Influence of proprioceptive neuromuscular facilitation on the muscle tonus and amplitude of movement in HTLV-1-infected patients with HAM/TSP

Abstract An HTLV-1-infected patient can develop paraparesis that limits their movements. Rehabilitation techniques could improve the motor abilities of these patients. The present study investigates five cases of physical therapy intervention in HTLV-1 patients to evaluate the influence of functional rehabilitation on the tonus and range of motion (ROM) of HTLV-1 patients with spasticity. The patients had a gain of ROM, especially in the lower limb, and reduction in hypertonia/spasticity after functional treatment. The reduction in hypertonia increased the ROM. Thus, functional methods may be valuable for the rehabilitation of HTLV-1 patients with neurological damage.

Balance, functional mobility, and fall occurrence in patients with human T-cell lymphotropic virus type-1-associated myelopathy/tropical spastic paraparesis: a cross-sectional study

Abstract INTRODUCTION: Human T-cell lymphotropic virus type-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) may lead to reduced functional mobility and balance. It is important to establish specific parameters that identify these changes and predict the risk of falls in these patients. The aim was to compare balance, functional mobility, and occurrence of falls among patients with and without HAM/TSP and to suggest values to predict the risk of falls in these patients. METHODS: A cross-sectional study in patients with and without HAM/TSP involved balance assessments based on the berg balance scale (BBS) and functional mobility evaluation based on the timed up and go (TUG) test. From reports of falls, the sensitivity, specificity, and best cutoff points for the risk of falls assessed by these instruments were established using the receiver-operating characteristic (ROC) curve; 5% alpha was considered. RESULTS: We selected 42 participants: 29 with HAM/TSP and 13 without HAM/TSP. There was a statistically significant difference in the occurrence of falls, balance, and functional mobility between the groups (p<0.05). Good accuracy was determined for the BBS (77%) and TUG test (70%) and the cutoff points for the risk of falls were defined as 50 points for the BBS and 12.28 seconds for the TUG test. CONCLUSIONS: Patients with HAM/TSP present reduced functional mobility and balance in relation to those without HAM/TSP. The risk of falls increased for these patients can be evaluated by the values ​​of 50 points using the BBS and 12.28 seconds using the TUG test.

Muscular weakness represents the main limiting factor of walk, functional independence and quality of life of myelopathy patients associated to HTLV-1 / Fraqueza muscular representa o principal fator limitante da marcha, independência funcional e qualidade de vida de pacientes com mielopatia associada ao HTLV-1

ABSTRACT HTLV-1-associated myelopathy is a progressive disabling disease associated with gait abnormalities. Objective To identify and quantify the main muscles affected by weakness and spasticity, their impact on gait, functional capacity and on quality of life of HTLV-1-associated myelopathy patients. Method We evaluated lower limbs muscular strength according to the Medical Research Council scale, spasticity according to the modified Ashworth scale, daily activities according to the Barthel Index and quality of life according to the Short-Form Health Survey-36 of 26 HTLV-1-associated myelopathy patients. Results The muscles most affected by weakness included the dorsal flexors and knee flexors. Spasticity predominated in the hip adductor muscles and in plantar flexors. Assistance for locomotion, minimal dependence in daily activities, limitations in functional capacity and physical aspects were the most common findings. Conclusion The impairment of gait, functional dependence and quality of life were predominantly a consequence of intense muscle weakness in HTLV-1-associated myelopathy patients.

RESUMO Mielopatia associada ao HTLV-1 é uma doença inflamatória, incapacitante e progressiva que acomete o sistema nervoso central. Objetivo Identificar e quantificar os principais músculos comprometidos pela fraqueza e espasticidade, o impacto na capacidade funcional e na qualidade de vida dos pacientes com mielopatia associada ao HTLV-1. Método Força muscular ( Medical Research Council), espasticidade (escala Ashworth modificada), atividades de vida diária (Índice de Barthel) e qualidade de vida ( Short-Form Health Survey-36) foram avaliados em 26 pacientes . Resultados Os principais músculos comprometidos pela fraqueza incluíram os flexores dorsais e flexores do joelho. A espasticidade predominou nos músculos adutores do quadril e nos flexores plantares. Assistência para locomoção, dependência mínima nas atividades diárias, limitações na capacidade funcional e os aspectos físicos representaram os achados mais frequentes. Conclusão Dificuldade de deambulação, dependência funcional e prejuízo na qualidade de vida foram as principais consequências da intensa fraqueza muscular nos pacientes com mielopatia associada ao HTLV-1.

Proprioceptive neuromuscular facilitation in HTLV-I-associated myelopathy/tropical spastic paraparesis

Introduction: Human T cell lymphotropic virus type I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) can impact the independence and motricity of patients. The aims of this study were to estimate the effects of physiotherapy on the functionality of patients with HAM/TSP during the stable phase of the disease using proprioceptive neuromuscular facilitation (PNF) and to compare two methods of treatment delivery. Methods: Fourteen patients with human T cell lymphotropic virus type I (HTLV-I) were randomly allocated into two groups. In group I (seven patients), PNF was applied by the therapist, facilitating the functional activities of rolling, sitting and standing, walking and climbing and descending stairs. In group II (seven patients), PNF was self-administered using an elastic tube, and the same activities were facilitated. Experiments were conducted for 1h twice per week for 12 weeks. Low-back pain, a modified Ashworth scale, the functional independence measure (FIM) and the timed up and go test (TUG) were assessed before and after the interventions. Results: In the within-group evaluation, low-back pain was significantly reduced in both groups, the FIM improved in group II, and the results of the TUG improved in group I. In the inter-group analysis, only the tone was lower in group II than in group I. Conclusions: Both PNF protocols were effective in treating patients with HAM/TSP. .

Os efeitos de um programa de exercícios domiciliares em pacientes com Paraparesia Espástica Tropical/Mielopatia Associada ao HTLV-1 (PET/MAH) / The effects of a home-based exercise program in Tropical Spastic Paraparesis/HTLV-1 Associated Myelopathy (TSP/HAM) patients

Os efeitos da prática de exercícios sobre a funcionalidade de indivíduos com Paraparesia Espástica Tropical/Mielopatia Associada ao HTLV-1 (PET/MAH) são desconhecidos. O objetivo deste estudo é avaliar os efeitos de um Programa de Exercícios Domiciliares (PED) e a sua taxa de adesão em indivíduos com PET/MAH. Métodos: Vinte e três participantes com o diagnóstico de PET/MAH, marcha preservada e que não praticavam exercícios há pelo menos um mês foram submetidos ao PED de 20 semanas. Os desfechos primários incluíram os escores de força muscular, contração isométrica voluntária máxima (CIVM) e comprimento muscular dos membros inferiores, dor lombar e nos membros inferiores, EDSS, Escala de Incapacidade do IPEC, Índice de Barthel e SF-36. A taxa de adesão e os eventos adversos também foram mensurados e caracterizados. Resultados: No momento da análise os pacientes foram divididos em dois grupos de acordo com o teste Timed Up and Go (TUG) (<20s vs maior ou igual 20s). O comprimento dos músculos isquiotibiais e plantiflexores, a CIVM dos membros inferiores e o componente Aspectos Sociais da SF-36 apresentaram melhora significativa no grupo TUG <20s. Os indivíduos do grupo TUG maior ou igual 20s melhoraram significativamente o componente Capacidade Funcional da SF-36. A taxa de adesão foi de 90 por cento no total e os eventos adversos, como fadiga, dor muscular e caimbras foram de intensidade leve a moderada. Discussão: Foi observada uma boa adesão ao PED, além de melhora significativa da incapacidade e da qualidade de vida dos indivíduos com PET/MAH. É possível que o grupo TUG maior ou igual 20s apresente um maior componente neurodegenerativo e, portanto, uma menor probabilidade de incremento da funcionalidade. Conclusões: O PED foi eficaz em melhorar algumas incapacidades e a qualidade de vida dos indivíduos com PET/MAH...

The effect of exercises on functioning in Tropical Spastic Paraparesis/HTLV-1 Associated Myelopathy (TSP/HAM) individuals is unknown. This study aimed to investigate the effects of a home-based exercise program and its adherence in TSP/HAM individuals. Methods: Twenty-three TSP/HAM ambulators participants that did not practice any kind of exercise for at least one month were submitted to a 20-week home-based exercise program. Primary outcomes included lower limb muscular strength scores, maximum voluntary isometric contraction (MVIC) and muscle length, low back and lower limb pain, EDSS, IPEC Disability Scale, Barthel Index and SF-36. Adherence and adverse effects were also measured and characterized.Results: At analysis the participants were divided in two groups according to Timed Up and Go Test (TUG) (<20s vs greater than or equal to 20s). Muscle length of hamstrings and ankle flexors; hip flexors and total score of lower limb MVIC and the social functioning domain in SF-36 improved significantly in TUG <20s group. The individuals in the TUG greater than or equal to 20s group improved significantly the physical functioning domain in SF-36. The total adherence to the 20-weekhome-based exercise program was 90 percent. There were mild to moderate adverse events, like fatigue, muscle soreness and cramp. Discussion: There was a very good adherence to the home-based exercise program, besidesimprovements in disabilities and quality of life in TSP/HAM participants. It is possible that TUG greater than or equal to 20s group has a worse neurodegenerative component and then a lower probability tofunctioning increments. Conclusions: The home-based exercise program was effective in improving disabilities andquality of life in individuals with TSP/HAM. Those results reinforce the need for alternative strategies to face-to-face model that improve participation of these people to rehabilitationprograms...

Implications of HAM/TSP functional incapacity in the quality of life / Implicações da incapacidade funcional na qualidade de vida de pacientes com HAM/TSP

OBJECTIVE: To identify the functional status and quality of life of HAM/TSP patients. METHOD: We evaluated prospectively 30 HAM/TSP patients (20 females) seen in the Neuroinfection Clinic of the HUGG. The functional capacity was analyzed by the functional independence measure (FIM), the expanded disability status (EDSS) scale and the Osame's motor disability score (OMDS). The quality of life was assed by the Short-Form Health Survey 36 (SF-36)TM. RESULTS: All patients need assistance device. The FIM, OMDS and EDSS scores classified 70 percent, 67 percent and 67 percent of the patients as dependent, respectively. The lowest scores of the SF-36 survey were found in the domains related to the physical health (D1, D2), role-emotional functioning (D7) and social functioning (D6). CONCLUSION: Our data suggest that the HAM/TSP physical impairment has an impact in the emotional and social issues, considering the limitation in the daily activities.

OBJETIVO: Avaliar a capacidade funcional e sua interferência na qualidade de vida de pacientes com HAM/TSP. MÉTODO: Foram analisados prospectivamente 30 casos (20 mulheres) de HAM/TSP, atendidos no Ambulatório de Neuroinfecção do HUGG. As escalas para avaliação da capacidade funcional consistiram em: medida de independência funcional (FIM), escala de incapacidade expandida (EDSS) e pontuação da incapacidade motora de Osame (OMDS). A qualidade de vida foi analisada pelo Short-Form 36 Health Survey (SF-36)TM. RESULTADOS: Todos os pacientes necessitavam de assistência para deambular. As escalas FIM, OMDS e EDDS classificaram 70 por cento, 67 por cento e 67 por cento dos pacientes como dependentes, respectivamente. A avaliação pelo SF-36 demonstrou menores escores nos domínios físico (D1, D2), emocional (D7) e social (D6). CONCLUSÃO: Os achados sugerem que a limitação nas atividades diárias decorrentes do envolvimento físico comprometem aspectos emocionais e sociais na HAM/TSP.

The motor evoked potential in aids and HAM/TSP State of the evidence / El potencial evocado motor en SIDA y HAM/PET

OBJECTIVE: We aimed to better understand the involvement of the corticospinal tract, assessed by non-invasive transcranial stimulation, in order to determine the actual involvement of the motor system in patients with HAM/TSP and AIDS. METHOD: An exhaustive MEDLINE search for the period of 1985 to 2008 for all articles cross-referenced for "HTLV-I, HTLV-II, HTLV-III and HIV, HIV1, HIV2, evoked potential, motor evoked potential, high voltage electrical stimulation, transcranial magnetic stimulation, magnetic stimulation, corticomotor physiology, motor pathways, acquired immunodeficiency syndrome, AIDS, SIDA, tropical spastic paraparesis, HTLV-I-associated myelopathy, HAM, TSP, and HAM/TSP" were selected and analysed. RESULTS: Eighteen papers published in English, Spanish, Portuguese, French and Japanese were identified. Only the central motor conduction time has been analyzed in seropositive patients to human retroviruses. The investigations done on HAM/TSP support the involvement of the pyramidal tract mainly at lower levels, following a centripetal pattern; in AIDS, such an involvement seems to be more prominent at brain levels following a centrifugal pattern. CONCLUSION: The central motor conduction time abnormalities and involvement differences of the corticospinal tract of patients with AIDS and HAM/TSP dissected here would allow to re-orient early neurorehabilitation measures in these retroviruses-associated neurodegenerative disorders. Besides this, more sophisticated and sensitive non-invasive corticospinal stimulation measures that detect early changes in thalamocortical-basal ganglia circuitry will be needed in both clinically established as well as asymptomatic patients at times when the fastest corticospinal fibers remain uninvolved.

OBJETIVO: Investigar el compromiso del tracto piramidal, evaluado por estimulación trascranial no invasiva, en pacientes afectados por SIDA y HAM/TSP. MÉTODO: Se realizó una búsqueda en la base de datos MEDLINE, que abarcó el período de 1985 a 2008; se incluyeron los términos "HTLV-I, HTLV-II, HTLV-III and HIV, HIV1, HIV2, evoked potential, motor evoked potential, high voltage electrical stimulation, transcranial magnetic stimulation, magnetic stimulation, corticomotor physiology, motor pathways, acquired immunodeficiency syndrome, AIDS, SIDA, tropical spastic paraparesis, HTLV-I-associated myelopathy, HAM, TSP, and HAM/TSP". RESULTADOS: Se obtuvieron 18 artículos publicados en inglés, español, portugués, francés y japonés. El tiempo de conducción central es el único parámetro que se ha estudiado en individuos seropositivos a retrovirus humanos. Las investigaciones hechas en HAM/PET apoyan el compromiso del tracto piramidal, principalmente a nivel dorso-lumbar, de manera centrípeta. En SIDA, el compromiso parece ser mas prominente a nivel cortical, siguiendo un patrón centrifugo. CONCLUSION: El conocer las diferencias en el compromiso y anormalidades del tracto corticoespinal de los pacientes con SIDA y HAM/TSP podrán ser útiles para reorientar la neurorehabilitación temprana en estos desórdenes neurodegenerativos asociados a retrovirus. De otro lado, evaluaciones mas sensibles y sofisticadas del sistema piramidal, que permitan detectar cambios tempranos en los circuitos talamocorticoganglionicos serán mandatarios de realizar a partir de la fecha, bien sea que los individuos estén asintomáticos o no, en estadios clínicos donde las fibras corticoespinales rápidas no estén aun comprometidas.

Paraparesia espástica progresiva asociada a HTLV-I en Chile: estudio y seguimiento de 121 pacientes por diez años / Progresive spastic paraparesis associated to HTLV-I in Chile

Revision is made to 121 Chilean patients with progressive adult spastic paraparesis (PSPs) associated to HTLV-I. Epidemiologic, clinical, diagnosis and associated illnesses aspects are analyzed as well as the pathogenesis. The follow-up of patients during several years allowed defining the evolutional profile, establishing the causes of death and studying the virus' behavior. Pathogenesis hypothesis arose from the neuropathological search to define the mechanisms of damage supported on immunohystochemical studies. It was confirmed that the CNS illness is a degenerative process linked to a central axonopathy which expresses flaws in the axoplasmic transport, particularly affecting the corticospinal tracts, although there is a more extended myeloencephalic involvement. Furthermore, the virus is capable of producing a multisystemic illness that may simultaneously involve the nervous system; the hematological system; the exocrine glands; the hepatic, lung, muscular and bone parenchymas.

Se revisan las paraparesias espásticas progresivas del adulto (PEPAs) producidas por el HTLV-I, en 121 pacientes chilenos. Se analizan los aspectos epidemiológicos, clínicos, diagnósticos, las enfermedades asociadas, y la patogenia. El seguimiento de los pacientes durante varios años permitió definir el perfil evolutivo, establecer las causas de muerte y estudiar el comportamiento del virus. De los casos con anatomía patológica surgieron hipótesis, que han permitido definir mecanismos de daño, sustentados en estudios inmunohistoquímicos. Se pudo confirmar que la enfermedad del SNC es un proceso degenerativo, vinculado a una axonopatía central que expresa fallas del transporte axoplásmico, que afecta particularmente la vía corticoespinal, aunque existe un compromiso más extenso mielo-encefálico. Además, el virus es capaz de producir una enfermedad multisistémica, que puede comprometer simultáneamente el sistema nervioso, el sistema hematológico, las glándulas exocrinas, el parénquima hepático, pulmonar, muscular y óseo.

Urodynamic features of the voiding dysfunction in HTLV-1 infected individuals

OBJECTIVE:To describe urodynamic abnormalities in HTLV-1 infected individuals presenting urinary symptoms and verify if these findings and quality of life (QOL) evaluation correlate with overall neurological impairment. MATERIALS AND METHODS: From January/2001 to May/2004, 324 HTLV-1 seropositive subjects were evaluated to determine the occurrence of urinary symptoms. Urodynamic testing was performed in those who complained of frequency, urgency, or incontinence. They went through a complete clinical, neurological, and urological examination to investigate symptoms and signs of myelopathy. Neurological disability was assessed by Expanded Disability Status Scale (EDSS). RESULTS: From the 324 patients evaluated, 78 underwent the urodynamic testing. Fifty-seven individuals were females (73.1 percent) and age ranged from 23 to 76 years (mean = 48.7 years; SD ± 11.6). Urodynamic testing was abnormal in 63 patients (80.8 percent). The major abnormality was detrusor overactivity (DO), observed in 33 individuals (33/63; 52.4 percent), followed by detrusor-external sphincter dyssynergia (DESD), diagnosed in 15 subjects (15/63; 25.4 percent). HAM/TSP patients had significantly more DESD than the HTLV-I carriers (p = 0.005; OR = 5.5; CI: 1.6 to 19.4). QOL was severely compromised in HAM/TSP patients. CONCLUSIONS: Prominent urodynamic abnormalities were identified in individuals genuinely considered as HTLV-I carriers, suggesting an early compromise of the urinary tract; whereas HAM/TSP patients presented urodynamic findings, which posed a potential risk to the upper urinary tract (dyssynergia). Urodynamic evaluation should be performed in all HTLV-I-infected individuals with voiding complaints.

Achados dos potenciais evocados somatossensitivos e motores na mielopatia associada oa HTLV-I / Somatosensitive and motor evoked potentials in HTLV-I associated mylopathy

Foram estudados 63 pacientes com diagnóstico de paraparesia espástica tropical/ mielopatia associada ao HTLV-I (PET/ MAH) através de potencial evocado somatossensitivo (PESS) e 42 destes pacientes através de potencial evocado motor (PEM). Todos os pacientes apresentaram história clínica típica de MAH e sorologia para HTLV-I confirmada por Western blot. Dos pacientes estudados por PESS 51/63 (81 por cento) alterados em membros inferiores e 11 (17,5 por cento) deles estavam também em membros superiores. Dos pacientes estudados por PEM 37/42 estavam alterados, 34/42 (81 por cento) em membros inferiores e 25/42 (59,5 por cento) em membros superiores. Um alto percentual da amostra apresentou alterações das vias corticoespinhais nos quatro membros ao PEM, e ausência de alterações em membros superiores ao PESS, evidenciando a correlação entre o tempo de condução motora central para membros superiores e a gravidade clínica da PET/ MAH (p<0,01). Não foi evidenciado correlação entre o tempo de historia clínica e os achados dos PESS e PEM (p=0,69).

Paraparesia espástica tropical (ou mielopatia associada ao retrovírus HTLV-I - PET/MAH) / Tropical spastic paraparesis (or HTLV-I associated myelopathy - TSP/HAM)

A paraparesia espástica tropical (PET/MAH) é uma mielopatia associada em grande parte ao retrovírus HTLV-I, que pertence à família Retroviridade e subfamília Oncovirinae. Esse vírus é prevalente no Japão, Caribe, América do Sul, África e Melanésia , e no mundo existem cerca de 20 milhões de indivíduos infectados e mais de 2000 casos descritos de PET/MAH. O diagnóstico da infecção é feito por testes sorológicos ( Elisa, descritos de PET/MAH. O diagnóstico da infecção é feito por testes sorológicos ( (Elisa Western blot e mais recentemente INNO-LIA) e moleculares (PCR). Clinicamente , a PET/MAH se caracteriza por uma paraparesia espástica insidiosamente progressiva associada a variáveis distúrbios esfincterianos e sensitivos. Os critérios diagnósticos foram propostos pela OMS e têm sido usado como guia na definição clínica e laboratorial . tais como LCR, estudo de potenciais evocados somatossentivos e ressonância nuclear magnética, têm sido utilizados para confirmação e diagnóstico diferencial da PET/MAH. Do ponto de vista neuropatológico, a PET/MAH apresenta lesões inflamatórias e desmielinizantes centradas principalmente na medula torácica baixa.As hipóteses fisiopagênicas atuais apontam para mecanismos imunológicos, de onde tentativas terapêuticas pertinentes advêm.

HTLV-I associated myelopathy in the northern region of Brazil (BelÚm-Parß): serological and clinical features of three cases

Three patients (males, black, ages 37, 40 and 57) attended a university clinic with a progressive paraparesis of obscure origin. One patient who referred disease duration of more than 16 years, showed diminished deep reflexes, bilateral Babinski's sign, diminished sensation of vibration, abnormal bladder function and back pain. The other two patients (with one and six years of disease duration) complained of weakness in one leg, increased deep reflexes and back pain. Babinski's sign and bladder disturbance were also present in the patient with six years of disease. Blood samples tested by an enzyme immune assay and a discriminatory Western blot were positive for HTLV-I. The familial analysis of one patient showed a possible pattern of sexual and vertical transmission of the virus. To the best of our knowledge, these are the first cases of a proven association between HTLV-I and TSP/HAM in Belem, Para, and emphasize the need to actively look for cases of neurological disease associated to the virus.

Eletroneuromiografia e potenciais evocados somatossensitivos na mielopatia pelo HTLV-I / Electroneuromyography and somatosensory evoked potentials in HTLV-I associated myelopathy

Tivemos como objetivo investigar por eletroneuromiografia (ENMG) e potenciais evocados somatossensitivos a possibilidade de acometimento do nervo periférico na mielopatia pelo HTLV-I (HAM), correlacionando os achados com os parâmetros clínicos e com a síntese intratecal de anticorpos anti HTLV-I. Os pacientes tinham sorologia negativa para HIV e apresentaram VDRL negativo. Outras causas para mielopatia ou neuropatia periférica foram excluídas. De 32 pacientes que realizaram ENMG, em 34,3 por cento ela foi considerada sugestiva de neuropatia periférica. Esta foi sobretudo assimétrica (82 por cento), sensitivo-motora (90 por cento), com padrao axonal (54,5 por cento) ou misto (45,4 por cento). Em 63,6 por cento dos casos de neuropatia periférica, havia sintomas correlatos. O potencial evocado auditivo foi anormal em apenas um caso. O potencial evocado visual foi normal em 28,5 por cento dos casos. Nao havia sinais ou sintomas auditivos ou visuais. Em 85,5 por cento dos casos obteve-se potencial evocado sensitivo alterado. Desses, 50 por cento tinham manifestaçao clínica compatível. Em 28 por cento dos pacientes com potencial evocado sensitivo anormal houve concomitância de ENMG sugestiva de neuropatia periférica.

Further studies on HTLV-I associated myelopathy in Argentina

We report 10 HTLV-I virus seropositive subjects, eight of them with HTLV-I associated myelopathy (HAM), two of them also infected with HIV as well as two asymptomatic HTLV-I+ relatives of two unrelated patients. HTLV-I is endemic in several tropical areas, where it causes different neurological diseases. Only few patients have been reported in our country since 1994. We studied 8 patients, who fulfilled the clinical criteria for chronic spastic paraplegia, and 2 other non-symptomatic HTLV-I seropositive relatives, with electromyography (EMG), motor and sensory conduction velocities (NCV), somatosensory, visual and brainstem auditory evoked potentials (SSEP, VEP and BAEP), Magnetic Resonance Images (MRI) and cerobrospinal fluid (CSF) analysis. The latter was carried out only in seven symptomatic patients. In every case positive ELISA tests for HTLV-I/II were confirmed by Western Blot. The two asymptomatic persons were clinically and electromyographically assessed, one of them was also submitted to SSEPs studies. Three patients were males. Patient's ages ranged from 5 to 65 years old. All symptomatic patients showed muscular weakness, spasticity with pyramidal signs and sphincter disturbances. Five of them had paresthesias and 2 had burning pain on their feet. The EMGS and the NCVs were normal in 7 patients and in the 2 asymptomatic ones. SSEPs, obtained by stimulating the posterior tibial nerves, were impaired in 7 patients and in the asymptomatic person who received the procedure. The 7 symptomatic patients who underwnt lumbar puncture had positive tests for HTLV-I in CSF, 3 out of these 7 patients had also high protein levels and 4 had increased number of lymphocytes. In 2 patients intrathecal IgG production could also be demonstrated. MRI were normal in 7 patients and in the 2 asymptomatics, the exception being a female who had bilateral hypertense lesions in cerebral white matter in T2. In conclusion, tropical spastic paraparesis is apparently a rare disorder in Argentina. However, some cases have been reported recently. Most probably, its prevalence is currently underestimated. Its diagnosis should be considered in every patient with progressive spastic paraplegia.

Adult T-cell leukemia-lymphoma in a patient with HTLV-I/II associated myelopathy

Chronic myelopathy associated with T-lymphotropic virus type I (HAM) has been described as an endemic disease in several areas of the world, meanwhile there are few papers describing the association between HAM and adult T cell leukemia-lymphoma. We report the case of a man that, after four years of progressive spastic paraparesis and neurogenic bladder, developed a clinical picture of a lymphoproliferative disorder characterized by dermal and systemic envolviment, mimicking mycosis fungoides/Sézary syndrome.

Reflejo orbicular de los párpados en la paraparesia espástica tropical y otras enfermedades neurológicas

El reflejo orbicular de los ojos (ROO) obtenido por estimulación eléctrica produce usualmente dos respuestas (R1 y R2); sin embargo, unas poco comunes como la "respuesta temprana contralateral" (R1K), o el registro de dos o más componentes en la respuesta intermedia (R2) son más importantes de lo que hasta ahora se había creído. Estos "raros" hallazgos han sido apreciados en entidades que afectan el sistema nervioso periférico como la parálisis facial y el sistema nervioso central como la enfermedad de Parkinson (EP), varias clases de distonías y la paraparesia espástica tropical/mielopatía asociada al HTLV-I(HAM/TSP). Dichos registros poco comunes se han descrito en la esclerosis lateral amiotrófica (ELA) o la esclerosis múltiple (EM). Trastornos de la regeneración axonal, secundarios a un daño nervioso periférico y/o fallas en la inhibición interneuronal mediada por la corteza cerebra, el tracto piramidal o los ganglios basales (GB), parecen ser los mecanismos más importantes de estas respuestas principalmente a nivel patológico. De otro lado, la respuesta tardía (R3) del ROO, descrita más recientemente, viaja por rutas nerviosas diferentes a las conocidas para las dos primeras respuestas (R1, R1K y R2) y se considera esencialmente nociceptiva. Esta respuesta es modulada a nivel de tallo cerebral, y muy probablemente, regulada por la corteza cerebral, y además, parece ser importante en el estudio fisiopatológico de las vías relacionadas con el dolor en el ser humano. Las diferencias del ROO entre la HAM/TSP y la esclerosis múltiple (EM) podrían ser útiles para comprender los mecanismos de la espasticidad en éstas y otras entidades relacionadas en futuros estudios clínicos y neurofisiológicos.

Diagnostico y tratamiento de la neuropatia endemica cronica asociada con virus humano linfotropico de celulas T, tipo I (HTLV-1) / Diagnosis and treatment of chronic endemic neuropathy associated with human lymphotropic virus of T cells, type I (HTLV-1)

An abridged review of the current status of chronic endemic neuropathy associated with human lymphotropic virus type I is presented. Tropical and non trpopical spastic paraparesis are associated with HTL V -I antibodies and the virus has been isolated from the blood and spinal fluid of patients, and probably they are a similar process. The case of a 57 year old black female from St. Croix, USVI, with a six year history of progressive spastic paraparesis, sphincter disturbances and subjective sensory complaints is described. Antibodies to HTL V-I were detected by the Elisa and confirmed by Western blot. The patient was treated with Zidovudine 200 mgrs three times a day for six weeks. At the end of this period she was able to walk without assistance, dance, jump and squat. Spasticity, sensory and sphinter disturbances disappeared