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Clinical manifestations of meningococcal infection

INTRODUCTION

Neisseria meningitidis is the leading cause of bacterial meningitis in children and young adults in the United States, with an overall mortality rate of 13 percent, and it is the second most common cause of community-acquired adult bacterial meningitis [1]. The clinical manifestations of meningococcal disease can be quite varied, ranging from transient fever and bacteremia to fulminant disease with death ensuing within hours of the onset of clinical symptoms.

The clinical manifestations of both meningococcal meningitis and meningococcemia will be reviewed here. The microbiology, pathobiology, epidemiology, diagnosis, treatment, and prevention of N. meningitidis infection are discussed separately. (See "Microbiology and pathobiology of Neisseria meningitidis" and "Epidemiology of Neisseria meningitidis infection" and "Diagnosis of meningococcal infection" and "Treatment and prevention of meningococcal infection".)

MENINGITIS AND ACUTE MENINGOCOCCEMIA

Acute systemic meningococcal disease is most frequently manifest by three syndromes [2]:

Meningitis

Meningitis with accompanying meningococcemia

                    

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Literature review current through: Oct 2014. | This topic last updated: Jul 16, 2014.
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References
Top
  1. Durand ML, Calderwood SB, Weber DJ, et al. Acute bacterial meningitis in adults. A review of 493 episodes. N Engl J Med 1993; 328:21.
  2. Wolf RE, Birbara CA. Meningococcal infections at an army training center. Am J Med 1968; 44:243.
  3. Kaplan SL, Schutze GE, Leake JA, et al. Multicenter surveillance of invasive meningococcal infections in children. Pediatrics 2006; 118:e979.
  4. Heckenberg SG, de Gans J, Brouwer MC, et al. Clinical features, outcome, and meningococcal genotype in 258 adults with meningococcal meningitis: a prospective cohort study. Medicine (Baltimore) 2008; 87:185.
  5. van de Beek D, de Gans J, Spanjaard L, et al. Clinical features and prognostic factors in adults with bacterial meningitis. N Engl J Med 2004; 351:1849.
  6. Thompson MJ, Ninis N, Perera R, et al. Clinical recognition of meningococcal disease in children and adolescents. Lancet 2006; 367:397.
  7. CARPENTER RR, PETERSDORF RG. The clinical spectrum of bacterial meningitis. Am J Med 1962; 33:262.
  8. Migeon CJ, Kenny FM, Hung W, Voorhess ML. Study of adrenal function in children with meningitis. Pediatrics 1967; 40:163.
  9. Winkelstein A, Songster CL, Caras TS, et al. Fulminant meningococcemia and disseminated intravascular coagulation. Arch Intern Med 1969; 124:55.
  10. McGehee WG, Rapaport SI, Hjort PF. Intravascular coagulation in fulminant meningococcemia. Ann Intern Med 1967; 67:250.
  11. Nieuwland R, Berckmans RJ, McGregor S, et al. Cellular origin and procoagulant properties of microparticles in meningococcal sepsis. Blood 2000; 95:930.
  12. Smith OP, White B, Vaughan D, et al. Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans. Lancet 1997; 350:1590.
  13. Alberio L, Lämmle B, Esmon CT. Protein C replacement in severe meningococcemia: rationale and clinical experience. Clin Infect Dis 2001; 32:1338.
  14. White B, Livingstone W, Murphy C, et al. An open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemia. Blood 2000; 96:3719.
  15. Faust SN, Levin M, Harrison OB, et al. Dysfunction of endothelial protein C activation in severe meningococcal sepsis. N Engl J Med 2001; 345:408.
  16. Darmstadt GL. Acute infectious purpura fulminans: pathogenesis and medical management. Pediatr Dermatol 1998; 15:169.
  17. Wong VK, Hitchcock W, Mason WH. Meningococcal infections in children: a review of 100 cases. Pediatr Infect Dis J 1989; 8:224.
  18. Algren JT, Lal S, Cutliff SA, Richman BJ. Predictors of outcome in acute meningococcal infection in children. Crit Care Med 1993; 21:447.
  19. Feigin RD, Dodge PR. Bacterial meningitis: newer concepts of pathophysiology and neurologic sequelae. Pediatr Clin North Am 1976; 23:541.
  20. Thomas HM. Meningococcic meningitis and septicemia. Report of an outbreak in the fourth service command during the winter and spring of 1942 to 1943. JAMA 1943; 123:264.
  21. Ducker TB, Simmons RL. The pathogenesis of meningitis. Systemic effect of meningococcal endotoxin within the cerebrospinal fluid. Arch Neurol 1968; 18:123.
  22. Hardman JM. Fatal meningococcal infections: the changing pathologic picture in the '60s. Mil Med 1968; 133:951.
  23. Levin S, Painter MB. The treatment of acute meningococcal infection in adults. A reappraisal. Ann Intern Med 1966; 64:1049.
  24. KANTER DM, LEARNER N, MAURIELLO DA. Acute meningococcemia with vascular collapse: an analysis of 10 recently treated cases. Am J Med Sci 1956; 232:674.
  25. Pathan N, Hemingway CA, Alizadeh AA, et al. Role of interleukin 6 in myocardial dysfunction of meningococcal septic shock. Lancet 2004; 363:203.
  26. GORE I, SAPHIR O. Myocarditis; a classification of 1402 cases. Am Heart J 1947; 34:827.
  27. Boucek MM, Boerth RC, Artman M, et al. Myocardial dysfunction in children with acute meningococcemia. J Pediatr 1984; 105:538.
  28. Benes J, Dzupova O, Kabelkova M, et al. Infective endocarditis due to Neisseria meningitidis: two case reports. Clin Microbiol Infect 2003; 9:1062.
  29. Gotshall RA. Conus medullaris syndrome after meningococcal meningitis. N Engl J Med 1972; 286:882.
  30. Sivalingam P, Tully AM. Acute meningococcal epiglottitis and septicaemia in a 65-y-old man. Scand J Infect Dis 1998; 30:196.
  31. Finkelstein Y, Adler Y, Nussinovitch M, et al. A new classification for pericarditis associated with meningococcal infection. Eur J Pediatr 1997; 156:585.
  32. Herman RA, Rubin HA. Meningococcal pericarditis without meningitis presenting as tamponade. N Engl J Med 1974; 290:143.
  33. Morse JR, Oretsky MI, Hudson JA. Pericarditis as a complication of meningococcal meningitis. Ann Intern Med 1971; 74:212.
  34. Pierce HI, Cooper EB. Meningococcal pericarditis. Clinical features andd therapy in five patients. Arch Intern Med 1972; 129:918.
  35. Maron BJ, Macoul KL, Benaron P. Unusual complications of meningococcal meningitis. Johns Hopkins Med J 1972; 131:64.
  36. Brasier AR, Macklis JD, Vaughan D, et al. Myopericarditis as an initial presentation of meningococcemia. Unusual manifestation of infection with serotype W135. Am J Med 1987; 82:641.
  37. Brandtzaeg P, Oktedalen O, Kierulf P, Opstad PK. Elevated VIP and endotoxin plasma levels in human gram-negative septic shock. Regul Pept 1989; 24:37.
  38. Brandtzaeg P, Kierulf P, Gaustad P, et al. Plasma endotoxin as a predictor of multiple organ failure and death in systemic meningococcal disease. J Infect Dis 1989; 159:195.
  39. Brandtzaeg P, Ovstebøo R, Kierulf P. Compartmentalization of lipopolysaccharide production correlates with clinical presentation in meningococcal disease. J Infect Dis 1992; 166:650.
  40. Brandtzaeg P, Mollnes TE, Kierulf P. Complement activation and endotoxin levels in systemic meningococcal disease. J Infect Dis 1989; 160:58.
  41. Brandtzaeg P, Sandset PM, Joø GB, et al. The quantitative association of plasma endotoxin, antithrombin, protein C, extrinsic pathway inhibitor and fibrinopeptide A in systemic meningococcal disease. Thromb Res 1989; 55:459.
  42. Brandtzaeg P, Bryn K, Kierulf P, et al. Meningococcal endotoxin in lethal septic shock plasma studied by gas chromatography, mass-spectrometry, ultracentrifugation, and electron microscopy. J Clin Invest 1992; 89:816.
  43. Meningococcal Vaccine Information Statement. National Immunization Program Webpage. www.cdc.gov/nip/publications/VIS/vis-mening.pdf.
  44. Sharip A, Sorvillo F, Redelings MD, et al. Population-based analysis of meningococcal disease mortality in the United States: 1990-2002. Pediatr Infect Dis J 2006; 25:191.
  45. Kim GY, Sorvillo F, Kuo T. Update on meningococcal disease mortality in the United States since 2002. Pediatr Infect Dis J 2010; 29:681.
  46. Frank ST, Gomez RM. Chronic meningococcemia. Mil Med 1968; 133:918.
  47. Sayre JW. Case 1: Chronic meningococcemia. Pediatr Rev 1992; 13:269.
  48. Rompalo AM, Hook EW 3rd, Roberts PL, et al. The acute arthritis-dermatitis syndrome. The changing importance of Neisseria gonorrhoeae and Neisseria meningitidis. Arch Intern Med 1987; 147:281.
  49. Nielsen HE, Koch C, Mansa B, et al. Complement and immunoglobulin studies in 15 cases of chronic meningococcemia: properdin deficiency and hypoimmunoglobulinemia. Scand J Infect Dis 1990; 22:31.
  50. Schaad UB. Arthritis in disease due to Neisseria meningitidis. Rev Infect Dis 1980; 2:880.
  51. Kerttula Y, Leinonen M, Koskela M, Mäkelä PH. The aetiology of pneumonia. Application of bacterial serology and basic laboratory methods. J Infect 1987; 14:21.
  52. Holm, MI, Davison, WC. Meningococcus pneumonia: I. The occurrence of post-influenzal pneumonia in which diplococcus intracellularis meningitidis was isolated. Bull Johns Hopkins Hosp 1919; 30:324.
  53. Koppes GM, Ellenbogen C, Gebhart RJ. Group Y meningococcal disease in United States Air Force recruits. Am J Med 1977; 62:661.
  54. Putsch RW, Hamilton JD, Wolinsky E. Neisseria meningitidis, a respiratory pathogen? J Infect Dis 1970; 121:48.
  55. Irwin RS, Woelk WK, Coudon WL 3rd. Primary meningococcal pneumonia. Ann Intern Med 1975; 82:493.
  56. Young LS, LaForce FM, Head JJ, et al. A simultaneous outbreak of meningococcal and influenza infections. N Engl J Med 1972; 287:5.
  57. TOBIN JL. Complications of meningococcus infection in a series of sixty-three consecutive sporadic cases. Am J Med Sci 1956; 231:241.
  58. McCracken GH Jr. Rapid identification of specific etiology in meningitis. J Pediatr 1976; 88:706.
  59. Miller MA, Millikin P, Griffin PS, et al. Neisseria meningitidis urethritis. A case report. JAMA 1979; 242:1656.
  60. Faur YC, Weisburd MH, Wilson ME. Isolation of Neisseria meningitidis from the Genito-urinary tract and anal canal. J Clin Microbiol 1975; 2:178.
  61. Salit IE, Frasch CE. Seroepidemiologic aspects of Neisseria meningitidis in homosexual men. Can Med Assoc J 1982; 126:38.
  62. Conde-Glez CJ, Calderón E. Urogenital infection due to meningococcus in men and women. Sex Transm Dis 1991; 18:72.
  63. Katz AR, Chasnoff R, Komeya A, Lee MV. Neisseria meningitidis urethritis: a case report highlighting clinical similarities to and epidemiological differences from gonococcal urethritis. Sex Transm Dis 2011; 38:439.
  64. Goedvolk CA, von Rosenstiel IA, Bos AP. Immune complex associated complications in the subacute phase of meningococcal disease: incidence and literature review. Arch Dis Child 2003; 88:927.
  65. Sullivan TD, LaScolea LJ Jr. Neisseria meningitidis bacteremia in children: quantitation of bacteremia and spontaneous clinical recovery without antibiotic therapy. Pediatrics 1987; 80:63.