Abelardo de Q-C. Araújo

Progression of neurological disability in HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP).

HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is apparently a disease with a chronic evolution without spontaneous remissions. The real profile of its natural history and of the progression of the neurological disability, however, awaits confirmation. We devised the present study to evaluate the progression profile of the neurological disability of HAM/TSP in a series of 43 patients who have never received any kind of previous immune therapy. Patients were divided into different groups according to the duration of their disease. Age, gender and the Kurtzkes disability status scale (DSS) at the time of the first examination were compared. There were no statistically significant differences among groups with different disease duration. The present study suggests that the evolution of the neurological disability in HAM/TSP occurs mainly during the first year of the disease and becomes relatively stable after that. Therefore we speculate that the variable therapeutic success rates observed in many series of the literature could be due to the timing in the beginning of the pharmacological immunosuppression. Probably the therapeutic window in HAM/TSP lies within the first year of the disease. Thus it might be of utmost importance that future therapeutical trials take into consideration the duration of the disease since this factor can play an important role in the results of the trial.

Dysautonomia in human T‐cell lymphotrophic virus type I‐associated myelopathy/tropical spastic paraparesis

The frequency and importance of dysautonomia in human T‐cell lymphotrophic virus type I–associated myelopathy/tropical spastic paraparesis (HAM/TSP) have not been fully investigated. We describe the characteristics of dysautonomia in such patients in a case‐control study. Our results indicate that autonomic disturbances are more frequent in HAM/TSP than has been previously suggested, with a predominance of sympathetic nervous system dysfunction. In some of these patients, the symptoms may be severe enough to warrant specific treatment.

Spastic paraparesis of obscure origin: A case-control study of HTLV-I positive and negative patients from Rio de Janeiro, Brazil

In order to find clinical findings that could significantly discriminate between HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and HTLV-I negative spastic paraparesis of obscure origin (SPOneg) prior to serological testing, and to find risk factors significantly associated with HAM/TSP we devised a case-control study. Sixty consecutive SPO patients were studied without previous knowledge of their HTLV-I serological status. Thirty-four (56.7%) turned out to be HTLV-I positive and 26 (43.3%) HTLV-I negative. HTLV-I infected patients tended to have more commonly motor and bladder disturbances at the beginning of their illness and a disease that was still in progression at the time of the examination. Bladder dysfunction, constipation and penile impotence, and more widespread pyramidal signs, were also more frequent during the whole course of their illness. Likewise, an increased intrathecal synthesis of IgG was found more often in the HTLV-I positive group. The only risk factor for HTLV-I infection significantly associated to HAM/TSP was a prior history of sexually transmitted diseases. These results suggest that, at least in RJ, HAM/TSP might be mainly sexually acquired.

Perception of disability in a public health perspective: a model based on fuzzy logic

Measures of functional levels, commonly used to assess the safety and quality of life of individuals and populations, have not yet been derived from a fuzzy framework. The aim of this study is to estimate the degree of disability associated with varying functional levels, through a model based on fuzzy sets theory. A fuzzy linguistic model was developed to measure varying levels of functional disability, in accordance with the definitions of an individuals social and physical activities and mobility. One year of an adults life whose mobility, social and physical activities were somewhat limited, was judged to be equivalent to 0.575 years free of functional disability. Results obtained from the fuzzy model approach those obtained with the quality of well-being scale (QWB), used as a conceptual framework. Such findings are encouraging, since the QWB is considered a consistent and valid approach for disability assessment and quality-of-life evaluation.

Falls in patients with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP).

Study design:Cross-sectional study.Objectives:To determine the prevalence of falls in human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients and possible factors associated to their occurrence.Setting:Instituto de Pesquisa Clínica Evandro Chagas, Fundação Oswaldo Cruz (FIOCRUZ) - Brazil.Methods:Thirty-six HAM/TSP patients able to walk at least 20 m were assessed by a questionnaire. Data regarding gender, age, duration of disease (DD), HTLV-I proviral load (HPL), frequency of physical activity (FCA), use of walking aids, functional ambulation level, the number of falls and associated injuries in the last year were reviewed. Multiple correspondence analysis was used to group characteristics of this sample according to the fall occurrence.Results:The prevalence of falls was 63.9% and we observed injuries in 47.8% of the cases. Four groups were identified in the descriptive analysis. One group was formed by faller individuals, men <60 years, independent ambulation, FCA⩾3 times per week and HPL <6.6 copies per 100 cells (group B). The other one comprised non-faller patients, women ⩾60 years, restricted ambulation, DD ⩾7 years, use of orthosis, FCA 0-1 time per week and HPL ⩾6.6 copies per 100 cells (group D). The others two groups comprised individuals that did not use orthosis (group A) and those that FCA was two times per week and DD <7 years (group C).Conclusion:Falls occur in roughly two-thirds of ambulatory HAM/TSP patients and are associated with significant morbidity. Further studies with a larger number of patients are necessarily to identify risk factors in order to elaborate specific programs to prevent falls in this population.

Doença de Segawa: distonia progressiva sensível à L-dopa. Relato de caso

Segawas disease (SD) is a hereditary progressive dystonia with marked diurnal fluctuation with onset in childhood or adolescence and a striking responsiveness to L-dopa. Here we describe a typical case of SD in a 28 year old woman whose disease begun at the age of 18 years. This patient had a second cousin with probable hereditary spastic paraplegia (Strumpells familiar spastic paraplegia) who had no benefit on a recent L-dopa trial. Due to this family history our patient had been misdiagnosed as Strumpells disease for more than 10 years. There was no other apparent case of SD in the family. Her father had an atypical gait but was otherwise normal. Her daughter had motor developmental delay due to hypotonia. Pes cavus was a common feature to the patient, her father and her cousin.Segawas disease (SD) is a hereditary progressive dystonia with marked diurnal fluctuation with onset in childhood or adolescence and a striking responsiveness to L-dopa. Here we describe a typical case of SD in a 28 year old woman whose disease begun at the age of 18 years. This patient had a second cousin with probable hereditary spastic paraplegia (Strumpells familiar spastic paraplegia) who had no benifit on a recent L-dopa trial. Due to this family history our patient had been misdiagnosed as Strumpells disease for more than 10 years. There was no other apparent case of SD in the family. Her father had an atypical gait but was otherwise normal. Her daughter had motor developmental delay due to hypotonia. Pes cavus was a common feature to the patient, her father and her cousin.

A neuropatogenia do vírus da imunodeficiência humana

The spreading of human immunodeficiency virus (HIV) infection and its increasing scientific knowledge keep the medical staff involved with these patients in permanent need of updating themselves. The different neurologic manifestations caused by HIV are related to a variety of pathogenic mechanisms, as follows: immunodeficiency, autoimmunity, direct effects of the virus on the nervous system, and toxic and metabolic effects. The opportunistic infections are caused by the immunodeficiency due to the action of the virus on CD4+ T cells and on cells of the monocytic-macrophage lineage. Demyelinating polyradiculoneuropathy and polymyositis-like syndromes are related to autoimmune mechanisms involving, probably, the non-specific stimulation of T cells by viral proteins. The primary action of the virus on the nervous system brings out aseptic meningitis, cognitive dysfunction, dementia, vacuolar myelopathy and sensory polyneuropathy probably through liberation of neurotoxic products by the infected macrophages. Antiretroviral drugs and others used to treat patients with AIDS may also have neurotoxic effects. The better understanding of the neuropathogenesis of HIV infection will permit the use of new, and more specific, therapeutical options in the future as well as a more precocious control of its neurologic complications.