Recurrent

Mollaret's Meningitis

Mollaret described a syndrome, rather than a disease, of benign recurrent meningitis with characteristic spinal fluid picture in 1944. In 1962, Byrum proposed the clinical diagnostic criteria of:

• Recurrent episodes of sever headache, meningismus, and fever
• Cerebrospinal fluid (CSF) pleocytosis with large "endothelial" cells, neutrophils, and lymphocytes
• Attacks separated by symptom-free periods of weeks to months
• Spontaneous remission of symptoms and signs
• No causative etiologic agent detected

Then in 1979, Goldi observed that Mollaret's meningitis could occur without fever, have symptom-free periods from days to years, increased CSF gamma globulin, and transient neurologic signs and symptoms.

Information

General:
Mollaret's meningitis is characterized by repeated episodes of fever (up to 104 degrees F), meningismus, and severe headache separated by symptom-free intervals. It's a rare, poorly understood disorder, whose cause remains obscure and its diagnosis by exclusion. Even though Mollaret's meningitis doesn't pose a threat to patients, its early recognition may prevent the repeated extensive diagnostic investigations.

Long-term transient neurologic abnormalities such as seizures, diplopia, pathologic reflexes, cranial nerve paresis, hallucinations, and comas occur in over 50% of cases.

The most difficult to differentiate from Mollaret's meningitis is the aseptic recurrent meningitis caused by epidermoid tumors. The cyst ruptures and the contents, which become hard to find, contain cell debris and ghost cells identical to those seen in the CSF of patients with Mollaret's meningitis. Associated viral infection is often suspected but seldom confirmed. Viruses that have been associated with Mollaret's meningitis include Epstein-Barr virus, Coxsackie viruses B5 and B2, echoviruses 9 and 7, and herpes simplex virus types 1 and 2.

Recent data suggests that herpes simplex type 1 and 2 may be etiologic in some if not most cases of Mollaret's meningitis. Patients may present Mollaret's meningitis without Mollaret's cells being demonstrated, or they may represent a Mollaret's-like syndrome caused by herpes simplex type 2. Other etiologic agents that have been considered over the years include trauma and viral infections other than Herpes simplex.

Prevention:

There is no current vaccine available to prevent anyone from Mollaret's meningitis.


Symptoms:

• Irritability
• Fever (below normal)
• Headache
• Stiff neck
• Muscle aches

Individual attacks are sudden with symptoms reaching maximum intensity within a few hours. They persist for a few days, but can be present for 3 weeks. Symptoms can reoccur for years then disappear as quickly as they arrived.

Diagnosis:
All types of meningitis are diagnosed by growing bacteria from a sample of the infected person's spinal fluid, which is collected by performing a lumbar puncture (spinal tap).

CSF obtained from the patient demonstrates the large friable "endothelial" cells termed Mollaret's cells, which can be demonstrated by the Papanicolaou stain and are now considered to be large activated cells of monocyte/macrophage lineage. Mollaret's cells are the number one determination for its meningitis, but are only present in the first 24 and missed easily.

More criteria for diagnosing Mollaret's meningitis include recurrent attacks of meningitis with or without additional transient non-meningitic signs and symptoms of arthralgias, myalgia, vertigo, syncope, cranial nerve paralysis and coma.

Treatment:
Acyclovir is administered intravenously or orally and Valacyclovir is given orally to treat Mollaret's meningitis. Other therapies tried include steroids, which had no effect and colchicine 0.5 mg BID, which had an apparent prophylactic benefit in some cases.

Source(s):
Barakat, Muhammed H. MD, FACP. Archives of Neurology. August 1988, Vol. 4

Ruben, Stuart J. The Western Journal of Medicine.  May 1994, Vol. 160