Dr. Greenlee of the University of Utah School of Medicine has no relevant financial relationships to disclose.)
This article includes discussion of recurrent meningitis, recurrent bacterial meningitis, and recurrent nonpurulent meningitis. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
The term “recurrent meningitis” encompasses a variety of conditions, some of which are life-threatening, some spontaneously remitting, and some representing exacerbations of chronic infections. Recurrent meningitis may, thus, represent repeated episodes of bacterial meningitis, recurrent episodes of meningitis due to nonbacterial microorganisms, chemical meningitis due to rupture of dermoid or parasitic cysts, or meningitis in response to nonsteroidal or other agents. In some instances, as in protracted cases of meningitis due to Cryptococcus neoformans, what appears to be recurrent meningitis may actually represent periodic exacerbations of a chronic, ongoing infectious process. In this article, the author reviews the pathogenesis, clinical features, diagnosis, and treatment of this group of disorders.
• Episodes of recurrent meningitis fall into 2 groups: recurrent bacterial meningitis and recurrent episodes of nonpurulent meningitis.
• Recurrent bacterial meningitis is most frequently associated with defects in the skull base or spinal cord or with genetic defects, most commonly in the complement system.
• Nonbacterial recurrent meningitis has a much wider differential diagnosis and may include viral, fungal, protozoal, or non-infectious processes, as well as conditions such as sarcoid or meningeal reaction to nonsteroidal or other pharmacological agents.
Historical note and terminology
Episodes of recurrent meningitis fall into 2 groups: recurrent bacterial meningitis, and recurrent episodes of nonpurulent meningitis. Symptomatology and cerebrospinal fluid changes in recurrent bacterial meningitis are those typical of bacterial meningitis in general. Symptoms in recurrent nonpurulent meningitis are much more variable, and cerebrospinal fluid may contain lymphocytes, neutrophils, or a mixed pleocytosis. Recurrent bacterial meningitis did not exist as a clinical entity prior to the advent of antibiotics because a single episode of meningitis was almost invariably fatal. In modern times, up to 9% of patients surviving acute bacterial meningitis may go on to have further episodes (Durand et al 1993). In children, recurrent bacterial meningitis is most commonly associated with congenital defects of the middle ear or with persistent dermal sinuses along the spinal column (Khan et al 2013; Masri et al 2018). In adults, recurrent episodes of bacterial meningitis are most commonly associated with traumatic defects at the skull base (Adriani et al 2007). In a minority of cases, recurrent bacterial meningitis is associated with defects in the complement system or, rarely, with agammaglobulinemia, selective IgM deficiency, X-linked hyper- IgM syndrome, or, rarely, common variable immunodeficiency syndrome (Ersoy et al 1990; Goldstein et al 2008; Tebruegge et al 2008; Gaschignard et al 2014; Franca et al 2018; Yazdani et al 2018; Butters et al 2019). In recurrent bacterial meningitis, identification of the infectious agent is usually straightforward, and the major task, after the episode of meningitis has been successfully treated, is to identify and, if possible, treat the anatomical or immunological defects that allow recurrent infections to occur.
Recurrent episodes of nonpurulent meningitis were first recognized in patients with syphilis. Over the years, recurrent nonpurulent meningitis has been associated with both infectious and noninfectious conditions. Infectious agents associated with recurrent nonpurulent meningitis have included bacteria, spirochetes, fungi, protozoa, and viruses. Noninfectious causes of recurrent nonpurulent meningitis have included chemical meningitis due to intermittent leakage of intracranial epidermoid cysts; inflammatory conditions of unknown cause, such as sarcoid; and atypical reactions to nonsteroidal anti-inflammatory drugs or other therapeutic agents. The diversity of conditions that may cause recurrent nonpurulent meningitis and the relative insensitivity of diagnostic tests used in these conditions combine to make the diagnosis of recurrent or chronic nonpurulent meningitis one of the most difficult, and often most frustrating, areas in all of neurology. Recurrent bacterial meningitis and recurrent nonpurulent meningitis will be discussed separately under each topic heading.
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