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Tabes Dorsalis

Tabes dorsalis is a late manifestation of neurosyphilis that affects the posterior columns of the spinal cord and the dorsal roots. The disease process results in demyelination and inflammation with fibrosis of the spinal cord. Affected individuals most commonly present with sensory ataxia and stabbing, knife-like lancinating pain.


Presentation

The onset of symptoms attributed to tabes dorsalis may occur anywhere from 3 to 20 years following syphilis infection [1]. The most common presentation consists of loss of coordination (sensory ataxia) and sudden sharp, stabbing-like (lancinating) pain in the face, back, or lower extremities. Other symptoms include loss of pain and vibration sensations (paresthesia), seizures (status epilepticus), visceral crisis characterized by repeated attacks of severe epigastric pain, nausea, and vomiting, loss of sexual function, and bladder dysfunction (i.e., urinary retention and overflow incontinence) [2] [3] [4] [5] [6].

Depending on the duration or stage of tabes dorsalis, a patient may present with either preataxia, ataxia, or paralysis [1]. Patients with tabes dorsalis typically present with a slapping and/or wide-based gait during ambulation [7].

Falling
  • Case Report A 78-year-old man was injured in a fall while wheeling his bicycle on a snowy road.[omicsonline.org]
  • This is true even if your disability falls under the CAL program. In other words, even with a diagnosis of Tabes Dorsalis, automatic approval of benefits is not guaranteed.[disability-benefits-help.org]
Difficulty Walking
  • Symptoms - Syphilitic myelopathy (tabes dorsalis) * Abnormal sensations, often called \"lightning pains\" * Difficulty walking * Loss of coordination * Loss of reflexes * Muscle weakness * Wide-based gait (the person walks with the legs far apart) Causes[checkorphan.org]
  • The patient began to experience difficulty walking in the dark and urinary incontinence and sought other medical advice.[lksom.temple.edu]
Impaired Balance
  • Problem List: Impaired sensation Weakness Hypotonicity Impaired coordination Impaired gait Impaired balance 9. Goals for Physiotherapy: 1. Educate about sensory loss and educate on precautions to be taken 2. Strengthening of muscles 3.[de.slideshare.net]
Abdominal Pain
  • pain, etc Etymology: New Latin, literally: tabes of the back; see tabes , dorsal ' tabes dorsalis ' also found in these entries:[wordreference.com]
  • Muscular function impairment, abdominal pains, and severe gastrointestinal/excretory system crisis (sudden onset with rapid subsidence) are observed.[dovemed.com]
Foot Ulcer
  • Victims become unable to sense pain, limb position, and temperature; these sensory deficits cause loss of bladder and sometimes bowel control, severe foot ulcerations, and Charcot joints (an osteoarthritis that typically involves the knees and hip).[britannica.com]
  • Common symptoms can include: Episodes of intense pain and disturbed sensation Weakness Diminished reflexes Unsteady gait Foot slap Progressive degeneration of the joints (Charcot's joints) Foot ulcers Loss of coordination Personality changes Dementia[syphilis.emedtv.com]
  • Joint damage and foot ulcers occur and vision may be affected by optic nerve involvement. Treatment with penicillin can arrest the progress of the disease. Also known as locomotor ataxia.[medical-dictionary.thefreedictionary.com]
  • Constipation, impotence, deafness, painless foot ulcers and painless hip and knee arthritis are other features. Decreased memory, disorientation, personality changes and sometimes frank psychiatric illness can also occur.[encyclopedia.com]
Formication
  • Symptoms: Symptoms may not appear for some decades after the initial infection and include: weakness, diminished reflexes, paresthesias (shooting and burning pains, pricking sensations, and formication), hypoesthesias (abnormally diminished cutaneous,[medical-institution.com]
  • Signs and symptoms may not appear for decades after the initial infection and include weakness, diminished reflexes, paresthesias (shooting and burning pains, pricking sensations, and formication), hypoesthesias (abnormally diminished cutaneous, especially[en.wikipedia.org]
  • Results of Tabes Dorsalis • Weakness • Paresthesias (shooting and burning pains, pricking sensations and formication) • Hyposthesias (abnormally diminished cutaneous, especially tactile, sensory modalities) • Loss of coordination • Dementia • Deafness[slideshare.net]
Pupillary Abnormality
  • Pupillary abnormalities are present in almost all the cases. Pupils on two sides are unequal. Accomodation reflex is retained while light reflex is lost. Complete Argyll-Robertson pupil may develop later on.[healthdrip.com]
  • abnormalities cerebellar dysfunction Optic atrophy Pyramidal tract dysfunction Features suggesting psychiatric illness 10-20 years Lancinating pain Sensory ataxia Bowel dysfunction Bladder dysfunction Cranial nerve abnormalities Any time after infection[quizlet.com]
  • The mydriatic reaction to atropine is rather poor, and the pupil does not dilate with painful skin stimuli. [13] It must be realized, however, that pupillary abnormalities are not always of this typical pattern.[ijstd.org]
Back Pain
  • A 28-year-old man was hospitalized with lightning back pain, nausea, and bladder bowel dysfunction. Serum and cerebrospinal fluid (CSF) revealed high titers of Treponema pallidum antibody, and the serum IgA level was less than 5 mg/dl.[ncbi.nlm.nih.gov]
  • “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.[medical-institution.com]
  • 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.[en.wikipedia.org]
  • DTR are diminished or absent "Tabes dorsalgia" related back pain. "Tabetic gait" is a characteristic high- stepping gait - Tabetic Ataxia Deafness 6.[de.slideshare.net]
Leg Pain
  • The patient was treated with intravenous penicillin for 14 days, and gabapentin was started for the neuropathic leg pains.[nejm.org]
  • This form of Neurosyphilis has becoming increasingly rare with the commencement of penicillin antibiotic treatment Optic Atrophy Tertiary Syphilis: Neurosyphilis – Tabes Dorsalis signs and symptoms include: Intense back and leg pain (stabbing pain that[dovemed.com]
Neurogenic Arthropathy
  • arthropathy [Charcot's joint disease] ( 713.5 ) ICD-9-CM Volume 2 Index entries containing back-references to 094.0 : Ataxia, ataxy, ataxic 781.3 locomotor (progressive) 094.0 partial 094.0 progressive locomotor 094.0 spastic 094.0 spinal Atrophy, atrophic[icd9data.com]
  • arthropathy NEC Nonsyphilitic neurogenic spondylopathy NEC Type 1 Excludes spondylopathy (in): syringomyelia and syringobulbia ( G95.0 ) tabes dorsalis ( A52.11 ) ICD-10-CM Diagnosis Code M49 Spondylopathies in diseases classified elsewhere 2016 2017[icd10data.com]
Urinary Incontinence
  • BACKGROUND: Tabes dorsalis is a late manifestation of untreated syphilis that is characterized by ataxia, lancinating pains, and urinary incontinence.[ncbi.nlm.nih.gov]
  • Clinical findings Sharp pain, ataxia, optic-nerve degeneration leading to blindness, urinary incontinence, loss of position sense, joint degeneration (aslo known as Charcot’s joints). tabes dorsalis Tabes syphilis, tabetic neurosyphilis Neurology Slowly[medical-dictionary.thefreedictionary.com]
  • A 47-year-old man with a history of human immunodeficiency virus (HIV) infection presented with an 8-month history of severe paroxysmal shooting pains in his legs, progressive difficulty in walking, tinnitus, and urinary incontinence.[nejm.org]
  • Among the terrible features of tabes dorsalis are lancinating lightning-like pain, ataxia (wobbliness), deterioration of the nerves to the eyes (the optic nerves) leading to blindness, urinary incontinence, loss of the sense of position, and degeneration[medicinenet.com]
Overflow Incontinence
  • ., urinary retention and overflow incontinence). Depending on the duration or stage of tabes dorsalis, a patient may present with either preataxia, ataxia, or paralysis.[symptoma.com]
  • Urine overflow incontinence is very common as the bladder loses its muscular tone. Constipation, impotence, deafness, painless foot ulcers and painless hip and knee arthritis are other features.[encyclopedia.com]
Urinary Retention
  • ., urinary retention and overflow incontinence). Depending on the duration or stage of tabes dorsalis, a patient may present with either preataxia, ataxia, or paralysis.[symptoma.com]
Ataxia
  • (see also Paralysis) 344.9 Pel's crisis 094.0 Sclerosis, sclerotic posterior (spinal cord) (syphilitic) 094.0 Syphilis, syphilitic (acquired) 097.9 ataxia, locomotor (progressive) 094.0 cord, bladder 094.0 locomotor ataxia 094.0 spastic spinal paralysis[icd9data.com]
  • The most common presentation consists of loss of coordination (sensory ataxia) and sudden sharp, stabbing-like (lancinating) pain in the face, back, or lower extremities.[symptoma.com]
  • BACKGROUND: Tabes dorsalis is a late manifestation of untreated syphilis that is characterized by ataxia, lancinating pains, and urinary incontinence.[ncbi.nlm.nih.gov]
  • […] tabes dor·sa·lis \ -dȯr-ˈsa-ləs, -ˈsā-, -ˈsä- \ Definition of tabes dorsalis : a syphilitic disorder of the nervous system marked by wasting, pain, limb weakness, ataxia, and disorders of sensation and vision resulting from degeneration of the spinal[merriam-webster.com]
Abnormal Gait
  • The condition tends to present 15 to 20 years after the initial infection and is characterized by lightening-like pains in the lower extremities, Urinary incontinence ; Ataxia ; severely impaired position and vibratory sense, abnormal gait (see Gait disorders[jove.com]
  • The condition tends to present 15 to 20 years after the initial infection and is characterized by lightening-like pains in the lower extremities, URINARY INCONTINENCE; ATAXIA; severely impaired position and vibratory sense, abnormal gait (see GAIT DISORDERS[profiles.umassmed.edu]
  • The condition tends to present 15 to 20 years after the initial infection and is characterized by lightening-like pains in the lower extremities, urinary incontinence; ataxia; severely impaired position and vibratory sense, abnormal gait (see gait disorders[icd10data.com]
Hyporeflexia
  • Charcot joint, hyporeflexia, Romberg sign, are also relatively common findings, as seen in this patient. Syphilitic optic atrophy 6 is considered a separate entity that is seen in less than 10% of cases.[ncbi.nlm.nih.gov]
  • Ataxic gait and hyporeflexia are caused by purely sensory deficits due to irritation and destruction of the sensory afferents in the lumbosacral dorsal roots, affecting the incoming heavily myelinated proprioceptive fibers (later seen as demyelination[lksom.temple.edu]
Positive Romberg Sign
  • Romberg's sign. 05:36 And as you know, a positive Romberg is a measure of how the posterior columns are working. 05:45 So you have the patient stand with feet together, hands out in front, have them close their eyes, and see if they can maintain that[lecturio.com]
Broad-Based Gait
  • The patient's ataxia was evident as she walked with a broad-based gait. She was very nervous when asked to walk or stand with her eyes closed and demonstrated the Romberg sign.[lksom.temple.edu]

Workup

Workup consists of a thorough history, physical exam, and laboratory testing (serum and cerebrospinal spinal fluid analysis). The medical history should elicit information about the current or past diagnosis of syphilis, neurologic or ocular disease, and human immunodeficiency virus infection.

A neurological and ocular physical exam should be performed. Abnormalities of the pupils are common in patients with tabes dorsalis; Argyll-Robertson pupils are present in approximately half of patients [8]. Other neurologic exam findings include loss of reflexes, proprioceptive sense, sensory ataxia, and/or Charcot joints [9]. Examination of the skin may reveal trophic ulcers (mal perforans).

Serum testing will establish the diagnosis of syphilis. Commonly used tests for syphilis include:

  • Non-treponemal tests (venereal disease research laboratory -VDRL and rapid plasma reagin)
  • Treponemal tests (Fluorescent treponemal antibody absorption, micro hemagglutination assay-T pallidum, fluorescent Treponemal antibody-absorption double staining)

During early syphilis, both types of test are positive. However, during late syphilis, such as with tabes dorsalis, non-treponemal tests may be negative and treponemal tests will be positive. This testing pattern indicates that the patient had syphilis in the past and may be at risk for neurosyphilis [10] [11].

A cerebrospinal fluid (CSF) exam should be performed to establish the diagnosis of neurosyphilis. Neurosyphilis is consistent with the following CSF findings: a negative CSF-VDRL, CSF lymphocyte count >5 cells/µL, and elevated protein count (>45 mg/dL) [12]. The CSF-VDRL test may be false positive when there is contamination of the CSF with blood [13] [14]. A polymerase chain reaction (PCR) for detection of treponemal nucleic acids may also be performed. The CSF may be tested for diagnostic markers of neurosyphilis such as intrathecally produced anti-treponemal antibodies and oligoclonal bands.

Tests that aid in the diagnosis, but are not confirmatory, include computed tomography (CT) and magnetic resonance imaging (MRI) of the brain and spine showing patterns of parenchymal disease that is consistent with neurosyphilis such as ischemic lesions, frontocortical atrophy, cerebral gumma, or disseminated frontal high signal lesions in T2-weighted MRI sequences [11] [15]. Electrophysiologic testing may prove useful for ruling out neurological disorders; a triad of normal nerve conduction studies that reveal absent H-reflexes, normal nerve studies, and reduced posterior column conduction is highly indicative of tabes dorsalis. An electroencephalogram (EEG) findings may indicate complex partial or isolated status epilepticus [4] [5].

Cerebrospinal Fluid Abnormality
  • Cerebrospinal fluid abnormalities in patients with syphilis: association with clinical and laboratory features. J Infect Dis . 2004 Feb 1. 189(3):369-76. [Medline] . Holmes MD, Brant-Zawadzki MM, Simon RP.[medscape.com]

Treatment

  • Is there any treatment? Penicillin, administered intravenously, is the treatment of choice. Associated pain can be treated with opiates, valproate, or carbamazepine.[web.archive.org]
  • Abstract A man with a history of treatment for early syphilis presented with tabes dorsalis. Despite receiving a course of penicillin accepted as adequate by the World Health Organisation the illness progressed to tabo-paresis after 20 years.[ncbi.nlm.nih.gov]
  • Preventive treatment for those who come into sexual contact with an individual with tabes dorsalis is important. Treatment Penicillin, administered intravenously, is the treatment of choice.[ninds.nih.gov]
  • Abstract The demonstration of favorable results with the use of penicillin in the treatment of tabes dorsalis, shortly after the introduction of this drug in the treatment of syphilis, was of special interest, in view of the highly complex and uncertain[annals.org]

Prognosis

  • Prognosis If left untreated, tabes dorsalis can lead to paralysis, dementia, and blindness. Existing nerve damage cannot be reversed. x Prognosis If left untreated, tabes dorsalis can lead to paralysis, dementia, and blindness.[ninds.nih.gov]
  • Prognosis If left untreated, tabes dorsalis can lead to paralysis, dementia, and blindness. Existing nerve damage cannot be reversed.[brainfacts.org]
  • Frey, 2 from Schaffer's clinic, has studied 286 cases of incipient tabes, classifying these as oculo-pupillary, dysuric, lancinating and mixed forms, and studying sixty of them also from the standpoint of prognosis.[jamanetwork.com]
  • […] rule out other diseases Serum VDRL or serum RPR (used as a screening test for syphilis infection) If the serum VDRL or serum RPR test is positive, one of the following tests will be needed to confirm the diagnosis: FTA-ABS MHA-TP TP-EIA TP-PA Outlook (Prognosis[health.ridgeviewmedical.org]

Etiology

  • A few years later, Guillaume Duchenne 2 (1858), also known as Duchenne de Boulogne, made an almost complete clinical description of tabes dorsalis, which he named progressive locomotor ataxia, and made some comments about the etiology:[jamanetwork.com]
  • (Etiology) Syphilis is a contagious bacterial infection that is transmitted through sex with an affected individual.[dovemed.com]
  • Etiology Treponema bacteria (particularly during stages I /II) are highly contagious! Sexual contact with a partner who suffers from active syphilis will lead to infection in 30% of cases![amboss.com]

Epidemiology

  • Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified. Etiology Treponema bacteria (particularly during stages I /II) are highly contagious![amboss.com]
  • Epidemiology of infectious syphilis in Ottawa. Recurring themes revisited. Can J Public Health . 2008 Sep-Oct. 99(5):401-5. [Medline] . Peng RR, Wang AL, Li J, Tucker JD, Yin YP, Chen XS.[medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • References: [3] [4] Pathophysiology Clinical features Primary syphilis Primary lesion ( chancre ) Typically starts out as a solitary, raised papule (usually on the genitals) Evolves into painle ss, firm ulcer with indurated borde rs and smooth base Resolves[amboss.com]

Prevention

  • The NINDS supports and conducts research on neurodegenerative disorders, such as tabes dorsalis, in an effort to find ways to prevent, treat, and, ultimately, cure these disorders.[web.archive.org]
  • Health knowledge - a thorough and concise knowledge of the prevention, causes, and treatments of disease, simplified for home use (1920) (14595212919).jpg 734 1 534; 110 KB The glands of life - a contribution to the study of organotherapy (1914) (14593762060[commons.wikimedia.org]
  • Research The NINDS supports and conducts research on neurodegenerative disorders, such as tabes dorsalis, in an effort to find ways to prevent, treat, and, ultimately, cure these disorders. View research on this topic.[brainfacts.org]
  • Treating the infection helps prevent new nerve damage and may reduce symptoms. Treatment does not reverse existing nerve damage.[health.ridgeviewmedical.org]

References

Article

  1. Merritt HH, Adams RD, Solomon HC. Neurosyphilis, Oxford University Press, New York 1946.
  2. Conde-Sendín MA, Amela-Peris R, Aladro-Benito Y, Maroto AA. Current clinical spectrum of neurosyphilis in immunocompetent patients.Eur Neurol. 2004;52:29-35.
  3. Hooshmand H, Escobar MR, Kopf SW. Neurosyphilis. A study of 241 patients. JAMA. 1972;219:726.
  4. Ances BM, Shellhaus R, Brown MJ, Rios OV, Herman ST, French JA. Neurosyphilis and status epilepticus: case report and literature review. Epilepsy Res. 2004; 59:67-70.
  5. Vojvodic NM, Sokic DV, Jankovic SM, Delic S. Isolated episodes of status epilepticus as the manifestation of neurosyphilis: a case report. Epilepsia. 2003;44:623.
  6. Yao Y, Huang E, Xie B, Cheng Y. Neurosyphilis presenting with psychotic symptoms and status epilepticus. Neurol Sci. 2012;33:99-1
  7. Murugan S, Sanjeev B, Kaleelullah MC. Pattern of late syphilis: A decade study. Indian J Sex Transm Dis 1986;7:61-3.
  8. Moradi A, Salek S, Daniel E, et al. Clinical features and incidence rates of ocular complications in patients with ocular syphilis. Am J Ophthalmol. 2015;159:334-43.
  9. Berger JR, Dean D. Neurosyphilis. Handb Clin Neurol. 2014;121:1461-72. Neurol Sci. 2012;33:99-102.
  10. Solbrig MV, Healy JF, Jay CA. Infections of the Nervous System. A Bacterial Infection. Neurology in Clinical Practice. The Neurological Disorders. Bradley WG, Daroff RB, Gerald MF, Marsden CD (Eds). Third Edition. Butterword Heinemann. 2000;2:1317-51.
  11. Knudsen PR, Menezes MS de. Neurosyphilis. eMedicine, Neurology, Neurological infections. Editors: Pruitt AA, Talavera F, Thomas FP, Benbadis SR, Lorenzo N. Last updated: 2005;1-10.
  12. Wong T, Fonseca K, Chernesky MA, Garceau R, Levett PN, Serhir B. Canadian Public Health Laboratory Network laboratory guidelines for the diagnosis of neurosyphilis in Canada.Can J Infect Dis Med Microbiol. 2015;26 Suppl A:18A-22A.
  13. Marra CM, Maxwell CL, Smith SL, et al. Cerebrospinal fluid abnormalities in patients with syphilis: association with clinical and laboratory features. J Infect Dis. 2004;189:369.
  14. Solbrig MV, Healy JF, Jay CA. Infections of the Nervous System. A Bacterial Infection. Neurology in Clinical Practice. The Neurological Disorders. Bradley WG, Daroff RB, Gerald MF, Marsden CD (Eds). Third Edition. Butterword Heinemann. 2000;2:1317-51.
  15. Zhang HL, Lin LR, Liu GL, et al.Clinical spectrum of neurosyphilis among HIV-negative patients in the modern era. Dermatology. 2013;226:148-56.

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Last updated: 2018-06-22 10:38