Tropical spastic paraparesis


Tropical spastic paraparesis (TSP)

Description, Causes and Risk Factors:

For several decades the term "tropical spastic paraparesis" has been used to describe a chronic and progressive disease of the nervous system that affects adults living in equatorial areas of the World. 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 (HTLV-1).

HTLV-1 preferentially infects CD4+ lymphoid cells in vivo. Three molecules have been identified for binding and/or entry of HTLV-1: heparan sulfate proteoglycans, neuropilin-1, and glucose transporter 1. An efficient transfer of the virus from an infected cell to a target cell can occur through the formation of a viral synapse and/or by virofilm structure. As for all retroviruses, HTLV-1 genome possesses three major ORFs (Gag, Pol and Env) encoding the structural and enzymatic proteins. HTLV-1 encodes also some regulatory and auxillary proteins including the tax protein with transforming activities and the HBZ protein which plays a role in the proliferation and maintenance of the leukemic cells.

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.

TSP is a progressive disease, but it is rarely fatal. Most individuals live for several decades after the diagnosis. Their prognosis improves if they take steps to prevent UTI and skin sores, and if they participate in Physical & occupational therapy programs.

Symptoms:

    Muscle stiffness.

  • Muscle spasms.

  • Sensory disturbance.

  • Bladder symptoms.

  • Constipation.

  • Optic atrophy.

  • Nerve deafness and wasting.

  • Arthritis.

  • Polymyositis.

Pathologically, patchy thickening and opacity of the meninges of the base of brain and especially the spinal cord was observed. Microscopically, a mainly perivascular lymphocytic cellular infiltrate with parenchymal small vessel proliferation and reactive gliosis in damaged neuronal tissue was noted. Demyelination was most prominent in lateral and posterior columns and could extend into the posterior and anterior nerve roots.

Diagnosis:

Differential diagnosis:

    Syphilis.

  • Diabetes mellitus.

  • Transverse myelitis.

  • Human immunodeficiency virus (HIV) myelopathy.

  • Motor neuron disease - especially primary lateral sclerosis.

  • Familial spastic paraplegia.

  • Adrenoleucodystrophies.

  • Multiple sclerosis (MS).

  • Lupus erythematosus involving the spinal cord.

  • Midline parasagittal mass lesions.

  • Spinal arteriovenous malformation.

The diagnosis of TSP will be suggested in the context of the appropriate clinical history and physical findings with a positive HTLV-1 antibody test.

This antibody test demonstrates the presence of IgG antibodies to HTLV-1 by an enzyme-linked immunosorbent assay (ELISA) technique employing disrupted virions produced by HTLV-1 producing cell lines, such as the HUT 102 cell line, as antigen. This ELISA tests is also utilized on CSF. Confirmation by Western blot may be performed. In this test, nitrocellulose strips containing HTLV-1-specific proteins (fractionated according to molecular weight) are washed with phosphate-buffered saline (PBS) and Tween-20, incubated with PBS containing heat-inactivated normal goat serum and nonfat dry milk to minimize nonspecific immunoglobulin binding and then reacted with serum or CSF specimens in dilution and washed again. Human immunoglobulins to HTLV-1 proteins are visualized using biotin-labelled goat antihuman immunoglobulin, avidin-horseradish peroxidase conjugate and 4-chloro-1-napthol solution as developer. HTLV-1 proteins detected include the group antigen (gag) - encoded proteins p19, p24 and p53, the envelope-encoded glycoproteins (gp) gp61 and gp46, and p42 protein encoded in part in the long open reading frame portion. Other supplementary findings often seen include a positive VDRL serology, the detection of polylobated lymphocytes and abnormalities of CSF.

Treatment:

There is no established treatment program for TSP. Corticosteroids may relieve some symptoms, but aren't likely to change the course of the disorder. Clinical studies suggest that interferon alpha provides benefits over short periods and some aspects of disease activity may be improved favorably using interferon beta. Stiff and spastic muscles may be treated with LioresalSM or ZanaflexSM. Urinary dysfunction may be treated with OxytrolTM.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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