II. Epidemiology

  1. Most common in young adults and children with bimodal peak
    1. Age under 2 years accounts for 50% of cases
    2. Also peaks in teens
  2. Incidence
    1. Among top 2 causes of Bacterial Meningitis in U.S. (other is Streptococcal Pneumoniae Meningitis)
    2. Up to 2800 cases per year per year in the U.S.
    3. One case per 100,000 people
  3. Exposure risks
    1. Sporadic cases are most common
    2. Outbreaks in close quarters (e.g. Dormitory)

III. Pathophysiology

  1. Neisseria Meningitidis
    1. Gram Negative Cocci in pairs (diplococci)
    2. Normal oral flora in 5-10% (provides carriers natural Immunity after age 2 years)
  2. Transmission
    1. Respiratory secretions passed via aerosol or contact
  3. Polysaccharide capsule surrounds Neisseria Meningitidis
    1. Resistant to Phagocytosis in the absence of Opsonins (antibodies to capsule)
    2. Capsular Polysaccharides may become Antigens for Antibody response
    3. Serogroups are defined by the specific capsular Polysaccharides (13 have been defined)
      1. Meningitis is most commonly caused by serogroups A, B, C, as well as W-135, Y
  4. Endotoxin
    1. Lipopolysaccharide Endotoxin is released from Meningococcus
    2. Triggers vascular necrosis, Petechiae, Hemorrhage and Sepsis
  5. IgA1 Protease
    1. Enzyme expressed by some pathologic Meningococcus
    2. Lyses IgA in half
  6. Pili
    1. Allow Neisseria to adhere to the nasopharynx
    2. May also act as Antigen targets for immune response
  7. Other pathogenic mechanisms
    1. Extracts iron from human Transferrin

IV. Risk Factors

  1. Infants age 6 months to 2 years
    1. Vertical transmission of maternal Antibody provides protection for first few months
  2. Living in college dormitory or military barracks
    1. High rates of exposure to Meningococcus carriers with a wide variety of strains
  3. More common in white males
  4. Tobacco use or passive Tobacco exposure
  5. Recent Upper Respiratory Infection

V. Precautions: Presentations may be cryptic

  1. Nuchal Rigidity may be absent
  2. Severe Upper Respiratory Infection may be only initial presentation
  3. Petechiae and fever confers a 10-15% risk of Meningococcemia
  4. May present with concurrent other infections (e.g. Otitis Media, Conjunctivitis, Pneumonia)

VI. Symptoms

VII. Signs

  1. See Meningitis
  2. Fever
  3. Nuchal Rigidity may be absent
  4. Rash (70% of cases)
    1. Centripetal Rash (starts on distal extremities, wrists and ankles)
    2. May start with light pink maculopapular rash (may even blanch initially)
    3. Petechiae (non-blanching) are common
      1. If biopsied and cultures, petechial lesions will yield diplococci
    4. Fulminant Purpura (20% of cases)
      1. Central gun-metal gray center

VIII. Findings: Clinical Syndromes

  1. Meningococcemia
    1. Bacteremia resulting in toxic appearance, fever, chills, myalgias and petechial rash
  2. Waterhouse-Friderichsen Syndrome
    1. Fulminant Meningococcemia with bilateral adrenal Hemorrhage and Adrenal Insufficiency
    2. Presents with rapid progression within hours, Septic Shock, DIC, coma and very high mortality
  3. Bacterial Meningitis
    1. Most common presentation of Neisseria Meningitidis, typically combined with Meningococcemia

IX. Labs

  1. See Bacterial Meningitis
  2. Gram Stain
    1. Neisseria Meningitidis appears as Gram Negative diplocci under microscopy
    2. Each coccus is Kidney bean shaped, with pairs facing and overlapping one another
  3. Polymerase chain reaction (PCR)
    1. Neisseria Meningitidis may be identified from body fluids (e.g. blood, CSF)
  4. Culture
    1. Blood Agar Plates ("Chocolate agar")
      1. Named for the brown coloration after agar plates are heated
      2. Neisseria grow well on blood agar
    2. Thayer-Martin VCN Media
      1. Chocolate agar with 3 antimicrobials: Vancomycin, Colistin (Polymixin), Nystatin
      2. Antimicrobials kill all Gram Negatives except Neisseria, and all Gram Positives and fungi
    3. High CO2 Environment
      1. High CO2 concentrations further selects for Neisseria growth
    4. Acid production from Maltose Metabolism
      1. Distinguishes Neisseria Meningitidis (metabolizes maltose) from N. gonorrhoeae

X. Management

  1. See Bacterial Meningitis Management
  2. If considered, obtain Lumbar Puncture and start empiric Antibiotics immediately
  3. Manage shock
    1. See Sepsis (covers fluid boluses and pressor use)
    2. Consider adding Corticosteroids to the Antibiotic regimen
      1. Meningococcus may be associated with adrenal infarction Hemorrhage

XI. Complications

  1. Waterhouse-Friderichsen Syndrome
    1. Shock, Petechiae and adrenal infarction (bilateral adrenal Hemorrhage)

XII. Prevention

  1. See Meningococcal Vaccine
  2. Vaccination does on confer 100% protection (misses some sub-groups)
    1. Known exposures to Meningococcal Meningitis need Antibiotic prophylaxis despite Immunization

XIII. Prevention: Post-exposure Prophylaxis

  1. Indications
    1. Exposure from 7 days before onset of illness until 24 hours after Antibiotics initiated AND
    2. Significant exposure (treat if any concern)
      1. Household contacts (up to 800 fold increase in risk, up to 1 in 250 will be infected)
      2. Child care centers
      3. Oral secretion exposure (e.g. health care worker without adequate protection)
      4. Long-distance (>8 hours) travel next to source
  2. Prophylaxis options (pick one): Start as soon as possible (up to 14 days after exposure)
    1. Rifampin
      1. Age <1 month: 5 mg/kg orally every 12 hours for 2 days
      2. Age >1 month: 10 mg/kg (max: 600) orally every 12 hours for 2 days
      3. Adults: 600 mg orally every 12 hours for 2 days
    2. Ciprofloxacin (adults) 500 mg orally for one dose
    3. Ceftriaxone (Rocephin)
      1. Age <15 years: 125 mg IM for one dose
      2. Age >15 years: 250 mg IM for one dose
  3. References
    1. Bilukha (2005) MMWR Recomm Rep 54:1-21 [PubMed]

XIV. Prognosis

  1. Mortality: Approaches 14% despite treatment
  2. Serious residual morbidity approaches 19%

XV. References

  1. Claudius in Herbert (2012) EM:Rap 12(11): 8
  2. Kimmel (2005) Am Fam Physician 72:2049-56 [PubMed]

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Ontology: Meningococcal meningitis (C0025294)

Definition (MSH) A fulminant infection of the meninges and subarachnoid fluid by the bacterium NEISSERIA MENINGITIDIS, producing diffuse inflammation and peri-meningeal venous thromboses. Clinical manifestations include FEVER, nuchal rigidity, SEIZURES, severe HEADACHE, petechial rash, stupor, focal neurologic deficits, HYDROCEPHALUS, and COMA. The organism is usually transmitted via nasopharyngeal secretions and is a leading cause of meningitis in children and young adults. Organisms from Neisseria meningitidis serogroups A, B, C, Y, and W-135 have been reported to cause meningitis. (From Adams et al., Principles of Neurology, 6th ed, pp689-701; Curr Opin Pediatr 1998 Feb;10(1):13-8)
Concepts Disease or Syndrome (T047)
MSH D008585
ICD9 036.0
ICD10 A39.0
SnomedCT 28476005, 186363003, 154307005, 192644005
English Meningitis, Meningococcal, Meningitis, Meningococcic, Meningococcal Meningitis, MENINGOCOCCAL MENINGITIS, MENINGITIS MENINGOCOCCAL, meningococcal meningitis (diagnosis), meningococcal meningitis, Neisseria meningitides meningitis, Meningitis meningococcal, Meningitis, Meningococcal [Disease/Finding], Meningitis;meningococcal, meningitis meningococcal, meningococcic meningitis, epidemics meningitis, Epidemic meningitis, Meningococcal meningitis (disorder), Meningococcal meningitis (disorder) [Ambiguous], Meningococcal meningitis
French MENINGITE MENINGOCOCCIQUE, Méningite à Neisseria meningitides, Méningite cérébro-spinale, Méningite méningococcique, Méningite cérébrospinale à méningocoques, Méningite cérébrospinale, Méningite à Neisseria meningitidis, Méningite à méningocoques, Méningite à méningocoque
Portuguese MENINGITE MENINGOCOCICA, Meningite a Neisseria meningitidis, Meningite Meningoccócica, Meningite por Meningococos, Meningite meningocócica, Meningite Meningocócica
Italian Meningite menigococcica, Meningite da Neisseria meningitides, Meningite meningococcica
Dutch meningokokkenmeningitis, Neisseria meningitides meningitis, Meningitis door meningokokken, meningokokkenhersenvliesontsteking, Meningitis, meningokokken, Meningokokkenmeningitis
German Neisseria meningitides Meningitis, Meningokokken-Meningitis, MENINGITIS DURCH MENINGOKOKKEN, Meningokokkenmeningitis, Meningitis durch Meningokokken, Meningitis, Meningokokken-
Spanish Meningitis por Neisseria meningitidis, Meningitis Meningocóccica, meningitis epidémica, meningitis meningocócica (concepto no activo), meningitis meningocócica (trastorno), meningitis meningocócica, Meningitis meningocócica, Meningitis Meningocócica
Swedish Hjärnhinneinflammation, meningokock
Japanese ズイマクエンキンセイズイマクエン, 髄膜炎-髄膜炎菌性, 流行性脳脊髄膜炎, 流行性髄膜炎, 髄膜炎菌性脳脊髄膜炎, 髄膜炎菌性髄膜炎, 脳脊髄膜炎-流行性, 脳脊髄膜炎-髄膜炎菌性, 髄膜炎-流行性
Czech meningitida meningokoková, Meningitida vyvolaná Neisseria meningitidis, Meningokoková meningitida
Finnish Meningokokkimeningiitti
Russian MENINGIT MENINGOKOKKOVYI, МЕНИНГИТ МЕНИНГОКОККОВЫЙ
Korean 수막알균 수막염
Croatian MENINGITIS, MENINGOKOKNI
Polish Meningokokowe zapalenie opon mózgowo-rdzeniowych, Zapalenie opon meningokokowe
Hungarian Meningococcus okozta agyhártyagyulladás, meningococcalis meningitis, Neisseria meningitidis meningitis
Norwegian Meningokokkmeningitt

Ontology: Meningococcal Infections (C0025303)

Definition (MEDLINEPLUS)

Meningococci are a type of bacteria that cause serious infections. The most frequent is meningitis, which is an inflammation of the thin tissue that surrounds the brain and spinal cord. Meningococci can also cause other problems, including a serious bloodstream infection called sepsis.

Meningococcal infections can be spread from person to person. They are common in people living in close quarters, such as college students or military recruits.

In its early stages, you may have flu-like symptoms and a stiff neck. But the disease can progress quickly and can be fatal. Early diagnosis and treatment are extremely important. Treatment is with antibiotics. Since the infection spreads from person to person, family members may also need to be treated.

A vaccine can prevent meningococcal infections.

Definition (MSH) Infections with bacteria of the species NEISSERIA MENINGITIDIS.
Concepts Disease or Syndrome (T047)
MSH D008589
ICD9 036.9, 036
ICD10 A39 , A39.9
SnomedCT 186377000, 187328003, 154306001, 23511006
English Infections, Meningococcal, Meningococcal Infections, Infection, Meningococcal, Meningococcal Infection, Meningococal infectn,unspcf, Meningococcal infection, unspecified, [X]Meningococal infectn,unspcf, [X]Meningococcal infection, unspecified, INFECT MENINGOCOCCAL, MENINGOCOCCAL INFECT, meningococcal infection, meningococcal infection (diagnosis), Neisseria meningitidis infection NOS, Unspecified meningococcal infection, Meningococcal infect NOS, Meningococcal disease NOS, Meningococcal Infections [Disease/Finding], disease meningococcal, meningococcal diseases, meningococcal disease, meningococcal infections, Infection;meningococcal, Meningococcal infection NOS (disorder), [X]Meningococcal infection, unspecified (disorder), Meningococcal infection (disorder), Meningococcal infectious disease, Meningococcal infection, Infection due to Neisseria meningitidis, Meningococcal infectious disease (disorder), infection; meningococcal, meningococcal; infection, Meningococcal infection, NOS, Meningococcal infectious disease, NOS, Meningococcal infection NOS, Meningococcal diseases
Italian Infezione meningococcica, Infezione meningococcica, non specificata, Infezione meningococcica non specificata, Infezione da Neisseria meningitidis NAS, Infezioni meningococciche
Dutch Neisseria meningitidisinfectie NAO, meningokokkeninfectie, niet-gespecificeerd, niet-gespecificeerde meningokokkeninfectie, infectie; meningokokken, meningokokken; infectie, Meningokokkeninfectie, niet gespecificeerd, meningokokkeninfectie, Infectie, meningokokken-, Infecties, meningokokken-, Meningokokkeninfectie, Meningokokkeninfecties
French Infection méningococcique, non précisée, Infection méningococcique non précisée, Infection à méningocoques SAI, Infection méningococcique, Infections méningococciques, Infections à méningocoques, Méningococcies
German Neisseria meningitidis-Infektion NNB, unspezifische Meningokokken-Infektion, Meningokokken-Infektion unspezifisch, Meningokokkeninfektion, nicht naeher bezeichnet, Meningokokkeninfektion, Meningokokkeninfektionen
Portuguese Infecção a Neisseria meningitidis NE, Infecção meningocócica NE, Infecção miningocócica NE, Infecções por Meningococos, Infecções por Neisseria meningitidis, Infecção meningocócica, Infecções Meningocócicas
Spanish Infección meningocócica no especificada, Infección por Neisseria meningitidis NEOM, [X]infección meningocócica, no especificada, [X]infección meningocócica, no especificada (trastorno), infección meningocócica, SAI (trastorno), infección meningocócica, SAI, Infecciones por Meningococos, Infecciones por Neisseria meningitidis, enfermedad infecciosa meningocócica (trastorno), enfermedad infecciosa meningocócica, infección meningocócica (trastorno), infección meningocócica, infección por Neisseria meningitidis, Infección meningocócica, Infecciones Meningocócicas
Japanese 髄膜炎菌感染, 髄膜炎菌感染、詳細不明, 髄膜炎菌感染NOS, 詳細不明の髄膜炎菌感染, ズイマクエンキンカンセンNOS, ショウサイフメイノズイマクエンキンカンセン, ズイマクエンキンカンセンショウサイフメイ, ズイマクエンキンカンセン, 髄膜炎菌感染症
Swedish Meningokockinfektioner
Czech Meningococcus - infekce, Meningokoková infekce, Infekce Neisseria meningitidis NOS, Meningokoková infekce, blíže neurčená, Blíže neurčená meningokoková infekce
Finnish Meningokokki-infektiot
Russian MENINGOKOKKOVYE INFEKTSII, МЕНИНГОКОККОВЫЕ ИНФЕКЦИИ
Korean 수막알균 감염, 상세불명의 수막알균 감염
Croatian MENINGOKOKNE INFEKCIJE
Polish Zakażenie Neisseria meningitidis, Infekcja meningokokowa, Infekcje meningokokowe, Zakażenia meningokokowe
Hungarian nem meghatározott meningococcalis fertőzés, meningococcus fertőzés, Neisseria meningitidis fertőzés k.m.n., meningococcus fertőzés, nem meghatározott
Norwegian Meningokokkinfeksjoner

Ontology: Neisseria meningitidis (C0027575)

Definition (NCI_CDISC) Any bacterial organism that can be assigned to the species Neisseria meningitidis.
Definition (NCI) A species of aerobic, Gram-negative, diplococci shaped bacteria assigned to the phylum Proteobacteria. This species is catalase and oxidase positive, non-hemolytic, non-pigmented, does not reduce nitrate or nitrite, does not synthesize polysaccharides, grows on blood, chocolate, or Muller-Hinton agar, and produces acid from glucose and maltose but not fructose, sucrose, mannose, or lactose. N. meningitidis, known commonly as meningococcus, is the causative agent of cerebrospinal meningitis, but is also associated with other human infections, but can be commensal in the oropharynx and nasopharynx of carriers.
Definition (MSH) A species of gram-negative, aerobic BACTERIA. It is a commensal and pathogen only of humans, and can be carried asymptomatically in the NASOPHARYNX. When found in cerebrospinal fluid it is the causative agent of cerebrospinal meningitis (MENINGITIS, MENINGOCOCCAL). It is also found in venereal discharges and blood. There are at least 13 serogroups based on antigenic differences in the capsular polysaccharides; the ones causing most meningitis infections being A, B, C, Y, and W-135. Each serogroup can be further classified by serotype, serosubtype, and immunotype.
Definition (CSP) gram-negative, aerobic bacteria; a commensal and pathogen only of humans, and can be carried asymptomatically in the nasopharynx; when found in cerebrospinal fluid it is the causative agent of cerebrospinal meningitis; also found in venereal discharges and blood; there are at least 13 serogroups based on antigenic differences in the capsular polysaccharides; the ones causing most meningitis infections being A, B, C, Y, and W-135; each serogroup can be further classifed by serotype, serosubtype, and immunotype.
Concepts Bacterium (T007)
MSH D009345
SnomedCT 17872004
LNC LP16660-0, MTHU006713
English Micrococcus intracellularis, Meningococcus, Neisseria meningitidis, N. meningitidis, neisseria menningitidis, Neisseria meningitidis (Albrecht and Ghon 1901) Murray 1929, Beta-Lactamase Negative Neisseria meningitidis, meningitidis n, meningitidis neisseria, meningococcus, n. meningitidis, neisseria meningitidis, NEISSERIA MENINGITIDIS, Diplokokkus intracellularis meningitidis, Micrococcus meningitidis, Micrococcus meningitidis cerebrospinalis, Neisseria weichselbaumii, BETA-LACTAMASE NEGATIVE NEISSERIA MENINGITIDIS, Neisseria intracellularis, Neisseria meningitidis (organism)
French Meningococcus, Méningocoque, Neisseria meningitidis
Swedish Neisseria meningitidis
Czech Neisseria meningitidis
Finnish Meningokokki
Italian Meningococco, Neisseria meningitidis
Russian MENINGOKOKKI, NEISSERIA MENINGITIDIS, МЕНИНГОКОККИ
Japanese 髄膜炎菌, ナイセリア・メニンジティディス, ナイセリアメニンギチジス
Croatian NEISSERIA MENINGITIDIS, MENINGOKOK
Polish Dwoinki zapalenia opon mózgowych, Ziarenkowce oponowe, Neisseria meningitidis, Meningokoki
Norwegian Neisseria meningitidis, Meningococcus
Spanish Neisseria meningitidis (organismo), meningococo, Neisseria meningitidis, Meningococcus
German Meningokokkus, Neisseria meningitidis
Dutch Meningococcus, Meningokokken, Neisseria meningitidis
Portuguese Meningococcus, Neisseria meningitidis

Ontology: Waterhouse-Friderichsen Syndrome, Meningococcal (C1403891)

Concepts Disease or Syndrome (T047)
MSH D014884
ICD9 036.3
ICD10 A39.1
SnomedCT 154309008, 36102002
English Waterhouse-Friderichsen syndrome, meningococcal, Ac adr insuf + meningococ sept, Meningo haemorr adrenalitis, Waterhouse-Friderichsen synd, Friderichsen-Waterhouse syndrome, meningococcemia with Waterhouse-Friderichsen syndrome (diagnosis), meningococcemia with Waterhouse-Friderichsen syndrome, Meningococc adrenal synd, Meningococcic adrenal syndrome, Waterhouse-Friderichsen disease, Waterhouse-Friderichsen syndrome, Meningococcal Waterhouse-Friderichsen syndrome, Meningococcal adrenal syndrome, Meningococcal hemorrhagic adrenalitis, Acute adrenal insufficiency with meningococcal septicaemia, Acute adrenal insufficiency with meningococcal septicemia, Meningococcal haemorrhagic adrenalitis, Waterhouse-Friderichsen syndrome (disorder), infection; meningococcal, adrenalitis, hemorrhagic (manifestation), meningococcus; infection, adrenalitis, hemorrhagic (manifestation), Waterhouse-Friderichsen syndrome, NOS, Fulminant Meningococcal Sepsis with Adrenal Apoplexy, Adrenalitides, Meningococcal Hemorrhagic, Adrenalitis, Meningococcal Hemorrhagic, Hemorrhagic Adrenalitides, Meningococcal, Hemorrhagic Adrenalitis, Meningococcal, Meningococcal Hemorrhagic Adrenalitides, Meningococcal Hemorrhagic Adrenalitis, Meningococcal Waterhouse Friderichsen Syndrome, Syndrome, Meningococcal Waterhouse-Friderichsen, Waterhouse-Friderichsen Syndrome, Meningococcal, Meningococcal Waterhouse-Friderichsen Syndrome, Waterhouse-Friderichsen syndrome NOS
Italian Sindrome di Waterhouse-Friderichsen, Sindrome di Friderichsen-Waterhouse, Sindrome Waterhouse-Friderichsen, meningococcica, Sepsi meningococcica fulminante con apoplessia surrenalica, Sindrome di Waterhouse-Friderichsen meningococcica
Dutch syndroom van Waterhouse-Friderichsen, meningokokken, infectie; meningokokken, adrenalitis, hemorragisch, meningokokken; infectie, adrenalitis, hemorragisch, Syndroom van Waterhouse-Friderichsen, syndroom van Waterhouse-Friderichsen, Hemorragische meningokokkenadrenalitis, Meningokokkensepsis, fulminante
French Syndrome de Waterhouse-Friderichsen, méningococcique, Syndrome de Friderichsen-Waterhouse, Purpura Fulminans à méningocoques, Syndrome de Waterhouse-Friderichsen méningococcique, Syndrome de Waterhouse-Friderichsen, Surrénalite hémorragique à méningocoques, Syndrome méningococcique surrénalien
German Waterhouse-Friderichsen Syndrom, Meningokokken, Friderichsen-Waterhouse-Syndrom, Waterhouse-Friderichsen-Syndrom durch Meningokokken, Waterhouse-Friderichsen-Syndrom, Waterhouse-Friderichsen Syndrom, Fulminante Meningokokkensepsis mit Nebennierenapoplexie, Meningokokkensepsis, fulminante, Hämorrhagische Adrenalitis durch Meningokokken, Hämorrhagische Entzündung der Nebenniere durch Meningokokken
Portuguese Síndrome de Waterhouse-Friderichsen meningocócica, Sepse Meningocócica Fulminante com Apoplexia Suprarrenal, Síndrome de Waterhouse-Friderichsen, Adrenalite Meningocócica Hemorrágica
Spanish Síndrome de Waterhouse Friederichsen, Síndrome de Waterhouse-Friderichsen meningocócico, adrenalitis hemorrágica meningocócica, enfermedad de Waterhouse - Friderichsen, síndrome de Waterhouse - Friderichsen (trastorno), síndrome de Waterhouse - Friderichsen meningocócico, síndrome de Waterhouse - Friderichsen, síndrome suprarrenal meningocócico, Síndrome de Waterhouse-Friederichsen, Adrenalitis Meningocócica Hemorrágica, Sepsis Meningocóccica Fulminante con Apoplejia Suprarrenal
Japanese フリーデリクセン・ウォーターハウス症候群, ウォーターハウス・フリーデリクセン症候群, ウォータハウス・フリデリクセン症候群、髄膜炎菌性, ウォータハウスフリデリクセンショウコウグンズイマクエンキンセイ, フリーデリクセンウォーターハウスショウコウグン, ウォーターハウスフリーデリクセンショウコウグン
Czech Waterhouse-Friderichsenův syndrom, Meningokokový Waterhouse-Friderichsenův syndrom, Friderichsen-Waterhouseův syndrom
Korean 워터하우스-프리데릭센 증후군
Hungarian Waterhouse-Friderichsen-syndroma, meningococcalis, Waterhouse-Friderichsen-syndroma, Friderichsen-Waterhouse syndroma
Norwegian Fulminant meningokokksepsis med binyreinfarkt, Hemoragisk meningokokkadrenalitt