FAQ

1) What is recurrent viral meningitis?

Recurrent viral meningitis is meningitis that occurs more than once due to a viral infection. The current known causes of recurrent viral meningitis are herpes simplex virus type 1 (HSV-1), herpes simplex virus type 2 (HSV-2) and epstein-barr virus (HHV-4).

Definition according to the US Genetic and Rare Diseases Information Center for recurrent viral meningitis (Mollaret’s meningitis): “Mollaret meningitis is a rare type of viral meningitis that is characterized by repeated episodes of fever, stiff neck (meningismus), muscle aches, and severe headaches separated by weeks or months of no symptoms. About half of affected individuals may also experience long-term abnormalities of the nervous system that come and go, such as seizures, double vision, abnormal reflexes, some paralysis of a cranial nerve(paresis), hallucinations, or coma. Mollaret meningitis is poorly understood and the exact cause remains unknown. However, recent data suggests that herpes simplex virus (HSV-2 and, less frequently, HSV-1) may cause some, if not most cases. Other causes may include trauma and viral infections other than herpes simplex. There is no specific treatment, so management involves supportive measures such as rest, fluids, and medicine to relieve the fever or headache.”

2) What are the symptoms of recurrent viral meningitis?

Symptoms of a full-blown recurrent viral meningitis infection include:

• severe headaches

• fever (only seen in about half of cases)

• nausea

• vomiting

• sensitivity to light and/or sound

• malaise

• neck rigidity

Rarer symptoms include:

• tachycardia

• double vision

• hallucinations

Because of the similarity in symptoms to benign migraine headache, sufferers of recurrent viral meningitis have often been misdiagnosed by medical personnel. Without proper care, symptoms can escalate to seizures and even coma. Thus, a greater understanding and knowledge of this disease will facilitate better patient care in emergency situations.

Periods of severe illness usually last 3-4 days, separated by weeks or months between recurrences. However, many sufferers experience milder relapses, which although debilitating, do not necessitate hospitalization.

Unfortunately, along with each recurrence, whether mild or severe, comes the risk of further disability. Nearly half of those with the disease experience long-term negative effects of the nervous system. Permanent disabilities that can develop over time include:

• memory loss

• difficulty retaining information

• lack of concentration

• abnormal reflexes

• clumsiness

• coordination problems

• loss of balance

• residual headaches

• hearing problems

• tinnitus

• speech problems

• dizziness

• learning difficulties (ranging from temporary learning deficiencies all the way to long term mental impairment)

• tachycardia

• epilepsy

• seizures

• paralysis

• muscle spasms

• cerebral palsy

• loss of sight

• changes in sight

3) How is recurrent viral meningitis diagnosed?

Recurrent viral meningitis can be diagnosed through lumbar puncture (spinal tap) in order to examine the cerebrospinal fluid (the clear, colorless liquid that fills and surrounds the brain and the spinal cord and provides a mechanical barrier against shock). Diagnosis is made by detecting Herpes simplex virus DNA in the cerebrospinal fluid. Early diagnosis may prevent prolonged hospital admissions, unnecessary investigations, and exposure to unnecessary medications, along with their associated costs. If a patient has had a recent head injury or a problem with their immune system, they may not qualify for a lumbar puncture because of a possible fatal brain herniation; therefore, a CT or MRI scan may be performed prior to any lumbar puncture. All of the different types of meningitis (viral, bacterial, fungal and parasitic) are diagnosed by the growing presence of bacteria in the spinal fluid, a sample of which is collected with the help of a lumbar puncture. The fluid will reveal if the CSF (cerebral spinal fluid) has raised or lowered white blood cell production.

There are no vaccines currently available to prevent the development of viral meningitis. Many people who have a rare disease understandably want to find healthcare professionals or researchers with knowledge of their condition. However, with a condition as rare as recurrent viral meningitis, it can be extremely difficult to find medical professionals who have treated more than one or two cases.

4) How is recurrent viral meningitis treated?

Currently, Acyclovir is the treatment of choice for recurrent viral meningitis. Acyclovir has proven helpful in many cases, particularly when administered intravenously, where it can cross the blood/brain barrier. It is often the immediate treatment of choice for recurrences that require hospitalization. Some have also seen a reduction in recurrences from daily oral administration of Acyclovir, but it hasn’t been shown to definitively alter the recurrence of viral meningitis.

Valcyclovir is another medication similar to Acyclovir that has also been found helpful. Other common medications also administered for the subsequent pain during recurrences include:

• NSAIDS (Motrin, Advil)

• Hydrocodone (Norco, Vicodin Lortab)

• morphine

• hydromorphone (Dilaudid).

Anti-seizure medications:

• Gabapentin (Neurontin)

• Pregabalin (Lyrica)

• Lamotrigine (Lamictal)

With recurrent viral meningitis, it is difficult to measure the effectiveness of any drug therapy because the very nature of the disease is spontaneous and recurrent. The rarity of the disease also makes it difficult to create solid documentation of clinical trials studying the effectiveness of different antiviral drugs. However, there are some medications known to be contraindicated for the treatment of recurrent viral meningitis, those include:

• phenylbutazonum

• steroids

• antihistamines

• colchicine

• estrogen.

For those that have obtained an allergy to Acyclovir, and other antivirals, Lysine seems to be effective at helping to minimize symptoms according to patients.

1) What is a lumbar puncture?

Lumbar puncture is a diagnostic procedure, also known as a spinal tap, where a doctor samples fluid from the spinal column without putting any medication in.

Lumbar puncture is different from what is commonly known as an epidural, where an anesthesia doctor puts medication into the spine for child birth or surgical anesthesia.

2) What are the indications for a lumbar puncture?

To make a diagnosis of a potentially life threatening neurologic or infectious disease. The only way to perform analysis safely of the spinal fluid is by lumbar puncture in that case. It’s routinely required to perform a lumbar puncture, especially in cases of suspected infection of the brain or spinal cord known as meningitis or encephalitis.

3) What is the difference between Meningitis and Encephalitis?

Meningitis is an infection of the lining of the spine and the brain, whereas encephalitis is infection of the brain itself. Both can be diagnosed by lumbar puncture.

4) What is involved in a lumbar puncture procedure?

A needle enters the lower back through the skin, and soft tissue, and then the needle enters the spinal column to sample out the spinal fluid.

5) Is the lumbar puncture procedure safe?

The lumbar puncture procedure is very safe and has very few potential side effects.

6) What are the risks of lumbar puncture?

The main risk of lumbar puncture is pain at the site. The pain at the site can be minimized using numbing medicine, such as lidocaine which often stings itself.

Another relatively common effect of lumbar puncture, with sample removal, is a post lumbar puncture headache where the patient up to five days out from a lumbar puncture can experience a certain type of headache characterized by pain at the top of the head, or back, that is positional. It is better lying down and worse when seated up, or standing, or walking around. That is reported in up to 30% of patients undergoing lumbar puncture and is usually a treatable complication with either medication or another procedure called a blood patch where the patient’s own blood is put into the lower back to treat the post lumbar puncture headache.

Other very rare complication of a lumbar puncture include a formation of a collection of blood or hematoma inside the spinal column, which can be diagnosed and treated. It is greatly reduced by being aware of patients bleeding tendencies and also medications that might be able to be held for the lumbar puncture that could increase the risk of bleeding during the procedure or afterwards.

**You will need to speak with your doctor about the risks and benefits of the procedure in your specific case.**

7) Can there be neurologic symptoms, or neurologic injury, from a lumbar puncture?

It is thought to be very rare that a permanent neurologic injury could occur from lumbar puncture. When we do the lumbar puncture procedure we enter below the level of the spinal cord, and the spinal cord is most important in terms of avoiding neurologic injuries. So we go below the spinal cord.

There are nerve roots inside and outside the spine that maybe come apparent during the procedure. Sometimes when the needle enters the space where the fluid is, a patient can experience a lightning bolt sensation down their leg. That usually goes away almost immediately and if it doesn’t go away immediately, it certainly goes away at the end of the procedure. It is very unusual to have a persistent symptom like that after the procedure is completed.

8) Other things to consider regarding lumbar punctures.

We reduce the risk of infection, or causing an infection, by using sterile techniques.

• A mask for the operator

• Cleansing the skin

• Using a sterile field when performing a lumbar puncture.

9) Lumbar puncture information specific to those with recurrent viral meningitis.

Mollaret’s meningitis is a recurrent type of meningitis and the only way to diagnose meningitis, inflammation of the lining of the spinal cord, is by lumbar puncture looking for inflammation there. That requires sampling of the fluid. There is no known risks of repeat lumbar puncture beyond the risks of the lumbar puncture already addressed for each time the procedure is performed.

**We recommend talking to your doctor about the specifics of your case.**