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Rabies

Rabies

Definition

Rabies is an acute viral disease of the central nervous system that affects humans and other mammals but is most common in carnivores (flesh-eaters). It is sometimes referred to as a zoonosis, or disease of animals that can be communicated to humans. Rabies is almost exclusively transmitted through saliva from the bite of an infected animal. Another name for the disease is hydrophobia, which literally means "fear of water," a symptom shared by half of all people infected with rabies. Other symptoms include fever, depression, confusion, painful muscle spasms, sensitivity to touch, loud noise, and light, extreme thirst, painful swallowing, excessive salivation, and loss of muscle tone. If rabies is not prevented by immunization, it is almost always fatal.

Description

Cases of rabies in humans are very infrequent in the United States and Canada, averaging one or two a year (down from over 100 cases annually in 1900), but the worldwide incidence is estimated to be between 30,000 and 50,000 cases each year. These figures are based on data collected by the World Health Organization (WHO) in 1997 and updated in 2002. Rabies is most common in developing countries in Africa, Latin America, and Asia, particularly India. Dog bites are the major origin of infection for humans in developing countries, but other important host animals may include the wolf, mongoose, raccoon, jackal, and bat. A group of researchers in India found that monkeys as well as dogs were frequent vectors of rabies. The team also reported that the male:female ratio of rabies patients in India is 4:1.

Most deaths from rabies in the United States and Canada result from bat bites; the most recent fatality was a 66-year-old man in California who died in September 2003. The death of a nine-year-old girl in Quebec in the fall of 2000 was the first case of human rabies in Canada since 1985. Public health officials eventually determined that the girl had been bitten while she was sleeping by a silver-haired bat that had gotten into the family's home.

On October 18, 2004, a Wisconsin teenager was diagnosed with full-blown rabies after suffering from a minor bat bite on September 12, 2004. Miraculously, she was cured of rabies after doctors induced coma and administered four antiviral drugs to her. Since the therapy was only given and successful for one case, its curative properties needs to be corroborated by other cases before it will be considered a viable treatment option. The case and the physicians' findings will be published in a medical journal.

People whose work frequently brings them in contact with animals are considered to be at higher risk than the general population. This would include those in the fields of veterinary medicine, animal control, wildlife work, and laboratory work involving live rabies virus. People in these occupations and residents of or travelers to areas where rabies is a widespread problem should consider being immunized.

In late 2002, rabies re-emerged as an important public health issue. Dr. Charles E. Rupprecht, director of the World Health Organization (WHO) Collaborating Center for Rabies Reference and Research, has listed several factors responsible for the increase in the number of rabies cases worldwide:

  • Rapid evolution of the rabies virus. Bats in the United States have developed a particularly infectious form of the virus.
  • Increased diversity of animal hosts for the disease.
  • Changes in the environment that are bringing people and domestic pets into closer contact with infected wildlife.
  • Increased movement of people and animals across international borders. In one recent case, a man who had contracted rabies in the Philippines was not diagnosed until he began to feel ill in the United Kingdom.
  • Lack of advocacy about rabies.

Causes and symptoms

Rabies is caused by a rod- or bullet-shaped virus that belongs to the family Rhabdoviridae. The rabies virus is a member of a genus of viruses called lyssaviruses, which include several related viruses that infect insects as well as mammals. The rabies virus is usually transmitted via an animal bite, however, cases have also been reported in which the virus penetrated the body through infected saliva, moist tissues such as the eyes or lips, a scratch on the skin, or the transplantation of infected tissues. Inhalation of the virus in the air, as might occur in a highly populated bat cave, is also thought to occur.

From the bite or other area of penetration, the virus multiplies as it spreads along nerves that travel away from the spinal cord and brain (efferent nerves) and into the salivary glands. The rabies virus may lie dormant in the body for several weeks or months, but rarely much longer, before symptoms appear. Initially, the area around the bite may burn and be painful. Early symptoms may also include a sore throat, low-grade fever, headache, loss of appetite, nausea and vomiting, and diarrhea. Painful spasms develop in the muscles that control breathing and swallowing. The individual may begin to drool thick saliva and may have dilated or irregular pupils, increased tears and perspiration, and low blood pressure.

Later, as the disease progresses, the patient becomes agitated and combative and may exhibit increased mental confusion. The affected person usually becomes sensitive to touch, loud noises, and bright lights. The victim also becomes extremely thirsty, but is unable to drink because swallowing is painful. Some patients begin to dread water because of the painful spasms that occur. Other severe symptoms during the later stage of the disease include excessive salivation, dehydration, and loss of muscle tone. Death usually occurs three to 20 days after symptoms have developed. Unfortunately, recovery is very rare.

Diagnosis

After the onset of symptoms, blood tests and cerebrospinal fluid (CSF) analysis tests will be conducted. CSF will be collected during a procedure called a lumbar puncture in which a needle is used to withdraw a sample of CSF from the area around the spinal cord. The CSF tests do not confirm diagnosis but are useful in ruling out other potential causes for the patient's altered mental state.

The two most common diagnostic tests are the fluorescent antibody test and isolation of the rabies virus from an individual's saliva or throat culture. The fluorescent antibody test involves taking a small sample of skin (biopsy) from the back of the neck of the patient. If specific proteins, called antibodies, that are produced only in response to the rabies virus are present, they will bind with the fluorescent dye and become visible. Another diagnostic procedure involves taking a corneal impression in which a swab or slide is pressed lightly against the cornea of the eye to determine whether viral material is present.

Treatment

Until the most recent successful cure of a late-term rabies case can be validated with further success and validation from the medical community, the historic treatment options for rabies prevention immediately following a bite remains the most viable treatment. Because of the extremely serious nature of a rabies infection, the need for rabies immunizations will be carefully considered for anyone who has been bitten by an animal, based on a personal history and results of diagnostic tests.

If necessary, treatment includes the following:

  • The wound is washed thoroughly with medicinal soap and water. Deep puncture wounds should be flushed with a catheter and soapy water. Unless absolutely necessary, a wound should not be sutured.
  • Tetanus toxoid and antibiotics will usually be administered.
  • Rabies vaccination may or not be given, based on the available information. If the individual was bitten by a domestic animal and the animal was captured, the animal will be placed under observation in quarantine for ten days. If the animal does not develop rabies within four to seven days, then no immunizations are required. If the animal is suspected of being rabid, it is killed, and the brain is examined for evidence of rabies infection. In cases involving bites from domestic animals where the animal is not available for examination, the decision for vaccination is made based on the prevalence of rabies within the region where the bite occurred. If the bite was from a wild animal and the animal was captured, it is generally killed because the incubation period of rabies is unknown in most wild animals.
  • If necessary, the patient is vaccinated immediately, generally through the administration of human rabies immune globulin (HRIG) for passive immunization, followed by human diploid cell vaccine (HDCV) or rabies vaccine adsorbed (RVA) for active immunization. Passive immunization is designed to provide the individual with antibodies from an already immunized individual, while active immunization involves stimulating the individual's own immune system to produce antibodies against the rabies virus. Both rabies vaccines are equally effective and carry a lower risk of side effects than some earlier treatments. Unfortunately, however, in underdeveloped countries, these newer vaccines are usually not available. Antibodies are administered to the patient in a process called passive immunization. To do this, the HRIG vaccine is administered once, at the beginning of treatment. Half of the dose is given around the bite area, and the rest is given in the muscle. Inactivated viral material (antigenic) is then given to stimulate the patient's own immune system to produce antibodies against rabies. For active immunization, either the HDCV or RVA vaccine is given in a series of five injections. Immunizations are typically given on days one, three, seven, 14, and 28.

In those rare instances in which rabies has progressed beyond the point where immunization would be effective, the groundbreaking treatment involving a drug-induced coma and the administration of four different antiviral drugs will most likely be a radical treatment option. The traditional approach prior to October 2004 was to provide as much relief from pain and suffering as possible through medical intervention while waiting to see if survival was possible. The patient would be given medication to prevent seizures, relieve some of the anxiety, and relieve painful muscle spasms. Pain relievers would also be given. In the later stages, aggressive supportive care would be provided to maintain breathing and heart function. Survival via the traditional treatment is rare but can occur.

Prognosis

If preventative treatment is sought promptly, rabies need not be fatal. Immunization is almost always effective if started within two days of the bite. Chance of effectiveness declines, however, the longer vaccination is put off. It is, however, important to start immunizations, even if it has been weeks or months following a suspected rabid animal bite, because the vaccine can be effective even in these cases. If immunizations do not prove effective or are not received, rabies is nearly always fatal with a few days of the onset of symptoms.

KEY TERMS

Active immunization Treatment that provides immunity by challenging an individual's own immune system to produce antibody against a particular organism, in this case the rabies virus.

Antibody A specific protein produced by the immune system in response to a specific foreign protein or particle called an antigen.

Biopsy The removal of a small sample of tissue for diagnostic purposes.

Efferent nerves Nerves that convey impulses away from the central nervous system to the periphery.

Fluorescent antibody test (FA test) A test in which a fluorescent dye is linked to an antibody for diagnostic purposes.

Lumbar puncture A procedure that involves withdrawing a small sample of cerebrospinal fluid from the back around the spinal cord.

Lyssavirus A genus of viruses that includes the rabies virus and related viruses that infect insects as well as mammals.

Passive immunization Treatment that provides immunity through the transfer of antibodies obtained from an immune individual.

Rhabdovirus A type of virus named for its rod- or bullet-like shape. The rabies virus belongs to a family of viruses called Rhabdoviridae.

Vector An animal or insect that carries a disease-producing organism.

Zoonosis Any disease of animals that can be transmitted to humans. Rabies is an example of a zoonosis.

As of October 2004, the medical community awaits the publication of findings by the doctors that administered a life-saving treatment that cured a Wisconsin teenager of full-blown rabies. Further test cases will prove whether or not this treatment option will be a historic development in the search for a cure for rabies.

Prevention

One promising preventive strategy that has been used since the early 2000s is the distribution of wildlife baits containing an oral vaccine against rabies. This strategy has been used in Germany to vaccinate wild foxes, which are frequent carriers of the disease in Europe. In the United States, veterinary researchers at Kansas State University have developed an oral vaccine for fruit bats; early trials of the vaccine have given promising results.

The following precautions should be observed in environments where humans and animals may likely come into contact.

  • Domesticated animals, including household pets, should be vaccinated against rabies. If a pet is bitten by an animal suspected to have rabies, its owner should contact a veterinarian immediately and notify the local animal control authorities. Domestic pets with current vaccinations should be revaccinated immediately; unvaccinated dogs, cats, or ferrets are usually euthanized (put to sleep). Further information about domestic pets and rabies is available on the American Veterinary Medical Association (AVMA) web site.
  • Wild animals should not be touched or petted, no matter how friendly they may appear. It is also important not to touch an animal that appears ill or passive, or whose behavior seems odd, such as failing to show the normal fear of humans. These are all possible signs of rabies. Many animals, such as raccoons and skunks, are nocturnal and their activity during the day should be regarded as suspicious.
  • People should not interfere in fights between animals.
  • Because rabies is transmitted through saliva, a person should wear rubber gloves when handling a pet that has had an encounter with a wild animal.
  • Garbage or pet food should not be left outside the house or camp site because it may attract wild or stray animals.
  • Windows and doors should be screened. Some victims of rabies have been attacked by infected animals, particularly bats, that entered through unprotected openings.
  • State or county health departments should be consulted for information about the prevalence of rabies in an area. Some areas, such as New York City, have been rabies-free, only to have the disease reintroduced at a later time.
  • Preventative vaccination against rabies should be considered if one's occupation involves frequent contact with wild animals or non-immunized domestic animals.
  • Bites from mice, rats, or squirrels rarely require rabies prevention because these rodents are typically killed by any encounter with a larger, rabid animal, and would, therefore, not be carriers.
  • Travelers should ask about the prevalence of the disease in countries they plan to visit.

Resources

BOOKS

Beers, Mark H., MD, and Robert Berkow, MD., editors. "Central Nervous System Viral Diseases: Rabies (Hydrophobia)." Section 13, Chapter 162 In The Merck Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck Research Laboratories, 2004.

PERIODICALS

Chhabra, M., R. L. Ichhpujani, K. N. Tewari, and S. Lal. "Human Rabies in Delhi." Indian Journal of Pediatrics 71 (March 2004): 217-220.

Deshaies, D., P. A. Pilon, L. Valiquette, and J. Carsley. "A Public Health Intervention at the Time of a Case of Rabies in Quebec." [in French] Canadian Journal of Public Health 95 (March-April 2004): 138-141.

Fooks, A. R., N. Johnson, S. M. Brookes, et al. "Risk Factors Associated with Travel to Rabies Endemic Countries." Journal of Applied Microbiology 94, Supplement (2003): 31S-36S.

"Human Death Associated with Bat RabiesCalifornia, 2003." Morbidity and Mortality Weekly Report 53 (January 23, 2004): 33-35.

Messenger, S. L., J. S. Smith, L. A. Orciari, et al. "Emerging Pattern of Rabies Deaths and Increased Viral Infectivity." Emerging Infectious Diseases 9 (February 2003): 151-154.

Peters, C., R. Isaza, D. J. Heard, et al. "Vaccination of Egyptian Fruit Bats (Rousettus aegyptiacus ) with Monovalent Inactivated Rabies Vaccine." Journal of Zoo and Wildlife Medicine 35 (March 2004): 55-59.

Rosenthal, Elisabeth. "Girl is first to survive rabies without a shot." The New York Times November 25, 2004: A28.

Smith, J., L. McElhinney, G. Parsons, et al. "Case Report: Rapid Ante-Mortem Diagnosis of a Human Case of Rabies Imported Into the UK from the Philippines." Journal of Medical Virology 69 (January 2003): 150-155.

Stringer, C. "Post-Exposure Rabies Vaccination." Nursing Standard 17 (February 5-11, 2003): 41-42.

Thulke, H. H., T. Selhorst, T. Muller, et al. "Assessing Anti-Rabies BaitingWhat Happens on the Ground?" BMC Infectious Diseases 4 (March 9, 2004): 9.

Weiss, R. A. "Cross-Species Infections." Current Topics in Microbiology and Immunology 278 (2003): 47-71.

ORGANIZATIONS

American Veterinary Medical Association (AVMA). 1931 North Meacham Road, Suite 100, Schaumburg, IL 60173-4360. http://www.avma.org.

Centers for Disease Control and Prevention. 1600 Clifton Rd., NE, Atlanta, GA 30333. (800) 311-3435, (404) 639-3311. http://www.cdc.gov.

Institut Pasteur. 25-28, rue du Dr. Roux, 75015 Paris, France. +33 (0) 1 45 68 80 00. http://www.pasteur.fr/haut_ext.html.

OTHER

CDC. "Epidemiology of Rabies." http://www.cdc.gov/ncidod/dvrd/rabies/Epidemiology/Epidemiology.htm.

National Association of State Public Health Veterinarians, Inc. "Compendium of Animal Rabies Prevention and Control, 2003." Morbidity and Mortality Weekly Report Recommendations and Reports 52 (March 21, 2003) (RR-5): 1-6.

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Anderson, Janet; Frey, Rebecca. "Rabies." Gale Encyclopedia of Medicine, 3rd ed.. 2006. Encyclopedia.com. 25 Jul. 2016 <http://www.encyclopedia.com>.

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Rabies

Rabies

Definition

Rabies is an acute viral disease of the central nervous system that is transmitted through saliva from the bite of an infected animal.

Description

Rabies affects humans and other mammals but is most common in carnivores (flesh eaters). It is sometimes referred to as a zoonosis, or disease of animals that can be communicated to humans. Rabies is almost exclusively transmitted through saliva from the bite of an infected animal. Another name for the disease is hydrophobia, which literally means fear of water, a symptom shared by half of all people infected with rabies. Other symptoms include fever , depression, confusion, painful muscle spasms , sensitivity to touch, loud noise, and light, extreme thirst, painful swallowing, excessive salivation, and loss of muscle tone. If rabies is not prevented by immunization, it is almost always fatal.

In late 2002, rabies re-emerged as an important public health issue. Charles E. Rupprecht, director of the World Health Organization (WHO) Collaborating Center for Rabies Reference and Research, listed several factors responsible for the increase in the number of rabies cases worldwide:

  • Rapid evolution of the rabies virus. Bats in the United States have developed a particularly infectious form of the virus.
  • Increased diversity of animal hosts for the disease.
  • Changes in the environment that are bringing people and domestic pets into closer contact with infected wildlife.
  • Increased movement of people and animals across international borders. In one case, a man who had contracted rabies in the Philippines was not diagnosed until he began to feel ill in the United Kingdom.
  • Lack of advocacy about rabies.

Demographics

Cases of rabies in humans are very infrequent in the United States, averaging one or two a year (down from over 100 cases annually in 1900), but the worldwide incidence is estimated to be between 30,000 and 50,000 cases each year. These figures are based on data collected by the World Health Organization (WHO) in 1997 and updated in 2002. Rabies is most common in developing countries in Africa, Latin America, and Asia, particularly India. Dog bites are the major origin of infection for humans in developing countries, but other important host animals are the wolf, mongoose, and bat. Worldwide, the highest risk groups for contracting rabies are boys under the age of fifteen. Most deaths from rabies in the United States result from bat bites.

People whose work frequently brings them in contact with animals are also considered to be at higher risk than the general population. This group includes those in the fields of veterinary medicine, animal control, wildlife work, and laboratory work involving live rabies virus. People in these occupations and residents of or travelers to areas where rabies is a widespread problem should consider being immunized.

Causes and symptoms

Rabies is caused by a rod- or bullet-shaped virus that belongs to the family Rhabdoviridae. The virus is usually transmitted via an animal bite; however, cases have also been reported in which the virus penetrated the body through infected saliva, moist tissues such as the eyes or lips, a scratch on the skin, or the transplantation of infected tissues. Inhalation of the virus from the air, as might occur in a highly populated bat cave, is also thought to occur.

From the bite or other area of penetration, the virus multiplies as it spreads along nerves that travel away from the spinal cord and brain (efferent nerves) and into the salivary glands. The rabies virus may lie dormant in the body for several weeks or months, but rarely much longer, before symptoms appear. Initially, the area around the bite may burn and be painful. Early symptoms may also include a sore throat , low-grade fever, headaches, loss of appetite, nausea and vomiting , and diarrhea . Painful spasms develop in the muscles that control breathing and swallowing. The individual may begin to drool thick saliva and may have dilated or irregular pupils, increased tears and perspiration, and low blood pressure.

As the disease progresses, the patient becomes agitated and combative and may exhibit increased mental confusion. The affected person usually becomes sensitive to touch, loud noises, and bright lights. The victim also becomes extremely thirsty but is unable to drink because swallowing is painful. Some patients begin to dread water because of the painful spasms that occur. Other severe symptoms during the later stage of the disease are excessive salivation, dehydration , and loss of muscle tone. Death usually occurs three to 20 days after symptoms have developed. Recovery is very rare.

Diagnosis

After the onset of symptoms, blood tests and cerebrospinal fluid (CSF) analysis tests will be conducted. CSF will be collected during a procedure called a lumbar puncture in which a needle is used to withdraw a sample of CSF from the area around the spinal cord. The CSF tests do not confirm diagnosis but are useful in ruling out other potential causes for the patient's altered mental state.

The two most common diagnostic tests are the fluorescent antibody test and isolation of the rabies virus from an individual's saliva or throat culture . The fluorescent antibody test involves taking a small sample of skin (biopsy) from the back of the neck of the patient. If specific proteins, called antibodies, that are produced only in response to the rabies virus are present, they will bind with the fluorescent dye and become visible. Another diagnostic procedure involves taking a corneal impression in which a swab or slide is pressed lightly against the cornea of the eye to determine whether viral material is present.

Treatment

Because of the extremely serious nature of a rabies infection, the need for rabies immunizations should be carefully considered for anyone who has been bitten by an animal, based on a personal history and results of diagnostic tests.

If necessary, treatment includes the following:

  • The wound is washed thoroughly with medicinal soap and water. Deep puncture wounds should be flushed with a catheter and soapy water. Unless absolutely necessary, a wound should not be sutured.
  • Tetanus toxoid and antibiotics will usually be administered.
  • Rabies vaccination may or not be given, based on the available information. If the individual was bitten by a domestic animal and the animal was captured, the animal will be placed under observation in quarantine for ten days. If the animal does not develop rabies within four to seven days, then no immunizations are required. If the animal is suspected of being rabid, it is killed, and the brain is examined for evidence of rabies infection. In cases involving bites from domestic animals in which the animal is not available for examination, the decision for vaccination is made based on the prevalence of rabies within the region where the bite occurred. If the bite was from a wild animal and the animal was captured, it is generally killed because the incubation period of rabies is unknown in most wild animals.
  • If necessary, the patient is vaccinated immediately, generally through the administration of human rabies immune globulin (HRIG) for passive immunization, followed by human diploid cell vaccine (HDCV) or rabies vaccine adsorbed (RVA) for active immunization. Passive immunization is designed to provide the individual with antibodies from an already immunized individual, while active immunization involves stimulating the individual's own immune system to produce antibodies against the rabies virus. These rabies vaccines are equally effective and carry a lower risk of side effects than some earlier treatments. Unfortunately, however, in underdeveloped countries, these vaccines are usually not available. Antibodies are administered to the patient in a process called passive immunization. To do so, the HRIG vaccine is administered once, at the beginning of treatment. Half of the dose is given around the bite area, and the rest is given in the muscle. Inactivated viral material (antigenic) is then given to stimulate the patient's own immune system to produce antibodies against rabies. For active immunization, either the HDCV or RVA vaccine is given in a series of five injections. Immunizations are typically given on days 1, 3, 7, 14, and 28.

In those rare instances in which rabies has progressed beyond the point where immunization would be effective, the patient is given medication to prevent seizures, relieve some of the anxiety , and relieve painful muscle spasms. Pain relievers are also given. In the later stages, aggressive supportive care will be provided to maintain breathing and heart function. Survival is rare but can occur.

Prognosis

If preventative treatment is sought promptly, rabies need not be fatal. Immunization is almost always effective if started within two days of the bite. Chance of effectiveness declines, however, the longer vaccination is put off. It is, however, important to start immunizations, even if it has been weeks or months following a suspected rabid animal bite, because the vaccine can be effective even in these cases. If immunizations do not prove effective or are not received, rabies is nearly always fatal within a few days of the onset of symptoms.

Prevention

The following precautions should be observed in environments where humans and animals are likely to come into contact:

KEY TERMS

Active immunization Treatment that provides immunity by challenging an individual's own immune system to produce antibody against a particular organism.

Antibody A special protein made by the body's immune system as a defense against foreign material (bacteria, viruses, etc.) that enters the body. It is uniquely designed to attack and neutralize the specific antigen that triggered the immune response.

Biopsy The surgical removal and microscopic examination of living tissue for diagnostic purposes or to follow the course of a disease. Most commonly the term refers to the collection and analysis of tissue from a suspected tumor to establish malignancy.

Efferent nerves Peripheral nerves that carry signals away from the brain and spinal cord.

Fluorescent antibody test A test in which a fluorescent dye is linked to an antibody for diagnostic purposes.

Lumbar puncture A procedure in which the doctor inserts a small needle into the spinal cavity in the lower back to withdraw spinal fluid for testing. Also known as a spinal tap.

Passive immunization Treatment that provides immunity through the transfer of antibodies obtained from an immune individual.

Rhabdovirus A family of viruses named for their rod- or bullet-like shapes. The rabies virus is a rhabdovirus.

Vector A carrier organism (such as a fly or mosquito) which serves to deliver a virus (or other agent of infection) to a host. Also refers to a retrovirus that had been modified and is used to introduce specific genes into the genome of an organism.

Zoonosis Any disease of animals that can be transmitted to humans. Rabies is an example of a zoonosis.

  • Domesticated animals, including household pets, should be vaccinated against rabies. If a pet is bitten by an animal suspected to have rabies, its owner should contact a veterinarian immediately and notify the local animal control authorities. Domestic pets with current vaccinations should be revaccinated immediately; unvaccinated dogs, cats, or ferrets are usually euthanized (killed).
  • Wild animals should not be touched or petted, no matter how friendly they may appear. It is also important not to touch an animal that appears ill or passive or whose behavior seems odd, such as failing to show the normal fear of humans. These are all possible signs of rabies. Many animals, such as raccoons and skunks, are nocturnal and their activity during the day should be regarded as suspicious.
  • People should not interfere in fights between animals.
  • Because rabies is transmitted through saliva, a person should wear rubber gloves when handling a pet that has had an encounter with a wild animal.
  • Garbage or pet food should not be left outside the house or camp site because it may attract wild or stray animals.
  • Windows and doors should be screened. Some victims of rabies have been attacked by infected animals, particularly bats, that entered through unprotected openings.
  • State or county health departments should be consulted for information about the prevalence of rabies in an area. Some areas, such as New York City, have been rabies-free, only to have the disease reintroduced at a later time.
  • Preventative vaccination against rabies should be considered if one's occupation involves frequent contact with wild animals or non-immunized domestic animals.
  • Bites from mice, rats, or squirrels rarely require rabies prevention because these rodents are typically killed by any encounter with a larger, rabid animal, and would, therefore, not be carriers.
  • Travelers should ask about the prevalence of the disease in countries they plan to visit.

Parental concerns

Parents should speak with their children about the importance of avoiding contact with wild animals and reporting strange behavior in any animal, even a pet.

Resources

BOOKS

Adams, William G. "Rabies." In Nelson Textbook of Pediatrics. Edited by Richard E. Behrman et al. Philadelphia: Saunders, 2004.

Plotkin, Stanley A. "Rabies Virus." In Principles and Practice of Pediatric Infectious Diseases, 2nd ed. Edited by Sarah S. Long et al. St. Louis, MO: Elsevier, 2003.

PERIODICALS

Fooks, A. R., et al. "Risk Factors Associated with Travel to Rabies Endemic Countries." Journal of Applied Microbiology 94 Suppl. (2003): 31S6S.

"Human RabiesIowa, 2002." Morbidity and Mortality Weekly Report 52 (January 24, 2003): 478.

Messenger, S. L., et al. "Emerging Pattern of Rabies Deaths and Increased Viral Infectivity." Emerging Infectious Diseases 9 (February 2003): 1514.

National Association of State Public Health Veterinarians Inc. "Compendium of Animal Rabies Prevention and Control, 2003." Morbidity and Mortality Weekly Report Recommendations and Reports 52 (March 21, 2003): 16.

Smith, J., et al. "Case Report: Rapid Ante-Mortem Diagnosis of a Human Case of Rabies Imported into the UK from the Philippines." Journal of Medical Virology 69 (January 2003): 1505.

Stringer, C. "Post-Exposure Rabies Vaccination." Nursing Standard 17 (February 511, 2003): 412.

Weiss, R. A. "Cross-Species Infections." Current Topics in Microbiology and Immunology 278 (2003): 4771.

ORGANIZATIONS

American Veterinary Medical Association (AVMA). 1931 North Meacham Road, Suite 100, Schaumburg, IL 601734360. Web site: <www.avma.org>.

Centers for Disease Control and Prevention. 1600 Clifton Rd., NE, Atlanta, GA 30333. Web site: <www.cdc.gov>.

WEB SITES

"Epidemiology [of rabies]." National Center for Infectious Diseases. Available online at <www.cdc.gov/ncidod/dvrd/rabies/Epidemiology/Epidemiology.htm> (accessed January 9, 2005).

Janet Byron Anderson Rebecca J. Frey, PhD Rosalyn Carson-DeWitt, MD

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Anderson, Janet; Frey, Rebecca; Carson-DeWitt, Rosalyn. "Rabies." Gale Encyclopedia of Children's Health: Infancy through Adolescence. 2006. Encyclopedia.com. 25 Jul. 2016 <http://www.encyclopedia.com>.

Anderson, Janet; Frey, Rebecca; Carson-DeWitt, Rosalyn. "Rabies." Gale Encyclopedia of Children's Health: Infancy through Adolescence. 2006. Encyclopedia.com. (July 25, 2016). http://www.encyclopedia.com/doc/1G2-3447200471.html

Anderson, Janet; Frey, Rebecca; Carson-DeWitt, Rosalyn. "Rabies." Gale Encyclopedia of Children's Health: Infancy through Adolescence. 2006. Retrieved July 25, 2016 from Encyclopedia.com: http://www.encyclopedia.com/doc/1G2-3447200471.html

Rabies

Rabies

Definition

Rabies is a viral illness that can affect any mammal but is most common in carnivores (flesh-eaters). It is sometimes referred to as a zoonosis, or disease of animals that can be communicated to humans. Rabies is usually transmitted in the saliva through a bite wound. The virus attacks the central nervous system, and is fatal once symptoms begin, with very rare exceptions.

Description

Rabies, also known as hydrophobia, belongs to the rhabdovirus family. Fewer than 10% of animal cases reported in the United States in 1998 were in domestic animals. Raccoons accounted for the largest number of cases in wild animals. Cases of rabies in humans are very infrequent in the United States, averaging one or two a year (down from over 100 cases annually in 1900), but the worldwide incidence is estimated to be between 30,000 and 50,000 cases each year. These figures are based on data collected by the World Health Organization (WHO) in 1997 and updated in 2002. Rabies is most common in developing countries in Africa, Latin America, and Asia, particularly India. Dog bites are the major origin of infection for humans in developing countries, but other important host animals may include the wolf, mongoose, and bat. Most deaths from rabies in the United States result from bat bites; the most recent victim was a man in Iowa who died in September 2002.

People whose work frequently brings them in contact with animals are considered to be at higher risk than the general population. This would include those in the fields of veterinary medicine, animal control, wildlife work, and laboratory work involving live rabies virus. People in these occupations and residents of or travelers to areas where rabies is a widespread problem should consider being immunized.

In late 2002, rabies re-emerged as an important public health issue. Dr. Charles E. Rupprecht, director of the World Health Organization (WHO) Collaborating Center for Rabies Reference and Research, has listed several factors responsible for the increase in the number of rabies cases worldwide:

  • Rapid evolution of the rabies virus. Bats in the United States have developed a particularly infectious form of the virus.
  • Increased diversity of animal hosts for the disease.
  • Changes in the environment that are bringing people and domestic pets into closer contact with infected wildlife.
  • Increased movement of people and animals across international borders. In one recent case, a man who had contracted rabies in the Philippines was not diagnosed until he began to feel ill in the United Kingdom.
  • Lack of advocacy about rabies.

Causes & symptoms

The most common way to contract rabies is from the bite of an infected animal. Although bats are the most frequent source of human infection in the United States, dogs are the primary vector of rabies in most parts of the world. The disease may also be transmitted by tissues and body fluids other than saliva. Rare cases have occurred as a result of infection through corneal transplantation.

Rabies travels from the site of the bite along the peripheral nerves to the brain. The average incubation period in humans is 3050 days, although it varies from 10 days to over a year. The initial symptoms are flu-like and nonspecific. They may include fever, headache , muscle pain, sore throat, fatigue, nausea , and vomiting . Altered sensation and muscle twitching in the area of the bite are signs that are more suspicious of rabies. When the virus reaches the brain, signs related to encephalitis (local or general inflammation of brain tissue) appear. This typically involves agitation, progressing to confusion,

combativeness, seizures, and localized areas of paralysis. There may also be hypersensitivity to light, sound, and touch. The patient may be coherent at times, but less so as the disease progresses. Many viruses causing encephalitis may produce similar signs. The next stage is dysfunction of the brainstem. The well-known phenomenon of foaming at the mouth is caused by excessive saliva production combined with difficulty swallowing. Many patients will refuse liquids at this point due to the painful muscle contractions caused by swallowing. This is how rabies came to be known as hydrophobia, which means "fear of water." Coma ensues soon after brainstem involvement, and death occurs when the respiratory center is affected. The course of the disease is four to 20 days after symptoms appear, unless life support is used.

Diagnosis

Early in its course, and without a known history of an animal bite, rabies can be difficult to diagnose. Symptoms of the early encephalitic (brain tissue inflammation) phase are similar to those of most viral types of encephalitis. When signs of brainstem dysfunction appear shortly after this time, rabies becomes a more likely possibility. Several tests are available for rabies diagnosis, but none are extremely reliable in the living patient. Part of the challenge is that rabies is so limited to nerve tissue until the late phases of the disease. The examination of brain tissue reveals a characteristic known as a Negri body, which is diagnostic. Direct fluorescent antibody (dFA) staining of saliva, skin biopsy, and corneal impressions may also yield a diagnosis.

Treatment

Local wound cleansing is important. Anyone who has experienced an animal bite should wash it thoroughly with soap and water. Rabies is a fatal illness, so a bite that breaks the skin warrants a call to a health care provider for evaluation of whether post-exposure prophylaxis (PEP) is necessary. Alternative treatments are recommended as a complementary therapy to conventional treatment in the case of rabies. Observation of the animal for signs of rabies is recommended whenever possible.

Allopathic treatment

If a person is bitten by a domestic animal and the owner is known, vaccination status should be checked. People bitten by healthy, immunized animals are unlikely to need post-exposure prophylaxis (PEP). The animal can be confined for 10 days. If it is healthy at the end of that time, it is presumed not to have been capable of transmitting rabies at the time of the bite, so PEP for the person bitten is not necessary.

Wild animals that have bitten can be captured, destroyed, and tested for rabies. Postexposure vaccine and specific immune globulin can be given if deemed necessary. In the United States, if the person who was bitten has not had prophylactic immunization and has a high-risk bite, generally five rabies vaccinations and one injection of human rabies immune globulin are given. There have been no cases in this country of people contracting rabies after receiving correctly administered PEP.

Bites from mice, rats, or squirrels rarely require rabies prevention because these rodents are typically killed by any encounter with a larger, rabid animal, and would therefore be less likely to be carriers. Bites from raccoons, bats, or unvaccinated dogs or cats are more suspect. Anyone bitten by a bat in the United States should receive PEP unless the bat is captured and proven not to be rabid.

If a pet is bitten by an animal suspected to have rabies, its owner should contact a veterinarian immediately and notify the local animal control authorities. Domestic pets with current vaccinations should be revaccinated immediately; unvaccinated dogs, cats, or ferrets are usually euthanized (put to sleep). Further information about domestic pets and rabies is available on the American Veterinary Medical Association (AVMA) web site.

Expected results

Survival of rabies after the appearance of symptoms is exceedingly rare.

Prevention

The following precautions should be observed in environments where humans and animals may likely come into contact. Domesticated animals, including household pets, should be vaccinated against rabies. Booster shots, given according to the manufacturer's recommendations, are required to maintain immunity. Wild animals should not be touched or petted, no matter how friendly an animal may appear. It is also important not to touch an animal that appears ill or passive, or whose behavior seems odd, such as failing to show the normal fear of humans. These are all possible signs of rabies. Many animals, such as raccoons and skunks, are nocturnal and their activity during the day should be regarded as suspicious. People should not interfere with fights between animals. Because rabies is transmitted through saliva, a person should wear rubber gloves when handling a pet that has had an encounter with a wild animal. Windows and doors should be screened. Some victims of rabies have been attacked by infected animals, particularly bats, that entered through unprotected openings. Finally, garbage or pet food should not be left outside because it may attract wild or stray animals.

Members of the high-risk occupations mentioned above should consider prophylactic immunization. Those who receive this pre-exposure vaccine still require PEP in the event of a potentially infective episode, but they have several advantages. One is that they require fewer post-exposure vaccines. A second advantage is that the timing of the PEP may be less critical for people who are in remote areas, or don't have ready access to vaccine for other reasons. Last, some people may be exposed without being aware of it, and the prophylactic vaccine might protect them.

Resources

BOOKS

"Central Nervous System Viral Diseases: Rabies (Hydrophobia)." Section 13, Chapter 162 in The Merck Manual of Diagnosis and Therapy, edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 1999.

Corey, Lawrence. "Rabies Virus and Other Rhabdoviruses." Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci et al., 14th ed.; New York: McGraw-Hill, 1998.

PERIODICALS

Fooks, A. R., N. Johnson, S. M. Brookes, et al. "Risk Factors Associated with Travel to Rabies Endemic Countries." Journal of Applied Microbiology 94 (2003) (Supplement): 31S36S.

"Human RabiesIowa, 2002." Morbidity and Mortality Weekly Report 52 (January 24, 2003): 4748.

Messenger, S. L., J. S. Smith, L. A. Orciari, et al. "Emerging Pattern of Rabies Deaths and Increased Viral Infectivity." Emerging Infectious Diseases 9 (February 2003): 151154.

Smith, J., L. McElhinney, G. Parsons, et al. "Case Report: Rapid Ante-Mortem Diagnosis of a Human Case of Rabies Imported Into the UK from the Philippines." Journal of Medical Virology 69 (January 2003): 150155.

Stringer, C. "Post-Exposure Rabies Vaccination." Nursing Standard 17 (February 5-11, 2003): 4142.

Weiss, R. A. "Cross-Species Infections." Current Topics in Microbiology and Immunology 278 (2003): 4771.

ORGANIZATIONS

American Veterinary Medical Association (AVMA). 1931 North Meacham Road, Suite 100, Schaumburg, IL 60173-4360. <http://www.avma.org>.

Centers for Disease Control and Prevention. 1600 Clifton Rd., NE, Atlanta, GA 30333. (800) 311-3435, (404) 639-3311. <http://www.cdc.gov>.

Institut Pasteur. 25-28, rue du Dr. Roux, 75015 Paris, France. +33 (0) 1 45 68 80 00. <http://www.pasteur.fr/haut_ext.html>.

OTHER

CDC. "Epidemiology of Rabies." http://www.cdc.gov/ncidod/dvrd/rabies/Epidemiology/Epidemiology.htm.

National Association of State Public Health Veterinarians, Inc. "Compendium of Animal Rabies Prevention and Control, 2003." Morbidity and Mortality Weekly Report Recommendations and Reports 52 (March 21, 2003) (RR-5): 16.

Judith Turner

Rebecca J. Frey, PhD

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Turner, Judith; Frey, Rebecca. "Rabies." Gale Encyclopedia of Alternative Medicine. 2005. Encyclopedia.com. 25 Jul. 2016 <http://www.encyclopedia.com>.

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Turner, Judith; Frey, Rebecca. "Rabies." Gale Encyclopedia of Alternative Medicine. 2005. Retrieved July 25, 2016 from Encyclopedia.com: http://www.encyclopedia.com/doc/1G2-3435100647.html

Rabies

Rabies

Rabies is a viral brain disease that is almost always fatal if it is not prevented with prompt treatment. The disease, which typically spreads to humans from animals through a scratch or a bite, causes inflammation of the brain. The disease is also called hydrophobia (meaning fear of water) because it causes painful muscle spasms in the throat that prevent swallowing. In fact, this is what leads to most fatalities in untreated cases: victims become dehydrated and die. Carriers of rabies include dogs, cats, bats, skunks, raccoons, and foxes; rodents are not likely to be infected. About 70% of rabies cases develop from wild animal bites that break the skin. Though a vaccine used first in 1885 is widely used, fatalities still occur due to rabies. Most fatalities take place in Africa and Asia, but some also occur in the United States. The cost of efforts to prevent rabies in the United States may be as high as $1 billion per year.

While many animal diseases cannot be passed from animal to man, rabies has long been known as an easy traveler from one species to the next. The disease was known among ancient people. The very name rabies, Latin for rage or madness, suggests the fear with which early men and women must have viewed the disease. For centuries there was no treatment, and the disease was left to run its rapid course leading to death.

Rabies is described in medical writings dating from 300 B.C., but the method of transmission or contagion was not recognized until 1804. In 1884, the French bacteriologist Louis Pasteur developed a preventive vaccine against rabies, and modifications of Pasteur's methods are still used in rabies therapy today. The Pasteur program, or variations of it, has greatly reduced the fatalities in humans from rabies. Modern treatment, following a bite by a rabid or presumed rabid animal, consists of immediate and thorough cleansing of the bite wound and injection into the wound and elsewhere of hyper-immune antirabies serum. Post exposure treatment consists of five injections of vaccine given over a one-month period, along with one dose of rabies immune globulin injected near the wound and intramuscularly.

The standard vaccine contains inactivated rabies virus grown in duck eggs. It is highly effective but causes neuroparalysis in about one in 30,000 persons receiving it. In the 1970s, a new vaccine was developed in France and the United States that contains virus prepared from human cells grown in the laboratory. This vaccine is safer and requires a shorter course of injections. With the widespread use of vaccine, rabies cases in the U.S. declined to fewer than five per year.

The transmission of rabies is almost invariably through the bite of an infected animal. The fact that the virus is eliminated in the saliva is of great significance, and unless saliva is introduced beneath the skin, the disease is seldom transmitted. The virus has been demonstrated in the saliva of dogs 38 days before the onset of symptoms. However, it has also been reported that only about 5060% of the infected dogs shed the virus in the saliva. Rare cases of rabies have been reported where only clawing and scratching occurred, or where the skin was contaminated with saliva. The virus is most concentrated in the central nervous system and saliva, but it has also been demonstrated in various organs of the body and milk from infected animals.

In humans, the rabies virus, in addition to entering the body by the usual route through skin broken by a bite or scratch, can enter the body through intact mucous membranes, can be inhaled as an aerosol, and can be transplanted in an infected corneal graft. These four cases are the only virologically documented examples of transmission of rabies from one person to another. Vertical transmission from mother to fetus and from lactating mother to suckling young has been described in nonhuman mammals.

The incubation period in natural cases of rabies is variable. In general, the quantity of virus introduced into the wound is correlated with the length of incubation before symptoms occur. In dogs, the minimum period is ten days, the average 2160 days, but may be as long as six months. In man, the incubation period is one to three months, with the minimum of ten days.

Rabies is caused by a number of different viruses that vary depending on geographic area and species. While the viruses are different, the disease they cause is singular in its course. The bullet-shaped virus is spread when it breaks through skin or has contact with a mucous membrane. The virus begins to reproduce itself initially in muscle cells near the place of first contact. At this point, within the first five days or so, treatment by vaccination has a high rate of success.

Once the rabies virus passes to the nervous system, immunization is no longer effective. The virus passes to the central nervous system, where it replicates itself in the system and moves to other tissues such as the heart, the lung, the liver, and the salivary glands. Symptoms appear when the virus reaches the spinal cord.

A bite from a rabid animal does not guarantee that one will get rabies; only about 50% of people who are bitten and do not receive treatment ever develop the disease. If one is bitten by or has had any exposure to an animal that may have rabies, medical intervention should be sought immediately. Treatment virtually ensures that one will not come down with the disease. Any delay could diminish the treatment's effectiveness.

In humans and in animals, rabies may be manifest in one of two forms: the furious (agitated) type or the paralytic (dumb) type. Furious rabies in animals, especially in the dog, is characterized by altered behavior such as restlessness, hiding, depraved appetite, excitement, unprovoked biting, aimless wandering, excessive salivation, altered voice, pharyngeal paralysis, staggering, general paralysis, and finally death. Death usually occurs within three to four days after the onset of symptoms. The paralytic form of rabies is frequently observed in animals inoculated with fixed virus, and is occasionally observed in other animals with street virus contracted under natural conditions. Animals showing this type usually show a short period of excitement followed by uncoordination, ataxia, paralysis, dehydration, loss of weight, followed by death.

In humans, furious rabies patients typically show bizarre behavior, ranging from episodes of severe agitation to periods of depression. Confusion becomes extreme as the disease progresses, and the patient may become aggressive. Hydrophobia is always seen with this type of disease, until the patient becomes comatose while showing intermittently uncontrollable inspiratory spasms. This type of rabies is also characterized by hypersalivation, from 11.6 qt (11.5 L) of saliva in 24 hours, and excessive sweating.

The paralytic form of rabies in humans is often indistinguishable from that of most viral encephalitis, except for the fact that a patient suffering from rabies remains conscious during the course of the disease. Paralysis usually begins at the extremity exposed to the bite and gradually involves other extremities finally affecting the pharyngeal and respiratory muscles.

The dog is a most important animal as a disseminator of rabies virus, not only to man but also to other animals. Wild carnivora may be infected and transmit the disease. In the United States, foxes, raccoons and skunks are the most commonly involved. These animals are sometimes responsible for infecting domestic farm animals.

The disease in wildlife (especially skunks, foxes, raccoons, and bats) has become more prevalent in recent years, accounting for approximately 85% of all reported cases of animal rabies every year since 1976. Wildlife now constitutes the most important potential source of infection for both human and domestic animals in the United States. Rabies among animals is present throughout the United States with the exception of Hawaii, which has remained consistently rabies-free. The likelihood of different animals contracting rabies varies from one place to the next. Dogs are a good example. In areas where public health efforts to control rabies have been aggressive, dogs make up less than 5% of rabies cases in animals. These areas include the United States, most European countries, and Canada.

However, dogs are the most common source of rabies in many countries. They make up at least 90% of reported cases of rabies in most developing countries of Africa and Asia and many parts of Latin America. In these countries, public health efforts to control rabies have not been as aggressive. Other key carriers of rabies include the fox in Europe and Canada, the jackal in Africa, and the vampire bat in Latin America.

In the United States, 60% of all rabies cases were reported in raccoons. The high number of cases in raccoons reflects an animal epidemic, or, more properly, an epizootic. The epizootic began when diseased raccoons were carried from further south to Virginia and West Virginia. Since then, rabies in raccoons has spread up the eastern seaboard of the United States. Concentrations of animals with rabies include coyotes in southern Texas, skunks in California and in south and north central states, and gray foxes in southeastern Arizona. Bats throughout the United States also develop rabies. When rabies first enters a species, large numbers of animals die. When it has been around for a long time, the species adapts, and smaller numbers of animals die.

There are few deaths from rabies in the United States. Between 1980 and the middle of 1994, a total of 19 people in the United States died of rabies, far fewer than the 200 Americans killed by lightning, for example. Eight of these cases were acquired outside the United States. Eight of the 11 cases contracted in the United States stemmed from bat-transmitted strains of rabies.

Internationally, more than 33,000 people die annually from rabies, according to the World Health Association. A great majority of cases internationally stem from dog bites. Different countries employ different strategies in the fight against rabies. The United States depends primarily on vaccination of domestic animals and on immunization following exposure to possibly rabid animals. Great Britain, in which rabies has never been established, employs a strict quarantine for all domestic animals entering the country.

Continental Europe, which has a long history of rabies, developed an aggressive program in the 1990s of airdropping a new vaccine for wild animals. The vaccine is mixed with pellets of food for red foxes, the primary carrier there. Public health officials have announced that fox rabies may be eliminated from Western Europe by the end of the decade. The World Health Organization is also planning to use the vaccine in parts of Africa.

Though the United States have been largely successful in controlling rabies in humans, the disease remains present in the animal population, a constant reminder of the serious threat rabies could become without successful prevention efforts.

See also Viruses and responses to viral infection

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Rabies

RABIES

Rabies is a viral disease of wild and domestic animals. It is particularly prevalent in feral dogs, while humans are occasional victims. The virus is transmitted in saliva and enters the body through puncture wounds caused by bites, or via abrasions, open cuts, or sores. The virus attacks the central nervous system by migrating up peripheral nerves from the site of entry. It can take several months to reach the central nervous system, so there can be a very long incubation period. In humans and most animals it is almost invariably fatal, but bats may be symptomless carriers. Rabies occurs almost worldwide, but it has been eliminated from Britain, Iceland, and Scandinavia through rigorously enforced animal quarantine, which has also prevented it from ever gaining entry to Australia. Because of the long incubation period, exposed animals must be quarantined for many months. In much of the world, including the United States and Canada, rabies is endemic in foxes, raccoons, skunks, bats, and other wild animals, and these occasionally infect domestic animals and humans.

The French bacteriologist Louis Pasteur developed a postexposure vaccine against rabies in 1885, using desiccated nerve tissue containing the virus. For many years, Pasteur's prolonged and painful course of injections was used to treat all persons who had been bitten by suspected rabid animals. Prophylactic immunizations for animal and human use have been much improved by the human diploid cell vaccine (HDCV), developed in the 1970s. Rabies immune globulin is used for postexposure prophylaxis. When humans are bitten by a suspected rabid animal, the animal should be killed and its brain examined for evidence of infection. Vaccination of wild animals utilizes an oral vaccine delivered in baits.

John M. Last

(see also: Communicable Disease Control; Immunizations; Pasteur, Louis; Veterinary Public Health )

Bibliography

National Center for Infectious Diseases. Rabies. Available at http://www.cdc.gov/ncidod/dvrd/rabies.

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rabies

rabies (rā´bēz, ră´–) or hydrophobia (hī´drəfō´bēə), acute viral infection of the central nervous system in dogs, foxes, raccoons, skunks, bats, and other animals, and in humans. The virus is transmitted from an animal to a person, or from one animal to another, via infected saliva, most often by biting but also by the contact of torn skin with infected saliva. The virus travels from the bite or contact location to the spinal cord and brain. In humans the incubation period ranges from 10 days to a year or more. Symptoms are fever, uncontrollable excitement, and pronounced spasms of the throat muscles. Salivation is extreme, and despite great thirst the victim cannot swallow water; hence the misnomer hydrophobia (fear of water). Once symptoms develop, death (caused by convulsions, exhaustion, or paralysis) is usually inevitable.

Following a bite from a rabid or possibly rabid animal, preventive treatment involves administration of immune globulin for passive immunization followed by vaccinations over several weeks for active immunization. The only treatment after symptoms appear is rest and sedation. Dogs have been immunized from the time Louis Pasteur demonstrated a successful vaccine in 1885. Since then, human rabies has become rare in the United States and other industrialized countries due to comprehensive vaccination programs for domestic animals. Mass vaccination of susceptible animals in the wild with vaccine-laced bait has been used in an effort to stem an increase of rabies cases in the United States and Canada that began in the late 1980s. A similar wild animal vaccination program has been used with some success in parts of Europe.

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Rabies

RABIES

DEFINITION


Rabies is a rare but serious disease caused by a virus. The virus that causes rabies is carried in saliva. It is transmitted when an infected animal bites another animal. Rabies affects humans and other mammals.

Another name for rabies is hydrophobia (pronounced HI-dro-fo-bee-uh). Hydrophobia means "fear of water." About half the people infected with rabies develop this symptom. Other symptoms include fever; depression; confusion; painful muscle spasms; sensitivity to touch, loud noise, and light; extreme thirst; painful swallowing; excessive salivation; and loss of muscle quality. Rabies can be prevented and treated by immunization (a protective treatment that causes the body's immune system to build up resistance to a particular disease; usually given as a shot). Without treatment, however, a person who is infected with rabies will almost certainly die.

DESCRIPTION


Worldwide, approximately fifteen thousand cases of human rabies occur each year. Remarkably, although more than one million persons in the United States are bitten each year by animals, only one or two die of rabies each year. Nonetheless, rabies is likely to remain a public-health problem in the future. Humans are continually moving into lands occupied by wild animals. As they do, they run the risk of being bitten by an animal with rabies.

Both domestic and wild animals can transmit rabies. At one time, domestic animals, such as cats and dogs, were the main source of rabies bites in the United States. In 1955, 47 percent of all reported rabies cases were caused by dog bites.

That situation has changed. Most cities, towns, and counties now require that dogs be vaccinated for rabies. The number of dogs infected with the virus has dropped dramatically. In 1994, fewer than 2 percent of all dogs tested positive for the rabies virus. Today, the vast majority of rabies cases in humans are caused by bites from wild animals, such as bats, raccoons, skunks, foxes, wolves, and coyotes.

Anyone who has been bitten by an animal can contract rabies. Age and sex make no difference in people's chances of getting the disease. However, people who work in certain occupations are at higher risk for rabies than the general public. These populations include farm and ranch workers, animal trainers and caretakers, forest rangers, animal exterminators, and veterinarians. People in these occupations often handle wild animals or domestic animals that may not have been vaccinated.

CAUSES


Rabies is caused by a virus that belongs to the family Rhabdoviridae. The virus is usually transmitted by way of an animal bite. The virus is present in the saliva of an infected animal. When an infected animal bites another animal, the virus in its saliva may flow into the second animal's bloodstream. The second animal becomes infected with the virus.

On rare occasions, the rabies virus can be transmitted in other ways. It can enter an animal's body through moist tissues around the eyes or lips or through a scratch in the skin. Some scientists believe the virus can even be inhaled. For example, the air in a cave occupied by bats may contain high levels of the rabies virus. A person who walks through the cave may breathe in some of the virus.

Rabies: Words to Know

Antibody:
A protein produced by the immune system in response to a specific foreign substance that enters the body.
Biopsy:
A procedure by which a small sample of tissue is removed and studied for the purpose of diagnosis.
Lumbar puncture:
A procedure in which a long, thin needle is inserted between the vertebrae in a person's spine in order to remove a sample of cerebrospinal fluid.
Rhabdovirus:
The virus that causes rabies.

Once it enters the body, the rabies virus travels to the nerves branching from the spinal cord and brain. It also travels to the salivary glands. The virus may lie dormant for several weeks or months. In its dormant stage, the virus is still alive, but it does not attack the body or produce any symptoms. It may continue to reproduce and spread through the body in its dormant stage. The only sign of a rabies infection at first may be a burning or painful sensation around the bite wound.

SYMPTOMS


Eventually, early symptoms of rabies begin to appear. These symptoms include a sore throat, low-grade fever (above normal, but in the lower ranges), headache, loss of appetite, nausea and vomiting, and diarrhea. Painful spasms (contractions) may develop in the muscles that control breathing and swallowing. The infected person may begin to drool thick saliva. Other symptoms include dilated (enlarged) pupils, increased flow of tears and perspiration, and low blood pressure.

As the disease progresses the patient may begin to feel excited, confused, and sensitive to bright lights, loud noises, and touch. He or she becomes very thirsty but is unable to drink because swallowing is so painful. Just looking at water can cause painful spasms. It is this reaction that accounts for the name hydrophobia. Other severe symptoms include excessive salivation, dehydration, and loss of muscle tone (quality). Death usually occurs three to twenty days after symptoms first appear. Once a person or animal has developed rabies, recovery is very rare.

DIAGNOSIS


A doctor who sees these symptoms will order tests to confirm the diagnosis. One test is called a lumbar puncture or spinal tap. A lumbar puncture is a procedure in which a long, thin needle is inserted between the vertebrae in a person's spinal cord. A sample of the fluid surrounding the vertebrae is removed. This fluid is called cerebrospinal fluid (CSF). A lumbar puncture is important because it rules out other possible causes of the patient's symptoms.

Tests can also be performed to detect the virus or antibodies produced by the body against the virus. Antibodies are chemicals produced by the immune system. They are manufactured when some kind of foreign substance enters the body. A different kind of antibody is produced for each different foreign substance. If the rabies virus is present, the immune system will have produced a very specific kind of antibody.

Some tests can detect the virus itself. For example, a small amount of liquid material in the cornea of the eye can be collected on a slide. The liquid can then be examined under a microscope. If the rabies virus is present in the body, it can be seen under a microscope.

TREATMENT


Any animal bite should be treated first with standard cleansing procedures. The wound should be washed thoroughly with medical soap and water. Antibiotics and tetanus injections (see tetanus entry) are usually given as well. These injections protect against bacterial infections.

The most important aspect of treatment is deciding whether a rabies immunization should be given. In some cases, the bite may have come from a domestic animal, such as a dog or a cat. If the animal is caught, it is placed in seclusion and observed. If the animal shows no signs of rabies in four to seven days, it is probably not rabid (not infected with rabies). The person who was bitten does not need a rabies immunization. If there is doubt as to whether the animal is rabid, it is killed. Its brain can then be examined to see if the rabies virus is present.

Sometimes the animal is not caught. In that case, a doctor has to decide whether to give the person who was bitten an immunization. That decision is usually based on how common rabies is in the area. If the disease is very rare, an immunization may not be necessary. If rabies is fairly common, an immunization will probably be given.

A rabies immunization usually consists of two parts. One part consists of injecting the patient with rabies antibodies taken from a person who has already been immunized. These antibodies will be of some help in destroying the rabies virus in the infected person's body. This part of the immunization process is given once, at the beginning of the treatment.

The second part of the immunization consists of injecting the patient with dead rabies viruses. These viruses will cause the patient's body to start manufacturing antibodies of its own without causing infection because they are dead. This part of the rabies immunization is given in a series of five injections, usually given one, three, seven, fourteen, and twenty-eight days after the animal bite. If successful, the antibodies from these two different sources will fight off the rabies infection.

Until the 1970s the rabies vaccination was a painful and frightening procedure that consisted of between fourteen and twenty-one shots that had to be given in the abdomen. Today the vaccine is given in the arm muscle, like a flu or tetanus shot.

Sometimes it may be too late to start immunizations. In that case, the main goal of treatment is to relieve the symptoms of the disease. For example, pain relievers can be given for painful muscle spasms. Other forms of medication can prevent seizures and relieve a person's anxiety. In the late stages of the disease, a patient may need mechanical devices to aid with breathing and heart function. Survival in such cases is rare but not unheard of.

PROGNOSIS


The key to successful treatment of rabies is timing. An infected person must begin a series of immunizations as soon as possible after being bitten. If immunizations begin within two days of the bite, chances of survival are very good. Even if the immunizations do not begin until later, there is a chance that the patient can survive. The longer the delay in starting immunizations, however, the less hopeful the prognosis for recovery is. Without immunizations, a patient will almost certainly die of the disease.

PREVENTION


Rabies can be prevented to a large extent by following some simple rules. These rules govern the way people interact with domestic and wild animals. They include:

  • Domesticated animals, including household pets, should be vaccinated against rabies on a regular basis.
  • Wild animals should not be touched or petted. Even if they seem friendly, they should be avoided. Special care should be taken with animals that behave strangely. For example, an animal may seem to have no fear of humans. Or animals that are normally out only at night may show up during the day. Such behaviors may be symptoms of rabies.
  • Do not interfere in fights between animals.
  • Use extra caution in handling a pet that has come into contact with a wild animal. Wear rubber gloves with the pet until you are sure that it has not been infected with rabies.
  • Windows and doors should be covered with screens. Animals sometimes enter a building through unprotected openings. People have been bitten by rabid animals that got into their houses by this route.
  • Citizens can become informed about the frequency of rabies in an area by calling the local health department.
  • People who work with domestic or wild animals should be vaccinated against rabies.
  • Bites from mice, rats, and squirrels rarely transmit rabies. Small animals like these that are bitten by a larger rabid animal usually die. Therefore, they are not likely to carry the disease.
  • Ask about the prevalence of rabies in new areas to which you might be traveling.

FOR MORE INFORMATION


Books

Cockrum, E. Lendell. Rabies, Lyme Disease, Hanta Virus, and Other Animal-Borne Human Diseases in the United States and Canada. Tucson, AZ: Fisher Books, 1997.

Finley, Don. Mad Dogs: The New Rabies Plague. Texas Station: Texas A&M University Press, 1998.

Silverstein, Alvin, Virginia Silverstein, and Robert Silverstein. Rabies. Hillside, NJ: Enslow Publishers, Inc., 1994.

Web sites

Rabies.com. [Online] http://www.rabies.com (accessed on October 31, 1999).

"RabiesWhat You Need To Know." [Online] http://www.cfainc.org/articles/rabies.html (accessed on October 31, 1999).

"Rabies." Who Information Fact Sheets. [Online] http://www.who.int/inffs/en/fact099.html (accessed on October 31, 1999).

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rabies

rabies (hydrophobia) Viral disease of the central nervous system. It can occur in all warm-blooded animals, but is especially feared in dogs due to the risk of transmission to human beings. The incubation period varies from a week or two to more than a year. It is characterized by severe thirst, although attempting to drink causes painful spasms of the larynx; other symptoms include fever, muscle spasms, and delirium. Once the symptoms have appeared, death usually follows within a few days. Anyone bitten by a rabid animal may be saved by prompt injections of rabies vaccine and antiserum.

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rabies

rabies (hydrophobia) (ray-beez) n. an acute virus disease of the central nervous system that may be transmitted to humans by a bite from an infected dog. Symptoms include malaise, fever, difficulty in breathing, salivation, and painful muscle spasms of the throat induced by swallowing. In the later stages of the disease the mere sight of water induces convulsions and paralysis; death occurs within 4–5 days. Injections of rabies vaccine and antiserum may prevent the disease from developing in a person bitten by an infected animal.
rabid (rab-id) adj.

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Rabies

Rabies

What Is Rabies?

How Common Is Rabies?

Is Rabies Contagious?

What Are the Signs and Symptoms of Rabies?

Making the Diagnosis

How Do Doctors Treat Rabies?

How Long Does the Disease Last and What Are the Complications?

Can Rabies Be Prevented?

Resources

Rabies (RAY-beez) is a viral infection of the central nervous system that usually is transmitted to humans by the bite of an infected animal.

KEYWORDS

for searching the Internet and other reference sources

Central nervous system infection

Encephalitis

Rhabdoviridae

Vaccination

Zoonotic diseases/Zoonoses

What Is Rabies?

Because of its devastating effects, rabies has been one of the most feared diseases in the world since it was first described in ancient times. A member of the Rhabdoviridae family of viruses causes rabies. In the United States, the virus lives primarily in wild bats, raccoons, skunks, and foxes; it sometimes is found in other animals as well, such as wolves, coyotes, or ferrets. Small animals such as hamsters, squirrels, mice, and rabbits typically do not carry the virus. More than 90 percent of reported rabies cases in animals occur in wild animals. The most common domestic animals that become infected, or rabid, are cats, dogs, and cattle. Throughout the world most cases of human exposure to rabies are to rabid dogs, but in the United States cases related to dog bites are rare. Indeed, most cases are linked to bats.

When a rabid animal bites a person, the rabies virus, which lives in the animals saliva, is transmitted through the body, where it can attack the central nervous system (the part of the nervous system that includes the brain and spinal cord), leading to encephalitis* and death. After symptoms begin, the disease can progress very quickly and can be fatal within a few days.

*encephalitis
(en-seh-fuh-LYE-tis) is an inflammation of the brain, usually caused by a viral infection.

How Common Is Rabies?

Almost all reported cases of rabies in the United States occur in animals. In the year 2000, 7,369 cases of rabies in animals were reported to the U.S. Centers for Disease Control and Prevention; 509 of those infections were in domestic animals (pets and farm animals). Only five people were infected with rabies that year, and all of them died. On average, only one or two deaths from rabies are seen in humans each year in the United States.

Is Rabies Contagious?

Rabies has not been found to be contagious from person to person. In theory, if a person infected with rabies were to bite someone else, the virus might spread, but no such cases have been recorded. Animal bites

are the most common cause of rabies in people. Rarely, the virus may spread when saliva or tissue from an infected animal enters an open wound or mucous membrane*, such as when an infected animal licks a persons broken skin. In rare instances, exposure to bats, with no known bite or scratch, causes human rabies.

*mucous membranes
are the moist linings of the mouth, nose, eyes, and throat.

What Are the Signs and Symptoms of Rabies?

Once a person has been infected with the rabies virus, symptoms may appear as soon as 10 days or as long as 90 days after exposure. These symptoms typically happen in stages. The first stage may include fever, a general ill feeling, sore throat, loss of appetite, nausea (NAW-zee-uh), vomiting, depression, and headache. If the person has been bitten by an animal, there may be a tingling sensation around the area of the bite. As the disease progresses and attacks the nervous system, a person may have difficulty sleeping and experience anxiety, confusion, aggressiveness, or hallucinations (ha-loo-sin-AY-shuns, seeing or hearing things that are not really there).

Other symptoms of rabies infection include partial paralysis*, seizures* or muscle spasms, inability to speak, sensitivity to light or sound, and hydrophobia (hi-druh-FOE-bee-uh), or avoiding drinking water or other liquids due to trouble swallowing. In the final stages, the person may experience double vision* or find it difficult to swallow saliva (which can make someone appear to be foaming at the mouth). The disease can progress to coma* and death.

*paralysis
(pah-RAH-luh-sis) is the loss or impairment of the ability to move some part of the body.
*seizures
(SEE-zhurs) are sudden bursts of disorganized electrical activity that interrupt the normal functioning of the brain, often leading to uncontrolled movements in the body and sometimes a temporary change in consciousness.
*double vision
is a vision problem in which a person sees two images of a single object.
*coma
(KO-ma) is an unconscious state in which a person cannot be awakened and cannot move, see, speak, or hear.

The Strange Facts in The Case of Edgar Allan Poe

Edgar Allan Poe, whose name is synonymous with horror and mystery, died as perplexing and shocking a death as any that he concocted in the many suspenseful and macabre tales that he wrote. Poe set out on a trip from Richmond, Virginia, to Philadelphia at the end of September 1849. The next anyone heard of himabout a week after his departurehe had collapsed outside a tavern in Baltimore and was found quivering and raving. Four days later, on October 7, he died in a nearby hospital. For many years it was thought that his symptoms of delirium*, cold sweat, confusion, memory loss, and difficulty in swallowing could be attributed to severe alcoholism. In 1996, however, R. Michael Benitez, a doctor at the University of Maryland Medical Center, concluded in a review of the historical record that Poe, in fact, might have died of rabies.

*delirium
(dih-LEER-e-um) is a condition in which a person is confused, is unable to think clearly, and has a reduced level of consciousness.

Making the Diagnosis

People who may have been exposed to rabies need immediate medical attention. If a person has symptoms of the disease, a doctor will perform a physical examination and ask questions to figure out whether the person might have been exposed to a rabid animal. To diagnose rabies in a human, doctors can perform several laboratory tests, including examination of blood and spinal fluid for antibodies* to the rabies virus. Skin biopsies* and saliva tests also may be done to search for signs of the infection. One of the best diagnostic tests is done on brain tissue from the potentially infected animal. The results can tell doctors whether the animal was rabid. Animals with less risk of being infected (such as a pet dog) can be isolated and observed by the local health department to see whether any signs of rabies develop.

*antibodies
(AN-tih-bah-deez) are protein molecules produced by the bodys immune system to help fight specific infections caused by microorganisms, such as bacteria and viruses.
*biopsies
(BI-op-seez) are tests in which small samples of skin or other body tissue are removed and examined for signs of disease.

How Do Doctors Treat Rabies?

It is recommended that someone who might have been exposed to rabies wash the site thoroughly with warm, soapy water. A person who has been bitten by or who has come into direct contact with an animal that may be rabid can receive immediate treatment by a doctor to prevent the disease from developing. This is called postexposure prophylaxis*. The person receives one dose of rabies immune globulin*, which provides the body with antibodies against rabies, followed by five doses of rabies vaccine* given over a period of 28 days. This treatment has proved to be very effective in preventing the development of rabies when it is started within a day of exposure. Once a person has symptoms of rabies, treatment typically is limited to life support in the hospital. Approximately 40,000 people in the United States and an estimated 10 million people throughout the world are treated as a precaution each year after having been exposed to animals suspected of being rabid.

*prophylaxis
(pro-fih-LAK-sis) means taking specific measures, such as using medication or a device (such as a condom), to help prevent infection, illness, or pregnancy.
*immune globulin
(ih-MYOON GLAH-byoo-lin), also called gamma globulin, is the protein material that contains antibodies.
*vaccine
(vak-SEEN) is a preparation of killed or weakened germs, or a part of a germ or product it produces, given to prevent or lessen the severity of the disease that can result if a person is exposed to the germ itself. Use of vaccines for this purpose is called immunization.

How Long Does the Disease Last and What Are the Complications?

Once symptoms have appeared the disease can progress very quickly to coma and death, generally within 1 to 3 weeks. Rabies infection that is not treated immediately almost always causes death. In rare cases where people have survived, they often have severe and permanent brain damage.

Can Rabies Be Prevented?

Vaccinating household pets against rabies is very important in preventing the spread of the disease. This method has dramatically limited the number of rabies cases seen in domestic animals in the United States. People who are at greatest risk of exposure, such as veterinarians, travelers to areas of the world where rabid animals are common,

Louis Pasteurs Vaccine Breakthrough

In 1885, Louis Pasteur (18221895), a French scientist known for his remarkable contributions to modern medicine, developed a vaccine that can prevent rabies in humans who have been exposed to the virus. This breakthrough led to the creation of the Pasteur Institute, a medical research organization dedicated to the understanding and prevention of infectious diseases, where Pasteur continued his important work for the last years of his life.

and laboratory workers who handle material that may contain the rabies virus, often are immunized (vaccinated) against the disease. Taking these safety measures also can help prevent rabies:

  • avoiding contact with unfamiliar or wild animals, especially bats and raccoons
  • never feeding or handling a wild or unknown animal
  • keeping trash that is stored outside carefully sealed, to avoid attracting raccoons and other animals
  • consulting a doctor for advice about receiving the rabies vaccine before traveling to an area where rabies is more common, such as Asia or Africa

See also

Encephalitis

Vaccination (Immunization)

Zoonoses

Resources

Organization

U.S. Centers for Disease Control and Prevention (CDC), 1600 Clifton Road, Atlanta, GA 30333. The CDC tracks outbreaks of rabies in the United States and publishes information about the disease at its website.

Telephone 800-311-3435 http://www.cdc.gov

Website

KidsHealth.org. KidsHealth is a website created by the medical experts of the Nemours Foundation and is devoted to issues of childrens health. It contains articles on a variety of health topics, including rabies.

http://www.KidsHealth.org

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rabies

ra·bies / ˈrābēz/ • n. a contagious and fatal viral disease of dogs and other mammals that causes madness and convulsions, transmissible through the saliva to humans. Also called hydrophobia.

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Rabies

340. Rabies

See also 122. DISEASE and ILLNESS .

hydrophobia
rabies in human beings. Also called lyssa .
lyssophobia
an abnormal fear of rabies. See also hydrophobophobia .

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rabies

rabiesAchinese, Ambonese, appease, Assamese, Balinese, Belize, Beninese, Bernese, bêtise, Bhutanese, breeze, Burmese, Cantonese, Castries, cerise, cheese, chemise, Chinese, Cingalese, Cleese, Congolese, Denise, Dodecanese, ease, éminence grise, expertise, Faroese, freeze, Fries, frieze, Gabonese, Genoese, Goanese, Guyanese, he's, Japanese, Javanese, jeez, journalese, Kanarese, Keys, Lebanese, lees, legalese, Louise, Macanese, Madurese, Maltese, marquise, Milanese, Nepalese, Nipponese, officialese, overseas, pease, Pekinese, Peloponnese, Piedmontese, please, Portuguese, Pyrenees, reprise, Rwandese, seise, seize, Senegalese, she's, Siamese, Sienese, Sikkimese, Sinhalese, sleaze, sneeze, squeeze, Stockton-on-Tees, Sudanese, Sundanese, Surinamese, Tabriz, Taiwanese, tease, Tees, telegraphese, these, Timorese, Togolese, trapeze, valise, Viennese, Vietnamese, vocalese, wheeze •superficies • Héloïse • Averroës •rabies • pubes • Maccabees •headcheese

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