Encephalomyelopathy associated with HTLV-I a primary disease or coexisting with multiple sclerosis / Encefalomielopatia associada ao HTLV-I: doença primária ou coexistência com esclerose múltipla

HTLV-I-associated myelopathy (HAM/TSP) is the most common neurological manifestation of HTLV-I, causing progressive weakness, sensory disturbance, and sphincter dysfunction. Although motor disorders have been well described, few studies have associated cognitive disorders and HTLV-I infection. In areas endemic for HTLV-I infection, the differential diagnosis between HAM/TSP and other myelopathy etiologies can be difficult, particularly if the patient has signs and symptoms of brain involvement, since seropositive HTLV-I patients can present other neurological diseases. Here, we report one case initially diagnosed as Multiple Sclerosis (MS) which, upon further investigation, was found to be HTLV-I seropositive.

HTLV-I causando fraqueza progressiva, alterações de sensibilidade e disfunção esfincteriana. As alterações motoras são bem descritas, mas ainda são poucos os estudos que examinam a possibilidade de ocorrência de transtornos cognitivos na infecção pelo HTLV-I. Em áreas endêmicas para o HTLV-I, o diagnóstico diferencial com outras causas de mielopatias pode ser difícil, particularmente se o paciente tem sinais e sintomas de acometimento encefálico, já que a sorologia positiva para o HTLV-I pode ser detectada em pacientes com outras doenças neurológicas. Aqui relata-se o caso de uma paciente inicalmente diagnosticada com Esclerose Múltipla e que, na investigação posterior, foi encontrado soropositividade para HTLV-I.

Incidence of human T cell lymphotropic virus type 1-associated myelopathy/tropical spastic paraparesis in a long-term prospective cohort study of initially asymptomatic individuals in Brazil.

The incidence of human T cell lymphotropic virus type 1 (HLTV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is not well defined in the literature. Several studies have reported different incidence rates, and recent publications suggest a higher incidence and prevalence of HAM/TSP. The interdisciplinary HTLV Research Group (GIPH) is a prospective open cohort study of individuals infected with HTLV-1/2. This study describes the demographic data and HAM/TSP incidence rate observed in 181 HTLV-1-seropositive individuals and compares the results with previous reports in the literature. HAM/TSP was diagnosed on the basis of the World Health Organization diagnostic criteria and De Castro-Costa et al. [Proposal for diagnostic criteria of tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM). AIDS Res Hum Retroviruses 2006;22:931-935]. Seven HAM/TSP incident cases were observed during the follow-up. The HAM/TSP incidence density was 5.3 cases per 1,000 HTLV-1-seropositive cases per year (95% confidence interval: 2.6-10.9), with a mean follow-up of 7±4 years (range: 1 month to 15 years). HAM/TSP was more frequent in women in their 40s and 50s with probable infection via the sexual route. The HAM/TSP incidence density among HTLV-1-seropositive cases observed in the present study is higher than that in previous studies. HAM/TSP may be underdiagnosed in countries like Brazil where HTLV infection is prevalent. Orientation and prevent transmission of HTLV programs are needed. Currently, preventing HTLV-1 transmission is the most effective way to reduce the impact of HAM/TSP on society.

Encephalomyelopathy associated with HTLV-I a primary disease or coexisting with multiple sclerosis?

HTLV-I-associated myelopathy (HAM/TSP) is the most common neurological manifestation of HTLV-I, causing progressive weakness, sensory disturbance, and sphincter dysfunction. Although motor disorders have been well described, few studies have associated cognitive disorders and HTLV-I infection. In areas endemic for HTLV-I infection, the differential diagnosis between HAM/TSP and other myelopathy etiologies can be difficult, particularly if the patient has signs and symptoms of brain involvement, since seropositive HTLV-I patients can present other neurological diseases. Here, we report one case initially diagnosed as Multiple Sclerosis (MS) which, upon further investigation, was found to be HTLV-I seropositive.

A mielopatia associada ao HTLV­I (HAM/TSP) é a manifestação neurológica mais frequente do HTLV-I causando fraqueza progressiva, alterações de sensibilidade e disfunção esfincteriana. As alterações motoras são bem descritas, mas ainda são poucos os estudos que examinam a possibilidade de ocorrência de transtornos cognitivos na infecção pelo HTLV-I. Em áreas endêmicas para o HTLV-I, o diagnóstico diferencial com outras causas de mielopatias pode ser difícil, particularmente se o paciente tem sinais e sintomas de acometimento encefálico, já que a sorologia positiva para o HTLV-I pode ser detectada em pacientes com outras doenças neurológicas. Aqui relata-se o caso de uma paciente inicalmente diagnosticada com Esclerose Múltipla e que, na investigação posterior, foi encontrado soropositividade para HTLV-I.

[HTLV-1 associated myelopathy: clinical and epidemiological profile in a 10-year case series study]. / Mielopatia associada ao HTLV-1: análise clínico-epidemiológica em uma série de casos de 10 anos.

INTRODUCTION: Human T cell lymphotropic virus type 1 (HTLV-I) myelopathy (HAM/TSP) is a progressive disabling disorder. This work aimed to analyze clinical features and epidemiology in a sample of HAM/TSP. METHODS: All HTLV-1 infected patients with diagnostic criteria for HAM/TSP, consecutively admitted to the Sarah Hospital from 1998 to 2007, were included in the study. RESULTS: 206 patients (67% females; mean age: 53.8 years-old) were diagnosed with HAM/TSP. The mean time of evolution was 9.0 years. The most common neurological symptoms were chronic progressive spastic paraparesis, spasticity, pain, neurogenic bladder and neurogenic bowel. The neurological findings were hyperreflexia, Babinsky, Hoffman and peripheral neuropathy. Pain, spasticity and spinal cord atrophy, observed in MRI, were associated with time of disease (p<0.05). CONCLUSIONS: HAM/TSP is a very disabling disorder, in which pain is reported early, while spasticity and thoracic spinal cord atrophy appear in a later phase of the disease. Cases of HAM/TSP exist with a probable vertical viral transmission.

Mielopatia associada ao HTLV-1: análise clínico-epidemiológica em uma série de casos de 10 anos / HTLV-1 associated myelopathy: clinical and epidemiological profile in a 10-year case series study

INTRODUÇÃO: A mielopatia associada ao retrovírus HTLV-1 (HAM/TSP) é uma doença progressiva e incapacitante. O objetivo deste trabalho é determinar características clínico-epidemiológicas de pacientes com HAM/TSP. MÉTODOS: Série de casos admitidos de 01/1998 a 12/ 2007, em hospital de reabilitação utilizando os critérios diagnósticos de HAM/TSP. RESULTADOS: Participaram 206 pacientes, dos quais, 67 por cento eram mulheres, com 53 anos de média de idade, nove anos de média de duração de doença. Os sintomas mais frequentes foram a diminuição da força em membros inferiores, espasticidade, dor, presença de bexiga neurogênica e a constipação intestinal. Os sinais neurológicos foram hiperreflexia, Babinsky, Hoffmann e neuropatia periférica. A presença de dor, de espasticidade muscular e de atrofia medular à ressonância nuclear magnética de medula espinhal foram associadas à duração da doença (p<0,05). CONCLUSÕES: A HAM/TSP é uma doença de curso incapacitante e progressiva, em que a dor é relatada precocemente, enquanto a atrofia medular torácica e a espasticidade surgem em fase mais tardia. Existem casos de HAM/TSP com provável transmissão do vírus por via vertical.

INTRODUCTION: Human T cell lymphotropic virus type 1 (HTLV-I) myelopathy (HAM/TSP) is a progressive disabling disorder. This work aimed to analyze clinical features and epidemiology in a sample of HAM/TSP. METHODS: All HTLV-1 infected patients with diagnostic criteria for HAM/TSP, consecutively admitted to the Sarah Hospital from 1998 to 2007, were included in the study. RESULTS: 206 patients (67 percent females; mean age: 53.8 years-old) were diagnosed with HAM/TSP. The mean time of evolution was 9.0 years. The most common neurological symptoms were chronic progressive spastic paraparesis, spasticity, pain, neurogenic bladder and neurogenic bowel. The neurological findings were hyperreflexia, Babinsky, Hoffman and peripheral neuropathy. Pain, spasticity and spinal cord atrophy, observed in MRI, were associated with time of disease (p<0.05). CONCLUSIONS: HAM/TSP is a very disabling disorder, in which pain is reported early, while spasticity and thoracic spinal cord atrophy appear in a later phase of the disease. Cases of HAM/TSP exist with a probable vertical viral transmission.

HTLV-1 associated myelopathy diagnosed during lepromatous leprosy reaction treatment: a case report.

Leprosy and human T cell lymphotropic virus type 1 infection are prevalent in Brazil. Coinfection by Mycobacterium leprae and HTLV-1 is reviewed and a case is reported. A 59 year-old woman was followed and HTLV-1 associated myelopathy was diagnosed during leprosy treatment. The clinical and neurological aspects of this unusual association were initially reviewed. Immunological markers and the possible prognoses due to the association of the diseases were discussed. The unexpected association of leprosy and HTLV-1 associated myelopathy may occur in endemic areas and causes difficulties in determining the correct diagnosis and adequate management of the neurological manifestations.

HTLV-1 associated myelopathy diagnosed during lepromatous leprosy reaction treatment: a case report / Mielopatia associada ao HTLV-1 diagnosticada durante tratamento de hanseníase virchowiana reacional: relato de caso

Leprosy and human T cell lymphotropic virus type 1 infection are prevalent in Brazil. Coinfection by Mycobacterium leprae and HTLV-1 is reviewed and a case is reported. A 59 year-old woman was followed and HTLV-1 associated myelopathy was diagnosed during leprosy treatment. The clinical and neurological aspects of this unusual association were initially reviewed. Immunological markers and the possible prognoses due to the association of the diseases were discussed. The unexpected association of leprosy and HTLV-1 associated myelopathy may occur in endemic areas and causes difficulties in determining the correct diagnosis and adequate management of the neurological manifestations.

Hanseníase e infecção pelo HTLV-1 são prevalentes no Brasil. A associação de hanseníase e infecção por HTLV-1 é revista e é relatado um caso de coinfecção. Paciente feminina de 59 anos teve diagnóstico de mielopatia associada ao HTLV-1 durante o tratamento para hanseníase. Aspectos clínicos e neurológicos desta associação, ainda não descrita, são revistos e os marcadores imunológicos e possíveis evoluções relacionadas com a associação dessas doenças discutidos. A associação de hanseníase e mielopatia associada ao HTLV-1, aparentemente pouco usual, pode ocorrer em áreas endêmicas e trazer dificuldades para o diagnóstico e tratamento das manifestações neurológicas.

Epidemiology, treatment, and prevention of human T-cell leukemia virus type 1-associated diseases.

Human T-cell leukemia virus type 1 (HTLV-1), the first human retrovirus to be discovered, is present in diverse regions of the world, where its infection is usually neglected in health care settings and by public health authorities. Since it is usually asymptomatic in the beginning of the infection and disease typically manifests later in life, silent transmission occurs, which is associated with sexual relations, breastfeeding, and blood transfusions. There are no prospects of vaccines, and screening of blood banks and in prenatal care settings is not universal. Therefore, its transmission is active in many areas such as parts of Africa, South and Central America, the Caribbean region, Asia, and Melanesia. It causes serious diseases in humans, including adult T-cell leukemia/lymphoma (ATL) and an incapacitating neurological disease (HTLV-associated myelopathy/tropical spastic paraparesis [HAM/TSP]) besides other afflictions such as uveitis, rheumatic syndromes, and predisposition to helminthic and bacterial infections, among others. These diseases are not curable as yet, and current treatments as well as new perspectives are discussed in the present review.

Vestibular-evoked myogenic potential (VEMP) to evaluate cervical myelopathy in human T-cell lymphotropic virus type I infection.

STUDY DESIGN: Cross-seccional analysis. OBJECTIVE: To define the clinical usefulness of vestibular-evoked myogenic potential (VEMP) in detecting cervical medullar involvement related to human T-cell lymphotropic virus type 1 (HTLV-1) associated myelopathy/tropical spastic paraparesis (HAM/TSP). SUMMARY OF BACKGROUND DATA: VEMP is generated by acoustic or galvanic stimuli, passing through the vestibulo-spinal motor tract, the spinal nerves and recorded by means of surface electrodes on the sternocleidomastoid muscle. HAM/TSP is a progressive inflammatory myelopathy with predominant lesions at the thoracic spinal cord level, although the cervical spine can be affected. VEMP may be of value to investigate cervical myelopathy. METHODS: Seventy-two individuals were evaluated of whom 30 HTLV-1 were seronegative and 42 HTLV-1 seropositive (22 asymptomatic, 10 with complaints of walking difficulty without definite HAM/TSP and 10 with definite HAM/TSP). VEMP was recorded using monaural delivered short tone burst (linear rise-fall 1 millisecond, plateau 2 milliseconds, 1 KHz) 118 dB NA, stimulation rate of 5 Hz, analysis time of 60 milliseconds, 200 stimuli, band pass filtered between 10 and 1.500 Hz. RESULTS: VEMP was normal in the seronegative group (30 controls). In the seropositive, abnormal VEMP was seen in 11 of 22 (50%) of the HTLV-1 asymptomatic carriers, in 7 of 10 (70%) of those with complaints of walking difficulty and in 8 of 10 (80%) of the HAM/TSP patients. In this last group, the pattern of response was different. No VEMP response was more frequent when compared with the HTLV-1 asymptomatic group (2-tailed P-value = 0.001). CONCLUSION: VEMP may possibly be useful to identify patients with cervical myelopathy and to distinguish variable degrees of functional damage. Minor injury would be related to latency prolongation and major injury to no potential-evoked response.

[Schistosomal myeloradiculopathy]. / Mielorradiculopatia esquistossomótica.

Schistosomal myeloradiculopathy is the most severe and disabling ectopic form of schistosomiasis mansoni. Its prevalence in endemic areas has been underestimated. The diagnosis relies on the presence of low thoracic/upper lumbar neurological symptoms, demonstration of the Schistosoma mansoni infection by microscopic or serologic techniques, and exclusion of other causes of transverse myelitis. When treatment with antischistosomal drugs and corticosteroids is started early, the clinical response is surprisingly good and those left untreated do not improve and frequently die. There is no consensus about doses and duration of treatment, but a recent study suggests that when steroids are given for at least 6 months clinical improvement is enhanced. As the diagnosis of SMR is presumptive and treatment is essentially clinical, physicians should be aware of the disease and more research is needed to increase the accuracy of the diagnostic methods and, hence, to avoid routine laminectomy. With the advent of magnetic resonance imaging of the spinal cord the diagnosis of this ectopic form of the disease was facilitated. In accordance, the number of cases of schistosomal myelopathy reported is increasing rapidly.

Mielorradiculopatia esquistossomótica / Schistosomal myeloradiculopathy

A mielorradiculopatia esquistossomótica é a forma ectópica mais grave e incapacitante da infecção pelo Schistosoma mansoni. A sua prevalência em área endêmica tem sido subestimada. O diagnóstico baseia-se na presença de sintomas neurológicos decorrentes de lesões da medula espinhal em nível torácico baixo e/ou lombar alto, na demonstração da infecção esquistossomótica por técnicas microscópicas ou sorológicas e na exclusão de outras causas de mielite transversa. O tratamento precoce, com esquistossomicidas e corticoesteróides, mostra-se eficaz na maioria dos casos e os pacientes não tratados não se recuperam ou morrem. Não há consenso sobre doses e duração do tratamento, mas estudo recente sugere que os corticoesteróides devam ser usados por pelo menos seis meses. Como o diagnóstico é presuntivo e o tratamento essencialmente clínico, há que se manter alerta para a presença da doença, aperfeiçoar a propedêutica e, dessa forma, evitar-se a laminectomia rotineira. Com o advento da ressonância magnética da medula espinhal houve grande avanço no diagnóstico da esquistossomose medular. Como conseqüência, o número de casos de mielopatia esquistossomótica relatados tem aumentado rapidamente.

Infecçäo e doença pelos vírus linfotrópicos humanos de células T (HTLV-I/II) no Brasil / Infection and disease caused by the human T cell lymphotropic viruses type I and II in Brazil

A infecçäo pelos vírus HTLV-I/II encontra-se presente em todas as regiöes brasileiras, mas as prevalências variam de um estado para outro, sendo mais elevadas na Bahia, Pernambuco e Pará. As estimativas indicam que o Brasil possui o maior número absoluto de indivíduos infectados no mundo. Testes de triagem de doadores e estudos conduzidos em grupos especiais (populaçöes indígenas, usuários de drogas intravenosas e gestantes) constituem as principais fontes de informaçäo sobre essas viroses em nosso país. O HTLV-I causa a leucemia/linfoma de células T do adulto (LLTA), a paraparesia espástica tropical/mielopatia associada ao HTLV (TSP/HAM), uveíte associada ao HTLV (HAU) e anormalidades dermatológicas e imunológicas. O HTLV-II näo se mostrou associado a nenhuma doença até o momento. O diagnóstico é feito com testes de triagem (ELISA, aglutinaçäo) e confirmatórios (Western Blot, PCR). Estes vírus säo transmitidos pelo sangue e agulhas contaminadas, através de relaçöes sexuais e de mäe para filho, especialmente através do aleitamento materno. Medidas de prevençäo devem focalizar a orientaçäo de doadores soropositivos, mäes infectadas e usuários de drogas intravenosas

[Human T-cell lymphotropic virus type 1(HTLV-1)-associated myelopathy]. / Mielopatia associada ao vírus linfotrópico humano de células T do tipo 1 (HTLV-1).

HTLV-1-associated myelopathy (HAM), also known as tropical spastic paraparesis (TSP), is a chronic progressive demyelinating disease that affects the spinal cord and white matter of the central nervous system. The lifetime incidence of HAM in HTLV-1 carriers is estimated to be less than 5%. Typical time of onset is in the fourth decade of life, with a female-to-male rate of 2:1. Gait disturbance and weakness and stiffness of the lower limbs are common presenting signs and symptoms of HAM. Lower extremities are affected to a much greater degree than upper extremities. Spasticity may be moderate to severe, and lower back pain is common. As the disease progresses, bladder and bowel dysfunction can occur. Sensory involvement is generally mild and can result in a variable degree of sensory loss and dysesthesia. Results of magnetic resonance imaging may be normal, or the scans show atrophy of the spinal cord and nonspecific lesions in the brain. Immunologic evidence suggests that an immune mechanism may play a role in the development of HAM. There is no effective treatment for the myelopathy. Corticosteroids, and INF-gamma may produce transient responses. Danazol, an anabolic steroid, does not improve gait and bladder function. The value of zidovudine (anti-retroviral agent) in the treatment has not been defined yet.

Mielopatia associada ao vírus linfotrópico humano de cÚlulas T do tipo 1 (HTLV-1) / Human T-cell lymphotropic virus type 1(HTLV-1)-associated myelopathy

HTLV-1-associated myelopathy (HAM), also known as tropical spastic paraparesis (TSP), is a chronic progressive demyelinating disease that affects the spinal cord and white matter of the central nervous system. The lifetime incidence of HAM in HTLV-1 carriers is estimated to be less than 5. Typical time of onset is in the fourth decade of life, with a female-to-male rate of 2:1. Gait disturbance and weakness and stiffness of the lower limbs are common presenting signs and symptoms of HAM. Lower extremities are affected to a much greater degree than upper extremities. Spasticity may be moderate to severe, and lower back pain is common. As the disease progresses, bladder and bowel dysfunction can occur. Sensory involvement is generally mild and can result in a variable degree of sensory loss and dysesthesia. Results of magnetic resonance imaging may be normal, or the scans show atrophy of the spinal cord and nonspecific lesions in the brain. Immunologic evidence suggests that an immune mechanism may play a role in the development of HAM. There is no effective treatment for the myelopathy. Corticosteroids, and INF-gamma may produce transient responses. Danazol, an anabolic steroid, does not improve gait and bladder function. The value of zidovudine (anti-retroviral agent) in the treatment has not been defined yet.

[Infection and disease caused by the human T cell lymphotropic viruses type I and II in Brazil]. / Infecção e doença pelos vírus linfotrópicos humanos de células T (HTLV-I/II) no Brasil.

HTLV-I/II infection is present in all regions of Brazil, but its prevalence varies according to the geographical area, being higher in Bahia, Pernambuco and Pará. It has been estimated that Brazil has the highest absolute number of infected individuals in the world. Blood donors screening and research conducted with special groups (indigenous population of Brazil, IV drug users and pregnant women) are the major sources of information about these viruses in our Country. HTLV-I causes adult T cell leukemia/lymphoma (ATLL), HTLV associated myelopathy/tropical spastic paraparesis (HAM/TSP), HTLV associated uveitis (HAU), dermatological and immunological abnormalities. HTLV-II is not consistently associated with any disease. Diagnosis is established using screening (enzymatic assays, agglutination) and confirmatory (Western blot, PCR) tests. The viruses are transmitted by blood and contaminated needles, by sexual relations and from mother to child, especially by breast feeding. Prevention efforts should focus on education of positive blood donors, infected mothers and IV drug users.

Piomiosite tropical / Tropical pyomyosistis: case report

A piomiosite é uma doença bacteriana, típica do clima tropical e de reconhecimento crescente em climas temperados. Afeta os grandes grupos musculares do esqueleto, formamdo abcessos profundos, usualmente associados ao S. Aureus. Em nosso meio näo há uma comunicaçäo sistemática quanto à sua ocorrência. Descreveremos quatro casos de nossa causuística, diagnosticados no período de março/91 a julho/92.