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Tabes Dorsalis

Disease Details

Family Health Simplified

Description
Tabes dorsalis is a late manifestation of untreated syphilis, which results in the degeneration of the dorsal columns of the spinal cord, leading to neurological impairments such as unsteady gait, sharp pain, and sensory disturbances.
Type
Tabes dorsalis is a type of late neurosyphilis. It is not genetically transmitted; rather, it results from a chronic infection by the bacterium *Treponema pallidum*, the causative agent of syphilis, which is sexually transmitted.
Signs And Symptoms
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 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, 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 eye pain, tearing of the eyes and sensitivity to light."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. In daylight the person can avoid some unsteadiness by watching their own feet.
Prognosis
Left untreated, tabes dorsalis can lead to paralysis, dementia, and blindness. Existing nerve damage cannot be reversed.
Onset
The onset of tabes dorsalis typically occurs years to decades after the initial infection with Treponema pallidum, the bacterium responsible for syphilis. It primarily affects individuals in the late stages of untreated syphilis.
Prevalence
Tabes dorsalis is a rare late manifestation of untreated syphilis, specifically neurosyphilis. Due to both the advent of antibiotics and aggressive public health measures for the treatment of syphilis, its prevalence is now extremely low in developed countries. Precise prevalence data are limited, but it is generally considered rare.
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.
Intractability
Tabes dorsalis is generally considered intractable in its advanced stages. Early detection and treatment of the underlying cause, notably syphilis, with antibiotics like penicillin can halt its progression. However, once significant neurological damage has occurred, reversing the damage is typically not possible.
Disease Severity
Tabes dorsalis is a late manifestation of untreated syphilis.

For disease severity:
- **Advanced:** Symptoms can be severe and include progressive degeneration of the sensory neurons leading to ataxia (loss of coordination), intense pain, deteriorating reflexes, and neurological deficits. These symptoms can significantly impair daily functioning and quality of life.

For "nan" (Not a Number) or if you meant "not applicable," please clarify as it is not a standard term used for discussing disease characteristics. If you meant "not available," specific disease severity can vary by individual case and progression of the disease.
Healthcare Professionals
Disease Ontology ID - DOID:10027
Pathophysiology
Tabes dorsalis is a late manifestation of untreated syphilis, typically occurring years to decades after the initial infection. The pathophysiology involves the demyelination and degeneration of the dorsal columns and dorsal roots of the spinal cord, primarily due to Treponema pallidum spirochetes. This results in a loss of proprioception, vibration sense, and fine touch. Additionally, patients may experience severe lancinating pains, ataxia, Argyll Robertson pupils, bladder dysfunction, and areflexia. The progression of this condition reflects extensive syphilitic damage to the nervous system.
Carrier Status
For tabes dorsalis, there is no carrier status because it is not a genetic condition. Tabes dorsalis is a late manifestation of untreated syphilis, specifically tertiary syphilis, affecting the spinal cord and peripheral nerves.
Mechanism
Tabes dorsalis is a late manifestation of untreated syphilis, specifically the neurosyphilis form. It primarily affects the dorsal columns and dorsal roots of the spinal cord.

**Mechanism:**
Tabes dorsalis arises from chronic infection with the bacterium *Treponema pallidum*. The infection leads to the progressive degeneration of the dorsal columns and dorsal roots, which are responsible for carrying sensory information such as vibration, proprioception, and fine touch to the brain. This degeneration results in severe neurological deficits.

**Molecular Mechanisms:**
1. **Direct Bacterial Invasion:** *Treponema pallidum* crosses the blood-brain barrier and invades the central nervous system (CNS), leading to inflammation and eventual destruction of nervous tissue.
2. **Immune Response:** The body's immune response to the infection results in chronic inflammation and auto-immune-like mechanisms targeting neuronal components, especially in the dorsal columns.
3. **Demylination:** Chronic inflammation and direct bacterial damage lead to demyelination of the neurons in the affected areas, disrupting normal signal transmission.
4. **Neuronal Death:** Persistent infection and inflammation cause neuronal death and degeneration in the dorsal roots and columns.

Symptoms of tabes dorsalis include progressive sensory ataxia, severe pain (often described as "lightning pains"), loss of reflexes, and eventual neurogenic issues such as bladder dysfunction and sexual dysfunction. If left untreated, it can lead to significant disability.
Treatment
Intravenously administered penicillin is the treatment of choice. Associated pain can be treated with opiates, valproate, or carbamazepine. 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.
Compassionate Use Treatment
Tabes dorsalis is a late neurological manifestation of untreated syphilis, leading to progressive degeneration of the spinal cord. The primary treatment focuses on eliminating the underlying Treponema pallidum infection with penicillin, the standard care. However, for advanced cases, compassionate use or experimental treatments might be considered to manage symptoms and complications.

1. **Compassionate Use Treatment:**
- **Proprietary Neurotrophic Factors:** Used under compassionate care to promote nerve health and repair, but their efficacy is not well-established.
- **Novel Antibiotics:** In rare cases, where patients have severe penicillin allergy or resistance issues, other antibiotics like ceftriaxone may be explored, though options remain limited.

2. **Off-Label or Experimental Treatments:**
- **Intravenous Immunoglobulins (IVIG):** Sometimes used experimentally to modulate immune response and reduce inflammation, though evidence in tabes dorsalis is limited.
- **Stem Cell Therapy:** An emerging area of research, leveraging the potential of stem cells to regenerate damaged neural tissues.
- **Gene Therapy:** Although still in very early stages, this aims to address underlying genetic susceptibilities or damage.
- **Neuroprotective Agents:** Drugs such as riluzole, used off-label to protect neurons from degeneration, are under investigation.

These treatments should be pursued only under strict medical supervision and typically within clinical trials or special programs due to their experimental nature.
Lifestyle Recommendations
Tabes dorsalis is a late manifestation of untreated syphilis, specifically affecting the spinal cord and peripheral nervous system. Given its nature, lifestyle recommendations for individuals with tabes dorsalis primarily focus on managing symptoms and preventing complications:

1. **Regular Medical Follow-up**: Continuous monitoring by a healthcare provider to manage symptoms and any underlying infections.
2. **Medications**: Adherence to prescribed antibiotic treatment for syphilis and medications to manage pain and neurological symptoms.
3. **Physical Therapy**: Engagement in physical therapy to maintain mobility, strength, and balance.
4. **Assistive Devices**: Use of canes, walkers, or braces to aid in walking and prevent falls, if necessary.
5. **Healthy Diet**: Maintaining a balanced diet to support overall health and potentially aid in healing.
6. **Avoiding Alcohol and Drugs**: Not consuming substances that could impair neurological function or overall health.
7. **Mental Health Support**: Seeking psychological support to cope with any cognitive or emotional challenges associated with the condition.

Preventing syphilis through safe sexual practices and regular health screenings plays a critical role in averting tabes dorsalis.
Medication
Tabes dorsalis is a late form of neurosyphilis, which results from untreated syphilis infection. The primary treatment involves:

1. **Antibiotics**: The cornerstone of treatment for tabes dorsalis is penicillin. Intravenous penicillin G is typically administered for an extended period, often 10-14 days.

2. **Symptomatic Treatment**: Pain may be managed with analgesics, and physical therapy can help with coordination and mobility issues.

It is important for patients to receive proper follow-up care to monitor the effectiveness of treatment and manage any residual symptoms. Prompt treatment of syphilis can prevent the progression to tabes dorsalis.
Repurposable Drugs
Tabes dorsalis is a late manifestation of neurosyphilis, characterized by the degeneration of the dorsal columns of the spinal cord, leading to sensory ataxia and lancinating pains.

**Repurposable Drugs:**
1. **Penicillin G** - This antibiotic is the first-line treatment for neurosyphilis, including tabes dorsalis. While not a repurposable drug in the conventional sense, it is critical here due to its efficacy in treating the underlying syphilitic infection.
2. **Gabapentin** - Traditionally used for neuropathic pain, gabapentin can help manage the severe shooting pains associated with tabes dorsalis.
3. **Baclofen** - Though primarily used for spasticity in conditions like multiple sclerosis, baclofen can aid in managing muscle spasms and discomfort in tabes dorsalis.

These are the key repurposable drugs that might be relevant for managing symptoms but treating the underlying condition primarily involves appropriate antibiotic therapy.
Metabolites
Tabes dorsalis is a late manifestation of neurosyphilis, primarily affecting the spinal cord. There are no specific metabolites uniquely associated with tabes dorsalis. Instead, it is diagnosed based on clinical features, serological tests for syphilis (e.g., VDRL, RPR), and the presence of symptoms like ataxia, sharp pains, and impaired reflexes. Metabolic profiling does not play a significant role in the diagnosis or management of tabes dorsalis.
Nutraceuticals
As of now, there is no established evidence supporting the use of nutraceuticals in the treatment of tabes dorsalis. Tabes dorsalis is a late manifestation of neurosyphilis, and the primary treatment involves antibiotic therapy, particularly with penicillin, to eradicate the underlying Treponema pallidum infection. Management may also include supportive therapies for symptom relief. If you are considering alternative or adjunct treatments, consulting with a healthcare professional is essential.
Peptides
Tabes dorsalis is a late form of neurosyphilis, caused by the bacterium Treponema pallidum. It affects the dorsal columns and dorsal roots of the spinal cord. There are no specific peptides or nanotechnology treatments currently approved for tabes dorsalis. Management typically involves addressing the underlying syphilitic infection, primarily through antibiotic therapy such as penicillin, and symptomatic relief for neurological deficits. Advanced research may explore novel approaches, but standard treatments remain focused on antimicrobial therapy and supportive care.