Meningococcal meningitis

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released December 7, 2004; last reviewed April 19, 2017; expires April 19, 2020

Overview

The author explains the clinical presentation, pathophysiology, diagnostic workup, and management of meningococcal meningitis. Meningococcal meningitis may be indistinguishable from other bacterial meningitides, although the classic triad of fever, neck stiffness, and change in mental status is significantly less common in patients with meningococcal meningitis (27%) than in patients with pneumococcal meningitis (58%). Although less than one third of affected patients present with the classic triad of fever, nuchal rigidity, and change in mental status, nearly 90% have at least 2 of the following 4 signs on presentation: fever, nuchal rigidity, change in mental status, and rash. Guidelines for use of the various meningococcal vaccines are summarized.

Key points

 

• Meningococcemia presents abruptly with fever, chills, nausea, vomiting, headache, myalgias, malaise, prostration, and rash. Rash is somewhat variable and may be urticarial, maculopapular, or petechial.

 

• Acute fulminating cases of meningococcal septicemia (Waterhouse-Friderichsen syndrome) occur mainly in children younger than 10 years of age and are characterized by vomiting, diarrhea, extensive purpura, disseminated intravascular coagulation, cyanosis, convulsions, shock, coma, and often death within hours despite appropriate treatment; many of these cases have meningitis and adrenal insufficiency with hemorrhage into the adrenal glands or adrenal infarction.

 

• Meningococcal meningitis may be indistinguishable from other bacterial meningitides, although the classic triad of fever, neck stiffness, and change in mental status is significantly less common in patients with meningococcal meningitis (27%) than in patients with pneumococcal meningitis (58%).

 

• Although less than one third of affected patients present with the classic triad of fever, nuchal rigidity, and change in mental status, nearly 90% have at least 2 of the following 4 signs on presentation: fever, nuchal rigidity, change in mental status, and rash.

 

• The strains of Neisseria meningitidis most commonly implicated in systemic disease are A, B, C, W, and Y. In the United States, groups B, C, and Y are the most common serogroups implicated, and each account for about 30% of reported cases.

 

• North American outbreaks (as opposed to isolated cases) are confined primarily to serogroup C (less commonly to Y and W), although the frequency of serogroup Y outbreaks increased markedly during the 1990s.

 

• Although rates of disease are highest among children less than 2 years of age, almost two thirds of meningococcal disease in the United States occurs in those older than 10 years. The vast majority of cases (95% to 98%) are sporadic. However, outbreaks of meningococcal disease have been occurring with increasing frequency in the United States.

 

• Meningococcal disease outbreaks are particularly likely to occur in semiclosed communities, such as day care centers, schools, colleges, nursing homes, and military recruit camps.

 

• Almost all secondary cases in an outbreak occur within 8 days of the index case.

 

• The U.S. Centers for Disease Control and Prevention recommends routine vaccination with quadrivalent meningococcal conjugate vaccine of adolescents 11 to 18 years of age and vaccination of persons 2 to 55 years of age who have an increased risk of invasive meningococcal disease.

 

• The case-fatality ratio for meningococcal disease is approximately 10%, and 11% to 26% of survivors have serious sequelae, including neurologic disability (eg, focal neurologic deficits, seizures, etc.), limb loss, and deafness.

 

• Because of the risks of severe morbidity and death, appropriate antibiotic therapy should be rapidly initiated in patients suspected of having meningococcal disease. Prior to confirmation of meningococcal disease as the cause of the illness, empiric antibiotic coverage should be given directed at the most likely pathogens based on epidemiologic considerations (eg, age, geographic location, etc.) and the known prevalence of antibiotic resistance for these organisms. No investigations should delay initiating antibiotic therapy once the diagnosis of meningococcal meningitis is suspected.

 

• Children who have suspected bacterial meningitis or meningococcal disease should be immediately treated with intravenous ceftriaxone, with the addition of amoxicillin or ampicillin for children younger than 3 months, and the addition of vancomycin for those who have recently travelled outside of the country or who have had prolonged or multiple exposures to antibiotics.

 

• Recommendations for initial empiric antimicrobial therapy in adults with community-acquired bacterial meningitis are vancomycin plus a third-generation cephalosporin (eg, cefotaxime or ceftriaxone) for those aged 16 to 50 years and vancomycin plus a third-generation cephalosporin plus ampicillin for those older than 50 years, or for those with alcoholism or altered immune status. Generally, combination therapy should be continued until results of in vitro susceptibility testing are available.

Historical note and terminology

In the first decade of the 20th century, untreated meningococcal meningitis had a case-fatality rate of 70% to 80% (Rosenstein et al 2001; Swartz 2004). Following the introduction of intrathecal equine meningococcal antiserum in 1913, the case-fatality rate dropped to 20% to 31% by the late 1920s and early 1930s (Swartz 2004). In the 1930s, with the introduction of sulfonamides, the case-fatality rate dropped to 5% to 15%. Later therapies included high-dose penicillin and third-generation cephalosporins, but the case-fatality rate has generally stayed around 6% to 14% (Centers for Disease Control and Prevention 2000; Boras et al 2004; Casella et al 2004; Swartz 2004; van de Beek et al 2004b; Bilukha et al 2005; Nathan et al 2005; Sotir et al 2005; Kaplan et al 2006; O'Brien et al 2006; Sharip et al 2006; Smith et al 2006). Meningococcal meningitis continues to be a major cause of morbidity and mortality around the world and a significant contributor to healthcare costs, even in developed countries (Nathan et al 2005; Artenstein and LaForce 2012; Darbà et al 2014).

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