Recurrent Meningitis Causes1
Recurrent meningitis is defined by at least two episodes of meningitis with associated CSF pleocytosis with no symptoms between episodes. Recurrent meningitis was first described in the 1940s by physician-scientist Pierre Mollaret, who noted a handful of cases of recurrent acute aseptic meningitis with CSF showing evidence of lymphocytic and mononuclear pleocytosis. This was later attributed to HSV-2 infection. The condition was later named Mollaret meningitis after him and the “Mollaret cells” he identified, although it is now known that many of the infectious and noninfectious etiologies of chronic meningitis can occur in a fluctuating pattern consistent with a recurrent meningitis. While triggers for recurrent viral meningitis are unclear, significant risk factors should be investigated in those who present with recurrent bacterial meningitis, including host factors such as anatomic defects that provide a nidus for bacterial CNS invasion (eg, skull-base defects, neural tube defects) and acquired and congenital immunodeficiencies that predispose individuals to recurrent ear, nose, and throat infections. Likewise, recurrent meningitis due to fungi or parasites should trigger an investigation for an underlying immunocompromised state or ongoing environmental exposures. This should go beyond testing for HIV and should include evaluation of T-cell and B-cell profiles, complement levels, IgG subclass quantification, and screening for rheumatologic conditions. Recently an association between acute meningoencephalitis infection and positive paraneoplastic antibodies in recurrent meningitis cases was identified, although further studies are required to determine the significance of autoimmune antibodies in recurrent meningitis.2
Behcet’s Disease, also known as Behcet’s syndrome, is a rare, chronic, autoimmune, autoinflammatory disorder of unknown origin.
Granulomatosis with Polyangitis
Granulomatosis with polyangiitis (GPA/formerly called Wegener’s granulomatosis) is systemic, meaning that the effect of inflammation can be present in the entire body. It affects the upper (sinuses and nose), and lower (lungs), respiratory system and frequently involves the kidneys, lungs, eyes, ears, throat, skin and other body organs.
Rheumatoid arthritis (RA) is an autoimmune disease in which the body’s immune system – which normally protects its health by attacking foreign substances like bacteria and viruses – mistakenly attacks the joints.
Sarcoidosis (pronounced SAR-COY-DOE-SIS) is an inflammatory disease characterized by the formation of granulomas, tiny clumps of inflammatory cells, in one or more organs of the body.
Sjögren’s (“SHOW-grins”) is a systemic autoimmune disease that affects the entire body. Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies, and lymphomas.
Systemic lupus erythematosus
Systemic lupus is the most common form of lupus—it’s what most people mean when they refer to “lupus.” Systemic lupus can be mild or severe.
Vogt-Koyanagi-Harada disease is a rare disorder of unknown origin that affects many body systems, including as the eyes, ears, skin, and the covering of the brain and spinal cord (the meninges).
Craniopharyngioma is a slow-growing, non-cancerous brain tumor that develops near the pituitary gland (a small endocrine gland at the base of the brain which produces several important hormones) and the hypothalamus (an endocrine organ which controls the release of hormones by the pituitary gland).
A dermoid cyst is a saclike growth that is present at birth. It contains structures such as hair, fluid, teeth, or skin glands that can be found on or in the skin.
Epidermoid (ep-ih-DUR-moid) cysts are noncancerous small bumps beneath the skin. They can appear anywhere on the skin, but are most common on the face, neck and trunk.
Known bacterial causes for recurrent meningitis include Streptococcus pneumoniae, Haemophilus influenzae, Neisseria meningitidis, Staphylococcus aureus, Escherichia coli.
Known fungal causes for recurrent meningitis include Cryptococcus neoformans, Candida species, Histoplasma capsulatum, Coccidiodes immitis, and Blastomyces dermatitidis.
Known parasites that can cause recurrent meningitis include Echinococcosis species, Strongyloides stercoralis, and Toxoplasma gondii.
Known viral causes for recurrent meningitis include HSV-2, HSV-1, and EBV (HHV-4).
Leptomeningeal Metastasis of Solid Tumors
Leptomeningeal metastasis (LM), i.e. the seeding of tumor cells to the cerebrospinal fluid (CSF) and the leptomeninges, is a devastating and mostly late-stage complication of various solid tumors.
In leukemic meningitis, cancer cells have spread from the original (primary) tumor to the meninges (thin layers of tissue that cover and protect the brain and spinal cord).
In lymphomatous meningitis, cancer cells have spread from the original (primary) tumor to the meninges (thin layers of tissue that cover and protect the brain and spinal cord).
Antibiotics known to cause recurrent meningitis include trimethoprim-sulfamethoxazole, cephalosporins, amoxicillin, and ciprofloxacin.
Anticonvulsants known to cause recurrent meningitis include lamotrigine and carbamazepine.
Chemotherapeutic drugs known to cause recurrent meningitis include cetuximab and intrathecal chemotherapy.
Immunosuppressive agents known to cause recurrent meningitis include IVIG, methotrexate, azathioprine and TNF inhibitors (e.g., adalimumab, infliximab, etanercept).
Non-steroidal Anti-inflammatory Agents
NSAIDs known to cause recurrent meningitis include ibuprofen, diclofenac, naproxen, and sulindac.