Mollaret's meningitis
Mollaret's meningitis | |
---|---|
Other names | Benign recurrent lymphocytic meningitis |
Meninges of the central nervous system: dura mater, arachnoid, and pia mater. | |
Specialty | Neurology |
Mollaret's meningitis is a recurrent or chronic inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. Since Mollaret's meningitis is a recurrent, benign (non-cancerous), aseptic meningitis, it is also referred to as benign recurrent lymphocytic meningitis.[1][2] It was named for Pierre Mollaret, the French neurologist who first described it in 1944.[3][4]
Although chronic meningitis has been defined as "irritation and inflammation of the meninges persisting for more than 4 weeks being associated with pleocytosis in the cerebrospinal fluid",[2] cerebrospinal fluid abnormalities may not be detectable for the entire time.[5] Diagnosis can be elusive, as Helbok et al. note: "in reality, many more weeks, even months pass by until the diagnosis is established. In many cases the signs and symptoms of chronic meningitis not only persist for periods longer than 4 weeks, they even progress with continuing deterioration, i. e. headache, neck stiffness and even low grade fever. Impairment of consciousness, epileptic seizures, neurological signs and symptoms may evolve over time."[2]
Signs and symptoms
Mollaret's meningitis is characterized by chronic, recurrent episodes of headache, stiff neck,
While
Cause
Although for a long time, the cause of Mollaret's meningitis was not known, recent work has associated this problem with
Cases of Mollaret's resulting from varicella zoster virus infection, diagnosed by polymerase chain reaction (PCR), have been documented. In these cases, PCR for herpes simplex was negative.[9][10] Some patients also report frequent
A familial association, where more than one family member had Mollaret's, has been documented.[12]
Diagnosis
Diagnosis starts by examining the patient's symptoms. Symptoms can vary. Symptoms can include headache, sensitivity to light, neck stiffness, nausea, and vomiting. In some patients, fever is absent. Neurological examination and MRI can be normal.[5]
Mollaret's meningitis is suspected based on symptoms, and can be confirmed by HSV 1 or HSV 2 on PCR of Cerebrospinal fluid (CSF), although not all cases test positive on PCR. PCR is performed on spinal fluid or blood, however, the viruses do not need to enter the spinal fluid or blood to spread within the body: they can spread by moving through the axons and dendrites of the nerves.[13]
During the first 24 h of the disease the spinal fluid will show predominant polymorphonuclear neutrophils and large cells that have been called endothelial (Mollaret's) cells.[14]
A study performed on patients who had diffuse symptoms, such as persistent or intermittent headaches, concluded that although PCR is a highly sensitive method for detection, it may not always be sensitive enough for identification of viral DNA in CSF, due to the fact that viral shedding from latent infection may be very low. The concentration of viruses in CSF during subclinical infection might be very low.[15]
Investigations include
During the lumbar puncture procedure, the opening pressure is measured. A pressure of over 180 mm H2O is suggestive of bacterial meningitis.
It is likely that Mollaret meningitis is underrecognized by physicians, and improved recognition may limit unwarranted antibiotic use and shorten or eliminate unnecessary hospital admission.[12]
PCR testing has advanced the state of the art in research, but PCR can be negative in individuals with Mollaret's, even during episodes with severe symptoms. For example, Kojima et al. published a case study for an individual who was hospitalized repeatedly, and who had clinical symptoms including genital herpes lesions. However, the patient was sometimes negative for HSV-2 by PCR, even though his meningitis symptoms were severe. Treatment with acyclovir was successful, indicating that a herpes virus was the cause of his symptoms.[16]
Treatment
The IHMF recommends that patients with benign recurrent lymphocytic meningitis receive intravenous acyclovir in the amount of 10 mg/kg every 8 hours, for 14–21 days. More recently, the second-generation antiherpetic drugs valacyclovir and famciclovir have been used to successfully treat patients with Mollaret's. Additionally, it has been reported that Indomethacin administered in the amount of 25 mg 3 times per day after meals, or 50 mg every 4 hours, has resulted in a faster recovery for patients, as well as more extended symptom-free intervals, between episodes.[1]
Prognosis
Recurring Mollaret meningitis attacks generally resolve within 3 to 5 years after the first occurrence, but some patients live with the disease for much longer.[1] With suppressive antiviral therapy, some patients who have Mollaret's report experiencing fewer attacks. However, there are some that have flare ups all throughout the year.[1]
See also
References
- ^ PMID 17029141.
- ^ S2CID 6218001.
- Who Named It?
- ^ Mollaret P (1944). "Méningite endothélio-leucocytaire multirécurrente bénigne. Syndrome nouveau ou maladie nouvelle? (Documents cliniques)". Revue neurologique, Paris. 76: 57–76.
- ^ PMID 20375513.
- PMID 18680414.
- ISBN 9780071802154.
- ^ Tarakad S Ramachandran, MBBS, FRCP(C), FACP (Feb 12, 2010). "Aseptic Meningitis". Emedicine. Retrieved 9 January 2011.
- S2CID 41674924.
- PMID 12553305.
- ^ Mollaret's meningitis at patient.co.uk
- ^ PMID 20825883.
- PMID 23435239.
- PMID 19329344.
- PMID 21849074.
- PMID 12195049. Archived from the original(PDF) on 2013-01-22. Retrieved 2014-09-21.