A high proportion of recent human rabies cases diagnosed in the United States have been acquired outside the country and have lacked a history of animal bite exposure. On June 21, 1994, a 40-year-old man died in a hospital in Miami following a subacute and progressive neurologic syndrome; rabies had not been clinically suspected but was diagnosed postmortem on July 13. This report summarizes the case investigation, which indicated that the case was acquired outside the United States.

During 1979-1993, the man frequently visited Haiti. After temporarily residing in Haiti during most of 1993, the man returned to Miami in December. During February 1994, he sought medical care for severe neck pain and headache; he was treated as an outpatient and returned to Haiti in March. On April 19, after returning to the United States, he presented to a hospital in Miami with acute renal failure attributed to mild mesangial proliferative glomerular nephritis and was hospitalized. He recovered following hemodialysis and was discharged on April 29.

During May 1994, he made four visits to the hospital's outpatient clinic for different problems, including frontal headache, acute anxiety, epigastric pain, and chest and back pain. During each visit, he also complained of pain in the neck, extremities, or back. Negative diagnostic studies during these visits included an electroencephalogram, electrocardiogram (ECG), and magnetic resonance imaging (MRI).

On June 9, he presented again with a 6-day history of neck pain, headaches, photophobia, feverishness, nausea and vomiting, and right-sided weakness. He was hospitalized and started on ceftriaxone for presumed meningitis. Laboratory studies included a white blood cell count of 10,300/mm3 (normal: 5000-10,000/mm3), hemoglobin of 12 g (normal: 14-18 g), and hematocrit of 36 units (normal: 40-54 units); findings were within normal limits for chest radiographs, ECG, and a brain MRI. Cerebrospinal fluid obtained by a lumbar puncture was clear with a protein level of 16 mg/dL and glucose of 111 mg/dL. The patient was started on acyclovir and high-dose steroids for presumed central nervous system vasculitis. Because of progressive lethargy and disorientation, he was admitted to the medical intensive care unit (MICU); two computed tomographies of the head done on admission on June 9 and again on June 11 were negative for evidence of a cerebrovascular event or meningeal involvement.

The patient's neurologic function continued to deteriorate, and despite mechanical ventilation, he died on June 21. Hypersalivation was not a documented symptom during the clinical course.

On July 13, microscopic examination of brain tissue taken at autopsy showed diffuse, severe encephalomyelitis. Ultrastructural examination of residual neurons detected viral particles consistent with rabies virus. Immunofluorescent antibody staining of formalin-fixed tissue at CDC was strongly suggestive of rabies. On July 21, rabies was confirmed by identification of rabies virus RNA from formalin-fixed brain tissue by reverse transcription-polymerase chain reaction (RT-PCR) amplification with rabies-specific primers. Nucleotide sequence of the RT-PCR product revealed a rabies virus variant sharing 99% homology with a recent dog rabies sample from Haiti; this variant has not been documented in the United States.

Interviews with the patient's family members who resided in Florida indicated that he had not reported an animal bite and that he avoided contact with domestic and wild animals. In addition, they reported that he never had had a pet dog and were unaware of stray dogs in his neighborhood. However, the incubation period, onset of symptoms, travel history, and finding of the rabies virus variant suggest the patient had been exposed to rabies in Haiti during late 1993 or March 1994.

Although postexposure prophylaxis was not indicated for any relatives, 16 hospital personnel (i.e., a morgue technician who cut himself during the autopsy, 10 respiratory therapists, four medical residents, and one nurse in the MICU) received postexposure treatment. The Pan American Health Organization was alerted to contact appropriate Haitian authorities about the case. Reported by: B Elser, MD, CK Petito, MD, Jackson Memorial Hospital; V Sneller, PhD, ED Sfakianaki, MD, MB Ares, MD, M Edouard, MD, Dade County Public Health Unit; WG Hlady, MD, RS Hopkins, MD, State Epidemiologist, Florida Dept of Health and Rehabilitative Svcs. Viral and Rickettsial Zoonoses Br, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: The case described in this report is the 20th case of human rabies to have been reported in the United States since 1980. Of these 20 cases, 10 probably were acquired outside the United States, and 14 had no documented history of animal bite exposure. As a result of exposures to the 20 cases, 832 persons received post-exposure rabies prophylaxis, representing an estimated cost of $850,000 (1).

In the United States, vaccination programs for pets have reduced the potential for rabies exposure from domestic animals (2). In comparison, the occurrence of rabies in dogs remains a common problem in Haiti (3,4) and many other developing countries (3). Because of the risk for rabies in these countries, travelers are advised to avoid contact with dogs and other animals, and rabies preexposure prophylaxis is recommended for persons planning to stay at least 30 days (5).

The case described in this report is the third since 1993 that was diagnosed approximately 1 month postmortem. Rabies should be considered early in the differential diagnosis of rapidly progressive encephalitic syndromes of suspected viral etiology, regardless of whether the patient has a history of an animal bite. Although early diagnosis alters neither the patient's treatment course nor prognosis, advantages of this approach include the prompt implementation of appropriate infection-control measures, limitation of the number of persons exposed who require postexposure prophylaxis, and prompt administration of prophylaxis to exposed persons.

References

1. Fishbein DB, Robinson LE. Rabies. N Engl J Med 1993;329:1632-8.

2. Krebs JW, Strine TW, Childs JE. Rabies surveillance in the United States during 1992. J Am Vet Med Assoc 1993;203:1718-31.

3. World Health Organization. World survey of rabies XXVII (for year 1991). Geneva: World Health Organization, Division of Communicable Diseases, Veterinary Public Health Unit, 1993; publication no. WHO/Rabies/93.209.

4. Pan American Health Organization. Epidemiologic surveillance of rabies for the Americas, 1990. Buenos Aires: Pan American Health Organization, 1991.

5. CDC. Human rabies -- Texas and California, 1993. MMWR 1994;43:93-

7. Whitley RJ. Viral encephalitis. N Engl J Med 1990;323:242-50.

   
   

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