HAM
MCID: MYL057
MIFTS: 39

Myelopathy, Htlv-1-Associated (HAM)

Categories: Infectious diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Myelopathy, Htlv-1-Associated

MalaCards integrated aliases for Myelopathy, Htlv-1-Associated:

Name: Myelopathy, Htlv-1-Associated 56 54
Tropical Spastic Paraparesis 58 71
Human T-Lymphotropic Virus Type I-Associated Myelopathy/tropical Spastic Paraparesis 58
Human T-Lymphotropic Virus Type-1-Associated Myelopathy/tropical Spastic Paraparesis 58
Htlv-1-Associated Myelopathy/tropical Spastic Paraparesis 58
Familial Spastic Paraparesis, Htlv-1-Associated 56
Htlv-1 Associated Myelopathy 53
Htlv1-Associated Myelopathy 36
Ham/tsp 58
Ham 56
Tsp 58

Characteristics:

Orphanet epidemiological data:

58
tropical spastic paraparesis
Inheritance: Not applicable; Age of onset: Adult;

OMIM:

56
Inheritance:
possible genetic factors


Classifications:

Orphanet: 58  
Rare neurological diseases
Rare infectious diseases


External Ids:

OMIM 56 159580
KEGG 36 H01724
ICD10 via Orphanet 33 G04.1
UMLS via Orphanet 72 C0030481
Orphanet 58 ORPHA289326
MedGen 41 C0030481
SNOMED-CT via HPO 68 14648003 312444006 48522003
UMLS 71 C0030481

Summaries for Myelopathy, Htlv-1-Associated

NINDS : 53 For several decades the term “tropical spastic paraparesis” (TSP) has been used to describe a chronic and progressive disease of the nervous system that affects adults living in equatorial areas of the world and causes progressive weakness, stiff muscles, muscle spasms, sensory disturbance, and sphincter dysfunction. The cause of TSP was obscure until the mid-1980s, when an important association was established between the human retrovirus — human T-cell lymphotrophic virus type 1 (also known as HTLV-1) — and TSP. TSP is now called HTLV-1 associated myelopathy/ tropical spastic paraparesis or HAM/TSP. The HTLV-1 retrovirus is thought to cause at least 80 percent of the cases of HAM/TSP by impairing the immune system. In addition to neurological symptoms of weakness and muscle stiffness or spasms, in rare cases individuals with HAM/TSP 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), polymyositis (an inflammatory muscle disease), keratoconjunctivitis sicca (persistent dryness of the cornea and conjunctiva), and infectious dermatitis (inflammation of the skin). The other serious complication of HTLV-1 infection is the development of adult T-cell leukemia or lymphoma. Nervous system and blood-related complications occur only in a very small proportion of infected individuals, while most remain largely without symptoms throughout their lives. The HTLV-1 virus is transmitted person-to-person via infected cells: breast-feeding by mothers who are seropositive (in other words, have high levels of virus antibodies in their blood), sharing infected needles during intravenous drug use, or having sexual relations with a seropositive partner. Less than 2 percent of HTLV-1 seropositive carriers will become HAM/TSP patients.

MalaCards based summary : Myelopathy, Htlv-1-Associated, also known as tropical spastic paraparesis, is related to htlv-1 associated myelopathy/tropical spastic paraparesis and tropical spastic paraparesis. An important gene associated with Myelopathy, Htlv-1-Associated is CNTN2 (Contactin 2), and among its related pathways/superpathways is Human T-cell leukemia virus 1 infection. The drugs Pentoxifylline and Zidovudine have been mentioned in the context of this disorder. Affiliated tissues include t cells, spinal cord and skin, and related phenotypes are abnormal pyramidal sign and spastic paraparesis

KEGG : 36 Human T lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic progressive myelopathy characterized by bilateral pyramidal tract involvement with sphincteric disturbances. HTLV-1 is the etiologic agent of HAM/TSP. Although the majority of HTLV-1-infected individuals remain asymptomatic during their lifetime, approximately one percent of this population develops a myelopathy consisting of a chronic inflammation of the white and gray matter of the spinal cord. Early in the disease process the leptomeninges, blood vessels and parenchyma are infiltrated with CD4+, CD8+, B lymphocytes and foamy macrophages, whereas later in the disease CD8+ lymphocytes predominate with subsequent progression to a relatively acellular, atrophic pattern with axonal and myelin degeneration.

Wikipedia : 74 Tropical spastic paraparesis (TSP), is a medical condition that causes weakness, muscle spasms, and... more...

More information from OMIM: 159580

Related Diseases for Myelopathy, Htlv-1-Associated

Diseases related to Myelopathy, Htlv-1-Associated via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 302)
# Related Disease Score Top Affiliating Genes
1 htlv-1 associated myelopathy/tropical spastic paraparesis 12.2
2 tropical spastic paraparesis 11.9
3 human t-cell leukemia virus type 1 11.6
4 retrovirus-associated myelopathy 11.5
5 autoimmune polyendocrine syndrome, type i, with or without reversible metaphyseal dysplasia 11.2
6 spastic paraparesis 10.9
7 t-cell leukemia 10.7
8 adult t-cell leukemia 10.6
9 papillomatosis, confluent and reticulated 10.6
10 measles 10.4
11 yaws 10.3
12 leukemia, acute myeloid 10.2
13 posttransplant acute limbic encephalitis 10.2
14 t-cell adult acute lymphocytic leukemia 10.2
15 myeloid leukemia 10.2
16 spasticity 10.2
17 creutzfeldt-jakob disease 10.2
18 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
19 sexual disorder 10.2
20 mumps 10.2
21 lathyrism 10.2
22 triiodothyronine receptor auxiliary protein 10.2
23 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.2
24 foodborne botulism 10.2
25 listeriosis 10.2
26 neurogenic bladder 10.2
27 neutropenia 10.2
28 staphyloenterotoxemia 10.2
29 depression 10.2
30 major depressive disorder 10.1
31 detrusor sphincter dyssynergia 10.1
32 leukemia 10.1
33 uveitis 10.1
34 polyneuropathy 10.1
35 mental depression 10.1
36 myelitis 10.1
37 myositis 10.1
38 leukemia, acute lymphoblastic 10.1
39 chops syndrome 10.1
40 lymphocytic leukemia 10.1
41 acute cystitis 10.1
42 diarrhea 10.1
43 paraplegia 10.1
44 mite infestation 10.1
45 systemic lupus erythematosus 10.1
46 schistosoma mansoni infection, susceptibility/ 10.1
47 kuru 10.1
48 mycosis fungoides 10.1
49 neurodegeneration with brain iron accumulation 2a 10.1
50 subacute sclerosing panencephalitis 10.1

Graphical network of the top 20 diseases related to Myelopathy, Htlv-1-Associated:



Diseases related to Myelopathy, Htlv-1-Associated

Symptoms & Phenotypes for Myelopathy, Htlv-1-Associated

Human phenotypes related to Myelopathy, Htlv-1-Associated:

31
# Description HPO Frequency HPO Source Accession
1 abnormal pyramidal sign 31 HP:0007256
2 spastic paraparesis 31 HP:0002313
3 myelopathy 31 HP:0002196

Symptoms via clinical synopsis from OMIM:

56
Neuro:
myelopathy
pyramidal signs
tropical spastic paraparesis
mild or no sensory disturbance

Lab:
anti-htlv-i antibodies in both serum and csf

Clinical features from OMIM:

159580

Drugs & Therapeutics for Myelopathy, Htlv-1-Associated

Drugs for Myelopathy, Htlv-1-Associated (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 46)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Pentoxifylline Approved, Investigational Phase 3 6493-05-6 4740
2
Zidovudine Approved Phase 2, Phase 3 30516-87-1 35370
3
Lamivudine Approved, Investigational Phase 2, Phase 3 134678-17-4 60825
4
Miconazole Approved, Investigational, Vet_approved Phase 2, Phase 3 22916-47-8 4189
5
Mogamulizumab Approved, Investigational Phase 3 1159266-37-1
6 Anti-Infective Agents Phase 2, Phase 3
7 Anti-Retroviral Agents Phase 2, Phase 3
8 Antiviral Agents Phase 2, Phase 3
9 Anti-HIV Agents Phase 2, Phase 3
10 Immunologic Factors Phase 2, Phase 3
11 Platelet Aggregation Inhibitors Phase 3
12 Antioxidants Phase 3
13 Free Radical Scavengers Phase 3
14 Protective Agents Phase 3
15 Vasodilator Agents Phase 3
16 Phosphodiesterase Inhibitors Phase 3
17 Radiation-Protective Agents Phase 3
18 Antirheumatic Agents Phase 2, Phase 3
19 Dermatologic Agents Phase 2, Phase 3
20 Reverse Transcriptase Inhibitors Phase 2, Phase 3
21 Antimetabolites Phase 2, Phase 3
22 Cyclosporins Phase 2, Phase 3
23 Antifungal Agents Phase 2, Phase 3
24 Immunosuppressive Agents Phase 2, Phase 3
25 Calcineurin Inhibitors Phase 2, Phase 3
26
Interferon beta-1a Approved, Investigational Phase 2 145258-61-3 6438354
27
Infliximab Approved Phase 2 170277-31-3
28 Adjuvants, Immunologic Phase 2
29 interferons Phase 2
30 Interferon-beta Phase 2
31 Janus Kinase Inhibitors Phase 2
32 Gastrointestinal Agents Phase 2
33 Antibodies, Monoclonal Phase 1
34 Antibodies Phase 1
35 Immunoglobulins Phase 1
36
Valproic acid Approved, Investigational 99-66-1 3121
37 HIV Integrase Inhibitors Early Phase 1
38 Raltegravir Potassium Early Phase 1
39 Integrase Inhibitors Early Phase 1
40 Central Nervous System Depressants
41 Neurotransmitter Agents
42 Tranquilizing Agents
43 GABA Agents
44 Anticonvulsants
45 Psychotropic Drugs
46 Antimanic Agents

Interventional clinical trials:

(show all 20)
# Name Status NCT ID Phase Drugs
1 Open-Label, Exploratory Study of the Efficacy and Safety of Tamibarotene(AM80H) for HTLV-1 Associated Myelopathy/ Tropical Spastic Paraparesis (HAM/TSP) Unknown status NCT01343355 Phase 2, Phase 3 Tamibarotene
2 Effectiveness of Pentoxifylline in Attenuating Neurological Disease Associated With HTLV-1 and Negative Modulator of Pathological Immune Response. Completed NCT01472263 Phase 3 Pentoxifylline;Placebo
3 Zidovudine Plus Lamivudine in HTLV-I-associated Myelopathy: a Randomised Trial Completed NCT00272480 Phase 2, Phase 3 Zidovudine/lamivudine;Placebos
4 The HAM Ciclosporin Study : an Observational Trial of Therapy in Early or Progressing HAM/TSP Completed NCT00773292 Phase 2, Phase 3 ciclosporin
5 A Phase 3 Multicenter, Randomized, Double-Blind and Placebo-Controlled Study, and Open Study of KW-0761 in Patients With HTLV-1 Associated Myelopathy (HAM) Active, not recruiting NCT03191526 Phase 3 KW-0761 0.3 mg/kg IV;Placebo (saline)
6 Combined Virological and Immunological Evaluation of Treatment of Patients With Early HTLV-1-Associated Myelopathy With Recombinant Human Interferon Beta-1a Completed NCT00001785 Phase 2 Recombinant human interferon beta-1a
7 Phase II Trial Evaluating the Safety and Efficacy of Ruxolitinib in Patients With Smoldering and Chronic Adult T-cell Leukemia (ATL) Recruiting NCT01712659 Phase 2 Ruxolitinib;ruxolitinib
8 An Open, Non-randomised Pilot Study of Anti-TNF-alpha Therapy in Early or Progressing HAM/TSP Terminated NCT00823641 Phase 2 Infliximab
9 Immunotherapy of Natural Killer in HTLV-1 Associated Myelopathy Unknown status NCT02961712 Phase 1
10 Phase I Study of HTLV-I-Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP) Using the Humanized MiK-Beta-1 Monoclonal Antibody Directed Toward the IL-2L-15R-Beta; Subunit (CD122) That Blocks IL-15 Action Completed NCT00076843 Phase 1 Hu MiK-Beta-1
11 MRI Investigation Of The CNS In HTLV-1 Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP) Completed NCT00034723
12 Effects of Physiotherapy in the Treatment of Neurogenic Bladder in Patients Infected With Human T-Lymphotropic Virus 1 Completed NCT01651819
13 Pilot Study of Raltegravir, an Integrase Inhibitor, in Human T-Cell Lymphotrophic Virus-1(HTLV-1) Associated Myelopathy, Tropical Spastic Paraparesis (HAM/TSP) Completed NCT01867320 Early Phase 1 Raltegravir
14 A Study of Familial and Genetic Aspects of Adult T-Cell Leukemia/Lymphoma, Tropical Spastic Paraparesis and Infective Dermatitis in Jamaica Completed NCT00340821
15 Effect of Raltegravir in Patients With Myelopathy/Tropical Spastic Paraparesis Associated With Infection by Human T-Lymphotropic Virus 1 (HTLV-1). Pilot Study Completed NCT02655471 Early Phase 1 "Raltegravir" and "Zidovudine"
16 Influence of IL28B Genetic Variation on the Phenotype Infection of HTLV-1 Completed NCT01754311
17 Retrovirus Epidemiology Donor Study I (REDS I) Completed NCT00005278
18 Immuno-Virological Evaluation of Human T Cell Lymphotropic Virus Infection and Associated Neurological Diseases Recruiting NCT00001778
19 Electromiography Study in the Respiratory Muscle Training in Human Lymphotropic Virus Type 1 Recruiting NCT03829709
20 Open Label Study of the Clinical and Laboratory Effects of Valproic Acid In HAM/TSP Terminated NCT00519181 Valproic Acid

Search NIH Clinical Center for Myelopathy, Htlv-1-Associated

Genetic Tests for Myelopathy, Htlv-1-Associated

Anatomical Context for Myelopathy, Htlv-1-Associated

MalaCards organs/tissues related to Myelopathy, Htlv-1-Associated:

40
T Cells, Spinal Cord, Skin, Eye, Lung, Breast, Brain

Publications for Myelopathy, Htlv-1-Associated

Articles related to Myelopathy, Htlv-1-Associated:

(show top 50) (show all 1540)
# Title Authors PMID Year
1
Endemic tropical spastic paraparesis associated with human T-lymphotropic virus type I: a clinical and seroepidemiological study of 25 cases. 61 56
3030190 1987
2
HTLV-I and HTLV-III antibodies and tropical spastic paraparesis. 61 56
2866324 1985
3
Antibodies to human T-lymphotropic virus type-I in patients with tropical spastic paraparesis. 61 56
2863442 1985
4
Familial clustering of HTLV-I infection in southeastern Italy. 56
7603241 1995
5
Seminars in medicine of the Beth Israel Hospital, Boston. Pathogenesis of diseases induced by human lymphotropic virus type I infection. 56
8455685 1993
6
Familial spastic paraparesis syndrome associated with HTLV-I infection. 56
2388670 1990
7
Bottle-feeding can prevent transmission of HTLV-I from mothers to their babies. 56
2778340 1989
8
The first report of familial adult T-cell leukemia lymphoma in the United States. 56
2895582 1988
9
Familial occurrence of HTLV-I--associated myelopathy. 56
2894196 1988
10
Familial cases of HTLV-I-associated myelopathy. 56
2892464 1987
11
Intrafamilial clustering of anti-ATLA-positive persons. 56
2876624 1986
12
Intrafamilial transmission of adult T cell leukemia virus. 56
2877031 1986
13
HTLV-I associated myelopathy, a new clinical entity. 56
2871307 1986
14
High frequencies of Th1-type CD4(+) T cells specific to HTLV-1 Env and Tax proteins in patients with HTLV-1-associated myelopathy/tropical spastic paraparesis. 54 61
11964301 2002
15
Increased levels of soluble Fas ligand in CSF of rapidly progressive HTLV-1-associated myelopathy/tropical spastic paraparesis patients. 54 61
10430056 1999
16
Predominant expression of Fas ligand mRNA in CD8+ T lymphocytes in patients with HTLV-1 associated myelopathy. 54 61
9817448 1998
17
Immunophenotypic analysis of cerebrospinal fluid reveals concurrent development of ATL in the CNS of a HAM/TSP patient. 61
31930455 2020
18
Interferon-γ Secretion Enzyme-Linked Immunospot Assay Determined Among Human T Cell Lymphotropic Virus Type 1-Infected Subjects: A Potential Laboratory Marker for Early HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis Diagnosis. 61
31474114 2020
19
Abnormal vitamin D and lipid profile in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients. 61
31713009 2020
20
EOS, an Ikaros family zinc finger transcription factor, interacts with the HTLV-1 oncoprotein Tax and is downregulated in peripheral blood mononuclear cells of HTLV-1-infected individuals, irrespective of clinical statuses. 61
31856855 2019
21
Noninvasive Detection of Antibodies to Human T-Cell Lymphotropic Virus Types 1 and 2 by Use of Oral Fluid. 61
31597746 2019
22
HTLV-1-infected asymptomatic carriers compared to HAM/TSP patients over-express the apoptosis- and cytotoxicity-related molecules. 61
31317252 2019
23
HTLV-1 contains a high CG dinucleotide content and is susceptible to the host antiviral protein ZAP. 61
31842935 2019
24
HTLV-1 CTCF-binding site is dispensable for in vitro immortalization and persistent infection in vivo. 61
31864373 2019
25
Nutritional status of human T-lymphotropic virus 1 patients: A retrospective study. 61
31677708 2019
26
HTLV-1 proviral load in infective dermatitis associated with HTLV-1 does not increase after the development of HTLV-1-associated myelopathy/tropical spastic paraparesis and does not decrease after IDH remission. 61
31851683 2019
27
An insight to HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) pathogenesis; evidence from high-throughput data integration and meta-analysis. 61
31888669 2019
28
Complete genome sequence of human T-cell lymphotropic type 1 from patients with different clinical profiles, including infective dermatitis. 61
31883457 2019
29
TREX1 531C>T Polymorphism is Associated with High Proviral Load Levels in HTLV-1-Infected Persons. 61
31861565 2019
30
HTLV-1 infection: An emerging risk. Pathogenesis, epidemiology, diagnosis and associated diseases. 61
31648512 2019
31
HTLV-1 infection of myeloid cells: from transmission to immune alterations. 61
31870397 2019
32
Hormonal and Psychogenic Risk Factors for Erectile Dysfunction in Men with HTLV-1. 61
31521570 2019
33
Population-based prevalence of human T-lymphotropic virus type 1 in sub-Saharan Africa: a systematic review and meta-analysis. 61
31465629 2019
34
Delimitation of the upstream region of NFKBIA gene associated with HTLV-1-associated myelopathy/tropical spastic paraparesis using candidate Tag-SNPs in Peruvian HTLV-1 infected individuals. 61
31226330 2019
35
Tropical spastic paraparesis in autochthon patient. 61
31784111 2019
36
Immunovirological markers in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). 61
31783764 2019
37
Essential Role of Human T Cell Leukemia Virus Type 1 orf-I in Lethal Proliferation of CD4+ Cells in Humanized Mice. 61
31315992 2019
38
Real-world clinical course of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in Japan. 61
31639014 2019
39
Use of cerebrospinal fluid CXCL10 and neopterin as biomarkers in HTLV-1-associated myelopathy/tropical spastic paraparesis treated with steroids. 61
31662348 2019
40
Different roles of CXCR1 and CXCR2 in HTLV-1 carriers and HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients. 61
30341468 2019
41
Hybrid machine learning models for predicting types of Human T-cell Lymphotropic Virus. 61
31567100 2019
42
Silencers of HTLV-1 and HTLV-2: the pX-encoded latency-maintenance factors. 61
31492165 2019
43
Hodgkin-like adult T-cell leukemia/lymphoma that developed during the follow-up of HTLV-1 associated myelopathy/tropical spastic paraparesis. 61
31391405 2019
44
Curcumin increased the expression of c-FLIP in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients. 61
31074052 2019
45
Phenotypic and functional changes in gamma delta T lymphocytes from HTLV-1 carriers. 61
31287591 2019
46
Conflicting effects of poly(ADP-ribose) polymerase inhibitor on cell-mediated and virion-mediated HTLV-1 infection. 61
31299194 2019
47
Balance Impairments in Patients with Human T-Cell Lymphotropic Virus Type 1 Infection. 61
31391511 2019
48
Clinical trial of a humanized anti-IL-2/IL-15 receptor β chain in HAM/TSP. 61
31402625 2019
49
Current approaches for detection of human T-lymphotropic virus Type 1: A systematic review. 61
30633358 2019
50
Impact of host immunity on HTLV-1 pathogenesis: potential of Tax-targeted immunotherapy against ATL. 61
31438973 2019

Variations for Myelopathy, Htlv-1-Associated

Expression for Myelopathy, Htlv-1-Associated

Search GEO for disease gene expression data for Myelopathy, Htlv-1-Associated.

Pathways for Myelopathy, Htlv-1-Associated

Pathways related to Myelopathy, Htlv-1-Associated according to KEGG:

36
# Name Kegg Source Accession
1 Human T-cell leukemia virus 1 infection hsa05166

GO Terms for Myelopathy, Htlv-1-Associated

Cellular components related to Myelopathy, Htlv-1-Associated according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 8.62 FASLG CNTN2

Sources for Myelopathy, Htlv-1-Associated

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
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56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
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72 UMLS via Orphanet
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