55 Kenosia Avenue
Danbury, CT 06810
Phone: 203.744.0100
Toll Free: 1.800.999.6673
http://rarediseases.org

Listeriosis

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 1989, 1992, 1997, 2004, 2009, 2012

NORD is very grateful to Benjamin J. Silk, PhD, MPH, Lieutenant Commander, U.S. Public Health Service and Barbara Mahon, MD, MPH, Deputy Chief, Enteric Diseases Epidemiology Branch, Centers for Disease Control and Prevention, for assistance in the preparation of this report.

Synonyms of Listeriosis

Disorder Subdivisions

General Discussion

Listeriosis is a rare but serious infectious disease caused by the bacterium Listeria monocytogenes, which is predominantly transmitted to humans through the consumption of contaminated food. Clinical syndromes associated with listeriosis include febrile gastroenteritis, invasive disease (sepsis, meningitis, meningoencephalitis), and fetal infections causing spontaneous abortions, stillbirth, premature labor, and neonatal disease. Approximately 20% of listeriosis cases are fatal. Listeriosis most often affects unborn fetuses, newborn infants, older adults, and individuals with weakened immune systems. Prompt recognition and treatment of the disease is necessary to avoid several serious complications.

Symptoms

Symptoms associated with listeriosis may vary widely. Nonpregnant adults most often present with bacteremia (bacteria in the blood), meningitis (infection of the meninges, the membrane surrounding the brain and spinal cord), or meningoencephalitis (infection of the meninges and brain). Common presenting symptoms in patients with bacteremia, meningitis, or meningoencephalitis include fever, muscle aches, headache, stiff neck, malaise, ataxia (loss of balance), convulsions, and mental status changes, such as confusion. Less common clinical syndromes include endocarditis (infection of the lining of the heart), pneumonia, osteomyelitis (infection of the bone), septic arthritis (infection of the joint), abscesses of the brain or liver, peritonitis (infection of the membranes lining the abdominal cavity), and endopthalmitis (infection of structures within the eye). Symptoms secondary to these conditions depend on the anatomical site of infection and may include abdominal pain, bone pain, respiratory distress, pneumonia, and heart failure. Outbreaks have demonstrated that listeriosis can cause febrile gastroenteritis, a noninvasive syndrome, in healthy individuals. Patients in these outbreaks typically experience diarrhea, fever/chills, fatigue, headache, abdominal pain, and nausea.

Listeriosis during pregnancy can result in fetal loss (spontaneous abortion and stillbirth), premature delivery, and neonatal bacteremia or meningitis. Listeriosis may occur at any time during pregnancy; however, it is most often detected in the third trimester. Among pregnant women diagnosed with listeriosis, most reported only mild flu-like symptoms including fever, muscle aches (myalgias), headaches, joint pain (arthralgia), and gastrointestinal symptoms. Some pregnant women do not recall having had any symptoms before the diagnosis of listeriosis in their infant. In contrast to maternal illness, fetal and newborn infections are serious and frequently (~30%) fatal. Infants with listeriosis (neonatal listeriosis) may present with fever, lethargy, irritability, diarrhea, poor feeding, vomiting, respiratory distress, or a characteristic skin rash consisting of widely spread, small, pale nodules (granulomatosis infantiseptica). Neonatal listeriosis may be classified as early-onset or late-onset. Early-onset neonatal listeriosis, which occurs in the first 7 days of life, is most often associated with bacteremia or sepsis. Early-onset infections occur following maternal bacteremia with transplacental transmission before birth. Late-onset neonatal listeriosis is most often associated with meningitis. The mode of transmission is less clear in late-onset listeriosis; environmental sources may be involved in some cases, and outbreaks of hospital-acquired, neonatal listeriosis have been reported.

Causes

Listeriosis is caused by the bacterium L. monocytogenes. Nearly all cases of listeriosis (other than neonatal listeriosis) occur from eating contaminated food products, especially ready-to-eat, refrigerated foods. Unlike most foodborne bacterial pathogens, L. monocytogenes can grow at refrigeration temperatures. Listeria organisms are commonly found in the environment, including in water, soil, mud, and decaying vegetation. L. monocytogenes can be introduced into abattoirs and food manufacturing plants, which results in the contamination of food products such as meat or dairy products, raw produce, and processed foods. Numerous outbreaks have been traced to contaminated food products such as unpasteurized or improperly pasteurized milk, soft cheeses, and dairy products, hot dogs, turkey delicatessen meats, and cantaloupe.

Although L. monocytogenes exposures are common, the interaction of several factors is thought to play a role in the development of invasive disease. These factors include the number of bacteria a person consumes (dose), the virulence of the bacterial strain, and the functioning of the patient's immune system.

Affected Populations

Invasive listeriosis usually affects pregnant women and their unborn fetuses, newborn infants, older adults, and individuals with weakened immune systems (immunocompromised). Medical risk factors for invasive listeriosis include hematologic malignancy, organ transplantation, HIV infection or AIDS, alcoholism, and liver and kidney disease. In the United States, L. monocytogenes accounts for ~5% of all cases of bacterial meningitis in infants <2 months of age and ~10% of all cases of bacterial meningitis in people =65years of age. Invasive listeriosis occurs very infrequently in young, healthy adults. Overall rates of invasive listeriosis in the United States have remained relatively constant over the past decade (~3.3 cases per million population), as measured where active surveillance for listeriosis has been conducted.

Related Disorders

Symptoms of the following disorders can be similar to those of listeriosis. Comparisons may be useful for a differential diagnosis:

The differential diagnosis of listeriosis includes all causes of meningitis or sepsis in newborn infants, such as group B streptococcus infection; all causes of meningitis and encephalitis in older adults and in individuals with weakened immune systems; and all causes of febrile illness during pregnancy, including viral syndromes.

Group B streptococcus (group B strep) is a type of bacteria that causes infection among newborns, pregnant women, or women after childbirth, women after gynecologic surgery, and older men and women with other serious diseases. Group B strep remains the most common cause among newborns (neonates) of infection of the blood and of the brain. The responsible bacterium, usually Streptococcus agalactiae, may be found most often in the vagina and rectum of females and may be transmitted as the infant passes through the birth canal. Group B strep disease is said to be early onset if it is obvious within the first week of life. It is said to be late onset if the disease is evident after the first week of life and before the end of the first three months. (For more information on this disorder, choose "Group B streptococcus" as your search term in the Rare Disease Database.)

Bacterial meningitis is a central nervous system infection caused by certain types of bacteria. Meningitis is characterized by inflammation of the membranes (meninges) around the brain or spinal cord. Inflammation can begin suddenly (acute) or develop gradually (subacute). Major symptoms may include fever, headache, and a stiff neck, sometimes with aching muscles. Nausea, vomiting and other symptoms may occur. Five types of bacteria are responsible for most cases of bacterial meningitis: Haemophilus influenzae, Streptococcus pneumoniae (pneumococcus), Group B streptococcus, Neisseria meningitidis (meningococcus), and Listeria monocytogenes (Listeria). (For more information on this disorder, choose "meningitis" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
A diagnosis of invasive listeriosis is confirmed through laboratory tests called cultures, which confirm the presence of L. monocytogenes in the body by isolating the bacteria from a clinical specimen. Cultures of blood, amniotic fluid, cerebrospinal fluid, placenta, or specimens from any affected organ systems may be performed to determine whether the bacterium L. monocytogenes is present. Antibodies against the bacteria can also be detected in blood samples, but serological tests are not commonly used in practice. Certain x-ray tests such as computed tomography (CT) scan or magnetic resonance imaging (MRI) might be used to detect abscesses that may form on internal organs, especially the brain or liver.

Treatment
Listeriosis is treated with antibiotics. The most commonly prescribed treatment is intravenous ampicillin. Many physicians also recommend treatment with the antibiotic gentamicin in combination with ampicillin. For affected individuals who cannot tolerate B-lactam antibiotics (such as ampicillin), trimethoprim-sulfamethoxazole is recommended. Erythromycin and vancomycin may also be used. Antibiotic treatment of pregnant women with documented listeriosis may prevent infection of the fetus. L. monocytogenes is resistant to all third generation cephalosporins. Other treatment is symptomatic and supportive.

Prevention
Knowing what foods to avoid and how to safely prepare and store foods is the best way to reduce the risk of listeriosis. This information is most important for those persons in groups at higher risk for invasive listeriosis, especially pregnant women, older adults, and persons who have weakened immune systems. To prevent listeriosis and other foodborne diseases, thoroughly cook all raw foods of animal origin, such as meat, because heat kills L. monocytogenes. Reheat leftovers or prepackaged foods, especially deli meats, until steaming hot. Wash fruits and vegetables thoroughly if eating raw. Do not eat or drink raw (unpasteurized) milk or other dairy products. Do not eat soft cheeses such as Brie, feta, and Mexican-style cheeses, unless they are labeled as made from pasteurized milk. Do not eat refrigerated smoked seafood, unless it is contained in a cooked dish, such as a casserole, or unless it is a canned or shelf-stable product. Follow recommended "sell by" or "best if used by" labels on processed foods. Keep unwashed and uncooked foods separate from foods that have been already prepared for eating.

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

Listeriosis Resources

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

Organizations:

References

TEXTBOOKS
Painter J, Slutsker L. Listeriosis in Humans. In: Ryser ET ME, ed. Listeria, Listeriosis, and Food Safety 3rd ed. Boca Raton, FL: Taylor and Francis Group, 2007:85-110.

Braden CR. Listeriosis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:290.

Slutsker L, Evans MC, Schuchat A. Listeriosis. In: Scheld WM, Craig WA, Hughes JM, eds. Emerging Infections. Washington DC: ASM Press, 2000:83-106.

JOURNAL ARTICLES
Jackson KA, Biggerstaff M, Tobin-D'Angelo, Sweat D, Klos R, Nosari J, Garrison O, Boothe E, Saathoff-Huber L, Hainstock L, Fagan RP. Multistate outbreak of Listeria monocytogenes associated with Mexican-style cheese made from pasteurized milk among pregnant, Hispanic women. J Food Prot. 2011;74(6):949-53.

Thigpen MC, Whitney CG, Messonnier NE, Zell ER, Lynfield R, Hadler JL, Harrison LH, Farley MM, Reingold A, Bennett NM, Craig AS, Schaffner W, Thomas A, Lewis MM, Scallan E, Schuchat A; Emerging Infections Programs Network. Bacterial meningitis in the United States, 1998-2007. N Engl J Med. 2011 May 26;364(21):2016-25.

Jackson KA, Iwamoto M, Swerdlow D. Pregnancy-associated listeriosis. Epidemiol Infect 2010;138(10):1503-9.

Swaminathan B, Gerner-Smidt P. The Epidemiology of Human Listeriosis. Microbes Infect. 2007;9(10):1236-43.

Voetsch AC, Angulo FJ, Jones TF, et al. Reduction in the incidence of invasive listeriosis in Foodborne Diseases Active Surveillance Network Sites, 1996-2003. Clin Infect Dis 2007;44:513-20.

Gottlieb SL, Newbern C, Griffin PM, et al. Multistate outbreak of listeriosis linked to turkey deli meat and subsequent changes in US regulatory policy. Clin Infect Dis 2006;42:29-36.

Arias Miranda IM, et al., Listeriosis in the adult. Revision of 10 cases. An Med Interna. 2004;21:75-8.

Roberts AJ, Wiedemann M. Pathogen, host and environmental factors contributing to the pathogenesis of listeriosis. Cell Mol Life Sci. 2003;60:904-18.

Benshushan A, et al., Listeria infection during pregnancy: a 10 year experience. Isr Med Assoc J. 2002;4:776-80.

Pierre V, et al., Prevention of Listeria infections. Bull Acad Natl Med. 2000;184:295-302.
Schlech WF. Foodborne listeriosis. Clin Infect Dis 2000;31;770-5.

Schlech III WF. Listeria gastroenteritis - new pathogen. N Engl J Med. 1997;336:130-2

Dalton CB, et al., An outbreak of gastroenteritis and fever due to Listeria monocytogenes in milk. N Engl J Med. 1997;366:100-5.

Schuchat A, Swaminathan B, Broome CV. Epidemiology of Human Listeriosis. Clin Micro Rev 1991;4;169-83.

Linnan MJ, Mascola L, Dong Lou X, et al. Epidemic listeriosis associated with Mexican-style cheese. N Engl J of Med 1988;319:823-8.

Teberg AJ, et al., Clinical manifestations of epidemic neonatal listeriosis. Pediatr Infect Dis J. 1987;6:817-20.

Boucher M, et al., Perinatal listeriosis (early-onset): correlation of antenatal manifestations and neonatal outcome. Obstet Gynecol. 1986;68:593-7.

INTERNET
Centers for Disease Control and Prevention. Listeriosis. www.cdc.gov/listeria. Updated January 23, 2012. Accessed February 7, 2012.

Zach T. Listeria Infection. Emedicine. . http://www.emedicine.com/ped/topic1319.htm. Updated Nov 15, 2011. Accessed February 7,2012.

Weinstein KB. Listeria Monocytogenes. Emedicine. 2003. http://emedicine.medscape.com/article/220684-overview. Updated: Jan 17, 2012 . Accessed February 7, 2012.

Report last updated: 2012/03/01 00:00:00 GMT+0