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Meningococcal Meningitis

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Copyright 1990, 1997, 1998, 2000, 2009, 2012

NORD is very grateful to Rino Rappuoli, PhD, Global Head of Vaccines Research, Novartis Vaccines and Diagnostics, Siena, Italy, for assistance in the preparation of this report.

Synonyms of Meningococcal Meningitis

Disorder Subdivisions

General Discussion

Meningococcal meningitis is a form of meningitis caused by a specific bacterium known as Neisseria meningitidis. Meningitis is characterized by inflammation of the membranes (meninges) around the brain or spinal cord. This inflammation can begin suddenly (acute) or develop gradually (subacute). Symptoms may include fever, headache, and a stiff neck, sometimes with aching muscles. Nausea, vomiting and other symptoms may also occur. Skin rashes occur in about half of all individuals with meningococcal meningitis.
Meningococcal meningitis is still associated with a high mortality rate and persistent neurological defects, particularly among infants and young children. Meningococcal meningitis without antibiotic therapy is uniformly fatal.

Symptoms

Meningococcal meningitis is one of the three most common types of bacterial meningitis. The incubation period averages 3-4 days (range 1-10 days), which is the period of communicability. It progresses more rapidly than any other acute form of bacterial meningitis. Meningococcal meningitis involves the central nervous system. In adults and children it is often preceded by respiratory illness or a sore throat. In its acute form, the disorder is characterized by fever, headache, a stiff neck, nausea, vomiting and altered mental state such as confusion or coma. Adults may become seriously ill within hours. In children the course of the infection may be even shorter.

Meningococcal meningitis evolves when the bacteria, Neisseria meningitidis (N.meningitidis) progresses from initial adherence to the nasopharyngeal (nose and throat) mucosa to invasion of the deeper mucosal layers (the submucosa). These bacteria rapidly multiply, and can lead to a mild (subclinical) infection. However, in approximately 10-20% of cases, the N.meningitidis enters the bloodstream (meningococcemia). This systemic form of the disease, meningococcemia, usually precedes the development of meningococcal meningitis by 24-48 hours.

Meningococcemia is characterized by severe, widespread vascular injury, with evidence of circulatory collapse and disseminated intravascular coagulation (DIC) Skin rashes occur in about half of all individuals with meningococcal meningitis. The rash is petechial (tiny, non-raised, purple-reddish lesions that do not blanch when pressed, and are the result of areas of intravascular bleeding.

Swelling or inflammation of the brain (cerebral edema or ventriculitis), or hydrocephalus (accumulation of fluid in the brain cavity) may also occur. Additional symptoms may include chills; sweating; weakness; loss of appetite; muscle pain (myalgia) of the lower back or legs; or inability to tolerate bright light (photophobia). (For more information on hydrocephalus, choose "hydrocephalus" as your search term in the Rare Disease Database).

Dehydration often occurs in individuals with meningococcal meningitis. In some cases, collapse of the blood vessels may lead to shock (Waterhouse-Friderichsen syndrome) when the meningococcus bacteria spread to the blood (septicemia). Later symptoms may include paralysis of one side of the body (hemiparesis), hearing loss, or additional neurological abnormalities.

The course of meningococcal meningitis is less predictable among infants between three months and two years of age. Fever, refusal of feedings, vomiting, irritability, and convulsions usually occur. A high-pitched cry and a bulging or tight soft spot (fontanel) on the crown of the head (where the parts of the skull's still unhardened bones join) may also occur. Since the incidence of most types of meningitis is highest among this age group, any unexplained fever needs to be closely watched. Cerebral fluid may accumulate just inside the tough outer membrane covering the brain (subdural effusions) after several days. Warning signs may include seizures, a persistent fever, and an enlarging head size. A brain abscess or subdural pus accumulation may also occur. Water accumulating in the brain (hydrocephalus), deafness and slowed mental and physical development are possible consequences of meningitis.

Causes

Meningococcal meningitis is caused by a bacterium known as Neisseria meningitidis. There are several types, or serogroups, of Neisseria meningitidis. The most common of these serogroups are A, B, C, D, X, Y, 29E, and W135. Serogroups A, B, C, and Y are responsible for most meningococcal diseases.

The bacterium is spread by droplets in the air or close contact with an infected person. It collects in the nasopharynx, or post-nasal space, that connects the nasal cavities with the throat. The bacterium is transported to the membranes (meninges) around the brain or spinal cord by the blood. It usually spreads from nearby infected areas such as the nasal sinuses or from the cerebrospinal fluid.

Affected Populations

Meningococcal meningitis primarily affects infants, children, and young adults. Males are affected slightly more than females, and account for 55% of all cases, with an incidence of 1.2 cases per 100,000 population, compared to 1 case per 100,000 population among females. Meningococcal meningitis can occur as an epidemic in subgroups such as people in the military services or students in dormitories. Vaccines can help control meningitis epidemics caused by serogroups A, B, C, Y, or W135.

The age-specific incidence of meningococcal disease is highest in young children, although the incidence of meningococcal disease in adolescents and college-aged young adults appears to have increased. College students living in dormitories seem to be the population at the most increased risk. This is due to the close proximity of students in college dormitories, which allows for faster spread of infection.

Related Disorders

Symptoms of the following disorders may resemble those of meningococcal meningitis. Comparisons may be useful for a differential diagnosis:

In general, meningitis is characterized by inflammation of the membranes (meninges) around the brain or spinal cord. This inflammation may be caused by different types of bacteria, viruses, fungi, malignant tumors, or reactions to certain injections into the spinal canal. (For more information on other types of Meningitis, choose "meningitis" as your search term in the Rare Disease Database.)

Encephalitis is a brain infection. There are different types of this disorder that are caused by different types of viruses. Encephalitis may also be caused by hypersensitivity initiated by a virus or proteins foreign to the body. Symptoms may include headache, drowsiness, hyperactivity, and/or general weakness. This disorder may have some symptoms similar to those of meningitis such as a stiff neck, altered reflexes, confusion, speech disorders, convulsions, paralysis and coma. (For more information choose "Encephalitis" as your search term in the Rare Disease Database.)

Rocky Mountain spotted fever is an acute infectious disorder transmitted to humans through the bite of an infected tick, usually in wooded areas of the midwest, eastern and southeastern United States. Fever and rash are among major symptoms. The rash may not develop in all cases, possibly making diagnosis difficult. Swelling (edema), headaches, chills, weakness, and muscle pains may also occur. Severe headaches, lethargy, confusion, delirium, focal neurological deficits, increased pressure in the skull leading to pressure on and swelling of the optic disk (papilledema), seizures and/or coma may occur in untreated cases as the nervous system is progressively affected. Some individuals may have a stiff neck due to muscle pain (myalgia) or irritation of membranes surrounding the brain and spinal cord tissue (meningismus). (For more information on this disorder, choose "Rocky Mountain Spotted Fever" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
Testing for meningococcal meningitis may include imaging techniques such as CT scans or magnetic resonance imaging (MRI). Other testing may include examination of the blood and/or skin. Diagnosis is made by laboratory examination of the cerebrospinal fluid that often reveals the presence of bacterial meningitis.

Treatment
There are 5 subtypes of N. meningitidis. Currently, (as of June, 2012) licensed vaccines for prevention of illness from 4 of the 5 subtypes exists. The vaccines are called Menveo, Menactra and Nimerix. Vaccines with narrow coverage have been used against serogroup B, and a vaccine with broad coverage is in late stage of development. Public health officials recommend that all college students take the meningococcal meningitis immunization. In particular, those who live in close quarters (dormitories, fraternities, and sororities), who frequent bars or consume alcohol, who smoke or are regularly around smokers are at higher risk and should consider vaccination. Students with certain chronic conditions (eg, have had their spleen removed) should be vaccinated. Students traveling to high-risk areas of the world (eg, sub-Sahara Africa) should consider vaccination. The conjugate vaccine is now also recommended for all children when they reach 11-12 years of age.

Meningococcal meningitis is usually treated with antibiotic drugs, administered intravenously, against the bacteria causing the infection. Initial treatment should always comprise a new generation cephalosporin (with or without Vancomycin) plus dexamethasone unless the patient is already under a specific antibiotic regimen.
The use of penicillins is limited due to their suboptimal penetration into the CSF. Early intravenous administration of dexamethasone is currently recommended as adjunctive therapy in an attempt to diminish the rate of permanent neurological sequelae. Antibiotic treatment is modified based on the CSF culture and antibiotic sensibility studies.

Family members of those infected can be treated with Rifampin as a preventative measure; however, for pregnant women, ceftriaxone is recommended.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Contact for additional information about meningococcal meningitis:

Rino Rappuoli, PhD
Global Head Vaccines Research
Novartis Vaccines and Diagnostics Srl
Via Fiorentina 1
53100 Siena, Italy
Ph: +39-0577-243414
Fax: +39-0577-278508
Email: rino.rappuoli@novartis.com

Organizations related to Meningococcal Meningitis

References

TEXTBOOKS
Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:1618-21.

JOURNAL ARTICLES
Nassif X. Microbiology. A furtive pathogen revealed. Science. 2000;287(5459):1767-8.

Ragunathan L, Ramsay M, Borrow R, Guiver M, Gray S, Kaczmarski EB. Clinical features, laboratory findings and management of meningococcal meningitis in England and Wales: report of a 1997 survey. Meningococcal meningitis: 1997 survey report. J Infect. 2000;40(1):74-9.

Kastenbauer S, Grabein B, Pfister HW. Prevention of meningococcal meningitis. Nervenarzt. 2000;71(2):134-7.

.Bonadio WA, Mannenbach M, Krippendorf R. Bacterial meningitis in older children. Am J Dis Child. 1990;144(4):463-5

Chang KH, Han MH, Roh JK, Kim IO, Han MC, Kim CW. Gd-DTPA-enhanced MR imaging of the brain in patients with meningitis: comparison with CT. AJR Am J Roentgenol. 1990;154(4):809-16.

Girgis NI, Abu el-Ella AH, Farid Z, Haberberger RL, Woody JN. Ceftriaxone alone compared to ampicillin and chloramphenicol in the treatment of bacterial meningitis. Chemotherapy. 1988;34 Suppl 1:16-20.

Masterton RG, Youngs ER, Wardle JC, Croft KF, Jones DM. Control of an outbreak of group C meningococcal meningitis with a polysaccharide vaccine. J Infect. 1988;17(2):177-82.

INTERNET
de Assis Aquino Gondim F, Singh MK, Croul SE. Meningococcal Meningitis. http://emedicine.medscape.com/article/1165557. Updated June 26, 2012. Accessed June 28, 2012.

Meningococcal Disease. Centers for Disease Control and Prevention. http://www.cdc.gov/meningococcal/vaccine-info.html. Updated March 15, 2012. Accessed June 28, 2012.

Smith DS, Hoffman TA, Chan JL. Meningococcal Infections. http://emedicine.medscape.com/article/221321. Updated October 17, 2011. Accessed June 28, 2012.

Report last updated: 2012/06/28 00:00:00 GMT+0