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Disease Profile

Mollaret meningitis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

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Europe Estimated

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Benign recurrent aseptic meningitis; Mollaret's meningitis; Benign recurrent lymphocytic meningitis;

Categories

Viral infections

Summary

Mollaret meningitis is a type of meningitis due to a viral infection (aseptic meningitis) that occurs multiple times.[1] It is characterized by repeated episodes of meningitis, typically lasting two to five days, occurring weeks to years apart.[1][2] Common signs and symptoms during an episode may include severe headache, fever, nausea, vomiting, sensitivity to light (photophobia), and stiff neck.[1][3] Some people also experience temporary neurological symptoms such as double vision, hallucinations, altered consciousness, cranial nerve palsy, or seizures.[1][2][3] Almost half of people with Mollaret meningitis develop long-term neurological impairment such as problems with memory, balance, coordination, and/or hearing.[1]

The virus most commonly responsible for Mollaret meningitis is the herpes simplex virus (usually HSV-2, the type generally responsible for genital herpes, but also HSV-1).[1][2] However, more than half of people with Mollaret meningitis due HSV-2 do not report a history of genital herpes.[4] Epstein-Barr virus has also been associated with Mollaret meningitis. The diagnosis typically involves detecting herpes simplex virus DNA in the cerebrospinal fluid, which is collected during a lumbar puncture (spinal tap).[1][2] This test can diagnosis other types of meningitis as well. Treatment may involve an antiviral drug such as acyclovir to treat and prevent recurrent episodes of viral meningitis, as well as various medications to treat specific symptoms present in each person.[1][2] While antiviral drugs have reportedly improved symptoms in many cases, the effectiveness of antiviral therapy is difficult to measure due to the rarity of the disease and its recurrent and spontaneous nature.[1] While aseptic meningitis usually is not fatal, serious complications such as encephalitis and coma can develop in severe cases.[3]

Symptoms

Mollaret meningitis is characterized by recurrent episodes of viral meningitis. The episodes of meningitis usually last from two to five days and then go away on their own, but the time in between episodes varies among people with the disease, from weeks to years.[1][2] Some people have milder symptoms during recurrences, which do not necessarily require hospitalization.[1]

Symptoms during an episode commonly include:[1][2][3]

  • severe headache
  • fever
  • stiff neck
  • nausea and vomiting
  • sensitivity to light (photophobia) or sound
  • feeling tired or unwell (malaise)
  • muscle pain

Some people experience temporary neurological symptoms, such as:[1][2][3]

Each recurrence of meningitis increases the risk for a person to develop permanent neurological problems or disability, which occur in about half of people with Mollaret meningitis. Various long-term symptoms have been reported, including:[1]

  • cognitive impairment
  • memory loss
  • problems with balance and coordination
  • hearing or vision problems
  • ringing in the ears (tinnitus)
  • speech problems
  • epilepsy

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Social Networking Websites

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Mollaret meningitis. Click on the link to view a sample search on this topic.

          References

          1. Information on Recurrent Viral Meningitis (Mollaret’s). Recurrent Meningitis Association. https://recurrentmeningitis.org/rvm-information/. Accessed 1/14/2019.
          2. Tunkel AR. Aseptic meningitis in adults. UpToDate. Waltham, MA: UpToDate; 2018; https://www.uptodate.com/contents/aseptic-meningitis-in-adults.
          3. Ramachandran TS. Aseptic Meningitis. Medscape Reference. July 17, 2018; https://emedicine.medscape.com/article/1169489.
          4. Rosenberg J, Galen BT. Recurrent Meningitis. Curr Pain Headache Rep. July, 2017; 21(7):33. https://www.ncbi.nlm.nih.gov/pubmed/28551737.

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