angular variation of the ankle, as well as with the BBS in the three angles and the TUG for lateral oscillation (p = 0.406). CONCLUSIONS: Each method has advantages and disadvantages, including cost accuracy. The best resources available at no additional cost for outpatient to use are the kinematic evaluation using a simple smartphone camera and free analysis software, and the TUG.

Benefits of inspiratory muscle training under indirect home supervision in patients with human T-cell lymphotropic virus type 1 / Benefícios do treinamento muscular inspiratório sob supervisão indireta domiciliar em pacientes com vírus linfotrópico de células T humana tipo 1

Abstract Introduction: The presence of human T-cell lymphotropic virus type 1 (HTLV-1) associated with neuropathy (myelopathy/tropical spastic paraparesis - HAM/TSP), can generate morphological and functional changes in the respiratory system. As a preventive therapeutic possibility for respiratory dysfunctions, it is expected that the already conceptualized inspiratory muscle training, when performed at home, can be a therapeutic resource that favors adherence to treatment. Objective: To evaluate respiratory muscle strength in patients with HTLV-1 after participating in a home respiratory muscle training protocol under indirect supervision. Method: This was a clinical, longitudinal, prospective, quantitative, and single-center trial approved by the Research Ethics Committee of the State University of Pará, opinion no. 2.695.505 and registered in clinical trials NCT03829709. Six HTLV-1 patients participated in a 5-week home respiratory muscle training protocol lasting 30 minutes daily through a linear load inspiratory muscle trainer. For the characterization of the imposed load, they were submitted to manovacuometry during pre (T0), peri (T3), and post (T5) treatment. Results: Six individuals completed the program, of which 83.33% were female and 16.66% male. With the application of respiratory muscle training, it was possible to achieve a significant increase (p < 0.011) of the maximum inspiratory pressure as shown when comparing T0 (66.8±12.58) to T5 (115.08±31.78). Conclusion: This study identified an increase in inspiratory muscle strength after HTLV-1 patients participated in a home muscle training protocol under indirect supervision.

Resumo Introdução: A presença do vírus linfotrópico de células T humana do tipo 1 (HTLV-1) associado à neuropatia (Paraparesia Espástica Tropical/Mielopatia - PET/MAH) pode gerar alterações morfológicas e funcionais no sistema respiratório. Como possibilidade terapêutica preventiva para disfunções respiratórias, vislumbra-se que o treinamento muscular inspiratório já conceituado, possa a nível domiciliar ser uma ferramenta terapêutica que favoreça a adesão ao tratamento. Objetivo: Avaliar a força muscular respiratória diante de um protocolo de treinamento muscular respiratório domiciliar, sob supervisão indireta em portadores do HTLV- 1. Método: Estudo clínico, longitudinal, prospectivo, quantitativo e de centro único, aprovado pelo Comitê de Ética em Pesquisa da Universidade do Estado do Pará, parecer no. 2.695.505 e registrado no Clinical Trials NCT03829709. Seis pacientes com HTLV-1 participaram de um protocolo de treinamento muscular respiratório domiciliar por 5 semanas com duração de 30 minutos diários por meio de um treinador muscular inspiratório de carga linear. Para a caracterização da carga imposta, os mesmos foram submetidos a manovacuometria, pré (T0), peri (T3) e pós (T5) tratamento. Resultados: Seis indivíduos completaram o programa, dos quais 83.33% eram do sexo feminino e 16.66% do sexo masculino. Com a aplicação do treinamento muscular respiratório foi possível obter um aumento significativo (p < 0,011) da pressão inspiratória máxima ao comparar T0 (66.8±12.58) ao T5 (115.08±31.78). Conclusão: Este estudo identificou um aumento na força muscular inspiratória após pacientes com HTLV-1 participarem de um protocolo de treinamento muscular domiciliar sob supervisão indireta.

Infección Por El Virus Linfotrópico De Células T Humano HTLV-1 Y Paraparesis Espástica Tropical En Ecuador: Paradigma De Enfermedad Tropical Desatendida / Human T-lymphotrophic Virus Infection (HTLV-1) And Tropical spastic Paraparesis: Paradigms Of An Overlooked Tropical Disease

Resumen La infección por el virus linfotrópico de células T humano de tipo 1 (HTLV-1) es considerada endémica en América del Sur. Desde hace más de dos décadas existen reportes de casos clínicos de mielopatías asociadas a infección por el retrovirus HTLV-1 en pacientes de Ecuador, patología denominada paraparesis espástica tropical. Sólo hay dos estudios de seroprevalencia de HTLV-1 en Ecuador, ambos con tamaños muestrales pequeños pero en ambos casos indicativos de una alta prevalencia de HTLV-1, al menos comunidades afrodescendientes e indígenas de Ecuador. Pese a ello, y a diferencia de países vecinos, no se realiza tamizaje de bancos de sangre y órganos por parte de las autoridades sanitarias competentes. Por tanto, esta infección y las patologías asociadas a la misma (como la paraparesis espástica tropical) se encontrarían desatendidas y sub diagnosticadas. En definitiva, urge desarrollar estudios epidemiológicos de prevalencia a nivel del país e implementar estrategias de control y prevención para evitar la diseminación de esta infección.

Abstract The human T cell lymphotropic virus (HTLV-1) infection is considered endemic in South America. For more than two decades clinical cases of HTLV-1 associated mielopaties have been reported in Ecuador, pathology known as tropical spastic paraparesis. There is only two studies about HTLV-1 seroprevalence in Ecuador, both of them with small sample size but showing a high prevalence of HTLV-1 infection, at least for afrodescendants and indigenous communities. Howewer, there is not a currently screening for blood and organ banks carried out by ecuadorian public health authorities. This scenario makes HTLV-1 infection and associated pathologies (like tropical spastic paraparesis) neglected diseases in Ecuador. More epidemiological studies need to be implemented in order to develope control and prevention strategies in the country.

Neurologic complications of HTLV-1: a review / Complicações neurológicas do HTLV: uma revisão HTLV-1

The human T-cell lymphotropic virus type 1 (HTLV-1) is a retrovirus that infects about 20 million people worldwide and causes immune-mediated diseases of the nervous system. The classical neurological presentation of HTLV-1 infection is the so-called HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). However, HAM/ TSP is not the only neurological outcome that can result from HTLV-1 infection. In this Review it is made an update on the many aspects of this important neurological condition, the HTLV-1 neurological complex.

O vírus linfotrópico de células T humanas tipo 1 (HTLV-1) é um retrovírus que infecta cerca de 20 milhões de pessoas em todo o mundo e causa doenças imunomediadas do sistema nervoso. A apresentação neurológica clássica da infecção pelo HTLV-1 é a chamada paraparesia espástica tropical / mielopatia associada ao HTLV-1 (HAM/TSP). HAM / TSP,no entanto, não é o único desfecho neurológico que pode resultar da infecção pelo HTLV-1. Nesta revisão, é feita uma atualização sobre vários aspectos desta importante condição neurológica, o complexo neurológico do HTLV-1.

Paraparesia espástica tropical y atrofia cerebelosa / Tropical spastic paraparesis and cerebelar atrophy

Resumen Antecedentes: El virus linfotrópico T tipo I (HTLV-I) origina la paraparesia espástica tropical (PET) en el 3% de los infectados, afectando predominante mujeres. Excepcionalmente la PET puede asociar un síndrome vestibular central y atrofia cerebelosa. Propósito: Presentar un nuevo y excepcional caso de paraparesia espástica y atrofia cerebelosa. Sugerir una interpretación patogénica del predominio femenino en esta patología Paciente: Mujer de 20 años de talla baja y menuda, infectada con HTLV-I durante la lactancia. Aproximadamente a los 15 años inició un síndrome ataxo-espástico progresivo, con grave alteración de la marcha, posteriormente agregó daño cognitivo y atrofia cerebelosa en la RM. Se constató a su ingreso una elevada carga viral y altos niveles de proteína Tax. Fue tratada con 4 mg betametasona diarios durante 10 días, que mejoraron la marcha. Conclusión: La PET es una axonopatía de la vía motora central, originada por la crónica perturbación del transporte axoplásmico, atribuible a la presencia de elevados niveles de la proteína Tax del virus. Circunstancialmente este aumento de Tax logra dañar axones del centro oval (deterioro cognitivo) o del vermis cerebeloso (síndrome vestibular central). La PET afecta mayoritariamente a mujeres 3:1, prevalencia que hace aparecer a las mujeres con una mayor vulnerabilidad en su SNC. Sin embargo, esta aparente minusvalía, sería debida a un aumento en la concentración de Tax en el SNC de ellas, causado por la adversa relación entre peso corporal y cantidad absoluta de Tax, que fue evidente en nuestra paciente, quien dio la clave para esta hipótesis.

Background: Lymphotropic Virus Type I (HTLV-I) causes Tropical Spastic Paraparesis (PET) in 3% of infected patients; in whom have been described exceptionally associated a central vestibular syndrome and cerebellar atrophy. Those alterations of CNS are predominating in women. Purpose: To present a new case of the exceptional form of spastic paraparesis and cerebellar atrophy. To suggest a pathogenic interpretation of female predominance in this pathology Patient: A 20-year-old woman of small size, infected with HTLV-I during lactation. Approximately at 15 years of age he started a progressive ataxo-spastic syndrome, later cognitive damage and cerebellar atrophy were added. Upon admission, high viral load and high levels of Tax protein, leukemoid lymphocytes and Sicca syndrome were observed. Conclusion: PET is an axonopathy of the central motor pathway, originated by a chronic disturbance of axoplasmic transport, attributable to the action of elevated levels of Tax protein in the CNS. In addition axons of the oval center (cognitive impairment) or the cerebellar vermis (central vestibular syndrome) are occasionally damaged. Although PET mainly affects 3: 1 women, this prevalence increases in accordance with the increase of neurological damage. The apparent greater vulnerability of the CNS in women would be due to the higher concentration of Tax in the CNS of them, originated in the adverse relationship between body weight and absolute amount of Tax, which was evident in our patient, who gave the key to this hypothesis.

Caso Clínico: Paraparesia Espástica Tropical Asociada a Infección por HTLV-1 / Case Report: Spastic Tropical Paraparesis Associated with HTLV-1

INTRODUCCIÓN: La mielopatía asociada al virus linfotrópico de células T humano tipo 1 o llamada comúnmente paraparesia espástica tropical, constituye un síndrome espástico caracterizado por una evolución insidiosa, crónica, lentamente progresiva y muy variable; es considerada por algunos autores como una patología desmielinizante que afecta la médula espinal. CASO CLÍNICO: Paciente de sexo femenino de 53 años de edad, procedente de Manabí y residente en Santo Domingo de los Tsáchilas, que acudió con un cuadro de 10 meses de evolución caracterizado por parestesias en miembro inferior derecho que progresó a temblores, parestesias, hiperalgesia y pérdida de fuerza muscular de las cuatro extremidades más afectación esfinteriana. Se realizaron los estudios necesarios para estudiar las causas más comunes de paraparesia; finalmente, se detectó infección por virus linfotrópico de células T humano tipo I. EVOLUCIÓN: ELa paciente permaneció hospitalizada durante 28 días y fue dada de alta en condiciones estables con los déficits neurológicos previamente establecidos. Se mantuvo en seguimiento durante 4 años posterior al diagnóstico con deterioro progresivo de la fuerza muscular en sus cuatro extremidades hasta la monoplejía con espasticidad de miembro inferior derecho. Se han reportado episodios de disartria y disnea que han remitido espontáneamente. CONCLUSIÓN: La mielopatía asociada a infección por HTLV-1 o paraparesia espástica tropical es una enfermedad de baja prevalencia, debe ser sospechada y estudiada en pacientes con un cuadro crónico de paraparesia, alteraciones sensitivas y disfunción vesical.(au)

BACKGROUND: The human T lymphotropic virus type-I associated myelopathy or also called spastic tropical paraparesis, is a spastic syndrome characterized by a slow progressive, insidious and chronic evolution. It is considered by some authors as a demyelinating spinal cord disease. CASE REPORT: 53-year-old female patient, born in Manabi and residing in Santo Domingo de los Tsáchilas. With 10 months history of right lower limb paresthesias that progressed to tremor, paresthesias, hyperalgesia and four limb muscle weakness plus sphincter disorders. An appropriate workup were performed in order to study the most common causes of paraparesis, infection with HTLV-1 was detected finally. EVOLUTION: The patient remained hospitalized for 28 days; she was discharged in stable conditions with all the prior established neurological deficits. A 4-year follow up was registered after diagnosis; muscle weakness progressed in four limbs causing right lower limb monoplegia. Dysarthria and dyspnea have been also reported, with spontaneous recovery. CONCLUSION: Human T lymphotropic virus type-I associated myelopathy or spastic tropical paraparesis is a low frequency disease, must be suspected and studied in patients with chronic paraparesis with sensory loss and sphincter disorders.(au)

Parâmetros para reabilitação da marcha em pessoas com Paraparesia Espástica Tropical: estudo transversal / Parameters for gait rehabilitation in Tropical Spastic Paraparesis: cross sectional study

INTRODUÇÃO: A marcha de pessoas com Mielopatia Associada ao HTLV-1 ou Paraparesia Espástica Tropical (HAM/TSP) é pouco conhecida. OBJETIVO: Avaliar o perfil cinemático da marcha em pessoas com HAM/TSP. MÉTODOS: Estudo transversal com 25 pessoas com HAM/TSP e 25 participantes saudáveis. Os dados espaço-temporais e angulares das filmagens da marcha foram submetidos à análise cinemática utilizando o software CVMob®. A marcha dos participantes com HAM/TSP foi analisada quantitativamente através do teste t-student (alfa de 5% e poder de 80%). O projeto foi aprovado pelo comitê de ética da Escola Bahiana de Medicina e Saúde Pública sob o CAAE 13568213.8.0000.5544. RESULTADOS: O grupo HAM/TSP apresentou alterações significativas em dois domínios distintos da análise biomecânica da marcha quando comparados aos controles saudáveis. As variáveis angulares apresentaram maior inclinação, flexão e extensão do tronco (p <0,05); aumento da flexão do quadril e diminuição da extensão do quadril (p <0,05); diminuição da flexão e extensão do joelho (p <0,05); diminuição da flexão dorsal e plantar do tornozelo (p <0,05). A amplitude de movimento também foi reduzida em todas essas articulações. As variáveis espaço-temporais mostraram diminuição do comprimento da passada, aumento do tempo da passada e velocidade do ciclo da marcha (p <0,001). Essas mudanças apontam para redução nas amplitudes articulares do quadril, joelho e tornozelo, mudanças na base de apoio e assimetria do tempo do duplo apoio entre os lados direito e esquerdo, redução do tempo do pré-balanço, elevação do quadril no balanço médio e queda do pé ao longo do balanço. CONCLUSÃO: As pessoas com HAM/TSP apresentam marcha caracterizada por redução nas amplitudes articulares do quadril, joelho e tornozelo, assimetria do tempo de suporte duplo entre os lados direito e esquerdo, redução do tempo do pré-balanço, elevação do quadril no balanço médio e queda do pé ao longo do balanço.

INTRODUCTION: The gait in people with HTLV-1 Associated Myelopathy or Tropical Spastic Paraparesis (HAM / TSP) is little known. OBJECTIVE: To evaluate the kinematic profile of gait in people with HAM/ TSP. METHODS: A cross-sectional study with 25 people with HAM/TSP and 25 healthy participants. Spatiotemporal and angular data from filming of gait were submitted to kinematic analysis using CVMob® software. The gait of partipants with HAM/TSP was analized quantitatively through t Student test (alpha 5% and Power of 80%). The project was approved by Ethical Committee of the Bahiana School of Medicine and Public Health with CAAE 13568213.8.0000.5544. RESULTS: The HAM/TSP group showed significant changes in two different domains of biomechanical gait analysis when compared to healthy controls. The angular variables showed increased trunk inclination, flexion and extension (p<0.05); increased hip flexion and decreased hip extension (p<0.05); decreased knee flexion and extension (p<0.05); decreased ankle dorsi and plantar flexion (p<0.05). Range of Motion was also reduced in all those joints. The spatiotemporal variables showed decreased stride length, increased stride time and speed gait cycle (p<0.001). Those changes point out to reduction in joint amplitudes of hip, knee and ankle, changes in support base and double support time asymmetry between right and left sides, reduction of pre-swing time, hip lift in the mid-swing, and foot drop throughout the swing. CONCLUSION: People with HAM/TSP have gait characterized by reduction in joint amplitudes of hip, knee and ankle, asymmetry between right and left sides and reduction of pre-swing time, hip lift in the mid-swing, and foot drop throughout the swing.

Low frequency of regulatory B-cells and increased CD4+ and CD8+ interferon-γ-producing cells in patients with tropical spastic paraparesis associated with human T-cell lymphotropic virus type

Abstract INTRODUCTION: Tropical spastic paraparesis/HTLV-1 associated myelopathy (TSP/HAM) is a disease caused by human T-cell lymphotropic virus type 1 (HTLV-I) that mainly infects CD4 T cells-for example, those of the CD4+CD25hiFOXP3+ [Treg] phenotype-where it inhibits forkhead box protein P3 (FOXP3) expression and promotes interferon-γ (IFN-γ) expression. However, the role it exerts on regulatory B cells (CD19+CD24hiCD38hi; Breg) is unknown. METHODS: The frequencies of Treg and Breg cells was evaluated and the Th1 profiles were assessed in TSP/HAM patients and healthy control subjects. RESULTS: Low percentages of Breg cells and high production of IFN-γ were observed in patients compared to those in healthy control subjects. CONCLUSIONS: The low percentage of Breg cells in patients and the increase in the frequency of Th1 cells suggest an imbalance in the control of the inflammatory response that contributes to the immunopathogenesis of TSP/HAM.

Paraparesia espástica por HTLV-I: 20 años después. A propósito de un caso / Spastic paraparesis by HTLV-I: 20 years later. About a case / Paraparesia espástica pelo HTLV-I: 20 anos depois. Sobre um caso

Resumen: Se describe un caso de paraparesia espástica asociada al retrovirus HTLV-I, en una paciente de 34 años, cuyo diagnóstico se realizó 17 años después del inicio de su enfermedad. El mismo se obtuvo mediante la determinación de anticuerpos en sangre y líquido cefalorraquídeo. En la literatura nacional existen solo dos casos publicados previamente. El objetivo de la presentación del caso es recordar las principales características epidemiológicas, clínicas y paraclínicas de dicha patología, así como restablecer la importancia de considerar al HTLV-Ia la hora de búsqueda etiológica de una mielopatía crónica, y mantener los controles en sangre de donantes y hemoderivados para eliminar una de las principales vías de contagio.

Abstract: A case of spastic paraparesis associated with HTLV-I retrovirus was described in a 34-year-old patient whose diagnosis was made 17 years after the onset of his disease. It was obtained by determining antibodies in blood and cerebrospinal fluid. In the national literature there are only two previously published cases. The objective of the presentation of the case is to recall the main epidemiological, clinical and paraclinical characteristics of said pathology, as well as to re-establish the importance of considering the HTLV-Ia the etiological search time of a chronic myelopathy, and to maintain the blood controls of donors and blood products to eliminate one of the main routes of infection.

Resumo: Um caso de paraparesia espástica associado a retrovírus HTLV-I foi descrito em um paciente de 34 anos cujo diagnóstico foi feito 17 anos após o início da doença. Foi obtido pela determinação de anticorpos no sangue e no líquido cefalorraquidiano. Na literatura nacional existem apenas dois casos publicados anteriormente. O objetivo da apresentação do caso é relembrar as principais características epidemiológicas, clínicas e paraclínicas da referida patologia, bem como restabelecer a importância de considerar o HTLV-Ia o tempo de busca etiológica de uma mielopatia crônica e manter o controle sanguíneo dos doadores. e hemoderivados para eliminar uma das principais vias de infecção.

Eficácia e segurança da toxina botulínica no tratamento da paraparesia espástica: revisão sistemática / Efficacy and safety of botulinum toxin in the treatment of spastic paraparesis: systematic review

A paraparesia espástica é caracterizada pela perda de função total ou parcial dos membros inferiores associado ao aumento do tônus muscular velocidade-dependente. A toxina botulínica é utilizada no tratamento de diversos padrões de espasticidade, sejam em flexão, extensão ou adução. Objetivo: determinar a eficácia e segurança do bloqueio químico com toxina botulínica em pacientes com paraparesia espástica. Método: foi realizada uma revisão sistemática com busca nas bases de dados do PUBMED, MEDLINE, LILACS e SCIELO. Os critérios de inclusão foram: ensaios clínicos que utilizaram a toxina botulínica para o tratamento de pacientes com paraparesia espástica e publicados em inglês a partir da década de 1980. Os desfechos considerados foram: a pontuação na Escala de Ashworth Modificada, a amplitude de movimento passiva e ativa e os efeitos adversos da toxina botulínica. Resultados: foram incluídos cinco artigos. Todos mostraram melhora da espasticidade nos pacientes estudados. Quatro artigos mostraram aumento da amplitude de movimento passivo e três relataram aumento da amplitude de movimento ativo. Três artigos trouxeram relatos de efeitos adversos após o uso da toxina botulínica, mas a maioria deles não eram graves e cessaram espontaneamente. Conclusão: os estudos analisados mostraram que a toxina botulínica é eficaz e segura em pacientes com paraparesia espástica.(AU)

Spastic paraparesis is the loss of total or partial lower limb function associated with increased speed-dependent muscle tone. Botulinum toxin is used in the treatment of several spasticity presentations that include flexion, extension and adduction. Objective: To determine both safety and efficacy of botulinum toxin as a blocking agent in the treatment of spastic paraparesis. Method:A systematic review was carried out with a search on PUBMED, MEDLINE, LILACS and SCIELO databases. The inclusion criteria were: clinical trials that used botulinum toxin for the treatment of patients with spastic paraparesis and published in English from the 1980s. The following outcomes were assessed by the studies: the Ashworth Modified scale score, the range of passive and active motion and botulinum toxin adverse effects. Results:Five articles were included. All of them showed spasticity improvements in the patients. Four studies showed increases in passive range of motion and three articles showed increase in active range of motion. Three papers reported adverse effects after botulinum toxin use but they were mostly mild and ceased spontaneously. Conclusion: Most analyzed studies indicated that botulinum toxin is safe and efficient inthe treatment of spastic paraparesis. (AU)

A Man with Adult-onset Progressive Spastic Paraparesis: Genetically Confirmed as Novel Mutation of ABCD1 Gene

Spastic paraparesis is caused by various etiologies such as autoimmune, infection, genetic and metabolic disorder. Adrenomyeloneuropathy (AMN) is very rare but one of important causes in spastic paraparesis. We experienced a patient presenting with adult-onset progressive spastic paraparesis, who was diagnosed as AMN with hemizygous c.431C>T (p.A144V), a novel mutation in exon1. The level of very long chain fatty acid should be included in diagnostic work-up for patients presenting with adult-onset progressive spastic paraparesis.

Tropical spastic paraparesis - anesthetic approach / Paraparesia espástica tropical - abordagem anestésica

Abstract Introduction HTLV-1 infection is endemic in Japan, Caribbean, Africa, and South America. It is transmitted from mother to child, sexual contact, blood transfusions, or sharing needles. Tropical spastic paraparesis (TSP) is a chronic degenerative neurological disease associated with this infection. It results from a spinal cord symmetrical degeneration at the thoracic level and is characterized by progressive motor weakness in the lower limbs, hyperreflexia, sensitivity changes, urinary incontinence, and bladder dysfunction. Clinical case Female, 53 years old, HTLV-1 infection and TSP. She had decreased strength in the lower limbs and hyperreflexia, paretic gait, spasticity, and neurogenic bladder symptoms, with recurrent urinary infections. She was scheduled for cystectomy. The patient was monitored according to standard ASA. Due to severe coagulopathy and the possibility of neurological worsening, epidural catheter was not placed. The induction of general anesthesia was performed with midazolam and fentanyl, followed by etomidate and cisatracurium. She was intubated with a tube size seven and maintained with desflurane and oxygen. Anesthesia was uneventful; the surgery lasted 1 hour and 50 min. There were no complications in the immediate postoperative period, during hospitalization, nor deterioration of the neurological examination. The patient was discharged 20 days later. Discussion/Conclusion There are reports of decreased electromyographic response and neurological deterioration associated with propofol in these patients, etomidate was used. The hepatic metabolism of rocuronium posed a risk, we chose to use cistracurium. It was concluded that the anesthesia chosen did not affect the course of the disease.

Resumo Introdução A infecção por HTLV- 1 é endêmica no Japão, nas Caraíbas, na África e na América do Sul. A transmissão ocorre de mãe para filho, por contatos sexuais, transfusões de sangue ou partilha de agulhas. A essa infeção está associada uma doença neurológica degenerativa crônica, a paraparesia espástica tropical (TSP). Essa resulta de uma degeneração simétrica da espinal medula em nível torácico. Caracteriza-se por diminuição progressiva da força nos membros inferiores, hiperreflexia, alterações de sensibilidade, incontinência urinária e disfunção vesical. Caso clínico Mulher de 53 anos, infecção por HTLV-1 e TSP. Apresentava diminuição da força nos membros inferiores e hiperreflexia, tinha uma marcha parética, espasticidade e sintomas de bexiga neurogênica com infecções urinárias de repetição. Foi proposta para cistectomia. Foi monitorada de acordo com o padrão da ASA. Devido à coagulopatia grave e à possibilidade de agravamento neurológico, não se colocou cateter epidural. A indução da anestesia geral foi feita com midazolam e fentanil seguidos de etomidato e cisatracúrio. Foi entubada com um tubo sete e mantida com desflurano e oxigênio. A anestesia decorreu sem intercorrências, a cirurgia terminou em uma hora e 50 minutos. Não houve quaisquer complicações no pós-operatório imediato, durante a internação, nem deterioração do exame neurológico. A doente teve alta 20 dias depois. Discussão/Conclusão Há relatos de diminuição da resposta eletromiográfica e deterioração neurológica associadas ao propofol nesses doentes, razão para uso de etomidato. A metabolização hepática do rocurônio representava um risco e se optou pelo cisatracúrio. Conclui-se que o plano anestésico escolhido não teve qualquer interferência no curso da doença.