Tabes dorsalis is a late consequence of

neurosyphilis Neurosyphilis is the infection of the central nervous system by '' Treponema pallidum'', the bacterium that causes the sexually transmitted infection syphilis. In the era of modern antibiotics, the majority of neurosyphilis cases have been report ...

, characterized by the slow degeneration (specifically, demyelination) of the neural tracts primarily in the

dorsal root ganglia A dorsal root ganglion (or spinal ganglion; also known as a posterior root ganglion) is a cluster of neurons (a ganglion) in a dorsal root of a spinal nerve. The cell bodies of sensory neurons known as first-order neurons are located in the dors ...

of the

spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue that extends from the medulla oblongata in the lower brainstem to the lumbar region of the vertebral column (backbone) of vertebrate animals. The center of the spinal c ...

(nerve root). These patients have lancinating nerve root pain which is aggravated by coughing, and features of sensory ataxia with ocular involvement.

Signs and symptoms

Signs and symptoms may not appear for decades after the initial infection and include

weakness Weakness is a symptom of many different medical conditions. 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 variety of skeletal muscle diseases, ...

, diminished reflexes,

paresthesia Paresthesia is a sensation of the skin that may feel like numbness (''hypoesthesia''), tingling, pricking, chilling, or burning. It can be temporary or Chronic condition, chronic and has many possible underlying causes. Paresthesia is usually p ...

s (shooting and burning pains, pricking sensations, and

formication Formication is the sensation resembling that of small insects crawling on (or delusional parasitosis, under) the skin, in the absence of actual insects. It is one specific form of a set of sensations known as paresthesias, which also include the ...

), hypoesthesias (abnormally diminished sense of touch), 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 Urinary incontinence (UI), also known as involuntary urination, is any uncontrolled leakage of urine. It is a common and distressing problem, which may have a significant effect on quality of life. Urinary incontinence is common in older women ...

dementia Dementia is a syndrome associated with many neurodegenerative diseases, characterized by a general decline in cognitive abilities that affects a person's ability to perform activities of daily living, everyday activities. This typically invo ...

deafness Deafness has varying definitions in cultural and medical contexts. In medical contexts, the meaning of deafness is hearing loss that precludes a person from understanding spoken language, an audiological condition. In this context it is writte ...

visual impairment Visual or vision impairment (VI or VIP) is the partial or total inability of visual perception. In the absence of treatment such as corrective eyewear, assistive devices, and medical treatment, visual impairment may cause the individual difficul ...

, 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 The patellar reflex, also called the knee reflex or knee-jerk, is a stretch reflex which tests the L2, L3, and L4 segments of the spinal cord. Many animals, most significantly humans, have been seen to have the patellar reflex, including dogs, cat ...

may be lacking ( Westphal's sign). A complication of tabes dorsalis can be transient neuralgic

paroxysmal Paroxysmal attacks or paroxysms are a sudden recurrence or intensification of symptoms, such as a spasm or seizure. These short, frequent symptoms can be observed in various clinical conditions. They are usually associated with multiple scleros ...

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 eye pain, tearing of the eyes and sensitivity to light.Basic Clinical Neuroscience, Young, Young, and Tolbert. Lippincott, Williams, and Wilkins, "Tabes dorsalgia" is a related lancinating back pain. "Tabetic gait" is a characteristic ataxic

gait Gait is the pattern of Motion (physics), movement of the limb (anatomy), limbs of animals, including Gait (human), humans, during Animal locomotion, locomotion over a solid substrate. Most animals use a variety of gaits, selecting gait based on s ...

of untreated syphilis where the person's feet slap the ground as they strike the floor due to loss of

proprioception Proprioception ( ) is the sense of self-movement, force, and body position. Proprioception is mediated by proprioceptors, a type of sensory receptor, located within muscles, tendons, and joints. Most animals possess multiple subtypes of propri ...

. In daylight the person can avoid some unsteadiness by watching their own feet.

Cause

Tabes dorsalis is caused by demyelination by advanced syphilis infection ( tertiary syphilis) when the primary infection by the causative

spirochete A spirochaete () or spirochete is a member of the phylum Spirochaetota (also called Spirochaetes ), which contains distinctive diderm (double-membrane) Gram-negative bacteria, most of which have long, helically coiled (corkscrew-shaped or ...

bacterium Bacteria (; : bacterium) are ubiquitous, mostly free-living organisms often consisting of one biological cell. They constitute a large domain of prokaryotic microorganisms. Typically a few micrometres in length, bacteria were among the ...

, ''

Treponema pallidum ''Treponema pallidum'', formerly known as ''Spirochaeta pallida'', is a Microaerophile, microaerophilic, Gram-negative bacteria, gram-negative, spirochaete bacterium with subspecies that cause the diseases syphilis, bejel (also known as endemic ...

'', is left untreated for an extended period of time (past the point of blood infection by the organism). The spirochete invades large myelinated fibers, leading to the involvement of the dorsal column medial leminiscus pathway rather than the spinothalamic tract.

Diagnosis

Routine screening for syphilis. Treponemal antibody usually positive both in blood and in CSF also. In CSF lymphocytosis and elevated protein found. Serological tests are usually positive.

Treatment

Intravenous Intravenous therapy (abbreviated as IV therapy) is a medical technique that administers fluids, medications and nutrients directly into a person's vein. The intravenous route of administration is commonly used for rehydration or to provide nutr ...

ly administered

penicillin Penicillins (P, PCN or PEN) are a group of beta-lactam antibiotic, β-lactam antibiotics originally obtained from ''Penicillium'' Mold (fungus), moulds, principally ''Penicillium chrysogenum, P. chrysogenum'' and ''Penicillium rubens, P. ru ...

is the treatment of choice. Associated pain can be treated with

opiate An opiate is an alkaloid substance derived from opium (or poppy straw). It differs from the similar term ''opioid'' in that the latter is used to designate all substances, both natural and synthetic, that bind to opioid receptors in the brain ( ...

valproate Valproate (valproic acid, VPA, sodium valproate, and valproate semisodium forms) are medications primarily used to treat epilepsy and bipolar disorder and prevent migraine headaches. They are useful for the prevention of seizures in those with ...

, or

carbamazepine Carbamazepine, sold under the brand name Tegretol among others, is an anticonvulsant medication used in the treatment of epilepsy and neuropathic pain. It is used as an adjunctive treatment in schizophrenia along with other medications and as ...

. Those with tabes dorsalis may also require

physical therapy Physical therapy (PT), also known as physiotherapy, is a healthcare profession, as well as the care provided by physical therapists who promote, maintain, or restore health through patient education, physical intervention, disease preventio ...

and

occupational therapy Occupational therapy (OT), also known as ergotherapy, is a healthcare profession. Ergotherapy is derived from the Greek wiktionary:ergon, ergon which is allied to work, to act and to be active. Occupational therapy is based on the assumption t ...

to deal with muscle wasting and weakness. Preventive treatment for those who come into sexual contact with an individual with syphilis is important.

Prognosis

Left untreated, tabes dorsalis can lead to

paralysis Paralysis (: paralyses; also known as plegia) is a loss of Motor skill, motor function in one or more Skeletal muscle, muscles. Paralysis can also be accompanied by a loss of feeling (sensory loss) in the affected area if there is sensory d ...

, and

blindness Visual or vision impairment (VI or VIP) is the partial or total inability of visual perception. In the absence of treatment such as corrective eyewear, assistive devices, and medical treatment, visual impairment may cause the individual difficul ...

. 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 HIV infection.

History

Although there were earlier clinical accounts of this disease, and descriptions and illustrations of the posterior columns of the spinal cord, it was the Berlin neurologist Romberg whose account became the classical textbook description, first published in German and later translated into English. Sir Arthur Conan Doyle, author of the

Sherlock Holmes Sherlock Holmes () is a Detective fiction, fictional detective created by British author Arthur Conan Doyle. Referring to himself as a "Private investigator, consulting detective" in his stories, Holmes is known for his proficiency with obser ...

stories, completed his

doctorate A doctorate (from Latin ''doctor'', meaning "teacher") or doctoral degree is a postgraduate academic degree awarded by universities and some other educational institutions, derived from the ancient formalism '' licentia docendi'' ("licence to teach ...

on tabes dorsalis in 1885.

Society and culture

Notable patients

* German storywriter E.T.A. Hoffmann appears to have had and died in 1822 from tabes dorsalis. *

Mary Todd Lincoln Mary Ann Todd Lincoln (Birth name, née Todd; December 13, 1818July 16, 1882) was First Lady of the United States from 1861 until the assassination of her husband, President Abraham Lincoln, in 1865. Mary Todd was born into a large and wealthy ...

ec 12, 1818 - Jul 16, 1882 wife of U.S. President Abraham Lincoln and America's First Lady from 1861 to 1865 most probably suffered from tabes dorsalis as early as 1869, at age 51

She died of a stroke at age 63 in Springfield, IL. * The French people, French novelist Alphonse Daudet 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 Barnes Julian Patrick Barnes (born 19 January 1946) is an English writer. He won the Man Booker Prize in 2011 with ''The Sense of an Ending'', having been shortlisted three times previously with ''Flaubert's Parrot'', ''England, England'', and ''Arthu ...

. * Poet

Charles Baudelaire Charles Pierre Baudelaire (, ; ; 9 April 1821 – 31 August 1867) was a French poet, essayist, translator and art critic. His poems are described as exhibiting mastery of rhythm and rhyme, containing an exoticism inherited from the Romantics ...

contracted syphilis in 1839 and resorted to opium to help alleviate the pain of tabes dorsalis ascending his spine. * Painter

Édouard Manet Édouard Manet (, ; ; 23 January 1832 – 30 April 1883) was a French Modernism, modernist painter. He was one of the first 19th-century artists to paint modern life, as well as a pivotal figure in the transition from Realism (art movement), R ...

died of syphilis complications, including tabes dorsalis, in 1883, aged 51. * Boxer Charley Mitchell * Meyer Nudelman, the father of author and doctor Sherwin Nuland, who described his father's condition extensively in his book ''Lost in America; A Journey with my Father'' (2003).

References

External links

* {{Spinal cord lesions Pain Histopathology Neurodegenerative disorders Syphilis