Tabes Dorsalis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Marshall Hall an English physician found a patient with loss of postural control in darkness caused by severely compromised proprioception In 1836, but He did not develop more information about it. Tabes dorsalis was first discovered by Moritz Heinrich Romberg, a german physician, in 1840. He described that excessive drinking and sexual activity may be the causes of tabes dorsalis and he named it progressive locomotor ataxia but he did not find the relation between syphilis and tabes doesalis. The association between syphilis and tabes dorsalis first time was considered by Guillaume Duchenne a French neurologist in 1858. In 1875, Jean-Alfred Fournier a French dermatologist, firmly described the syphilis as the main cause of tabes dorsalis. In 1888, Sir William R. Gowers a British neurologist gave accurate details of the modern Romberg’s test.

It is understood that tabes dorsalis is caused by tertiary syphilis from treponema pallidum infection.

Tabes dorsalis is a manifestation of invasion of treponema pallidum spirochetes to the dorsal column of spinal cord in tertiary syphilis.

In tabes dorsalis, the preganglionic portion of the dorsal roots of spinal nerves is infiltrated with lymphocytes and plasma cells, and invasion of treponema pallidum spirochetes to posterior columns of the spinal cord makes it atrophic.

The demyelination of the axones of the neurons is the main cause of symptoms and it affects the neurons in the dorsal root ganglia and posterior columns of the spinal cord.

Tabes dorsalis is a form of neurosyphilis, which is a complication of late or tertiary syphilis infection. Syphilis is a sexually transmitted infectious disease. The infection damages the spinal cord and peripheral nervous tissue.

Tabes dorsalis must be differentiated from other diseases that cause lightning pains, impaired sensation, ataxia and unsteady gait, such as vitamin B12 deficiency, extrinsic spinal cord compression, vitamin E deficiency and multiple sclerosis.

Tabes dorsalis is now very rare because syphilis is usually treated early in the disease.

In 2012, the incidence of syphilis was estimated to be 6 million cases worldwide. From year 2005 to 2014, the incidence of syphilis in the United States increased from 2.9 to 6.3 cases/100,000/year. The rate of reported cases increased by 15.1% between 2013 and 2014 in the United States. In 2012, the prevalence of syphilis was estimated to be approximately 18 million cases in men and women aged 15-29 worldwide. Among infected patients with Treponema pallidum only 3 to 5% develop neurosyphilis and only 5% of those individuals develop tabes dorsalis, 10–20 years later.

The most potent risk factor in the development of tabes dorsalis is untreated syphilis infection. Tabes dorsalis is a form of neurosyphilis, which is a complication of late or tertiary syphilis infection. Syphilis is a sexually transmitted infectious disease. The infection damages the spinal cord and peripheral nervous tissue.

The symptoms of tabes dorsalis usually develop secondary to long-term untreated syphilis, and start with symptoms such as lightning pains, impaired sensation and proprioception and hypesthesias. Common complications of tabes dorsalis include Dementia, stroke, eye disease, Paralysis, and Charcot arthropathy(Charcot joint).

A positive history of unprotected sexual activity or an proved treponemal infection is suggestive of tabes dorsalis. The most common symptoms of tabes dorsalis include lightning pains, impaired sensation and proprioception, hypesthesias, diminished reflexes or loss of reflexes, Poor coordination or loss of coordination, Unsteady gait(locomotor ataxia), sexual function problems and progressive sensory ataxia(inability to feel the lower limbs).

Patients with tabes dorsalis usually appear normal. Physical examination of patients with tabes dorsalis is usually remarkable for: Argyll-Robertson pupils, impaired vibratory and proprioception sense, broad base and sensory ataxic gait and positive romberg’s test.

Laboratory tests which may help diagnose syphilis include darkfield examinations and tests to detect T. pallidum in lesion exudate or tissue, PCR, nontreponemal (e.g., venereal disease research laboratory (VDRL) and rapid plasma reagent test) and treponemal tests (e.g., fluorescent treponemal antibody absorbed (FTA-ABS) tests, the T. pallidum passive particle agglutination (TP-PA) assay, various enzyme immunoassays, and chemiluminescence immunoassays).

Abnormalities in the CSF consistent with disease include; Pleocytosis, often lymphocytic predominant, mild protein elevation and positive CSF VDRL.

Spinal CT scan may be helpful in the diagnosis of tabes dorsalis. Findings on CT scan suggestive of of neurosyphilis include calcification in the soft tissues(posterior to the cord in cervical spine CT scan), ankylosis across the C4–5 disc and facet joints bilateral, areas of decreased density suggesting cerebral infarction, syphilitic gumma appear hypodense with precontrast, focal or diffuse extra-axial enhancement and non-specific white matter lesions.

Spinal and brain MRI may be helpful in the diagnosis of tabes dorsalis. Findings on MRI suggestive of tabes dorsalis include, longitudinal T2-weighted hyperintensity in the dorsal columns of the spinal cord, narrowing between the cervical intervertebral discs and partial ankylosis of the cervical disc space, bilateral high intensity signals on the T2 weighted sequence located in mesiotemporal, insular, frontal regions, calcification of the ligamentum flavum.

Penicillin, administered intravenously, is the treatment of choice of tabes dorsalis. Preventive treatment for those who come into sexual contact with an individual with tabes dorsalis is important. CNS involvement can occur during any stage of syphilis. However, CSF laboratory abnormalities are common in persons with early syphilis, even in the absence of clinical neurological findings. No evidence exists to support variation from recommended treatment for early syphilis for patients found to have such abnormalities. If clinical evidence of neurologic involvement is observed (e.g., cognitive dysfunction, motor or sensory deficits, ophthalmic or auditory symptoms, cranial nerve palsies, and symptoms or signs of meningitis), a CSF examination should be performed. Syphilitic uveitis or other ocular manifestations frequently are associated with neurosyphilis and should be managed according to the treatment recommendations for neurosyphilis. Patients who have neurosyphilis or syphilitic eye disease (e.g., uveitis, neuroretinitis, and optic neuritis) should be treated with the recommended regimen for neurosyphilis; those with eye disease should be managed in collaboration with an ophthalmologist. A CSF examination should be performed for all patients with syphilitic eye disease to identify those with abnormalities; patients found to have abnormal CSF test results should be provided follow-up CSF examinations to assess treatment response. Associated pain can be treated with opiates, valproate, or carbamazepine. Patients may also require physical or rehabilitative therapy to deal with muscle wasting and weakness.

There is no vaccine available for prevention of syphilis. However, effective measures for the primary prevention of syphilis include abstinence from intimate physical contact with an infected person, consistent use of latex condoms, limiting number of sexual partners, avoidance of sharing sex toys, practising safe sex, routine screening in pregnant females, individuals with high risk behaviours, and those residing in highly prevalent areas. In patients with diagnosed syphilis, early treatment with penicillin can completely prevent tabes dorsalis.

Secondary prevention strategies following syphilis include routine screening and follow up in patients with early syphilis to prevent complications, diagnosis and treatment of sexual partners of infected individuals, routine screening, diagnosis and treatment in pregnant females. In patients with diagnosed syphilis, early treatment with penicillin can completely prevent tabes dorsalis.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

In 1836, Marshall Hall an English physician found a patient with loss of postural control in darkness caused by severely compromised proprioception, but He did not develop more information about it. In 1840, Moritz Heinrich Romberg, a german physician was the first who discovered tabes dorsalis. He described excessive drinking and increase sexual activity may be the causes of tabes dorsalis. He named the disease as progressive locomotor ataxia. He was unable to find the relation between syphilis and tabes doesalis. In 1858, Guillaume Duchenne a French neurologist for the first time described the association between syphilis and tabes dorsalis. In 1875, Jean-Alfred Fournier, a French dermatologist conclusively described the syphilis as the main cause of tabes dorsalis. In 1888, Sir William R. Gowers a British neurologist gave accurate details of the modern Romberg’s test.

The main points in historical Perspective of tabes dorsalis are:^[1][2][3][4]

• In 1836, Marshall Hall, an English physician, found a patient with loss of postural control in darkness caused by severely compromised proprioception. but He did not develop more information about it.

• In 1840, Moritz Heinrich Romberg, a german physician was the first who discovered tabes dorsalis. He described that excessive drinking and sexual activity may be the causes of tabes dorsalis and he named it progressive locomotor ataxia but he was unable to find the relation between syphilis and tabes doesalis.

• In 1858, Guillaume Duchenne a French neurologist for the first time described the association between syphilis and tabes dorsalis.
• In 1875, Jean-Alfred Fournier a French dermatologist, firmly described the syphilis as the main cause of tabes dorsalis.
• In 1888, Sir William R. Gowers a British neurologist gave accurate details of the modern Romberg’s test.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

The syphilis disease is the sole cause of tabes dorsalis. Treponema pallidum is usually transmitted via direct contact with the infected lesion (sexual contact) or blood transfusion (rare). It is understood that tabes dorsalis is caused by tertiary syphilis from treponema pallidum infection. Tabes dorsalis is a manifestation of invasion of treponema pallidum spirochete to the dorsal column of spinal cord in tertiary syphilis. In tabes dorsalis, the preganglionic portion of the dorsal roots of spinal nerves is infiltrated with lymphocytes and plasma cells, and invasion of treponema pallidum spirochetes to posterior columns of the spinal cord makes it atrophic. The demyelination of the axones of the neurons is the main cause of symptoms and it affects the neurons in the dorsal root ganglia and posterior columns of the spinal cord.

• The syphilis disease is the sole cause of tabes dorsalis.
• The pathogenesis of syphilis may be described in the following steps:^{[1][2][3][4][5][6][7][8][9][10][11]}
  • Treponema pallidum is usually transmitted via direct contact with the infected lesion (sexual contact) or blood transfusion (rare).
  • The incubation period varies with the size of innoculum (9-90 days).

• Following transmission, Treponema pallidum uses the intact or abraded mucous membrane to enter the body.
• It then disseminates to the lymphatics and blood stream to gain access to any organ of the body.

• Syphilis uses fibronectin molecules to attach to the endothelial surface of the vessels in organs resulting in inflammation and obliteration of the small blood vessels causing vasculitis (endarteritis obliterans).
• Organism has slow replication rate (30-33 hrs) and evades the initial host immune response.
• It may seed to different organs of the body especially the cardiovascular system and central nervous system resulting in tertiary syphilis.

Different stages of syphilis results from the interaction between the antigen and the host immune response.^[1][2]

• The initial infection in primary syphilis is limited due to T helper 1 response and lack of the antibody response.
• It is speculated that there is a shift from T helper 1 to Th2 response during secondary syphilis.

• Cytotoxic T cells and an incomplete humoral response is mainly responsible for persistence of infection and tissue damage in tertiary syphilis.
• Ineffective type 4 delayed hypersensitivity reaction containing macrophages and sensitized T cells is mainly responsible for the gumma formation in various organs.

• It is understood that tabes dorsalis is caused by tertiary syphilis from treponema pallidum infection.
• Tabes dorsalis is a manifestation of invasion of treponema pallidum spirochetes to the dorsal column of spinal cord in tertiary syphilis.
• In tabes dorsalis, the preganglionic portion of the dorsal roots of spinal nerves is infiltrated with lymphocytes and plasma cells, and invasion of treponema pallidum spirochetes to posterior columns of the spinal cord makes it atrophic.
• The demyelination of the axones of the neurons is the main cause of symptoms and it affects the neurons in the dorsal root ganglia and posterior columns of the spinal cord.

There is no known genetic mutation that is associated with syphilis. However, neurosyphilis may be associated with the gene polymorphism for IL-10 production with increased levels seen in the patients with neurosyphilis.^[11]

• Syphilis is associated with increased transmission of HIV.^[9]
  • The underlying mechanism may be related to the accumulation of dendritic cells containing CCR5 co-receptors at the site of infection, the same receptor entity binding the HIV.

On microscopic histopathological analysis, characteristic findings of tabes dorsalis are:^[1]

• Small vessel inflammation (endarteritis obliterans)
• Granulomatous lesions (gumma) containing central necrosis, inflammatory cells, such as lymphocytes, macrophages, plasma cells and fibroblasts.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Tabes dorsalis is a form of neurosyphilis, which is a complication of late or tertiary syphilis infection. Syphilis is a sexually transmitted infectious disease. The infection damages the spinal cord and peripheral nervous tissue.

Tabes dorsalis is a form of neurosyphilis, which is a complication of late or tertiary syphilis infection. Syphilis is a sexually transmitted infectious disease. The infection damages the spinal cord and peripheral nervous tissue.^[1]

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Tabes dorsalis must be differentiated from other diseases that cause lightning pains, impaired sensation, ataxia and unsteady gait, such as vitamin B12 deficiency, extrinsic spinal cord compression, vitamin E deficiency and multiple sclerosis.

• Tabes dorsalis must be differentiated from other diseases that cause lightning pains, impaired sensation and proprioception, and progressive sensory ataxia and unsteady gait (locomotor ataxia) and sexual function problems, such as vitamin B12 deficiency, extrinsic spinal cord compression, vitamin E deficiency and multiple sclerosis.^[1][2][3][4]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: ;Mohamadmostafa Jahansouz M.D.[2] Aysha Anwar, M.B.B.S[3]; Nate Michalak, B.A.

Neurosyphilis is now very rare because syphilis is usually treated early. In 2012, the incidence of syphilis was estimated to be 6 million cases worldwide. From year 2005 to 2014, the incidence of syphilis in the United States increased from 2.9 to 6.3 cases/100,000/year. The rate of reported cases increased by 15.1% between 2013 and 2014 in the United States. In 2012, the prevalence of syphilis was estimated to be approximately 18 million cases in men and women aged 15-29 worldwide. Among infected patients with Treponema pallidum only 3 to 5% develop neurosyphilis and 5% of those individuals develop tabes dorsalis, 10–20 years later.

• Worldwide incidence of syphilis:
  • 2004: approximately 12 million cases^[1]
  • 2008: approximately 10.9 million cases^[2][3][4]
  • 2012: approximately 6 million cases^[5]
• Incidence of syphilis in the United States:
  • From 2005 to 2014, the incidence of primary and secondary syphilis increased from 2.9 to 6.3 cases/100,000/year.
  • From 2009 to 2014, the incidence of late and latent syphilis increased from 5.6 to 7.4 cases/100,000/year.
  • The rate of reported cases increased by 15.1% between 2013 and 2014.^[6]
  • Syphilis incidence increased in every region of the Untied States in 2014, with the highest rate in the West and lowest rate in the Midwest.
  • Neurosyphilis is not common disease now, because syphilis is usually treated early.^[6]
    • Among infected patients with Treponema pallidum only 3 to 5% develop neurosyphilis and only 5% of those individuals develop tabes dorsalis, 10–20 years later.^[7]
    • The incidence of tabes dorsalis is around 250 out of 100,000 patients with syphilis, and 5000 out of 100,000 patients with neurosyphilis.

• In 2008, the prevalence of syphilis was estimated to be 36.4 million cases worldwide.^[2]
• In 2012, the prevalence of syphilis is estimated to be 18 million cases in men and women aged 15-29 worldwide.^[5]
• In 2014, the prevalence of syphilis in the United States was estimated to be 20 cases/100,000 individuals.^[6]

The incidence and prevalence of syphilis may be affected by the following demographic factors:^[6][8]

In 2014, the highest rate of primary and secondary syphilis in the United States was seen in age groups 20-24 years and 25-29 years.^[6][9]

• There is increased incidence of primary and secondary syphilis in men who have sex with men (MSM).
  • In 2014, 61% of 19,999 total reported cases of primary and secondary syphilis were estimated to be among MSM.^{[6][8][10][7][11]}

• Syphilis is more prevalent in the African American population as compared to other racial groups.^[5]
• In 2014, the highest incidence of primary and secondary syphilis in the United States was reported to be in the African American population and the lowest incidence was observed in Caucasians, Asians and multiracial groups.^[6]

Worldwide, the highest incidence and prevalence of syphilis was seen in Africa and South East Asia, while the lowest was seen in Europe and the Eastern Mediterranean region in 2012.^[5]

United States

• Rates of reported cases of primary and secondary syphilis by state for 2014 is shown in the map below:

• Rates of reported cases of primary and secondary syphilis by region for 2014 is shown in the map below:

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

The most potent risk factor in the development of tabes dorsalis is HIV infection. Other risk factors include: Male gender, high serum RPR titer, advanced age, and African American race.

• The most potent risk factor in the development of tabes dorsalis is HIV infection. Other risk factors include: Male gender, high serum RPR titer, advanced age, and African American race.

• Common risk factors in the development of tabes dorsalis may be occupational, environmental, genetic, and viral.
• Common risk factors in the development of tabes dorsalis include:^[1][2]
  • HIV infection
  • Male patients
  • High serum rapid plasma reagent titer

• Less common risk factors in the development of tabes dorsalis include:^[2]
  • Advanced age
  • African American race

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

The symptoms of tabes dorsalis usually develop secondary to long-term untreated syphilis, and start with symptoms such as lightning pains, impaired sensation and proprioception, and hypesthesias. Common complications of tabes dorsalis include Dementia, stroke, eye disease, Paralysis, and Charcot arthropathy (Charcot joint).

The symptoms of tabes dorsalis usually develop secondary to long-term untreated syphilis, and start with symptoms such as:

• Lightning pains^[1]

• Impaired sensation and proprioception^[2]
• Hypesthesias^[3].

The symptoms of tabes dorsalis typically occurs 10 to 30 years after primary infection by treponema pallidum.^[4]

If left untreated, most patients with tabes dorsalis may progress to develop paralysis, dementia, and blindness.

Common complications of tabes dorsalis include:^[5][6]

• Dementia, stroke, eye disease
• Paralysis

• Charcot arthropathy (Charcot joint) of the foot may be seen
• Broad base gate and sensory ataxic gait is usually seen in patients with tabes dorsalis

Prognosis varies by site of involvement and duration of disease:^[7][8][9]

• Among patients with neurosyphilis, 90% respond to treatment.
• Gummatous lesions reverse with treatment.
• Mortality rates are high with cardiovascular complications.
• Mortality rate of patients with neurosyphilis is around 20% which is mainly due to related complications.

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