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HAM
MCID: MYL057
MIFTS: 39

Myelopathy, Htlv-1-Associated (HAM)

Categories: Infectious diseases, Neuronal diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Myelopathy, Htlv-1-Associated

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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:

MalaCards categories:
Global: Infectious diseases Rare diseases
Anatomical: Neuronal diseases
See all MalaCards categories (disease lists)
ICD10: 33
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
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Summaries for Myelopathy, Htlv-1-Associated

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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 t-cell lymphoblastic leukemia/lymphoma and htlv-1 associated myelopathy/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 eye, 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
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Related Diseases for Myelopathy, Htlv-1-Associated

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

(show top 50) (show all 300)
# Related Disease Score Top Affiliating Genes
1 t-cell lymphoblastic leukemia/lymphoma 29.9 FASLG CNTN2
2 htlv-1 associated myelopathy/tropical spastic paraparesis 12.2
3 tropical spastic paraparesis 11.9
4 human t-cell leukemia virus type 1 11.6
5 retrovirus-associated myelopathy 11.5
6 autoimmune polyendocrine syndrome, type i, with or without reversible metaphyseal dysplasia 11.3
7 spastic paraparesis 10.9
8 leukemia, t-cell, chronic 10.7
9 adult t-cell leukemia 10.6
10 papillomatosis, confluent and reticulated 10.6
11 dermatitis 10.4
12 measles 10.4
13 yaws 10.3
14 posttransplant acute limbic encephalitis 10.3
15 leukemia, acute myeloid 10.3
16 myeloid leukemia 10.3
17 t-cell adult acute lymphocytic leukemia 10.2
18 spasticity 10.2
19 creutzfeldt-jakob disease 10.2
20 sexual disorder 10.2
21 mumps 10.2
22 lathyrism 10.2
23 triiodothyronine receptor auxiliary protein 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 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.1
31 major depressive disorder 10.1
32 detrusor sphincter dyssynergia 10.1
33 leukemia 10.1
34 uveitis 10.1
35 polyneuropathy 10.1
36 mental depression 10.1
37 myelitis 10.1
38 myositis 10.1
39 leukemia, acute lymphoblastic 10.1
40 chops syndrome 10.1
41 acute cystitis 10.1
42 diarrhea 10.1
43 paraplegia 10.1
44 mite infestation 10.1
45 toxoplasmosis 10.1
46 systemic lupus erythematosus 10.1
47 schistosoma mansoni infection, susceptibility/ 10.1
48 kuru 10.1
49 mycosis fungoides 10.1
50 neurodegeneration with brain iron accumulation 2a 10.1

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



Diseases related to Myelopathy, Htlv-1-Associated
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Symptoms & Phenotypes for Myelopathy, Htlv-1-Associated

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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
Sources

Drugs & Therapeutics for Myelopathy, Htlv-1-Associated

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

(show all 42)
# 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
Clotrimazole Approved, Vet_approved Phase 2, Phase 3 23593-75-1 2812
5
Miconazole Approved, Investigational, Vet_approved Phase 2, Phase 3 22916-47-8 4189
6
Mogamulizumab Approved, Investigational Phase 3 1159266-37-1
7 Anti-Infective Agents Phase 2, Phase 3
8 Antiviral Agents Phase 2, Phase 3
9 Anti-HIV Agents Phase 2, Phase 3
10 Anti-Retroviral Agents Phase 2, Phase 3
11 Immunologic Factors Phase 2, Phase 3
12 Phosphodiesterase Inhibitors Phase 3
13 Antioxidants Phase 3
14 Platelet Aggregation Inhibitors Phase 3
15 Vasodilator Agents Phase 3
16 Protective Agents Phase 3
17 Radiation-Protective Agents Phase 3
18 Dermatologic Agents Phase 2, Phase 3
19 Antirheumatic 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 Immunosuppressive Agents Phase 2, Phase 3
24 Antifungal 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 Interferon-beta Phase 2
29 Adjuvants, Immunologic Phase 2
30 interferons Phase 2
31 Janus Kinase Inhibitors Phase 2
32 Gastrointestinal Agents Phase 2
33 Immunoglobulins Phase 1
34 Antibodies Phase 1
35 Antibodies, Monoclonal Phase 1
36
Valproic acid Approved, Investigational 99-66-1 3121
37 Integrase Inhibitors Early Phase 1
38 HIV Integrase Inhibitors Early Phase 1
39 Raltegravir Potassium Early Phase 1
40 Neurotransmitter Agents
41 Psychotropic Drugs
42 Anticonvulsants

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 Leukemia Virus Type-1 Associated Myelopathy (HAM/TSP) 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

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Genetic Tests for Myelopathy, Htlv-1-Associated

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Anatomical Context for Myelopathy, Htlv-1-Associated

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

40
T Cells, Spinal Cord, Eye, Lung, Skin, Breast, Brain
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Publications for Myelopathy, Htlv-1-Associated

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Articles related to Myelopathy, Htlv-1-Associated:

(show top 50) (show all 1557)
# 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. 56 61
2866324 1985
3
Antibodies to human T-lymphotropic virus type-I in patients with tropical spastic paraparesis. 56 61
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. 61 54
11964301 2002
15
Increased levels of soluble Fas ligand in CSF of rapidly progressive HTLV-1-associated myelopathy/tropical spastic paraparesis patients. 61 54
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
Molecular targeting of PD-1 signaling pathway as a novel therapeutic approach in HTLV-1 infection. 61
32283259 2020
18
Immunophenotypic analysis of cerebrospinal fluid reveals concurrent development of ATL in the CNS of a HAM/TSP patient. 61
31930455 2020
19
Tropical spastic paraparesis in autochthon patient. 61
31784111 2020
20
Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis. 61
32517313 2020
21
Phylogenetic and phylodynamic study of Human T-cell lymphotropic virus Type 1 (HTLV-1) in Iran. 61
32561293 2020
22
Frequent horizontal and mother-to-child transmission may contribute to high prevalence of STLV-1 infection in Japanese macaques. 61
32576215 2020
23
Mortality and risk of progression to adult T cell leukemia/lymphoma in HTLV-1-associated myelopathy/tropical spastic paraparesis. 61
32393644 2020
24
Signs, meanings and practices of people living with human t-cell lymphotropic virus type 1 or tropical spastic myelopathy. 61
32367401 2020
25
Neurologic, clinical, and immunologic features in a cohort of HTLV-1 carriers with high proviral loads. 61
32385802 2020
26
Complete genome sequence of human T-cell lymphotropic type 1 from patients with different clinical profiles, including infective dermatitis. 61
31883457 2020
27
Clinicopathological aspects and proviral load of adulthood infective dermatitis associated with HTLV-1: Comparison between juvenile and adulthood forms. 61
32330142 2020
28
Effective Treatment with Tocilizumab in a Rheumatoid Arthritis Patient Complicated with Human T-cell Leukemia Virus Type 1-associated Myelopathy: A Case Report. 61
32321893 2020
29
Acupuncture in the treatment of HTLV-I-associated myelopathy / tropical spastic Paraparesis. 61
32350814 2020
30
The IL-18, IL-12, and IFN-γ expression in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients, HTLV-1 carriers, and healthy subjects. 61
32270468 2020
31
First Description of Seronegative HTLV-1 Carriers in Argentina. 61
32043459 2020
32
Expression of TSLC1 in patients with HAM/TSP. 61
32285300 2020
33
Use of cerebrospinal fluid CXCL10 and neopterin as biomarkers in HTLV-1-associated myelopathy/tropical spastic paraparesis treated with steroids. 61
31662348 2020
34
The effect of home exercise on the posture and mobility of people with HAM/TSP: a randomized clinical trial. 61
32236329 2020
35
Infective dermatitis: A purely cutaneous manifestation of HTLV-1 infection. 61
31010607 2020
36
Clinical significance of soluble CADM1 as a novel marker for adult T-cell leukemia/lymphoma. 61
32054656 2020
37
Abnormal vitamin D and lipid profile in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients. 61
31713009 2020
38
The Role of Chemokines in the Pathogenesis of HTLV-1. 61
32231656 2020
39
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
40
HTLV-1 Infection and Rheumatic Diseases. 61
32117170 2020
41
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
42
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
43
HTLV-1 infection: An emerging risk. Pathogenesis, epidemiology, diagnosis and associated diseases. 61
31648512 2019
44
HTLV-1 infection of myeloid cells: from transmission to immune alterations. 61
31870397 2019
45
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
46
Noninvasive Detection of Antibodies to Human T-Cell Lymphotropic Virus Types 1 and 2 by Use of Oral Fluid. 61
31597746 2019
47
HTLV-1-infected asymptomatic carriers compared to HAM/TSP patients over-express the apoptosis- and cytotoxicity-related molecules. 61
31317252 2019
48
TREX1 531C>T Polymorphism is Associated with High Proviral Load Levels in HTLV-1-Infected Persons. 61
31861565 2019
49
Nutritional status of human T-lymphotropic virus 1 patients: A retrospective study. 61
31677708 2019
50
HTLV-1 contains a high CG dinucleotide content and is susceptible to the host antiviral protein ZAP. 61
31842935 2019
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Variations for Myelopathy, Htlv-1-Associated

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Expression for Myelopathy, Htlv-1-Associated

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

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

36
# Name Kegg Source Accession
1 Human T-cell leukemia virus 1 infection hsa05166
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GO Terms for Myelopathy, Htlv-1-Associated

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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
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Sources for Myelopathy, Htlv-1-Associated

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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