Tabes dorsalis
From The Art and Popular Culture Encyclopedia
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Tabes dorsalis is a slow degeneration of the sensory neurons that carry information. The degenerating nerves are in the dorsal columns (posterior 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 (proprioception), vibration, and discriminative touch.
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Cause
Tabes dorsalis is caused by demyelination. It is the result of an untreated syphilis infection.
Symptoms
Symptoms may not appear for some decades after the initial infection and include: weakness, diminished reflexes, paresthesias including morbid cutaneous sensations having no objective cause, shooting and burning pains, pricking sensations, and formication (a sensation like that produced by small insects crawling over skin), and hypoesthesias (abnormally diminished cutaneous, especially tactile, sensory modalities), tabetic gait (locomotor ataxia), progressive degeneration of the joints, loss of coordination, episodes of intense pain and disturbed sensation (including glossodynia), personality changes, dementia, deafness, visual impairment, and impaired response to light (Argyll Robertson pupil). The skeletal musculature is hypotonic due to destruction of the sensory limb of the spindle reflex. The deep tendon reflexes are also diminished or absent; for example, the "knee jerk" or patellar reflex may be lacking (this is known as Westphal's sign). A complication of Tabes Dorsalis can be transient neuralgic paroxysmal pain affecting the eyes and the ophthalmic areas, which used to be referred to as "Pel's Crises" - named after Dutch physician of internal medicine P.K. Pel. Now more commonly called "tabetic ocular crises," an attack is characterized by sudden, intense ocular pain, lacrimation and photophobia.
Tabes dorsalgia is a related back pain.
"Tabetic gait" is a characteristic sign of untreated syphilis where you hear the patient's feet as they strike the floor due to loss of proprioception.
Prognosis
If left untreated, tabes dorsalis can lead to paralysis, dementia, and blindness. Existing nerve damage cannot be reversed.
Incidence/Prevalence
The disease is more frequent in males than in females. Onset is commonly during mid-life. The incidence of tabes dorsalis is rising, in part due to co-associated HIV infection.
Treatment
Penicillin, administered intravenously, is the treatment of choice. Associated pain can be treated with opiates, valproate, or carbamazepine. Patients may also require physical therapy to deal with muscle wasting and weakness. Preventive treatment for those who come into sexual contact with an individual with tabes dorsalis is important.
Trivia
Sir Arthur Conan Doyle, author of the Sherlock Holmes stories, completed his doctorate on tabes dorsalis in 1885.
It was believed erroneously for some time that Tabes dorsalis was caused by sexual excess. This belief was corrected by the mid-nineteenth century.
Notable sufferers
- The French novelist Alphonse Daudet kept a journal of the pain he experienced from his Tabes Dorsalis. It was collected into the short book "In the Land of Pain" in 2002, translated by the English writer Julian Barnes.
See also