Many infections and disorders that cause inflammation can cause chronic meningitis.
Having a weakened immune system increases the risk of developing chronic meningitis.
Symptoms are usually similar to those of acute bacterial meningitis (headache, fever, and stiff neck) but may also include confusion, hearing loss, and double vision.
To diagnose chronic meningitis, doctors usually do imaging of the head, such as magnetic resonance imaging (MRI), followed by a spinal tap (lumbar puncture) with analysis of the cerebrospinal fluid.
The cause is treated.
(See also Introduction to Meningitis Introduction to Meningitis Meningitis is inflammation of the layers of tissue that cover the brain and spinal cord (meninges) and of the fluid-filled space between the meninges (subarachnoid space). Meningitis can be... read more .)
The brain and spinal cord are covered by three layers of tissue called meninges. The subarachnoid space is located between the middle layer and the inner layer of the meninges, which cover the brain and spinal cord. This space contains the cerebrospinal fluid, which flows through the meninges, fills the spaces within the brain, and helps cushion the brain and spinal cord.
Tissues Covering the Brain
Within the skull, the brain is covered by three layers of tissue called the meninges.
Subacute meningitis develops over a longer period of time than acute meningitis and over a shorter period than chronic—over days to a few weeks. Its causes, symptoms, diagnosis, and treatment are similar to those of chronic meningitis. Bacterial meningitis may be subacute rather than acute.
Chronic meningitis develops slowly, over weeks or longer, and may last for months to years. Rarely, chronic meningitis causes only mild symptoms and resolves on its own.
Causes of Subacute and Chronic Meningitis
Subacute or chronic meningitis is usually caused by an infection. Many microorganisms can cause subacute or chronic meningitis. Among the most important microorganisms are
Fungi Overview of Fungal Infections Fungi are neither plants nor animals. They were once thought to be plants but are now classified as their own kingdom. Some fungi cause infections in people. Because fungal spores are often... read more , including Cryptococcus neoformans, Cryptococcus gattii, Coccidioides immitis, Histoplasma capsulatum, and Blastomyces
The bacteria that cause tuberculosis cause a rapidly progressive form of chronic meningitis (called tuberculous meningitis). Meningitis may develop when people are first infected. Or the bacteria may remain in the body in an inactive state and become reactivated later and cause meningitis. The bacteria may be reactivated when people are treated with drugs that suppress the immune system (such as tumor necrosis factor inhibitors, including infliximab, adalimumab, golimumab, certolizumab, and etanercept).
Up to 8% of children and some adults with Lyme disease develop meningitis. Meningitis due to Lyme disease may be acute or chronic. Usually, it begins more slowly than acute viral meningitis.
The most common cause of chronic meningitis in the Western hemisphere is
These fungi are more likely to cause meningitis in people who have a weakened immune system due to disorders such as human immunodeficiency virus (HIV) infection Human Immunodeficiency Virus (HIV) Infection Human immunodeficiency virus (HIV) infection is a viral infection that progressively destroys certain white blood cells and can cause acquired immunodeficiency syndrome (AIDS). HIV is transmitted... read more or AIDS or who take drugs that suppress the immune system. Symptoms of meningitis due to Cryptococcus neoformans begin gradually and subtly, and they may come and go.
Less commonly, chronic meningitis is caused by the following:
Parasites such as the protozoa Toxoplasmosis gondii (which causes toxoplasmosis Toxoplasmosis Toxoplasmosis is infection caused by the single-celled protozoan parasite Toxoplasma gondii. Infection occurs when people unknowingly ingest toxoplasma cysts from cat feces or eat contaminated... read more ), usually in people with HIV infection or AIDS
Viruses, such as HIV and enteroviruses
Chronic meningitis is common among people who have HIV infection. Meningitis can result from the HIV infection. But many other organisms (including Cryptococcus neoformans, Mycobacterium tuberculosis, and several fungi) can also cause chronic meningitis in people with HIV infection.
Disorders that are not infections can also cause chronic meningitis. They include
Certain disorders that cause inflammation, such as systemic lupus erythematosus Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic autoimmune inflammatory connective tissue disorder that can involve joints, kidneys, skin, mucous membranes, and blood vessel walls. Problems in the... read more (lupus), rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is an inflammatory arthritis in which joints, usually including those of the hands and feet, are inflamed, resulting in swelling, pain, and often destruction of joints.... read more , Behçet syndrome Behçet Disease Behçet disease is chronic blood vessel inflammation (vasculitis) that can cause painful mouth and genital sores, skin lesions, and eye problems. The joints, nervous system, and digestive tract... read more , and Sjögren syndrome Sjögren Syndrome Sjögren syndrome is a common autoimmune rheumatic disorder and is characterized by excessive dryness of the eyes, mouth, and other mucous membranes. White blood cells can infiltrate and damage... read more
Leukemia Overview of Leukemia Leukemias are cancers of white blood cells or of cells that develop into white blood cells. White blood cells develop from stem cells in the bone marrow. Sometimes the development goes awry... read more , lymphoma Lymphomas , or other cancers that spread to the meninges
A few people have developed chronic fungal meningitis after they were given methylprednisolone (a corticosteroid) as an injection into the space around the spinal cord (called an epidural injection) in the lower back (for example, to relieve sciatica). In all cases, the drug had not been prepared using sterile techniques. Symptoms include headache, confusion, nausea, and/or fever. Most people also have a stiff neck, but about one third do not. Symptoms may occur up to 6 months after the injection. If people have any of these symptoms during the weeks or months after having a corticosteroid injection in their back, they should call their doctor.
Occasionally, chronic meningitis persists for months or even years, but no organisms are identified, and death does not result. This type of meningitis is called chronic idiopathic meningitis. Treatment with antifungal drugs or corticosteroids does not help. However, some people with chronic idiopathic meningitis eventually recover without treatment.
Symptoms of Subacute and Chronic Meningitis
The symptoms of subacute or chronic meningitis are similar to those of acute bacterial meningitis, except that they develop more slowly and gradually, usually over weeks rather than days. Also, fever is often less severe. Symptoms of chronic meningitis may last for years. Some people get better for a while, then worsen (relapse).
Headache, confusion, a stiff neck, and back pain are common. People may have difficulty walking. Weakness, pins-and-needles sensations, numbness, paralysis of the face, and double vision are also common. Paralysis of the face, double vision, and hearing loss develop when meningitis affects the cranial nerves Overview of the Cranial Nerves Twelve pairs of nerves—the cranial nerves—lead directly from the brain to various parts of the head, neck, and trunk. Some of the cranial nerves are involved in the special senses (such as seeing... read more (which go directly from the brain to various parts of the head, neck, and trunk).
Meningitis due to the bacteria that cause tuberculosis usually worsens fairly rapidly (over days to weeks) but may develop much more rapidly or gradually. Tuberculous meningitis can have serious effects. Pressure within the skull may increase. Blood vessels may become inflamed, sometimes leading to stroke. Vision, hearing, facial muscles, and balance may be affected.
Diagnosis of Subacute and Chronic Meningitis
Spinal tap and analysis of cerebrospinal fluid
Doctors ask about factors that increase the risk of chronic meningitis, such as a weakened immune system (as may be caused by HIV infection or AIDS) and travel to areas where Lyme disease or certain fungal infections are common. Doctors also ask about and look for symptoms that may suggest a cause.
To confirm the diagnosis, doctors do a spinal tap Spinal Tap Diagnostic procedures may be needed to confirm a diagnosis suggested by the medical history and neurologic examination. Electroencephalography (EEG) is a simple, painless procedure in which... read more (lumbar puncture) to obtain a sample of cerebrospinal fluid, which is then analyzed.
Analysis of cerebrospinal fluid
The cerebrospinal fluid is sent to a laboratory to be examined and analyzed. The results can usually enable doctors to distinguish between chronic and acute meningitis. In chronic meningitis, the number of white blood cells in cerebrospinal fluid is higher than normal but is usually lower than that in acute bacterial meningitis. Also, the type of white cells that are most common is usually different. Some infectious organisms that cause chronic meningitis, such as the fungus Cryptococcus neoformans, are visible under a microscope, but many, such as the bacteria that cause tuberculosis, are difficult to detect.
The cerebrospinal fluid is also cultured. Organisms, if present, are grown so that they can be identified. However, culturing may take weeks. Special techniques, which may provide results more quickly, may be used to identify fungi and the bacteria that cause tuberculosis and syphilis. For example, tests may be done to detect proteins released by Cryptococcus neoformans (called antigen testing).
The polymerase chain reaction (PCR) technique, which produces many copies of a gene, may identify the unique DNA sequence of the bacteria that cause tuberculosis. Doctors may also use an automated test called Xpert MTB/RIF, recommended by the World Health Organization (WHO) for the diagnosis of tuberculous meningitis, to detect the genetic material (DNA) of tuberculosis bacteria in samples of cerebrospinal fluid. Other tests may be done on samples of cerebrospinal fluid to document prior exposure to the bacteria that cause tuberculosis. Chest x-rays or computed tomography (CT) of the chest may detect evidence of prior or current tuberculosis.
Other tests on cerebrospinal fluid are done, depending on which disorders are suspected. For example, the fluid may be analyzed for cancer cells if cancer is suspected.
The cause of chronic meningitis may be difficult to determine, partly because detecting microorganisms in cerebrospinal fluid can be difficult. Thus, spinal taps may be repeated to obtain more cerebrospinal fluid for culture. If available, tests that can rapidly analyze large stretches of genetic material can be used to identify otherwise undetectable microorganisms in cerebrospinal fluid.
To identify the cause, doctors may also need to culture samples of blood and urine or to biopsy infected meninges or other tissues, which are identified using magnetic resonance imaging (MRI) or CT. Sometimes MRI or CT is done when the cause of symptoms is unclear.
Even after extensive testing, the cause often cannot be determined.
Treatment of Subacute and Chronic Meningitis
Treatment of the cause
Doctors focus on treating the cause. Depending on the cause, the following treatments are used:
For tuberculosis, syphilis, Lyme disease, or another bacterial infection: Antibiotics effective for the particular bacteria
For fungal infections: Usually antifungal drugs, such as amphotericin B, flucytosine, fluconazole, or voriconazole, given intravenously or by mouth
For disorders that are not infections, such as sarcoidosis and Behçet syndrome: Corticosteroids or other drugs that suppress the immune system (immunosuppressants), sometimes taken for a long time
For spread of cancer to the meninges: A combination of radiation therapy directed at the head and/or chemotherapy, depending on the cancer
Chronic meningitis due to Cryptococcus neoformans is commonly treated with amphotericin B plus flucytosine or fluconazole. When a fungal infection is particularly difficult to cure, amphotericin B is sometimes injected directly into the cerebrospinal fluid through an Ommaya reservoir. The Ommaya reservoir is a device that is placed under the scalp. The reservoir contains a large supply of drug, which it delivers slowly, over days or weeks, through a small tube running from the reservoir to the spaces within the brain.
Prognosis of Subacute and Chronic Meningitis
The prognosis for people with subacute or chronic meningitis depends on
What is the cause is
In many cases, how strong the person's immune system is
Syphilis and Lyme disease usually resolve after treatment. Meningitis due to fungal or parasitic infections is harder to treat and more likely to recur, especially in people with HIV infection.
If meningitis is due to leukemia, lymphoma, or cancer, the prognosis is often poor. In such cases, meningitis can be fatal.
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