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07-SEPTEMBER-2008 03:17:44 - Tropical spastic paraparesis Tropical spastic paraparesis Classification and external resources ICD-10 G04.1 DiseasesDB 29487 eMedicine med/1038 MeSH D015493 Tropical spastic paraparesis TSP 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. Contents 1 History 2 Presentation 3 Pathogenesis 4 Symptoms 5 Prevention 6 Treatment 7 Prognosis 8 References History 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. Presentation 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 When infected by HTLV-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. Symptoms Progressive muscle weakness; Sensory disturbance Sphincter dysfunction Urinary incontinence Uveitis arthritis Pulmonary lymphocyte alveolitis Polymyositis Keratoconjunctivitis sicca Infectious dermatitis Prevention Blood transfusion products are screened for HTLV-1 antibodies. Treatment 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. 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 Machigashira N, Yoshida Y, Wang S, Osame M 2001. HTLV-1-associated myelopathy/tropical spastic paraparesis with pseudohypoparathyroidism. Neurology 56 1: 104-6. PMID 11148245. v d e Pathology of the nervous system, primarily CNS G00-G47, 320-349 Inflammatory Meningitis Arachnoiditis, Aseptic meningitis, Tuberculous meningitis - Encephalitis - Myelitis - Encephalomyelitis Acute disseminated - Tropical spastic paraparesis - Cavernous sinus thrombosis Systemic atrophies Huntington's disease - Spinocerebellar ataxia Friedreich's ataxia, Ataxia telangiectasia, Herary spastic paraplegia - Spinal muscular atrophy: Werdnig-Hoffman - Kugelberg-Welander - Fazio-Londe - MND ALS, PMA, PBP, PP, PLS Extrapyramidal and movement disorders Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration - Dystonia/Dyskinesia Spasmodic torticollis, Meige's, Blepharospasm - Essential tremor - Myoclonus - Lafora - Chorea Choreoathetosis - Restless legs - Stiff person Other degenerative/ demyelinating diseases dementia: Alzheimer's - Pick's - Dementia with Lewy bodies - Frontotemporal lobar degeneration mitochondrial disease: Leigh's demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis - Marchiafava-Bignami disease - CAMFAK syndrome - Alpers' Seizure/epilepsy Focal - Generalised - Status epilepticus - Myoclonic epilepsy Headache Migraine Familial hemiplegic - Cluster - Vascular - Tension Vascular Transient ischemic attack Amaurosis fugax, Transient global amnesia Cerebrovascular disease MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke Sleep disorders Insomnia - Hypersomnia - Sleep apnea Obstructive, Ondine's curse - Narcolepsy - Cataplexy - Kleine-Levin - Circadian rhythm sleep - Delayed sleep phase - Advanced sleep phase Intracranial hypertension Hydrocephalus Normal pressure - Idiopathic intracranial hypertension Other encephalopathy Brain herniation - Cerebral edema - Reye's Other spinal cord disease Syringomyelia - Syringobulbia - Morvan's syndrome - Spinal cord compression v d e Infectious diseases - Virus diseases A80-B34, 042-079 CNS Encephalitis/ meningitis DNA virus: Progressive multifocal leukoencephalopathy RNA virus: Subacute sclerosing panencephalitis - Lymphocytic choriomeningitis - Tick-borne meningoencephalitis unknown: Encephalitis lethargica Eye DNA virus: Cytomegalovirus retinitis Other RNA virus: Rabies - Myelitis: Poliomyelitis Post-polio syndrome - Tropical spastic paraparesis Skin and mucous membrane lesions DNA virus, Herpesviridae: Herpes simplex - Chickenpox - Herpes zoster - KSHV DNA virus, other: Poxviridae Smallpox, Monkeypox, Cowpox, Vaccinia, Molluscum contagiosum - exanthem Roseola, Fifth disease - HPV Wart RNA virus: exanthem Measles, Rubella - picornavirus Hand, foot and mouth disease, Foot-and-mouth disease Digestive system Hepatitis DNA virus: B RNA virus: A - D - C - E - G Gastroenteritis DNA virus: Adenovirus RNA virus: Rotavirus - Norovirus - Astrovirus - Coronavirus Respiratory system RNA virus, IV: Acute viral nasopharyngitis - Severe acute respiratory syndrome RNA virus, V: Influenza/Avian influenza - Human parainfluenza viruses - RSV - hMPV Other/varied: Infectious mononucleosis - Viral pneumonia Sexually transmitted DNA virus: HPV Genital warts, Cervical cancer RNA virus, retrovirus: HIV AIDS, AIDS dementia complex - Adult T-cell leukemia Oncovirus DNA virus: Hepatitis B - HPV - Kaposi's sarcoma-associated herpesvirus RNA virus: Hepatitis C - HTLV Systemic DNA virus: Cytomegalovirus RNA virus: Mumps - Bornholm disease - Coxsackie B Retrieved from http://en..org/wiki/Tropical_spastic_paraparesis Categories: Viral diseases Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Español Polski This page was last modified on 6 August 2008, at 21:06
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