Tabes dorsalis is a late consequence of

neurosyphilis Neurosyphilis refers to infection of the central nervous system in a patient with syphilis. In the era of modern antibiotics the majority of neurosyphilis cases have been reported in HIV-infected patients. Meningitis is the most common neurologic ...

, characterized by the slow degeneration (specifically,

demyelination A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged. This damage impairs the conduction of signals in the affected nerves. In turn, the reduction in conduction ability causes deficiency i ...

) 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 dorsal ...

of the

spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the sp ...

(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 a number of different 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, i ...

, diminished reflexes, paresthesias (shooting and burning pains, pricking sensations, and formication), hypoesthesias (abnormally diminished sense of touch), tabetic gait (

locomotor ataxia Locomotor ataxia is the inability to precisely control one's own bodily movements. __TOC__ Disease People afflicted with this disease may walk in a jerky, non-fluid manner. They will not know where their arms and legs are without looking (i.e. a f ...

), progressive degeneration of the joints,

loss of coordination Ataxia is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in eye movements. Ataxia is a clinical manifestation indicating dysfunction of ...

, episodes of intense pain and disturbed sensation (including

glossodynia Burning mouth syndrome (BMS) is a burning, tingling or scalding sensation in the mouth, lasting for at least four to six months, with no underlying known dental or medical cause. No related medical sign, signs of disease are found in the mouth. ...

), 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 large impact on quality of life. It has been identified as an important issue in geri ...

dementia Dementia is a disorder which manifests as a set of related symptoms, which usually surfaces when the brain is damaged by injury or disease. The symptoms involve progressive impairments in memory, thinking, and behavior, which negatively affe ...

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 written ...

visual impairment Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment� ...

, 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 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 so ...

). A complication of tabes dorsalis can be transient neuralgic

paroxysmal Paroxysmal attacks or paroxysms (from Greek παροξυσμός) 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 usuall ...

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

proprioception Proprioception ( ), also referred to as kinaesthesia (or kinesthesia), is the sense of self-movement, force, and body position. It is sometimes described as the "sixth sense". Proprioception is mediated by proprioceptors, mechanosensory neurons ...

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

Cause

Tabes dorsalis is caused by

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 (), (synonym Spirochaetes) which contains distinctive diderm (double-membrane) gram-negative bacteria, most of which have long, helically coiled (corkscrew-shaped or s ...

bacterium Bacteria (; singular: 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 amon ...

, ''

Treponema pallidum ''Treponema pallidum'', formerly known as ''Spirochaeta pallida'', is a spirochaete bacterium with various subspecies that cause the diseases syphilis, bejel (also known as endemic syphilis), and yaws. It is transmitted only among humans. It is ...

'', 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. Treponomal antibody usually positive both in blood and in csf also. In CSF lymphocytosis and elevated protein found. Serological tests are usually positive.

Treatment

Intravenously administered penicillin is the treatment of choice. Associated pain can be treated with opiates,

valproate Valproate (VPA) and its valproic acid, 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 ...

, or

carbamazepine Carbamazepine (CBZ), sold under the trade name Tegretol among others, is an anticonvulsant medication used primarily in the treatment of epilepsy and neuropathic pain. It is used as an adjunctive treatment in schizophrenia along with other m ...

. Those with tabes dorsalis may also require physical therapy and occupational therapy 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,

, and

blindness Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment� ...

. 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 The human immunodeficiency viruses (HIV) are two species of ''Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the immune ...

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 Sir Arthur Ignatius Conan Doyle (22 May 1859 – 7 July 1930) was a British writer and physician. He created the character Sherlock Holmes in 1887 for '' A Study in Scarlet'', the first of four novels and fifty-six short stories about Ho ...

, author of the Sherlock Holmes stories, completed his

doctorate A doctorate (from Latin ''docere'', "to teach"), doctor's degree (from Latin ''doctor'', "teacher"), or doctoral degree is an academic degree awarded by universities and some other educational institutions, derived from the ancient formalism ''li ...

on tabes dorsalis in 1885.

Notable patients

German German(s) may refer to: * Germany (of or related to) ** Germania (historical use) * Germans, citizens of Germany, people of German ancestry, or native speakers of the German language ** For citizens of Germany, see also German nationality law **Ge ...

storywriter E.T.A. Hoffmann appears to have had and died from tabes dorsalis. * The French novelist

Alphonse Daudet Alphonse Daudet (; 13 May 184016 December 1897) was a French novelist. He was the husband of Julia Daudet and father of Edmée, Léon and Lucien Daudet. Early life Daudet was born in Nîmes, France. His family, on both sides, belonged to the ...

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 '' A ...

. * Poet

Charles Baudelaire Charles Pierre Baudelaire (, ; ; 9 April 1821 – 31 August 1867) was a French poet who also produced notable work as an essayist and art critic. His poems exhibit mastery in the handling of rhyme and rhythm, contain an exoticism inherited ...

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 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 to Impressionism. Bo ...

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

*
'An Essay Upon the Vasomotor Changes in Tabes Dorsalis' by Arthur Conan Doyle

External links

{{Spinal cord lesions Pain Histopathology Neurodegenerative disorders Syphilis