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Human Rabies -- Pennsylvania

The second case of human rabies occurring in the United States in 1984 was diagnosed September 21, in Danville, Pennsylvania. The patient, a 12-year-old male resident of Williamsport, Pennsylvania, had no history of exposure to a known rabid animal.

He was in good health until September 14, when he complained of a runny nose and sore throat. He was feverish and had teeth-chattering chills and drenching sweats. Later that day, he refused to eat, choking when he finally attempted to eat, and he was unable to swallow antipyretics. The next day, he felt somewhat better, but he was still unable to eat. On September 16, he complained of being chilly, hungry, and thirsty. The following morning, he suddenly developed muscle spasms and had difficulty breathing. When his mother attempted to take him to the hospital, he tried to run away.

On September 17, at the emergency room of a local hospital, the patient was agitated; his temperature was 39.4 C (103 F); and he appeared dehydrated. Diaphoresis, mydriasis, generalized hyperreflexia, and fasciculations of all muscle groups were noted. The admitting diagnosis was fever with dehydration. Hyperthyroidism, sepsis, and drug intoxication were ruled out shortly after admission. On September 18, after rehydration, he showed no changes. He refused to swallow liquids and expectorated all secretions. The diagnosis of rabies was considered, and contact isolation was instituted. Lumbar puncture was traumatic, revealing 149 white blood cells/mm((3)) and 17,560 red blood cells/mm((3)). Cardiac arrhythmias, including premature atrial and ventricular beats, were noted. His extremities became cold, and blood pressure was sometimes difficult to auscultate. He was transferred to a referral center, where physical examination revealed him to be both diaphoretic and shivering, intermittently responsive to commands, and at other times yelling inappropriately and violently. This became particularly marked when cool breezes, such as from an oxygen canula, crossed his face. He frequently gagged and expectorated. Periods of lucidity alternated with hallucinations and disorientation, and he exhibited facial grimaces and fasciculations. A skin biopsy from the nape of the neck performed on September 20 and examined at CDC on September 21 was positive for rabies by immunofluorescent staining. On September 23, the patient became less responsive, failing to follow commands to open his eyes. He was intubated because of expectoration of large amounts of foamy saliva and episodes of respiratory depression. On the morning of September 26, he became almost completely unresponsive, with only occasional grimaces to pain and asymmetric and sluggishly reactive pupils. Chest radiograph revealed consolidation of the left lower lung field and patchy densities in the right lung fields. An episode of bradycardia occurred. Further neurologic deterioration occurred, and the patient died after an episode of asystole on September 29.

Sera and cerebrospinal fluid from September 20, were negative for rabies neutralizing antibodies using the rapid fluorescent focus inhibition test performed at CDC. The definitive diagnosis was based on the positive fluorescent antibody of the neck biopsy and later isolation of rabies virus from the saliva.

Although the mid-Atlantic raccoon rabies epizootic now involves Pennsylvania, no rabid animals have been found since 1978 in the county where the patient lived. Testing of numerous terrestrial animals, including raccoons, failed to detect any animal rabies. For 1984, only one animal with rabies, a bat, has been reported from those counties adjacent to where the patient lived.

Monoclonal antibody analysis of the rabies virus isolated from the patient's saliva did not reveal the characteristic antigenic patterns found in rabies isolates from raccoons and other terrestrial mammals in the mid-Atlantic states. The similarity between this isolate and those obtained from insectivorous bats common to the eastern United States suggests a bat as the origin of this isolate, but an identical isolate has not been obtained (1). Further analyses and comparison of this isolate with virus isolates from rabid bats in the Pennsylvania area are under way. Reported by D Zeidner, MD, A Bowman, J Dennehy, MD, C Hufnal-Miller, MD, R Leipold, MD, T Royer, MD, M Ryan, MD, S Toor, MD, T Martin, MD, Geisinger Medical Center, Danville, J Maksimak, MD, RH Kaiser, MD, G Lattimer, MD, M Hart, C Sinner, Divine Providence Hospital, Williamsport, B Jones, DVM, S Bowen, MD, E Witte, VMD, C Hays, MD, State Epidemiologist, Pennsylvania State Dept of Health; Div of Viral Diseases, Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: This patient's classic hydrophobia, aerophobia, and furious behavior resulted in the early suspicion of rabies and early institution of proper isolation measures even in the absence of a history of rabies exposure. Although an exposure history can be identified in most cases of rabies, no exposure has been identified in nine (20%) of the 45 cases occurring in the United States from 1960 to the present (2). This case did not appear to have been acquired from a wild terrestrial mammal, and, to date, no case of human rabies has been associated with the mid-Atlantic raccoon rabies epizootic.

The absence of a known history of exposure, in conjunction with the impending hunting season, resulted in an unprecedented demand for preexposure immunization among many Pennsylvania hunters, trappers, and other outdoors enthusiasts. However, only professional trappers and hunters in rabies-endemic areas who are regularly exposed to potentially rabid animals should receive preexposure prophylaxis; the population at large, including individuals in rabies epizootic areas, does not require rabies preexposure prophylaxis (3). Sports trappers and hunters are at little risk of an inapparent exposure.

All hunters and trappers in rabies-endemic areas handling animals, such as raccoons, foxes, and skunks, known to be involved in endemic epizootic rabies problems should use care, especially when skinning animals, to avoid exposures (bites and contamination of mucous membranes and open wounds with potentially infectious materials Š(saliva and brain tissueŠ)). The use of gloves is recommended when wild mammals are handled. Any bite or nonbite exposure should be treated promptly with local-wound care and should be reported to appropriate local or state health officials. The animal head should be maintained at refrigerator temperature (approximately 4 C (39 F)) pending instructions on the need for testing.

Preexposure prophylaxis does not eliminate the need for postexposure prophylaxis. The use of rabies postexposure prophylaxis should be based on individual evaluations of each exposure and examination of the animal that was the source of the exposure after consultation with appropriate local and state health authorities. Rabies in humans is very rare in the United States, with an average of only two cases per year since 1960 (2), and no case has occurred in a person who has received the recommended postexposure treatment.


  1. Smith JS, Sumner JW, Roumillat LF, Baer GM, Winkler WG. Antigenic characteristics of isolates associated with a new epizootic of raccoon rabies in the United States. J Infect Dis 1984;149:769-74.

  2. Anderson LJ, Nicholson KG, Tauxe RV, Winkler WG. Human rabies in the United States, 1960 to 1979: epidemiology, diagnosis, and prevention. Ann Intern Med 1984;100:728-35.

  3. ACIP. Rabies prevention--United States, 1984. MMWR 1984;33:393-402, 407-8.

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