NINDS Tropical Spastic Paraparesis Information Page: NINDS
Article title: NINDS Tropical Spastic Paraparesis Information Page: NINDS
Main condition: Tropical Spastic Paraparesis
Conditions: Tropical Spastic Paraparesis
Table of Contents (click to jump to sections)What
is Tropical Spastic Paraparesis?
Is
there any treatment?
What
is the prognosis?
What
research is being done?
Organizations
Related
NINDS Publications and
Information
What is Tropical Spastic
Paraparesis?
For several decades the term “tropical spastic
paraparesis” (TSP) 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 one 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-1980's,
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.
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 or lymphoma.
Is there any
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. A current trial with interferon beta 1a is underway.
What is the
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.
What research is being
done?
The NINDS supports and conducts an extensive research program
on disorders of the nervous system. The goals of this research are to find
ways to prevent, treat, and, ultimately, cure these disorders
National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield, CT 06812-8923
orphan@rarediseases.org
http://www.rarediseases.org/
Tel:
203-746-6518 800-999-NORD (6673)
Fax: 203-746-6481
National Institute of Allergy and Infectious Diseases
(NIAID)
National Institutes of Health
31 Center Drive, Rm. 7A50
MSC 2520
Bethesda, MD 20892-2520
(see website)
http://www.niaid.nih.gov/
Tel:
301-496-5717
National Cancer Institute (NCI)
National Institutes of Health
Bldg. 31, Rm. 10A31
Bethesda, MD 20892-2580
cancermail@icicc.nci.nih.gov
http://cancernet.nci.nih.gov/
Tel:
301-435-3848 NCI's Cancer Information Service 800-4-CANCER (422-6237) TTY:
800-332-8615
Related NINDS Publications and Information
Polymyositis
information sheet compiled by NINDS, the National Institute of
Neurological Disorders and Stroke.
This fact sheet is in the public domain. You may copy it.Provided
by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD
20892
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