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                                A52.11  Tabes dorsalis




Tabes dorsalis is a slow degeneration of the nerve cells and nerve fibers that carry sensory information to the brain. The degenerating nerves are in the dorsal columns of the spinal cord (the portion closest to the back of the body) and carry information that help maintain a person's sense of position.

Tabes dorsalis, one of two manifestations of late neurosyphilis, is a slowly progressive parenchymatous degenerative disease of the dorsal column and dorsal root of the spinal cord as a result of infection with Treponema pallidum subspecies pallidum, one of three subspecies of Treponema pallidum that can cause sexually transmitted diseases in humans. Tabes generally occurs in the late tertiary stage of syphilis, but early involvement is reported. It may be accompanied by meningitis or meningomyelitis. Although CSF invasion often occurs early in infection with Treponema pallidum, the clinical syndrome of tabes dorsalis usually occurs many years, even decades, later. The disease has a delayed and insidious onset resulting in ataxia, dementia, painful crises, and skin, joint, and bladder abnormalities. This activity can help professionals' understanding of tabes dorsalis and can augment the interprofessional effort in caring for these individuals who often require institutionalization with support by several specialties like neurology, urology, and, eventually, sometimes palliative care

Tabes dorsalis is a slowly progressive degenerative disorder of the dorsal column and dorsal root of the spinal cord. Tabes dorsalis is caused by demyelination as a result of an untreated syphilis infection caused by Treponema pallidum. Treponema pallidum infection, if left untreated or partially treated, can lead to late neurosyphilis which has two forms, general paresis (also known as "syphilitic dementia," "dementia paralytica" or "paretic neurosyphilis") and tabes dorsalis (also known as "locomotor ataxia"). Neurosyphilis may be a more common and more aggressive complication of syphilis in those infected with HIV. It is more common in males than in females.

Causes And Symptoms

Syphilis is a sexually transmitted disease caused by a bacteria named Treponema pallidum. During initial infection, the bacteria spread through the blood stream into remote sites like the brain and spinal cord, but remain silent in these areas. If proper treatment is not instituted, neurological disorders arise about a decade later and is called neurosyphilis. Damage to the spinal cord substance due to syphilis is called tabes dorsalis.

Inflammation occurs in the dorsal columns of the spinal cord. These columns are in the portion of the spinal cord closest to the back and have nerve fibers that carry sensory information like deep pain and position sense (proprioception) from the legs and arms to the brain. As a result of this, the nerve fibers lose their insulation and start atrophying. The pathological process starts in the lower-most portion of the spinal cord that receives information from the legs and spreads upwards. The inflammation can also involve other nerves that control vision, hearing, eye movements, bladder and bowel.

In the twenty-first century, mostly atypical cases of tabes dorsalis are seen due to previous partial antibiotic treatment. Much of the description of the classic disease comes from scientific articles and patient reports more than fifty years ago. The earliest and probably the most troublesome symptom is pain. This is often described as "stabbing" or "lightning-like" and is quite intense. It appears very suddenly, usually in the legs, spreads rapidly to other parts of the body and then disappears quickly. Unfortunately, this cycle can repeat itself several times a day and for days together, making the patient's life miserable. They also experience uncomfortable abnormal sensations or "paresthesias," like tingling, burning, or coldness. Later the feet become progressively numb. "Visceral crisis" develops either spontaneously or after stress in about 15% of patients due to autonomic nerve dysfunction. These episodes are frightening and severe but rarely life threatening. They consist of excruciating abdominal pain and vomiting or vocal cord spasm or burning rectal pain.




A characteristic unsteady gait called "sensory ataxia" develops. Due to degeneration of nerves that carry position sense from the legs, patients are unable to judge the position of their feet in relation to the ground while walking. They become very unsteady especially while walking in a straight line, on uneven surfaces, or while turning suddenly. This becomes dramatically accentuated in the dark or while closing the eyes as visual compensation is removed. A person with tabes dorsalis walks stooped forward with a wide based "high-stepping" gait and eyes glued to the ground in order to prevent falling. With progression of the disease, they become unable to walk although muscle strength is intact.

Diagnosis

Diagnosis is mainly clinical. Syphilis has often been called "the great mimicker" and requires an astute physician to diagnose. There are three steps in diagnosis.

First, the physician has to suspect the diagnosis. The classic signs seen in tabes dorsalis are a triad of 3A's; Argyll-Robertson pupil, areflexia (absent tendon reflexes), and ataxia. Poor visual acuity, asymmetrical eye movement, deafness, clumsy hand and leg movements are other tell-tale signs.

Secondly, it has to be differentiated from other disorders that can present similarly. This is done with the help of CT scans , MRI scans, spinal tap and certain screening blood tests. The most common screening blood test is called the Venereal Disease Research Laboratory (VDRL) test. This measures the level of certain antibodies that are elevated in the blood in syphilis. It reflects disease activity and therefore may be falsely negative in very late "burnt out" cases of tabes. On the other hand, it maybe falsely elevated in a host of other medical conditions. Therefore, it is a sensitive but not a very specific test. It is only a screening test and any positive result has to be confirmed with other blood tests. The cerebrospinal fluid (CSF) circulates around the brain and spinal cord and reflects underlying inflammation. In tabes, the white cell count and protein level in the CSF are elevated. A positive VDRL test in the CSF is a definitive diagnostic test for tabes dorsalis.

Thirdly, confirmatory tests should be done on the spinal fluid and blood. There are two confirmatory tests for syphilis, namely the Fluorescent Treponemal Antibody Absorption (FTA-ABS) and Micro Hemagglutination of Treponema Pallidum (MHA-TP). These detect very specific antibodies in the blood that are present when the person has syphilis and not otherwise. FTA-ABS in the CSF is a very sensitive test and a negative result virtually rules out tabes dorsalis. It is mandatory that all patients with syphilis undergo testing for HIV.

There is no trial open for tabes dorsalis, but there is an ongoing phase III multicenter randomized trial as of early 2004 funded by the National Institute of Allergy and Infectious Diseases (NIAID) for assessing the antibiotic Azithromycin given orally in treatment of primary, secondary or early latent syphilis. The NIAID and the National Institute of Neurological Diseases and Stroke (NINDS) are carrying out research to develop a non-invasive test for detecting syphilis and to develop a vaccine. The genome of Treponema pallidum has been sequenced through NIAID-funded research. This is a wealth of information that will hopefully lead to clues to better diagnose, treat and vaccinate against syphilis.

Prognosis

Tabes dorsalis is a chronic, annoying and incapacitating disease but is per se seldom fatal. If tabes dorsalis is diagnosed in its very early stages, fairly good recovery is possible. Pain is quite bothersome and has a serious impact on quality of life. Ataxia, dementia and blindness are incapacitating. Death usually occurs due to rupture of enlarged blood vessels and damage to heart valves, which occur as a part of tertiary syphilis. Rarely, a urinary infection will lead to sepsis and death.

Special Concerns

Tabes dorsalis can affect thinking and memory and all patients must have neuropsychological testing for dementia. They will need to get legal advice for estate and financial planning and their wishes for future medical care.



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