Neurosyphilis: Causes, Symptoms & Treatment

Neurosyphilis is a syndrome that can develop as a late consequence of syphilis infection. It manifests as psychiatric and neurologic deficits. Neurosyphilis is also called neurolues or quaternary syphilis (fourth-stage syphilis).

What is neurosyphilis?

Neurosyphilis can develop when untreated or incompletely cured syphilis disease is far advanced. The disease then spreads to the central nervous system. Decades can pass between a syphilis infection and the outbreak of neurosyphilis. Accordingly, the age of onset is usually in middle to advanced age. Men are more frequently affected than women. If syphilis disease is treated thoroughly and in a timely manner, there is ample time to prevent the outbreak of neurosyphilis.

Causes

Neurosyphilis develops at an advanced stage of infection with syphilis. This is a sexually transmitted disease caused by the bacterium Treponema pallidum. Infection of the unborn child by an infected mother is also possible. Neurosyphilis develops in about 10-20% of all syphilis sufferers. While neurosyphilis was one of the most common reasons for psychiatric hospitalization in earlier centuries, it has now become rare in industrialized countries thanks to good treatment options and better diagnostics. In countries where adequate medical care and thus timely diagnosis and treatment of syphilis are not yet available, neurosyphilis continues to be a frequent complication.

Symptoms, complaints, and signs

In the early stages of neurosyphilis, symptoms of irritation of the meninges are most common. These are manifested in particular by severe headaches as well as paralysis of the cranial nerves. Depending on the cranial nerve affected, these pareses can cause different symptoms. For example, paralysis of the olfactory nerve leads to olfactory disorders, while paresis of the optic nerve results in visual disorders. Early neurosyphilis can also be manifested by the so-called polyradicular syndrome. Typical symptoms of this nerve root irritation are insensations such as tingling, pain or numbness in the area of the affected nerve root. Rarely, inflammation of the meninges (meningitis) with neck stiffness and exceptionally severe headache is seen in early neurosyphilis. In late neurosyphilis, which occurs in only one-tenth of patients with syphilis, inflammation of blood vessels of the meninges, brain, and spinal cord is common. The areas that are normally supplied by these arteries then show an undersupply of oxygen and nutrients. This results in symptoms such as full- or hemiplegia, cranial nerve deficits, sensory disturbances and seizures. In the course of the disease, the clinical picture tabes dorsalis may further develop. Spinal dorsalis is characterized primarily by a disturbance of vibratory sensation and disturbances of positional and motor sense.

Diagnosis and course

When neurosyphilis is suspected, the underlying syphilis is first detected by blood test. Neurosyphilis can be diagnosed by examination of the cerebrospinal fluid. This involves puncturing the spinal cord and taking a sample. Certain antibodies and elevated protein levels can be detected in the nerve fluid, which allow conclusions to be drawn about neurosyphilis. In the outbreak of neurosyphilis, there is initially an inflammation of the meninges, which manifests itself in visual disturbances – typical here is seeing double images. After a longer period of time, this so-called meningovascular stage can be followed by the parenchymatous stage, in which the tissue of the brain perishes. This manifests itself in headaches, sleep disorders, paralysis of the extremities, changes of character, delusions, memory disorders, hallucinations and seizures. Patients lose their mental abilities, appear severely confused and disoriented. Some of the symptoms resemble age-related dementia. While the transition from the meningovascular stage to the parenchymal stage often takes many years, the decline of brain tissue and thus the mental deterioration of the patient progresses rapidly.The final step is the so-called tabes dorsalis, in which the nerve sheaths, nerve roots and nerve nodes are destroyed. This leads to incontinence and insensitivity to pain and temperature stimuli, so that patients sometimes fail to notice infected wounds or accidentally scald themselves. In addition, there are gait disturbances and a loss of reflexes up to complete paralysis.

Complications

Neurosyphilis, if left untreated, always leads to late sequelae that can be fatal because of complications that develop. Because neurosyphilis is already a complication of syphilis, it is the latest to require special treatment. Complications associated with the destroyed nerve and brain tissue result, for example, from sensory disturbances. Affected persons may not notice wounds and inflammations, which can lead to tissue death. Hypersensitivity may also occur. Many everyday things can no longer be performed by those affected. The altered personality structure in advancing neurosyphilis causes social alienation. Thus, affected persons may often feel misunderstood and become aggressive towards relatives. This can lead to complete isolation, which is especially stressful for those affected if they become a care recipient at the same time. As the paralysis of the nerves progresses, those affected soon become a nursing case with all the associated complications (risk of bedsores, loneliness, malnutrition). This is mostly due to the advancing inflammation in the brain or meninges. This is referred to as chronic progressive meningitis or encephalitis. The development of tabes dorsalis (spinal cord atrophy) leads to severe motor limitations and thus to an increased risk of accidents due to falls or incorrect use of objects.

When should you see a doctor?

If the typical nodules are noticed in the intimate area, the gynecologist or urologist should be consulted. Growths with sunken centers indicate advanced neurosyphilis, which must be clarified and treated immediately. Medical advice is required at the latest when further symptoms such as fever or joint pain are added to the skin changes. The disease occurs in phases and can go unnoticed for weeks or months. This makes it all the more important to seek medical advice after unprotected sexual intercourse or contact with a possibly infected person. If unusual physical complaints are noticed in connection with the above triggers that cannot be attributed to any other cause, it is best to contact the doctor. Mothers who notice typical signs of syphilis after child birth should seek medical attention. So should people who notice corresponding symptoms after a blood transfusion and people who have already been diagnosed with STD once. People with hormonal symptoms or immunodeficiency talk to their general practitioner if neurosyphilis is suspected. In addition to the general practitioner, the gynecologist, the urologist or an internist may be consulted. The dermatologist may also be involved in the treatment. During treatment, close consultation with the physician is important so that the therapy can be adapted to the individual course of the disease.

Treatment and therapy

It is possible to treat syphilis disease even after it has progressed to the neurosyphilis stage. Penicillin is primarily used for this purpose. However, nerve tissue of the brain and spinal cord destroyed by neurosyphilis can no longer regenerate. However, causative treatment of syphilis infection can prevent further neuronal cell decline.

Outlook and prognosis

Neurosyphilis occurs as a late consequence of syphilis. If left untreated, the disease leads to premature death of the affected person. The affected person should consult a physician as soon as possible for relief of symptoms and a good prognosis so that necessary treatment steps can be initiated. In a drug therapy, the pathogens are prevented from spreading. At the same time, they are killed by the active substances contained in the preparations and subsequently removed from the organism.There is a gradual improvement in the general state of health. Complete recovery is also possible in the further course. The prerequisite for this is that no irreversible damage has occurred. If damage to the nerve tissue is detected in the affected person, the prognosis worsens. In these cases, long-term consequences occur because the spinal cord as well as the brain can no longer be regenerated. The goal of treatment is then the regular care of the individually occurring complaints and an improvement of the quality of life. Recovery is no longer achieved in this course of the disease despite the application of the best possible therapy methods. Nevertheless, a spread and thus a progression of the syphilis disease can be prevented. The life of the affected person is no longer endangered by the administration of the medication. The current health condition can be emotionally stressful. Therefore, there is an increased risk of developing a secondary mental disorder.

Prevention

Neurosyphilis is a complication of syphilis infection. So the best way of prevention is to prevent syphilis infection. Since this is a sexually transmitted disease, responsible sexual behavior is needed for this purpose. The use of condoms not only protects against infection with syphilis, but also against other serious diseases. It should be noted, however, that syphilis – unlike HIV, for example – can also be transmitted through oral sex and other sexual contacts. To prevent neurosyphilis, it is essential to treat an existing syphilis disease quickly and consistently. In this way, serious secondary diseases such as neurosyphilis can be avoided.

Follow-up

During follow-up, patients continue to be prescribed a medication such as penicillin to ensure that the syphilis is cured. After therapy, regular checkups are important. Physical examinations, regular patient interviews and laboratory tests ensure that the disease is completely cured or that complications are detected quickly. In particular, the cerebrospinal fluid is checked. The patient’s state of health can be precisely determined on the basis of the CSF findings and the serological tests. If the values are inconspicuous after 24 months, this indicates a cure. If neurosyphilis recurs, the necessary treatment measures can be initiated immediately. The STP Working Group recommends control examinations every three, six and twelve months after therapy. A prerequisite for extending the intervals is at least a fourfold drop in titer. Otherwise, treatment may have to be restarted. With successful therapy, the symptoms heal without further complications. Within nine to twelve months, the patient is symptom-free. Follow-up care can be terminated after two years, provided that no abnormalities were detected in the CSF examination. Follow-up measures are always based on the individual course of the disease, the patient’s constitution, and a whole range of other factors.

What you can do yourself

Neurosyphilis as a serious complication of untreated or even resistant syphilis does not allow any self-help options that come close to therapy. It is not possible to treat the bacterial pathogen of syphilis by home remedies, alternative therapies, or other remedies offered. The only possible cure is the use of strong antibiotics. Meanwhile, based on the current state of medical research, unapproved medications and substances that are not prescribed by licensed physicians are strongly discouraged. There are, however, ways in which the sufferer can better survive the symptoms. For example, massage, hot baths, or cooling can help with pain that occurs as a result of nerve damage or paralysis. This varies from person to person. However, it is only temporary relief of these symptoms. Meanwhile, other self-help measures depend on the symptoms caused. Especially in the case of the psychological symptoms associated with neurosyphilis, self-help measures are hardly available. Damage to the nervous system caused by neurolues can be partially compensated for by those affected through targeted training.Affected persons with neurosyphilis who have undergone drug therapy can therefore possibly make use of various exercise therapies and cognitive training. The type and extent should be determined with the treating physician.