MOLLARET’S MENINGITIS

• Mollaret’s meningitis is a rare form of recurrent meningitis originally described by Mollaret in 1944. In 1962, Bryun proposed the clinical diagnostic criteria:

• Recurrent episodes of severe headache, meningismus, and fever;

• Cerebrospinal fluid pleocytosis with large "endothelial" cells, neutrophils, and lymphocytes;

• Attacks separated by symptom-free periods of weeks to months;

• Spontaneous remission of symptoms and signs; and

• No causative etiologic agent detected. In 1979, Goldi observed that patients with Mollaret’s meningitis could present without fever, have symptom-free periods from days to years, have increased CSF gamma globulin, and have transient neurologic signs and symptoms.

Clinical Presentation

Mollaret’s meningitis is characterized by repeated episodes of fever, meningismus, and severe headache separated by symptom-free intervals. Individual attacks are sudden, with signs and symptoms reaching maximum intensity within a few hours. Headache, neck pain, generalized muscle aches, and neck stiffness usually persist from one to three days, but may be present for up to three weeks. Following a number of recurrences, which can span a period of years, the disease suddenly disappears. The long-term health of the patient seems not to be adversely affected. Transient neurologic abnormalities (seizures, diplopia, pathologic reflexes, cranial nerve paresis, hallucinations, and coma) occur in as many as 50% of cases. Persistence of neurologic defects should call the diagnosis into question. CSF obtained early in the course of the illness usually demonstrates large, friable "endothelial" cells termed Mollaret’s cells. Mollaret’s cells can be demonstrated by the Papanicolaou stain, and are now considered to be large activated cells of monocyte/macrophage lineage. Mollaret’s cells are considered by many to be the hallmark of Mollaret’s meningitis, and early on may comprise 60% to 70% of the CSF cells. These cells are usually present for only the first 24 hours and can be missed easily. Furthermore, "Mollaret’s cells" are not pathognomonic for Mollaret’s meningitis. After the first 24 hours, the CSF shows a lymphocytic predominance with cell counts usually less than 3,000/mm³. Hypoglycorrhachia (a low CSF glucose concentration), is reported in one-third of patients. CSF protein, especially the gamma globulin fraction, is usually mildly elevated.

Etiology
Recent data suggest that herpes simplex II and, less frequently, herpes simplex I may be etiologic in some if not most cases of Mollaret’s meningitis.

Therapy
Mollaret’s meningitis is a syndrome rather than a disease. As such, the syndrome of Mollaret’s meningitis appears to have multiple etiologies. Presently, herpes simplex II, and to a lesser extent herpes simplex I, appear to be etiologic in most cases. Because of the rarity of this syndrome, there are no large clinical trials comparing one therapy against another. However, acyclovir (intravenous or oral) or valacyclovir (oral only) are worthy of consideration for both therapy and prophylaxis. Other therapies tried include steroids (no benefit) and colchicine 0.5 mg BID (apparent prophylactic benefit in some cases).

Points to Remember

• Mollaret’s meningitis is usually a benign (but painful), self-limited, recurrent, often febrile meningitis.

• Transient neurologic deficits (seizures, cranial nerve paresis, pathologic reflexes) occur in 50% of cases

• Mollaret’s may be caused by herpes simplex II; acyclovir may play a role in prophylaxis and therapy.

Category: Medicine Notes