WordNet

an unskilled actor who overacts (同)ham actor
meat cut from the thigh of a hog (usually smoked) (同)jambon, gammon
a licensed amateur radio operator
the 8th letter of the Roman alphabet (同)h
the 20th letter of the Roman alphabet (同)t

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〈U〉〈C〉(豚肉の)『ハム』 / 〈C〉(動物,特に豚の)もも / 〈C〉《しばしば複数形で》(豚肉の)しりとももを合わせた部分 / 〈C〉アマチュア無線家(radio ham) / 〈C〉(大げさに演じる)へぼ役者 / 〈役〉‘を'大げさに演じる / 大げさに演技する
hydrogenの化学記号
鉛筆の硬度 / 《俗》heroin
tritiumの化学記号

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/02/22 19:46:46」(JST)

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Tropical spastic paraparesis (TSP), also known as HTLV-associated myelopathy or chronic progressive myelopathy, is an infection of the spinal cord by Human T-lymphotropic virus resulting in paraparesis, weakness of the legs. As the name suggests, it is most common in tropical regions, including the Caribbean and Africa.

History[edit]

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 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) was established. 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.

Presentation[edit]

Patients with HAM/TSP may also exhibit uveitis (inflammation of the uveal tract of the eye), arthritis (inflammation of one or more joints), pulmonary lymphocytic alveolitis (inflammation of the lung tissues), polymyositis (an inflammatory muscle disease), keratoconjunctivitis sicca (persistent dryness of the cornea and conjunctiva), and infectious dermatitis (inflammation of the skin). Co-factors that may play a role in transmitting the disorder include being a recipient of transfusion blood products (especially before 1989), breastmilk feeding from a seropositive mother, intravenous drug use, or being the sexual partner of a seropositive individual for several years. 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.

Pathogenesis[edit]

When infected by HTLV-1 the host mounts an antigen-specific immune response toward 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.

Vaccine Development[edit]

Currently, there is no licensed vaccine to protect against HTLV-1 or HTLB-2 in the US. At least 500,000 of the individuals infected with HTLV-1 eventually develop an often rapidly fatal leukemia, while others will develop a debilitative myelopathy, and yet others will experience uveitis, infectious dermatitis, or another inflammatory disorder. HTLV-2 is associated with milder neurologic disorders and chronic pulmonary infections. The novel HTLV-3 and HTLV-4 have been isolated only in a few cases. Human T cell lymphotropic virus types I and II (HTLV-I/II) are endemic in certain areas of the world. They cause two life-threatening diseases, adult T cell leukaemia/lymphoma and tropical spastic paraparesis. A vaccine is needed because in developing countries there are no other feasible preventive interventions against these diseases and in Western countries intravenous drug users at high risk for HTLV-I and HTLV-II infections and the health workers in contact with such populations must be protected. A recombinant adenovirus vector that expresses the HTLV-I envelope glycoprotein env in HeLa cells in the clinical trial stage. A pneumococcalpolysaccharide vaccine has elicited significant increases in concentrations of IgG against all 5 serotypes tested at 1 and 6 months after immunization. While there is no present licensed vaccine, there are many factors which make a vaccine against HTLV-1 feasible. The virus displays relatively low antibody production variability, natural immunity does occur in humans, and experimental vaccination using envelope antigens has been shown to be successful in animal models.

Symptoms[edit]

Progressive muscle weakness;
Sensory disturbance
Sphincter dysfunction
Urinary incontinence
Uveitis
Arthritis
Pulmonary lymphocyte alveolitis
Polymyositis
Keratoconjunctivitis sicca
Infectious dermatitis

Prevention[edit]

Blood transfusion products are screened for HTLV-1 antibodies.

Treatment[edit]

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 baclofen or tizanidine. Urinary dysfunction should be treated with self-catheterization or oxybutynin.

Prognosis[edit]

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.

Similar disorders[edit]

A similar disorder can occur in patients with HIV.^[1]

References[edit]

Machigashira N, Yoshida Y, Wang S, Osame M (9 January 2001). "HTLV-1-associated myelopathy/tropical spastic paraparesis with pseudohypoparathyroidism". Neurology 56 (1): 104–6. PMID 11148245.

^ The Merck Manual of Medical Information, Section HIV, Sidebar, Myleopathy

External links[edit]

International Retrovirology Association

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. ヒトTリンパ球好性ウイルスI型：疾患関連、診断、および治療 human t lymphotropic virus type i disease associations diagnosis and treatment
2. ヒトTリンパ球好性ウイルスI型：ウイルス学、病因、および疫学 human t lymphotropic virus type i virology pathogenesis and epidemiology
3. 脊髄に影響を与える疾患 disorders affecting the spinal cord
4. 遺伝性痙性対麻痺 hereditary spastic paraplegia
5. ポリオおよび前角細胞への感染性疾患 polio and infectious diseases of the anterior horn

English Journal

Humoral immune response to HTLV-1 basic leucine zipper factor (HBZ) in HTLV-1-infected individuals.

Enose-Akahata Y, Abrams A, Massoud R, Bialuk I, Johnson KR, Green PL, Maloney EM, Jacobson S.AbstractABSTRACT: BACKGROUND: Human T cell lymphotropic virus type 1 (HTLV-1) infection can lead to development of adult T cell leukemia/lymphoma (ATL) or HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in a subset of infected subjects. HTLV-1 basic leucine zipper factor (HBZ) gene has a critical role in HTLV-1 infectivity and the development of ATL and HAM/TSP. However, little is known about the immune response against HBZ in HTLV-1-infected individuals. In this study, we examined antibody responses against HBZ in serum/plasma samples from 436 subjects including HTLV-1 seronegative donors, asymptomatic carriers (AC), ATL, and HAM/TSP patients using the luciferase immunoprecipitation system.
Retrovirology.Retrovirology.2013 Feb 13;10(1):19. [Epub ahead of print]
ABSTRACT: BACKGROUND: Human T cell lymphotropic virus type 1 (HTLV-1) infection can lead to development of adult T cell leukemia/lymphoma (ATL) or HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in a subset of infected subjects. HTLV-1 basic leucine zipper factor (HBZ) gene has a
PMID 23405908

Altered Expression of Degranulation-Related Genes in CD8(+) T Cells in Human T Lymphotropic Virus Type I Infection.

Malta TM, Silva IT, Pinheiro DG, Santos AR, Pinto MT, Panepucci RA, Takayanagui OM, Tanaka Y, Covas DT, Kashima S.Source1 National Institute of Science and Technology in Stem Cell and Cell Therapy , Center for Cell Therapy and Regional Blood Center, Blood Center of Ribeirão Preto, Ribeirão Preto, São Paulo, Brazil .
AIDS research and human retroviruses.AIDS Res Hum Retroviruses.2013 Feb 5. [Epub ahead of print]
Abstract Human T lymphotropic virus type I (HTLV-1) is the etiological agent of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). CD8(+) T cells may contribute to the protection or development of HAM/TSP. In this study we used SAGE methodology to screen for differentially expres
PMID 23301858

Human T cell lymphotropic virus type 1 viral load variability and long-term trends in asymptomatic carriers and in patients with human T cell lymphotropic virus type 1-related diseases.

Demontis MA, Hilburn S, Taylor GP.SourceSection of Infectious Diseases, Faculty of Medicine, Imperial College London , London, United Kingdom .
AIDS research and human retroviruses.AIDS Res Hum Retroviruses.2013 Feb;29(2):359-64. doi: 10.1089/AID.2012.0132. Epub 2012 Sep 25.
Abstract Human T cell lymphotropic virus type 1 (HTLV-1) proviral load (PVL) in peripheral blood mononuclear cells (PBMCs) is high in patients with adult T cell leukemia/lymphoma or HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and in some asymptomatic carriers, but fluctuate
PMID 22894552

Japanese Journal

難病のバイオマーカー・創薬ターゲット分子探索技術 (特集難治性疾患の克服を目指して)

植田幸嗣
バイオインダストリー 28(4), 22-29, 2011-04
NAID 40018751575

Fulminant Myelopathy following Neurogenic Proximal Weakness Associated with Human T-Cell Lymphotropic Virus Type I Infection

Yamashita Satoshi,Ueda Akihiko,Hirahara Tomoo,Kimura En,Hirano Teruyuki,Uchino Makoto
Internal Medicine 50(8), 919-924, 2011
… The symptoms were inconsistent with the World Health Organization or El Escorial criteria for HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) or amyotrophic lateral sclerosis (ALS). …
NAID 130000650085

Related Pictures

★リンクテーブル★

リンク元	「HTLV-1関連脊髄症」「熱帯性痙性不全対麻痺症」
関連記事	「TS」「T」「TSP」「HAM」「HA」

「HTLV-1関連脊髄症」

Tropical spastic paraparesis
	Classification and external resources
ICD-10	G04.1
DiseasesDB	29487
eMedicine	med/1038
MeSH	D015493

　　[★]

英: HTLV-1-associated myelopathy HTLV-1 associated myelopaty, HTLV-I-associated myelopathy HTLV-I associated myelopathy、human T-lymphotropic virus type I-associated myelopathy, HAM
同: HTLV-I脊髄症、ヒトTリンパ好性ウイルスI型関連脊髄症、HTLV-I型関連ミエロパシー、ヒトTリンパ球向性ウイルスI型関連脊髄症
関: 熱帯性痙性不全対麻痺症 TSP tropical spastic paraparesis、HAM/TSP、ヒトTリンパ球向性ウイルス

[show details]

HTLV-1型関連ミエロパシー : nothing
HTLV-1型関連ミエロパチー : nothing
HTLV-1脊髄症 : 30 件
HTLV-I型関連ミエロパシー : 9 件
HTLV-I型関連ミエロパチー : nothing
HTLV-I脊髄症 : 70 件
ヒトT細胞白血病ウイルス関連脊髄症 : 4 件
ヒトTリンパ球向性ウイルス脊髄症 : 49 件
ヒトT細胞白血病ウイルスI型関連ミエロパシー : 2 件
ヒトTリンパ好性ウイルスI型関連脊髄症 : 10 件
ヒトTリンパ球関連ウイルス脊髄症 : nothing