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Mollaret's Meningitis

This is a form of benign, recurrent, aseptic meningitis.

It was first described by Pierre Mollaret (1898-1987) a French physician, in 1944 and he wrote several papers on the condition until 1968.1

Diagnostic criteria

GW Bruyn (a Dutch neurologist, co-editor of the Handbook of Clinical Neurology series, the most comprehensive source of information in neurology) refined the clinical diagnostic criteria in 1962:2

  • Recurrent episodes of severe 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 aetiological agent has been identified.
Pathogenesis
  • In recent years there has been increasing evidence to implicate the herpes simplex virus HSV 2 in many cases, (the usual cause of genital herpes) but also HSV 1, (the usual cause of oral herpes).3,4
  • The Epstein Barr virus has also been implicated, also a human herpes virus.5

It has been suggested that if the strict criteria above are followed, these cases should be excluded, and the term Mollaret's meningitis reserved for true idiopathic cases. However, this appears not to be rigorously applied.

Epidemiology

It is very rare and the literature is very limited.

Presentation
  • There is a rapid onset of symptoms that are typical of meningitis.
    These are:
    • Fever
    • Headache
    • Neck stiffness
  • The symptoms last between 1 and 7 days.
  • Symptoms resolve without any residual defect.
  • Symptom free periods may last from weeks to years, but the characteristic feature of this disease is the tendency to recur.
  • The clinical picture can be variable.
    • Some have been reported without fever, with transient neurological signs and symptoms, and with raised gamma globulin in the CSF.
    • More severe cases can occur with neurological abnormalities, including seizures, diplopia, abnormal reflexes, cranial nerve palsy, hallucinations and coma.
    • This may occur in as many as half of all patients, but full recovery is usual.
Investigations

Head CT or MRI is done before lumbar puncture if a brain mass is suspected e.g. from focal neurological signs or papilloedema.

  • The classical feature is the appearance of the CSF:6,7
    • Mollaret cells are seen, but they are not regarded as pathognomonic.
    • They may represent 60 to 70% of all cells in the CSF, but are often seen for only the first 24 hours.
    • The evidence suggests that these cells are monocytes.
  • After the first 24 hours the cells are predominantly lymphocytes, numbering less than 3,000 mm3.
  • In about a third of cases the CSF glucose is low.
  • Other causes of meningitis should be excluded.
Differential diagnosis

Other acute aseptic meningitis:8

  • Viral - enteroviruses (polio, Coxsackie, echo), HSV I&II, varicella-zoster, adenovirus, Epstein-Barr, LCMV, HIV, influenza A&B
  • Bacterial - partially treated meningitis, parameningeal infection, endocarditis, mycoplasma, TB, ehrlichiosis, borrelia, syphilis, brucella
  • Fungi
  • Parasites - toxoplasma and cysticercosis
  • Non-infectious:
  • Of the above, causes of recurrent meningitis including Behçet's, Vogt-Koyanagi-Harada syndrome, sarcoid, SLE, and undiagnosed viral e.g. encephalomyocarditis
Management
  • Pain and temperature should be treated in the usual way.
  • Intravenous fluid may be required.
  • Colchicine and indomethacin have been used and clinical observation suggests effectiveness.9
  • RCT on the the use of antiviral agents, are lacking. [This is probably because the disease is so rare that it is impossible to get even a small series.]
  • Most sources advocate the use of acyclovir, given the high prevalence of HSV on polymerase chain reaction testing.8,10
Prognosis
  • Herpes simplex encephalitis is a rare but very serious condition with a mortality around 70% that usually leaves survivors with neurological deficits.
  • This is in contrast to Mollaret's meningitis that may be recurrent and unpleasant in the acute attack but does not leave residual damage.

Document references
  1. Mollaret P, Cateigne G; A special type of benign endothelio-leukocytic meningitis with multiple recurrences and with synchronous inflammation of the parotid glands; isolation of an ultravirus from chick embryos. Bull Mem Soc Med Hop Paris. 1952 Jul 4-11;68(24-25):946-54.
  2. Bruyn GW, Straathof LJ, Raymakers GM; Mollaret's meningitis. Differential diagnosis and diagnostic pitfalls. Neurology. 1962 Nov;12:745-53.
  3. Jensenius M, Myrvang B, Storvold G, et al; Herpes simplex virus type 2 DNA detected in cerebrospinal fluid of 9 patients with Mollaret's meningitis. Acta Neurol Scand. 1998 Sep;98(3):209-12. [abstract]
  4. Achard JM, Duverlie G, Schmit JL, et al; Mollaret's meningitis and herpes simplex virus type 1. N Engl J Med. 1992 Mar 26;326(13):893-4.
  5. Graman PS; Mollaret's meningitis associated with acute Epstein-Barr virus mononucleosis. Arch Neurol. 1987 Nov;44(11):1204-5. [abstract]
  6. Stoppe G, Stark E, Patzold U; Mollaret's meningitis: CSF-immunocytological examinations. J Neurol. 1987 Feb;234(2):103-6. [abstract]
  7. Lowe E; Mollaret's meningitis: a case report. Acta Cytol. 1982 May-Jun;26(3):338-40. [abstract]
  8. MERCK manual. Aseptic meningitis.; Last updated November 2005
  9. Wynants H, Taelman H, Martin JJ, et al; Recurring aseptic meningitis after travel to the tropics: a case of Mollaret's meningitis? Case report with review of the literature. Clin Neurol Neurosurg. 2000 Jun;102(2):113-5. [abstract]
  10. Tyler KL; Herpes simplex virus infections of the central nervous system: encephalitis and meningitis, including Mollaret's. Herpes. 2004 Jun;11 Suppl 2:57A-64A. [abstract]
Internet and further reading Acknowledgements EMIS is grateful to Dr Hayley Willacy for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2008.
DocID: 2465
Document Version: 20
DocRef: bgp1266
Last Updated: 20 Dec 2007
Review Date: 19 Dec 2009




















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