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  • Updated 09.20.2021
  • Released 12.07.2004
  • Expires For CME 09.20.2024

Meningococcal meningitis

Introduction

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 two of the following four 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 two of the following four 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 semi-closed communities, such as daycare 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 traveled 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

Neisseria is a large genus of bacteria that colonize the mucosal surfaces of many animals. The genus is named after German physician Albert Ludwig Sigesmund Neisser (1885-1916), who discovered Neisseria gonorrhoeae in 1879.

German physician Albert Ludwig Sigesmund Neisser (1885-1916)

Neisser discovered Neisseria gonorrhoeae in 1879. (Photograph courtesy of the U.S. National Library of Medicine. Photograph restored by Dr. Douglas J Lanska.)

Of the 11 species of Neisseria that colonize humans, only two are pathogens: N. meningitidis (meningococcus) and N. gonorrhoeae. Neisseria species are gram-negative bacteria included among the proteobacteria. N. meningitidis is an aerobic or facultative anaerobic, non-motile, gram-negative, diplococcal bacterium that exclusively infects humans. Neisseria diplococci resemble coffee beans when viewed microscopically.

Meningococcal disease was first described by Geneva physician Gaspard Vieusseux (1746-1814) during an outbreak in Geneva in 1805. Another outbreak was described in 1806 in New Bedford, Massachusetts by Lothario Danielson (1765-1841), a Revolutionary War veteran, and Elias Mann (1778-1807). In 1884, Italian pathologists Ettore Marchiafava (1847-1935) and Angelo Celli (1886-1914) described intracellular micrococci in cerebrospinal fluid. In 1887, Austrian pathologist and bacteriologist Anton Wiechselbaum (1845-1920), working in Vienna, identified the meningococcus (designated as Diplococcus intracellularis meningitidis) in cerebrospinal fluid and established the connection between the organism and epidemic meningitis.

At the beginning of the twentieth century, the meningococcus was recognized as a habitant of the nasopharynx of healthy individuals, particularly at camps for military recruits.

Serum therapy was developed by German physiologist Emil von Behring (1854-1917) and German physician-scientist Paul Ehrlich (1854-1915) in the late 19th century for the treatment of diphtheria. They had produced their diphtheria serum by repeatedly injecting the bacterial toxin into a horse.

The serum was then used effectively during an epidemic in Germany. A chemical company offered a contract to both men, but Behring took the considerable financial rewards for himself. Behring was controversially awarded a solo Nobel Prize in Physiology or Medicine for this in 1901, but Ehrlich earned the 1908 Nobel Prize in Physiology or Medicine for his contributions to immunology. Commercial production of diphtheria serum depended at the time on injecting an increasing number of horses with the toxin to supply demand for the serum, which resulted in caricatures of the process in the mass media of the time (albeit inappropriately crediting Behring as sole developer.

The development of serum therapy paved the way for its application to meningococcal disease. Several investigators reported beneficial effects of serum therapy in animals from 1905 to 1907, and some among these began human experiments. An early effective serum therapy for meningococcal disease was introduced by Simon Flexner (1863-1946) at the Rockefeller Institute for Medical Research (where he was the director).

American physician-scientist Simon Flexner (1863-1946), c1905

(Photograph by Elias Goldensky, Philadelphia. Source: The World's Work: A History of Our Time. New York: Doubleday, Page & Company, 1904;9[2]:5550.)

Sulfonamides were introduced in 1937, but the emergence of resistance to sulfonamides in the 1960s stimulated the development of the first vaccines against meningococci.

In the first decade of the 20th century, untreated meningococcal meningitis had a case-fatality rate of 70% to 80% (151; 169). 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 (169). 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% (34; 17; 28; 169; 183; 15; 137; 164; 92; 130; 159; 162).

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 (137; 09; 55).

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