Tropical spastic paraparesis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Tropical spastic paraplegia
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Tropical spastic paraparesis is an infection of the spinal cord by Human T-lymphotropic virus resulting in paraparesis or weakness of the legs. As the name suggests, it is most common in tropical regions, including the Caribbean and Africa.
Treatment
Medical Therapy
There is no established treatment program for HAM/TSP although some patients may be given steroids. Clinical studies using interferon alpha and plasmapheresis have not shown significant patient improvement. Spasticity may be treated with lioresal or tizanidine. Urinary dysfunction should be treated with self-catheterization or oxybutynin.
Primary Prevention
Blood transfusion products are screened for HTLV-1 antibodies.
References
Historical Perspective
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Historical Perspective
For several decades the term tropical spastic paraparesis was used to describe a chronic and progressive clinical syndrome that affected adults living in equatorial areas of the world. This condition was initially thought to be associated with infectious agents (such as Treponema pertenue and Treponema pallidum which cause inflammation of the central nervous system) and with chronic nutritional deficiencies (such as avitaminosis) or exposure to potentially toxic foods (such as bitter cassava). Neurological and modern neuroepidemiological studies found that in some individuals no single cause could explain the progressive weakness, sensory disturbance, and sphincter dysfunction that affected individuals with TSP. In spite of public health programs created to eradicate the above-mentioned infectious and nutritional conditions in the tropics, large numbers of people continued to be affected.
During the mid-1980s, an important association was established between the first human retrovirus-human T-cell lymphotrophic virus type 1 (also known as HTLV-1)-and idiopathic TSP (idiopathic means of unknown origin). Since then, this condition has been named HTLV-1 associated myelopathy/ tropical spastic paraparesis or HAM/TSP and scientists now understand that it is a condition caused by a virus that results in immune dysfunction.
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Classification
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Pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Pathophysiology
When infected by HTLV-1[1] the host mounts an antigen specific immune response towards the HTLV-1 antigen. Cytotoxic T-lymphocytes of the host’s immune response release cytokines in an effort to fight the infection. These cytokines facilitate the transendothelial migration of lymphocytes across the blood-brain barrier. Once cytokines are within the central nervous system demyelination is brought as a result of bystander cell injury. The disease is chronic, progressing slowly, usually causing symptoms 20-30 years after infection.
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Causes
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Differentiating Tropical spastic paraparesis from other Diseases
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Epidemiology and Demographics
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Risk Factors
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Natural History, Complications and Prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Natural History, Complications and Prognosis
Complications
Not every HTLV-1 seropositive carrier will become a HAM/TSP patient. Fewer than 5% will exhibit neurological dysfunction or, eventually, hematological malignancy such as adult T-cell leukemia/lymphoma, suggesting that other host or viral factors are responsible for disease onset.
Prognosis
HAM/TSP is usually a progressive neurological disorder but it is rarely fatal. Most patients live for several decades after the diagnosis. Their prognosis improves if they take steps to prevent urinary tract infection and skin sore formation, and if they enroll in physical and occupational therapy programs.
References
Diagnosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | Other Diagnostic Studies
Treatment
Treatment
Medical Therapy | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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