Meningococcal disease is caused by Neisseria meningitides. Meningococcal meningitis causes swelling of the tissues around the brain and spinal cord.

Meningococcal Disease

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Ismail Munir

Overview

Meningococcal disease is a life-threatening bacterial infection caused by Neisseria meningitidis, also known as meningococcus<a href="#accordion">[1]</a>. This disease encompasses two illnesses, namely, meningitis and septicemia that can either occur on their own or coexist<a href="#accordion">[2]</a>.&nbsp;Meningitis refers to the inflammation of the meninges which are the membranes that envelop the brain and spinal cord<a href="#accordion">[3]</a>. Septicemia is a term used to describe the rampant spread of infection in the blood. It is the state of blood poisoning by bacteria and is considered the extreme stage of infection<a href="#accordion">[4]</a>. Septicemia is generally a more critical condition as compared to meningitis since it causes widespread damage to the body very rapidly<a href="#accordion">[2]</a>.

Meningococcal disease if not treated by antibiotics can ultimately be fatal<a href="#accordion">[5]</a>.&nbsp;Moreover, this disease is associated with increased morbidity (suffering from a disease) and mortality (death rate)<a href="#accordion">[3]</a>. As per the Center for Disease Control (CDC), 10 to 15 percent of individuals affected with meningococcal meningitis die, despite receiving treatment with antibiotics. Furthermore, 1 out of 5 people who survive the infection, face long-term complications<a href="#accordion">[3]</a>.

Meningococcal disease is considered a medical emergency. It may present as fever, stiff neck, headache, severe bacteremia (seeding of bacteria in the blood), or hypovolemic shock<a href="#accordion">[6]</a>. Therefore, prompt treatment is integral for the survival of an affected individual. Moreover, half of all individuals with meningococcal meningitis present with skin rash<a href="#accordion">[6]</a>.

Meningococcal disease has a worldwide occurrence but the highest incidence of the disease is recorded in the &#39;meningitis belt&#39; of sub-Saharan Africa<a href="#accordion">[1]</a>.

Meningococcus is known to cause 1.2 million infections each year with 135,000 deaths globally. Every year, approximately 1000 people in the United States get meningococcal meningitis and septicemia<a href="#accordion">[3]</a>.

In Pakistan, roughly 23,000 children die each year due to bacterial meningitis, and Neisseria meningitidis and Streptococcus pneumoniae are the common culprits of this disease. However, which of the two organisms is the commonest cause of infection in Pakistan is not yet known<a href="#accordion">[7]</a>.

<div>&nbsp;</div>

Neisseria meningitidis or meningococcus exist as multiple types based on the different serogroups. So far twelve meningococcal serogroups have been identified out of which six main serogroups namely, A, B, C, W, X, and Y are largely responsible for causing the disease globally<a href="#accordion">[8].</a>&nbsp;Three of these major serogroups, B, C, and Y cause most of the disease in the US<a href="#accordion">[1].</a> Whereas, in the UK, serogroup B causes most of the disease<a href="#accordion">[2]</a>. According to WHO, 90% of meningococcal disease is caused by serogroups A, B, and C. However, groups Y and W135 are gaining importance due to recent outbreaks<a href="#accordion">[9]</a>.

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Signs And Symptoms

Meningococcal disease initially appears as a flu-like illness and the condition rapidly worsens; it can be fatal in a few hours if treatment is not commenced<a href="#accordion">[1]</a>. The meningococcal disease consists of meningitis and septicemia and both these conditions have different presentations 
&nbsp;

<h5>Meningococcal Meningitis</h5>

It develops when the infection reaches the meninges through breaks in the small vessels present within the membranes. Through the damage in the vessels, the bacteria leaks into the cerebrospinal fluid that is present between the layers of meninges and causes them to become inflamed and swollen. Subsequently, pressure develops inside the brain and causes nerve damage. The following are the signs and symptoms of meningitis<a href="#accordion">[1]</a>: 
&nbsp;

<ul>
	<li>High-grade fever&nbsp;</li>
	<li>Severe and persistent headache</li>
	<li>Neck stiffness&nbsp;</li>
	<li>Photophobia (avoidance of bright light)</li>
	<li>Nausea and vomiting&nbsp;</li>
	<li>Confusion or altered mental state</li>
	<li>Drowsiness&nbsp;</li>
	<li>Loss of consciousness&nbsp;</li>
	<li>Seizures&nbsp; 
	&nbsp;</li>
</ul>

It can be difficult to identify meningitis in babies since they do not present with the classic symptoms listed above. Babies who have meningococcal meningitis may have different symptoms of meningitis as compared to adults which may include<a href="#accordion">[3][6]</a>: 
&nbsp;

<ul>
	<li>High-pitched cry&nbsp;</li>
	<li>Tense or bulging soft spot on the skull</li>
	<li>Stiff and jerky movements&nbsp;</li>
	<li>Irritability&nbsp;</li>
	<li>Becoming slow or inactive&nbsp;</li>
	<li>Poor feeding&nbsp;</li>
	<li>Sleepy</li>
	<li>Difficulty breathing&nbsp;</li>
	<li>Fever 
	&nbsp;</li>
</ul>

<h5>Meningococcal Septicemia</h5>

is the state of rapid multiplication of bacteria in the blood. Consequently, there are increased levels of bacterial toxins in the blood and the body&#39;s immune system has little effect on these toxins. It eventually damages blood vessels causing a reddish or purplish rash that does not turn white when a glass is placed against it. Additionally, it causes damage to the whole body.&nbsp; 
&nbsp;

Following are the signs and symptoms of meningococcal septicemia<a href="#accordion">[2]</a>:

<ul>
	<li>Fever and chills&nbsp;</li>
	<li>Cold hands and feet</li>
	<li>Stomach cramps</li>
	<li>Diarrhea&nbsp;</li>
	<li>Increased breathing&nbsp;</li>
	<li>Dark purplish bruises or spots that do not fade under pressure&nbsp;</li>
	<li>Severe muscle aches (myalgia) and joint aches (arthralgia)</li>
	<li>Vomiting&nbsp;</li>
</ul>

<div>&nbsp;</div>

Causes

Meningococcal disease is caused by the bacterium Neisseria meningitidis, also known as meningococcus. This bacterium is the major culprit of meningitis in children and adolescents. Whereas, in adults, it is the second most common cause of the disease. It is transmitted through saliva or oral secretions by coughing, sneezing, and kissing<a href="#accordion">[2][3]</a>.

Usually, children and adults, both carry the bacterium harmlessly in the back of their throats<a href="#accordion">[2]</a>. Furthermore, 10% of the general population is carriers of Neisseria meningitidis and these individuals develop immunity to the bacteria and hence do not develop the disease. However, sometimes, meningococcus defeats the body&#39;s defense mechanisms and manages to penetrate the bloodstream through a break in the lining in the back of the throat. In the blood, bacteria multiply at an increased rate, doubling in number every 30 minutes. From the blood, the infection travels to the meninges causing them to become inflamed, and presents with the symptoms of meningitis<a href="#accordion">[2]</a>.&nbsp;&nbsp;

<div>&nbsp;</div>

Meningococcal disease is difficult to distinguish from other types of meningitis. In order to screen the disease, the doctor should take a detailed history to evaluate risk factors and any past travel history to an area of outbreak of the disease. Furthermore, a detailed physical examination will provide more clues regarding the presence of infection. Lastly, identification of the causative organism in the spinal fluid or blood will help with the screening process.&nbsp;

&nbsp;

Diagnosis

Meningococcal disease is difficult to diagnose since the presentation of this disease overlaps with other medical illnesses such as flu<a href="#accordion">[10]</a>. However, the key investigation to diagnose meningococcal disease is a spinal tap or lumbar puncture<a href="#accordion">[2]</a>.&nbsp;In this procedure, the doctor collects the spinal fluid which is sent to a laboratory for analysis along with blood samples where the presence of Neisseria meningitidis is observed<a href="#accordion">[1]</a>.

The bacteria can be seen through microscopic examination of the spinal fluid and it yields a diagnosis in 85% of individuals with meningococcal meningitis<a href="#accordion">[6]</a>.&nbsp;Additionally, diagnosis can be confirmed by collecting the bacteria from the sample of blood and spinal fluid and growing the organism on a specific medium. This helps to figure out which type of bacteria is causing the disease and what antibiotics are the most appropriate for treatment<a href="#accordion">[1]</a>.

Polymerase chain reaction (PCR) or rapid diagnostic tests can also support the diagnosis of meningococcal disease, in case other tests do not yield a conclusive diagnosis<a href="#accordion">[8]</a>.&nbsp;&nbsp;

<div>&nbsp;</div>

Treatment

Roughly half of the individuals with meningococcal disease die if they are not treated, hence this disease is considered as a medical emergency<a href="#accordion">[8]</a>. Initially, it may be difficult to differentiate meningococcal meningitis from meningitis. Therefore, empirical therapy with antibiotics that rapidly penetrates the brain should be initiated before the diagnosis is confirmed, keeping the patient&#39;s age and current disease status in mind. Delay in initiating this treatment will result in adverse outcomes.

Initial empirical therapy includes dexamethasone (a steroid), along with different antibiotics such as third-generation cephalosporin (ceftriaxone, cefotaxime), and vancomycin. For meningococcal meningitis that is not complicated, a 7-day course of intravenous ceftriaxone or penicillin is sufficient to treat the infection<a href="#accordion">[11]</a>.

Once diagnosis is established, certain changes can be made to the treatment regimen. Third-generation cephalosporins (ceftriaxone, cefotaxime) are currently regarded as the drug of choice to treat meningococcal disease, even in the setting of an epidemic<a href="#accordion">[8][11]</a>.&nbsp;Other antibiotics that can be used as an alternative include, Penicillin G, chloramphenicol, ampicillin, fluoroquinolone, and aztreonam<a href="#accordion">[11]</a>. These antibiotics are prescribed over a period of 7 to 10 days, however, it may take longer to regain strength and to feel better<a href="#accordion">[10]</a>.

Furthermore, depending on the severity of the infection, individuals with meningococcal disease are offered other supportive treatments such as oxygen for breathing support and medications to reduce blood pressure. Surgery may be advised in certain cases for the removal of dead tissues. Certain parts of the body may have areas of damaged skin due to rash, therefore, wound care is advised in this case<a href="#accordion">[1]</a>.

<div>&nbsp;</div>

Most individuals with meningococcal meningitis achieve full recovery, but 15 percent of individuals with this disease develop long-term complications. Due to multiple regions in the brain being damaged as a result of infection, various complications ensue owing to the compression of nerves<a href="#accordion">[2]</a>.&nbsp;Moreover, due to septicemia, toxins from the bacteria poison the blood and damage the vessels, which eventually compromises the blood flow to vital organs of the body such as the lungs, brain, heart, kidney, and skin.&nbsp; 
&nbsp;

Subsequently, a set of complications surface due to meningococcal infection and they are as follows<a href="#accordion">[1][3][8]</a>:

<ul>
	<li><a href="https://merisehat.pk/disease/acute-flaccid-paralysis">Paralysis</a></li>
	<li>Brain damage</li>
	<li>Gangrene</li>
	<li>Deafness</li>
	<li>Hearing loss</li>
	<li>Seizure</li>
	<li>Limb weakness&nbsp;</li>
	<li>Difficulty with speech, language, memory, and communication</li>
	<li>Difficulty with vision&nbsp;</li>
	<li>Scarring</li>
	<li>Limb amputation&nbsp;</li>
</ul>

<div>&nbsp;</div>

The presence of certain factors makes an individual prone to getting infected with meningococcal disease. They are as follows: 
&nbsp;

<ul>
	<li>Age</li>
</ul>

Meningococcal disease can affect individuals of any age group, however, certain age groups are at an increased risk. For instance, young children and specifically babies under one year of age are the most susceptible group to get infected since their immune system is not fully developed. The second high-risk group includes teenagers and young adults due to their increased carrier state owing to heightened social interactions. Lastly, the third at-risk group includes individuals over 55 years of age since the body&#39;s defenses are weakened, decreasing the body&#39;s ability to overcome the infection<a href="#accordion">[2]</a>. 
&nbsp;

<ul>
	<li>Living Arrangements</li>
</ul>

There is a high risk of transmission of infection in close settings. Those individuals who live in college dorms or military barracks are at an increased risk to acquire this infection. Additionally, individuals who live in overcrowded households and in refugee camps are also at great risk of becoming infected<a href="#accordion">[1][9]</a>. 
&nbsp;

<ul>
	<li>Certain Medical Conditions</li>
</ul>

Individuals with certain medical conditions are at an increased risk such as those with an absent or injured spleen, or those having sickle cell disease. Moreover, individuals with immunodeficiency such as those with HIV are also at high risk to acquire the infection. Lastly, individuals with upper respiratory infections are more susceptible to getting the disease<a href="#accordion">[10]</a>. 
&nbsp;

<ul>
	<li>Traveling History</li>
</ul>

Individuals who have traveled to areas with a high burden of meningococcal disease are at an increased risk to get the infection due to high exposure<a href="#accordion">[1]</a>. 
&nbsp;

<ul>
	<li>Occupation&nbsp;</li>
</ul>

Individuals who work in laboratory settings are exposed to bacteria, hence they are at high risk to get infected. Additionally, individuals who are working in the region of disease outbreaks are also at an increased risk.&nbsp; 
&nbsp;

<ul>
	<li>Smoking</li>
</ul>

Both active and passive smoking is associated with increased susceptibility to infection<a href="#accordion">[10]</a>.

<div>&nbsp;</div>

Meningococcal disease is very much preventable and vaccines remain the cornerstone of prevention. Through vaccination, individuals develop life-long immunity to disease and this reduces the disease burden. Individuals who have been in contact with an infected individual should get themselves vaccinated. The following vaccines are available for preventing meningococcal disease<a href="#accordion">[9]</a>: 
&nbsp;

<ul>
	<li>Meningococcal A vaccines&nbsp;</li>
	<li>Meningococcal C vaccines</li>
	<li>Meningococcal polysaccharide vaccines (unconjugated) 
	&nbsp;</li>
</ul>

Antibiotics are also an effective way to prevent infection. Individuals who have had close contact with someone with meningococcal disease are advised to receive antibiotics to prevent themselves from acquiring the disease and this is known as prophylaxis. Through prophylactic treatment, the spread of the disease can be interrupted<a href="#accordion">[1]</a>.

<div>&nbsp;</div>

According to recent research, the time interval between the suspicion of meningococcal disease to the institution of antimicrobial therapy should not be any longer than 30 minutes, since the fatality rate increases with delay in treatment. Moreover, the disease shows a seasonal variation, with most cases seen during winter<a href="#accordion">[12]</a>.

It has been observed that the levels of endotoxins present in the blood fall by 50 percent following the administration of antibiotics and Neisseria meningitidis is killed 3 to 4 hours after the treatment has commenced<a href="#accordion">[12]</a>.

While choosing one of the two cephalosporins, the age of the patient should be considered, since patients under the age of 3 months should be given cefotaxime preferentially. This is because hepatic enzymes are not fully developed in the neonatal period and ceftriaxone is known to increase indirect bilirubin which in severe cases may lead to kernicterus (brain damage caused by increased bilirubin levels) in infants<a href="#accordion">[12]</a>.

<div>&nbsp;</div>

Key Points

<ul>
	<li>Meningococcal disease is a life-threatening medical condition that should be rapidly treated to prevent the condition from worsening.&nbsp;</li>
	<li>Despite treatment 15 percent of individuals with meningococcal disease die and a fraction of them face long-term complications.</li>
	<li>It is transmitted from human to human through respiratory droplets and oral secretions.&nbsp;</li>
	<li>This disease presents with fever, headache, stiff neck, and a red purplish rash.</li>
	<li>Vaccines are the most effective way to prevent infection while antibiotics can be used as prophylaxis.</li>
	<li>Ceftriaxone is the drug of choice for this disease.</li>
	<li>Meningococcal meningitis is a notification priority disease. Whenever any case of this disease is diagnosed it should be immediately informed to the public health officials and the individuals who have been in contact with the infected person should be promptly offered vaccination and prophylaxis to prevent the infection from transmitting.&nbsp;</li>
</ul>

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