What are the other Names for this Condition? (Also known as/Synonyms)
- Central Nervous System (CNS) Syphilis – Optic Atrophy Neurosyphilis
- Ocular Syphilis
- Symptomatic Neurosyphilis – Optic Atrophy
What is Optic Atrophy due to Neurosyphilis? (Definition/Background Information)
- Syphilis is a very common infectious disease caused by the bacterium Treponema pallidum. Syphilis is generally transmitted through sexual contact. A pregnant woman affected by Syphilis can transmit the infection to her child through the placenta, while the baby is still in the womb
- The disease leads to formation of lesions on the body, especially around the anus, genitalia, and mouth. Any contact with these ulcerative lesions result in a transmission of the condition
- There are 3 stages in the progression of Syphilis; Primary, Secondary, and Tertiary. The sooner the treatment is started, better is the outcome. If treatment is delayed or is lacking; it could develop into a serious medical issue with long-term neurological complications. In rare cases, the complications can even lead to death
- The universal treatment for this condition (at all stages) is by using the drug penicillin, which can bring about a complete recovery. However, early recognition and management of the condition is extremely important
Primary stage: After the initial infection (direct sexual contact with affected individual), the bacterium spreads all over the body using the blood vessels and lymphatic system, and multiply rapidly. Primary lesions form within a few hours after infection, which contain large quantities of the Treponema bacteria.
Secondary stage: The bacterium invades tissues, cerebrospinal fluid, skin, and mucus membranes. It causes inflammatory rashes on the body (secondary lesions), within 4-12 weeks after the primary stage. This continues till a stage of dormancy is reached; after which multiple relapse or remissions take place (for the next 1-2 years).
Tertiary stage: After the secondary stage, there is a period of latency, which could last for many years. A third of all infections, then gradually progresses to Tertiary Syphilis. In this stage, the central nervous system is affected and several vital organs (heart, liver) may develop infections. This may take place after 3 years or sometimes even after 20 years. Tertiary Syphilis is classified into three key types, principally based on the infestation location. These are: Neurosyphilis, Cardiovascular Syphilis, and Gummatous Syphilis
Neurosyphilis occurs when the infection affects the central nervous system. It is grouped into six principal types
- Neurosyphilis occurs when the infection affects the central nervous system. It is grouped into six principal types; Neurosyphilis - Optic Atrophy is one among them
- Optic Atrophy Neurosyphilis (also known as Ocular Syphilis) can cause vision disruption and may even result in complete blindness, if the disease is left untreated
Who gets Optic Atrophy due to Neurosyphilis? (Age and Sex Distribution)
- Sexually mature men and women who are infected by Treponema bacteria contract Syphilis. Occasionally, even teenagers (as young as 15 years) are affected by this infectious condition
- No gender preference or racial predilection is observed. However, according to a CDC report (CDC.gov release, 2006) the male-female incidence for Syphilis - Primary & Secondary, stands at 6:1. The male-female ratio for an untreated Syphilis developing to Tertiary Syphilis is around 2:1
- In certain geographical regions with lower socio-economic standards and insufficient access to proper healthcare (such as central African regions); it is found that the general incidence of sexually transmitted diseases (STDs), including Syphilis is higher
What are the Risk Factors for Optic Atrophy due to Neurosyphilis? (Predisposing Factors)
Neurosyphilis is the progressive worsening of Syphilis. Hence, the risk factors for Syphilis are mentioned. These include:
- Having a new sex partner (a carrier of Syphilis)
- Multiple sex partners
- Not practicing safe sex
- Having sex with individuals who have STDs
- Having a same-sex partner(s). The incidence of Syphilis is extremely high among men (according to a CDC study, up to 72% of all new cases in 2011, were owing to this single factor)
- Individuals suffering from HIV or AIDS have a high susceptibility to the syphilitic disease. In such individuals there is a quick advancement to the Neurosyphilis stage
- Increased use of drugs (cocaine, methamphetamine, Viagra); leading to high-risk behavior, such as ‘sex for drugs’
- Untreated pregnant women with syphilis, have a very high risk (90% chance) of passing on the infectious bacteria to the baby in the womb. This causes Congenital Syphilis
- Transfusion of contaminated blood or blood products (contaminated by the syphilitic bacteria)
It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.
Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.
What are the Causes of Optic Atrophy due to Neurosyphilis? (Etiology)
- Syphilis is a contagious bacterial infection that is transmitted through sex with an affected individual. It is caused by the bacterium Treponema pallidum
- An individual’s skin lesions and mucus membranes carry the pathogen, which then spreads to their partner(s) during all forms of sexual contact (even through kissing), causing an infection. It infects the mouth, anus, and genitals of males and females
- Syphilis has three distinct stages of progression; each stage more aggressive than the previous one: Primary, Secondary, and Tertiary
What are the Signs and Symptoms Optic Atrophy due to Neurosyphilis?
Each stage of Syphilis presents with a different set of signs and symptoms. The progression to higher stages occurs because of non-treatment. After the secondary stage, there is a dormant (asymptomatic) stage, known as Latent Syphilis which may last for many years (even decades). All infections do not progress to the final (tertiary) stage.
Tertiary stage: About 30% of the untreated Syphilis cases move into this final non-infectious state, also known as Late Syphilis. The central nervous system is affected, several vital organs (such as the heart and liver) may develop infections, and nerve cells are permanently destroyed. This occurs between 3-15 years, sometimes even two decades after the infection was first contracted.
Tertiary Syphilis is classified into three key types, based principally on the infestation location. These are not mutually exclusive; hence, they can all be present together. However, some forms may be more dominant than the other:
- Neurosyphilis: Severe infection of the central nervous system; it is divided into 6 categories based on the presentation and progression of the disease
- Cardiovascular Syphilis: Causing aortic aneurysms and regurgitation at aortic valve
- Gummatous (late benign) Syphilis: Characterized by the presence of soft tumor-like gummas
Neurosyphilis: Based on whether there is a participation of the tissue and organ (parenchyma) of the central nervous system (termed late Neurosyphilis); or whether it is confined to the membranes/meninges surrounding the nervous system alone (termed early Neurosyphilis), there are 6 principal types:
- Asymptomatic Neurosyphilis
- Meningeal Neurosyphilis (Acute Syphilitic Meningitis)
- Meningovascular Neurosyphilis
- General Paresis of the Insane
- Tabes Dorsalis
- Optic Atrophy: This is a relatively new form of Neurosyphilis that is marked by the loss of optic nerve endings, causing disrupted vision and light sensitivity. However, it shows better improvement with penicillin antibiotic treatment, than other late Neurosyphilis types
Tertiary Syphilis: Neurosyphilis – Optic Atrophy signs and symptoms include:
- Disorder of the eye pupils (known as Argyll Robertson pupils)
- Fixed pupils (when iris sticks to the lens)
- Dimmed vision
How is Optic Atrophy due to Neurosyphilis Diagnosed?
Diagnosis of Syphilis may be made at any stage of the disease. A few diagnostic tests performed for Syphilis include the following:
- Physical exam with evaluation of medical history
- Exam of cerebrospinal fluid, via a lumbar puncture
- CT scan of cranium
- Cerebral angiogram
- MRI scan of brain, spinal cord
- Blood test, for detecting antibodies against Syphilis
- Ophthalmological evaluation
Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.
What are the possible Complications of Optic Atrophy due to Neurosyphilis?
Complications due to Neurosyphilis are mainly due to the formation of destructive tumors, resulting in neurological abnormalities, which can cause life-threatening situations. Optic Atrophy may cause severely impaired vision or even complete blindness.
How is Optic Atrophy due to Neurosyphilis Treated?
Prompt, early diagnosis and appropriate treatment is the key to speedy recovery from Syphilis. However, with Syphilis – Tertiary, the bacterial infection has developed to a late stage (over many years) from which complete recovery with 100% return of normal body and brain function may not be possible. But, further advancement of Syphilis can be arrested, and fatalities avoided. The treatment measures include:
- All manifestations of syphilis are treated using the antibiotic drug penicillin. This is given as an intramuscular injection (for a couple of weeks), or intravenously. Worldwide, penicillin is a cheap and easily available drug that is also simple to administer
- If the individual is allergic to penicillin, then it is given in very small dosages and in a controlled manner, until the body gets sensitized (accustomed) to the drug
How can Optic Atrophy due to Neurosyphilis be Prevented?
- Neurosyphilis is a severe and advanced (Tertiary) stage of Syphilis. Syphilis is classified as a venereal disease (VD) transmitted from one individual to another. This occurs during sexual contact with the infected lesions. It is a preventable and curable disease
The onus is on all sexually mature individuals to ensure that they are generally well-informed (either by their healthcare providers or public health authorities) of the sexually transmitted syphilitic infection. They have to be also aware of the preventive measures and precautions to be taken, which include:
- Avoidance of multiple sex partners
- Practice of safe sex (use of condoms that prevent entry of the syphilis bacteria)
- Excessive craving for drugs and alcohol, may lead to unsafe practices, such as ‘sex for drugs’
- If you are frequently sexually active with multiple partners; then regular screening for sexually transmitted diseases is essential
What is the Prognosis of Optic Atrophy due to Neurosyphilis? (Outcomes/Resolutions)
- In many cases, Syphilis – Tertiary (stage) can be completely treated; if an early diagnosis and proper treatment is rendered. Nevertheless, on account of late management of the condition; damage to the central nervous system, bones, heart, lungs, and GI system may be permanent. The extent of the damage depends on many factors and other underlying disorders (if any present)
- The condition of infected individuals without any treatment will progressively worsen with the final/tertiary stage. In such cases the outcome is poor. Death may result from respiratory, neurological, cardiovascular, or liver complications
- The outcomes are poorer in individuals diagnosed with AIDS/HIV infection, or other opportunistic infections
Additional and Relevant Useful Information for Optic Atrophy due to Neurosyphilis:
- Given the fact that there may be many years of disease-free (latent) period; it is particularly important that regular screening and blood tests for Neurosyphilis are undertaken