U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

HTLV-1-associated myelopathy-tropical spastic paraparesis(HAM)

MedGen UID:
18298
•Concept ID:
C0030481
•
Disease or Syndrome
Synonyms: FAMILIAL SPASTIC PARAPARESIS, HTLV-1-ASSOCIATED; HAM; Human T-cell leukemia virus type 1 associated myelopathy/tropical spastic paraparesis; MYELOPATHY, HTLV-1-ASSOCIATED; Paraparesis, Tropical Spastic; Tropical spastic paraparesis (formerly)
SNOMED CT: Tropical spastic paraplegia (714279000); HTLV-I-associated myelopathy (714279000); Tropical spastic paraparesis (714279000); Human T-cell lymphotropic virus 1-associated myelopathy (714279000); Myelopathy caused by Human T-lymphotropic virus 1 (714279000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0008039
OMIM®: 159580
Orphanet: ORPHA289326

Definition

A progressive chronic inflammatory disease of the central nervous system with the aetiologic agent Human T cell lymphotropic virus type I (HTLV-I), the disease is characterised by unremitting myelopathic symptoms such as spastic paraparesis, bowel and/or bladder dysfunction and sensory changes of the lower limbs. [from SNOMEDCT_US]

Clinical features

From HPO
Spastic paraparesis
MedGen UID:
52432
•Concept ID:
C0037771
•
Sign or Symptom
Mild or moderate loss of motor function accompanied by spasticity in the lower extremities. This condition is a manifestation of CENTRAL NERVOUS SYSTEM DISEASES that cause injury to the motor cortex or descending motor pathways.
See: Feature record | Search on this feature
Myelopathy
MedGen UID:
11550
•Concept ID:
C0037928
•
Disease or Syndrome
A non neoplastic or neoplastic disorder that affects the spinal cord.
See: Feature record | Search on this feature
Abnormal pyramidal sign
MedGen UID:
68582
•Concept ID:
C0234132
•
Sign or Symptom
Functional neurological abnormalities related to dysfunction of the pyramidal tract.
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHTLV-1-associated myelopathy-tropical spastic paraparesis
Follow this link to review classifications for HTLV-1-associated myelopathy-tropical spastic paraparesis in Orphanet.

Professional guidelines

PubMed

Mogamulizumab for Treatment of Human T-lymphotropic Virus Type 1-Associated Myelopathy/Tropical Spastic Paraparesis: A Single-Center US-based Series.
Meyerowitz EA, Mukerji SS, Kyle Harrold G, Erdil RM, Chen ST, Rudmann EA, Tsibris A, Venna N, Robbins GK
Clin Infect Dis 2023 Sep 18;77(6):851-856. doi: 10.1093/cid/ciad281. PMID: 37157862Free PMC Article
The CC chemokine ligand (CCL) 1, upregulated by the viral transactivator Tax, can be downregulated by minocycline: possible implications for long-term treatment of HTLV-1-associated myelopathy/tropical spastic paraparesis.
Saito M, Sejima H, Naito T, Ushirogawa H, Matsuzaki T, Matsuura E, Tanaka Y, Nakamura T, Takashima H
Virol J 2017 Dec 4;14(1):234. doi: 10.1186/s12985-017-0902-6. PMID: 29202792Free PMC Article
Highly active antiretroviral treatment against STLV-1 infection combining reverse transcriptase and HDAC inhibitors.
Afonso PV, Mekaouche M, Mortreux F, Toulza F, Moriceau A, Wattel E, Gessain A, Bangham CR, Dubreuil G, Plumelle Y, Hermine O, Estaquier J, Mahieux R
Blood 2010 Nov 11;116(19):3802-8. Epub 2010 Jun 29 doi: 10.1182/blood-2010-02-270751. PMID: 20587783

Recent clinical studies

Etiology

T cell receptor repertoire analysis in HTLV-1-associated diseases.
Clauze A, Enose-Akahata Y, Jacobson S
Front Immunol 2022;13:984274. Epub 2022 Sep 15 doi: 10.3389/fimmu.2022.984274. PMID: 36189294Free PMC Article
Cardiovascular risk factors and brain white matter lesions in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP).
Filho ABV, Puccioni-Sohler M
J Neurovirol 2022 Feb;28(1):168-171. Epub 2022 Mar 2 doi: 10.1007/s13365-022-01063-6. PMID: 35235153
Spasticity distribution and severity in individuals with HTLV-1-associated myelopathy/tropical spastic paraparesis.
Sales M, de Almeida Scaldaferri GB, Barbosa Dos Santos JI, Melo A, da Silva Ribeiro NM
J Neurovirol 2021 Dec;27(6):857-863. Epub 2020 Oct 6 doi: 10.1007/s13365-020-00911-7. PMID: 33021698
Role of HTLV-1 orf-I encoded proteins in viral transmission and persistence.
Sarkis S, Galli V, Moles R, Yurick D, Khoury G, Purcell DFJ, Franchini G, Pise-Masison CA
Retrovirology 2019 Dec 18;16(1):43. doi: 10.1186/s12977-019-0502-1. PMID: 31852543Free PMC Article
Human T-Cell Lymphotropic Virus Type 1 and associated diseases in Latin America.
Eusebio-Ponce E, Candel FJ, Anguita E
Trop Med Int Health 2019 Aug;24(8):934-953. Epub 2019 Jul 9 doi: 10.1111/tmi.13278. PMID: 31183938

Diagnosis

Spasticity distribution and severity in individuals with HTLV-1-associated myelopathy/tropical spastic paraparesis.
Sales M, de Almeida Scaldaferri GB, Barbosa Dos Santos JI, Melo A, da Silva Ribeiro NM
J Neurovirol 2021 Dec;27(6):857-863. Epub 2020 Oct 6 doi: 10.1007/s13365-020-00911-7. PMID: 33021698
An update on human T-cell leukemia virus type I (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) focusing on clinical and laboratory biomarkers.
Yamauchi J, Araya N, Yagishita N, Sato T, Yamano Y
Pharmacol Ther 2021 Feb;218:107669. Epub 2020 Aug 21 doi: 10.1016/j.pharmthera.2020.107669. PMID: 32835825
Creation and validation of a bladder dysfunction symptom score for HTLV-1-associated myelopathy/tropical spastic paraparesis.
Yamakawa N, Yagishita N, Matsuo T, Yamauchi J, Ueno T, Inoue E, Takata A, Nagasaka M, Araya N, Hasegawa D, Coler-Reilly A, Tsutsumi S, Sato T, Araujo A, Casseb J, Gotuzzo E, Jacobson S, Martin F, Puccioni-Sohler M, Taylor GP, Yamano Y; Japan Clinical Research Group on HAM/TSP
Orphanet J Rare Dis 2020 Jul 3;15(1):175. doi: 10.1186/s13023-020-01451-3. PMID: 32620176Free PMC Article
Mortality and risk of progression to adult T cell leukemia/lymphoma in HTLV-1-associated myelopathy/tropical spastic paraparesis.
Nagasaka M, Yamagishi M, Yagishita N, Araya N, Kobayashi S, Makiyama J, Kubokawa M, Yamauchi J, Hasegawa D, Coler-Reilly ALG, Tsutsumi S, Uemura Y, Arai A, Takata A, Inoue E, Hasegawa Y, Watanabe T, Suzuki Y, Uchimaru K, Sato T, Yamano Y
Proc Natl Acad Sci U S A 2020 May 26;117(21):11685-11691. Epub 2020 May 11 doi: 10.1073/pnas.1920346117. PMID: 32393644Free PMC Article
HTLV-1 Associated Neurological Disorders.
Khan MY, Khan IN, Farman M, Al Karim S, Qadri I, Kamal MA, Al Ghamdi K, Harakeh S
Curr Top Med Chem 2017;17(12):1320-1330. doi: 10.2174/1568026616666161222141318. PMID: 28017149

Therapy

An update on human T-cell leukemia virus type I (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) focusing on clinical and laboratory biomarkers.
Yamauchi J, Araya N, Yagishita N, Sato T, Yamano Y
Pharmacol Ther 2021 Feb;218:107669. Epub 2020 Aug 21 doi: 10.1016/j.pharmthera.2020.107669. PMID: 32835825
Human T-lymphotropic virus type 1 (HTLV-1) and cellular immune response in HTLV-1-associated myelopathy/tropical spastic paraparesis.
Nozuma S, Kubota R, Jacobson S
J Neurovirol 2020 Oct;26(5):652-663. Epub 2020 Jul 23 doi: 10.1007/s13365-020-00881-w. PMID: 32705480Free PMC Article
Immunovirological markers in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP).
Enose-Akahata Y, Jacobson S
Retrovirology 2019 Nov 29;16(1):35. doi: 10.1186/s12977-019-0499-5. PMID: 31783764Free PMC Article
HTLV-1 Associated Neurological Disorders.
Khan MY, Khan IN, Farman M, Al Karim S, Qadri I, Kamal MA, Al Ghamdi K, Harakeh S
Curr Top Med Chem 2017;17(12):1320-1330. doi: 10.2174/1568026616666161222141318. PMID: 28017149
HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP): still an obscure disease.
Pillat MM, Bauer ME, de Oliveira AC, Ulrich H, Casseb J
Cent Nerv Syst Agents Med Chem 2011 Dec 1;11(4):239-45. doi: 10.2174/1871524911106040239. PMID: 22300227

Prognosis

T cell receptor repertoire analysis in HTLV-1-associated diseases.
Clauze A, Enose-Akahata Y, Jacobson S
Front Immunol 2022;13:984274. Epub 2022 Sep 15 doi: 10.3389/fimmu.2022.984274. PMID: 36189294Free PMC Article
An update on human T-cell leukemia virus type I (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) focusing on clinical and laboratory biomarkers.
Yamauchi J, Araya N, Yagishita N, Sato T, Yamano Y
Pharmacol Ther 2021 Feb;218:107669. Epub 2020 Aug 21 doi: 10.1016/j.pharmthera.2020.107669. PMID: 32835825
Mortality and risk of progression to adult T cell leukemia/lymphoma in HTLV-1-associated myelopathy/tropical spastic paraparesis.
Nagasaka M, Yamagishi M, Yagishita N, Araya N, Kobayashi S, Makiyama J, Kubokawa M, Yamauchi J, Hasegawa D, Coler-Reilly ALG, Tsutsumi S, Uemura Y, Arai A, Takata A, Inoue E, Hasegawa Y, Watanabe T, Suzuki Y, Uchimaru K, Sato T, Yamano Y
Proc Natl Acad Sci U S A 2020 May 26;117(21):11685-11691. Epub 2020 May 11 doi: 10.1073/pnas.1920346117. PMID: 32393644Free PMC Article
Role of HTLV-1 orf-I encoded proteins in viral transmission and persistence.
Sarkis S, Galli V, Moles R, Yurick D, Khoury G, Purcell DFJ, Franchini G, Pise-Masison CA
Retrovirology 2019 Dec 18;16(1):43. doi: 10.1186/s12977-019-0502-1. PMID: 31852543Free PMC Article
HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) inflammatory network.
Goncalves DU, Proietti FA, Barbosa-Stancioli EF, Martins ML, Ribas JG, Martins-Filho OA, Teixeira-Carvalho A, Peruhype-Magalhães V, Carneiro-Proietti AB
Inflamm Allergy Drug Targets 2008 Jun;7(2):98-107. doi: 10.2174/187152808785107642. PMID: 18691139

Clinical prediction guides

Cerebral and spinal cord changes observed through magnetic resonance imaging in patients with HTLV-1-associated myelopathy/tropical spastic paraparesis: a systematic review.
Bastos Ferreira AP, do Nascimento ADFS, Sampaio Rocha-Filho PA
J Neurovirol 2022 Feb;28(1):1-16. Epub 2022 Jan 3 doi: 10.1007/s13365-021-01043-2. PMID: 34981435
HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) versus adult T-cell leukemia/lymphoma (ATLL).
Zarei-Ghobadi M, Sheikhi M, Teymoori-Rad M, Yaslianifard S, Norouzi M, Yaslianifard S, Faraji R, Farahmand M, Bayat S, Jafari M, Mozhgani SH
BMC Res Notes 2021 Mar 23;14(1):109. doi: 10.1186/s13104-021-05521-y. PMID: 33757561Free PMC Article
An update on human T-cell leukemia virus type I (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) focusing on clinical and laboratory biomarkers.
Yamauchi J, Araya N, Yagishita N, Sato T, Yamano Y
Pharmacol Ther 2021 Feb;218:107669. Epub 2020 Aug 21 doi: 10.1016/j.pharmthera.2020.107669. PMID: 32835825
Mogamulizumab (Anti-CCR4) in HTLV-1-Associated Myelopathy.
Sato T, Coler-Reilly ALG, Yagishita N, Araya N, Inoue E, Furuta R, Watanabe T, Uchimaru K, Matsuoka M, Matsumoto N, Hasegawa Y, Yamano Y
N Engl J Med 2018 Feb 8;378(6):529-538. doi: 10.1056/NEJMoa1704827. PMID: 29414279
HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP): still an obscure disease.
Pillat MM, Bauer ME, de Oliveira AC, Ulrich H, Casseb J
Cent Nerv Syst Agents Med Chem 2011 Dec 1;11(4):239-45. doi: 10.2174/1871524911106040239. PMID: 22300227

Recent systematic reviews

Cerebral and spinal cord changes observed through magnetic resonance imaging in patients with HTLV-1-associated myelopathy/tropical spastic paraparesis: a systematic review.
Bastos Ferreira AP, do Nascimento ADFS, Sampaio Rocha-Filho PA
J Neurovirol 2022 Feb;28(1):1-16. Epub 2022 Jan 3 doi: 10.1007/s13365-021-01043-2. PMID: 34981435
Intrinsic and Extrinsic Cell Apoptotic Pathways in Patients with HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis: A Systematic Review.
Bastos Ferreira AP, Cassilhas APP, Moura P, Sampaio Rocha-Filho PA
Viral Immunol 2021 Jul-Aug;34(6):380-391. Epub 2021 Jan 19 doi: 10.1089/vim.2020.0131. PMID: 33470891
Population-based prevalence of human T-lymphotropic virus type 1 in sub-Saharan Africa: a systematic review and meta-analysis.
Ngoma AM, Omokoko MD, Mutombo PB, Mvika ES, Muwonga JM, Nollet KE, Ohto H
Trop Med Int Health 2019 Nov;24(11):1277-1290. Epub 2019 Sep 30 doi: 10.1111/tmi.13305. PMID: 31465629
Current approaches for detection of human T-lymphotropic virus Type 1: A systematic review.
Fani M, Rezayi M, Meshkat Z, Rezaee SA, Makvandi M, Abouzari-Lotf E, Ferns GA
J Cell Physiol 2019 Aug;234(8):12433-12441. Epub 2019 Jan 11 doi: 10.1002/jcp.28087. PMID: 30633358
Early Onset of HTLV-1 Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP) and Adult T-cell Leukemia/Lymphoma (ATL): Systematic Search and Review.
Oliveira PD, Kachimarek AC, Bittencourt AL
J Trop Pediatr 2018 Apr 1;64(2):151-161. doi: 10.1093/tropej/fmx039. PMID: 28582585

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...