Tabes dorsalis
Encyclopedia
Tabes dorsalis is a slow degeneration of the sensory neurons that carry afferent 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.
secondary to an untreated syphilis
infection
, diminished reflexes, paresthesia
s (shooting and burning pains, pricking sensations, and formication
), 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, urinary incontinence, dementia
, deafness, visual impairment
, positive Romberg's test
, 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 (Westphal's sign
). A complication of tabes dorsalis can be transient neuralgic paroxysmal pain affecting the eyes and the ophthalmic areas, previously called "Pel's crises" after Dutch physician 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 high-stepping gait
of untreated syphilis where the patient's feet slap the ground as they strike the floor due to loss of proprioception
.
, dementia
, and blindness
. Existing nerve damage cannot be reversed.
infection.
, administered intravenously, is the treatment of choice. Associated pain can be treated with opiate
s, 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 syphilis is important.
stories, completed his doctorate
on tabes dorsalis in 1885.
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
(the portion closest to the back of the body) and carry information that help maintain a person's sense of position (proprioception
Proprioception
Proprioception , from Latin proprius, meaning "one's own" and perception, is the sense of the relative position of neighbouring parts of the body and strength of effort being employed in movement...
), vibration, and discriminative touch.
Cause
Tabes dorsalis is caused by demyelinationDemyelinating disease
A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged. This impairs the conduction of signals in the affected nerves, causing impairment in sensation, movement, cognition, or other functions depending on which nerves are involved.The term...
secondary to an untreated syphilis
Syphilis
Syphilis is a sexually transmitted infection caused by the spirochete bacterium Treponema pallidum subspecies pallidum. The primary route of transmission is through sexual contact; however, it may also be transmitted from mother to fetus during pregnancy or at birth, resulting in congenital syphilis...
infection
Symptoms
Symptoms may not appear for some decades after the initial infection and include: weaknessWeakness
Weakness is a symptom represented, medically, by a number of different conditions, including: lack of muscle strength, malaise, dizziness, or fatigue. The causes are many and can be divided into conditions that have true or perceived muscle weakness. True muscle weakness is a primary symptom of a...
, diminished reflexes, paresthesia
Paresthesia
Paresthesia , spelled "paraesthesia" in British English, is a sensation of tingling, burning, pricking, or numbness of a person's skin with no apparent long-term physical effect. It is more generally known as the feeling of "pins and needles" or of a limb "falling asleep"...
s (shooting and burning pains, pricking sensations, and formication
Formication
Formication is the medical term for a sensation that resembles that of insects crawling on the skin. It is one specific form of a set of sensations known as paresthesia, which also include the more common prickling, tingling sensation of "pins and needles"...
), hypoesthesias (abnormally diminished cutaneous, especially tactile, sensory modalities), tabetic gait (locomotor ataxia
Locomotor ataxia
Locomotor ataxia is the inability to precisely control one's own bodily movements. Persons afflicted with this disease may walk in a jerky, nonfluid manner. They will not know where their arms and legs are without looking, but can, for instance, feel and locate a hot object placed against their feet...
), progressive degeneration of the joints, loss of coordination, episodes of intense pain and disturbed sensation (including glossodynia
Glossodynia
Glossodynia or burning mouth syndrome is a condition characterized by a burning or tingling sensation on the lips, tongue, or entire mouth....
), personality changes, urinary incontinence, dementia
Dementia
Dementia is a serious loss of cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging...
, deafness, visual impairment
Visual impairment
Visual impairment is vision loss to such a degree as to qualify as an additional support need through a significant limitation of visual capability resulting from either disease, trauma, or congenital or degenerative conditions that cannot be corrected by conventional means, such as refractive...
, positive Romberg's test
Romberg's test
Romberg's test or the Romberg maneuver is a test used by doctors in a neurological examination, and also as a test for drunken driving. The exam is based on the premise that a person requires at least two of the three following senses to maintain balanced while standing:Proprioception ; Vestibular...
, and impaired response to light (Argyll Robertson pupil
Argyll Robertson pupil
Argyll Robertson pupils are bilateral small pupils that constrict when the patient focuses on a near object , but do not constrict when exposed to bright light . They are a highly specific sign of neurosyphilis. In general, pupils that “accommodate but do not react” are said to show light-near...
). 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
Patellar reflex
The patellar reflex or knee-jerk is a deep tendon reflex and is a myotatic reflex.-Mechanism:Striking the patellar tendon with a tendon hammer just below the patella stretches the sensory nerve fiber of the femoral nerve which synapses at the level of L4 in the spinal cord, completely independent...
may be lacking (Westphal's sign
Westphal's sign
Westphal's sign is the clinical correlate of the absence or decrease of patellar reflex or knee jerk. Patellar reflex or knee jerk is a kind of deep or stretch reflex where an application of a stimulus to the patellar tendon such as strike by a solid object or hammer caused the leg to extend due to...
). A complication of tabes dorsalis can be transient neuralgic paroxysmal pain affecting the eyes and the ophthalmic areas, previously called "Pel's crises" after Dutch physician 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 high-stepping gait
Gait
Gait is the pattern of movement of the limbs of animals, including humans, during locomotion over a solid substrate. Most animals use a variety of gaits, selecting gait based on speed, terrain, the need to maneuver, and energetic efficiency...
of untreated syphilis where the patient's feet slap the ground as they strike the floor due to loss of proprioception
Proprioception
Proprioception , from Latin proprius, meaning "one's own" and perception, is the sense of the relative position of neighbouring parts of the body and strength of effort being employed in movement...
.
Prognosis
Left untreated, tabes dorsalis can lead to paralysisParalysis
Paralysis is loss of muscle function for one or more muscles. Paralysis can be accompanied by a loss of feeling in the affected area if there is sensory damage as well as motor. A study conducted by the Christopher & Dana Reeve Foundation, suggests that about 1 in 50 people have been diagnosed...
, dementia
Dementia
Dementia is a serious loss of cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging...
, and blindness
Blindness
Blindness is the condition of lacking visual perception due to physiological or neurological factors.Various scales have been developed to describe the extent of vision loss and define blindness...
. Existing nerve damage cannot be reversed.
Epidemiology
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 HIVHIV
Human immunodeficiency virus is a lentivirus that causes acquired immunodeficiency syndrome , a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thrive...
infection.
Treatment
PenicillinPenicillin
Penicillin is a group of antibiotics derived from Penicillium fungi. They include penicillin G, procaine penicillin, benzathine penicillin, and penicillin V....
, administered intravenously, is the treatment of choice. Associated pain can be treated with opiate
Opiate
In medicine, the term opiate describes any of the narcotic opioid alkaloids found as natural products in the opium poppy plant.-Overview:Opiates are so named because they are constituents or derivatives of constituents found in opium, which is processed from the latex sap of the opium poppy,...
s, valproate, or carbamazepine
Carbamazepine
Carbamazepine is an anticonvulsant and mood-stabilizing drug used primarily in the treatment of epilepsy and bipolar disorder, as well as trigeminal neuralgia...
. Patients may also require physical therapy
Physical therapy
Physical therapy , often abbreviated PT, is a health care profession. Physical therapy is concerned with identifying and maximizing quality of life and movement potential within the spheres of promotion, prevention, diagnosis, treatment/intervention,and rehabilitation...
to deal with muscle wasting and weakness. Preventive treatment for those who come into sexual contact with an individual with syphilis is important.
Trivia
Sir Arthur Conan Doyle, author of the Sherlock HolmesSherlock Holmes
Sherlock Holmes is a fictional detective created by Scottish author and physician Sir Arthur Conan Doyle. The fantastic London-based "consulting detective", Holmes is famous for his astute logical reasoning, his ability to take almost any disguise, and his use of forensic science skills to solve...
stories, completed his doctorate
Doctorate
A doctorate is an academic degree or professional degree that in most countries refers to a class of degrees which qualify the holder to teach in a specific field, A doctorate is an academic degree or professional degree that in most countries refers to a class of degrees which qualify the holder...
on tabes dorsalis in 1885.
Notable sufferers
- The FrenchFrench peopleThe French are a nation that share a common French culture and speak the French language as a mother tongue. Historically, the French population are descended from peoples of Celtic, Latin and Germanic origin, and are today a mixture of several ethnic groups...
novelist Alphonse DaudetAlphonse DaudetAlphonse Daudet was a French novelist. He was the father of Léon Daudet and Lucien Daudet.- Early life :Alphonse Daudet was born in Nîmes, France. His family, on both sides, belonged to the bourgeoisie. The father, Vincent Daudet, was a silk manufacturer — a man dogged through life by misfortune...
kept a journal of the pain he experienced from this condition which was posthumously published as La Doulou (1930) and translated into English as In the Land of Pain (2002) by Julian BarnesJulian BarnesJulian Patrick Barnes is a contemporary English writer, and winner of the 2011 Man Booker Prize, for his book The Sense of an Ending...
.