CHAPTER 17 Rabies Rabies is an acute fatal viral illness of the central nervous system (CNS). The word rabies is derived from the Latin verb to rage, which suggests the appearance of the rabid patient. It can affect all mammals and is transmitted between them by infected secretions, most often by bite. It was first recognized more than 3000 years ago and has been the most feared of infectious diseases. It is said that Aristotle recognized that rabies could be spread by a rabid dog. I. VIROLOGY 1. Enveloped RNA virus is bullet-shaped 2. Knob-like envelope glycoproteins elicit neutralizing and hemagglutination antibodies 3. Strains from different sources are antigenically heterogeneous 4. Negative sense RNA virus replicates in the cytoplasm 5. G protein containing lipoprotein envelope acquired from plasma membrane II.RABIES A. EPIDEMIOLOGY 1. Urban form is associated with unimmunized dogs or cats 2. Sylvatic form occurs in wild skunks, foxes, wolves, raccoons, and bats but not rodents or rabbits disease 3. Occasional case has resulted from aerosol exposure eg, bat caves 4. Human infection is blind-ended, and does not contribute to maintenance or transmission B. PATHOGENESIS 1. 2. 3. 4. Replicates initially in muscle and then enters peripheral nervous system Spreads to CNS gray matter Negri bodies found in neurons Incubation period can be prolonged for months III. RABIES: CLINICAL ASPECTS A. MANIFESTATIONS 1. Average incubation period of 20 to 90 days 1 2. Begins as a nonspecific illness marked by fever, headache, malaise, nausea, and vomiting 3. Abnormal sensations at or around the site of viral inoculation occur frequently and probably reflect local nerve involvement 4. Onset of encephalitis is marked by periods of excess motor activity and agitation 5. Hallucinations, combativeness, muscle spasms, signs of meningeal irritation, seizures, and focal paralysis occur 6. Combination of excess salivation and difficulty in swallowing produces the fearful picture of "foaming at the mouth." 7. Hydrophobia, the painful, violent involuntary contractions of the diaphragm and accessory respiratory, pharyngeal, and laryngeal muscles initiated by swallowing liquids 8. Once symptoms have developed, no drug or vaccine administration will improve survival 9. Recovery is exceedingly rare. B. DIAGNOSIS 1. Virus or antigen detected in brain tissue 2. Test of choice is detection of rabies antigen by immunofluorescent stain of a nape of the neck biopsy C. TREATMENT 1. Intensive supportive care has resulted in two or three long-term survivals 2. No specific treatment is available D. PREVENTION 1. Pasteur, noting the long incubation period of rabies, suggested that a vaccine to induce an immune response before the development of disease might be useful in prevention 2. Now believed that vaccination induces antibody that is either neutralizing or inhibits cell-to-cell spread of virus 3. Vaccine used in the United States for preexposure prophylaxis employs an attenuated rabies virus grown in human diploid cell culture and inactivated 4. Preexposure prophylaxis is recommended for individuals at high risk of contact with rabies virus, such as veterinarians, spelunkers, laboratory workers, and animal handlers 5. Postexposure prophylaxis consideration of: (1) whether the individual came into physical contact substance likely to contain rabies virus; (2) whether there was significant wound or abrasion; (3) whether rabies is known or suspected in the animal species (4) whether the bite was provoked or unprovoked; and (5) whether the animal is available for laboratory examination 6. Rabies immune globulin plus vaccine necessary in postexposure management 2