Human Rabies -- New York, 1993

In August 1993, a fatal case of human rabies in an 11-year-old girl was reported to the New York State Department of Health; this was the first indigenously acquired fatal case diagnosed in New York in 39 years. This report summarizes the investigation of this case.

On July 5, the girl complained of pain in the knuckles on her left hand. During July 6-7, she had increasing pain that extended up to the left shoulder. On July 8, a pediatrician diagnosed musculoskeletal pain and bilateral ear effusions; a throat culture was obtained and amoxacillin was prescribed.

On July 9, the patient developed fever, severe muscle spasms of the left arm, difficulty walking, and hallucinations. On evaluation in an emergency department on July 10, she had fever (101.1 F {38.6 C}), otitis media in her left ear, nonexudative pharyngitis, and a maculopapular rash on the chest; there were no focal neurologic or meningeal signs. The throat culture obtained July 8 was positive for presumed streptococcus group A, and recurrent streptococcal pharyngitis and otitis media were diagnosed. She was treated with intravenous ceftriaxone, normal saline, and oral anti-pyretics and was discharged with a prescription for cefaclor.

She subsequently would not drink, withdrew when offered a drink, and had difficulty swallowing oral secretions. On evaluation in a hospital emergency department on July 11, she had a temperature of 105.3 F (40.7 C), mild meningismus but no focal neurologic findings; a white blood cell (WBC) count was elevated at 13,300. A lumbar puncture revealed 23 WBCs per cubic millimeter (mm superscript 3) (100% lymphocytes) and 1200 red blood cells per mm superscript 3. Viral meningoencephalitis or meningococcal infection was diagnosed. She was treated with ceftriaxone and dexamethasone intravenously and transported by helicopter ambulance to a tertiary-care medical center.

On admission to the pediatric intensive-care unit, she was alert, oriented, and cooperative but agitated; her pupils were unequal but reactive. Acyclovir was added to her treatment regimen. The patient developed respiratory distress, hypertension, and tachycardia and was placed on mechanical ventilation; cardiac arrhythmias subsequently occurred, and she suffered nonreversible cardiac arrest.

An autopsy was performed on July 12; although unfixed brain tissue was not obtained for viral or bacterial diagnosis, cerebral edema was noted. During August 2-3, examination of routine histopathologic slides of brain tissue revealed encephalitis with severe involvement of the midbrain, pons and medulla, and possible Negri bodies. Culture of cerebrospinal fluid (CSF) obtained July 11 for rabies virus and tests of serum and CSF for rabies antibody were negative at the New York State Department of Health. However, specimens tested by the rabies fluorescent antibody technique (FA) indicated fluorescent inclusions in the brain stem, midbrain, and Purkinje cells of the cerebellum. Rabies diagnosis was confirmed at CDC by FA testing and histologic examination of formalin-fixed and paraffin-embedded tissue. The RNA extracted from formalin-fixed brain tissue was reverse transcribed and amplified by polymerase chain reaction. The nucleotide sequence identified a viral variant associated with rabies in insectivorous bats.

The patient lived in a heavily wooded area of the Catskill Mountains and had no history of foreign travel. She had no known history of contact with a bat, and examination of her home and outbuildings on the property revealed no evidence of bat infestation. She had been active outdoors, and her family kept horses, dogs, cats, rabbits, hamsters, and gerbils as pets; none of these pets had died with clinical signs consistent with rabies or disappeared. A survey of all neighbors on the same road indicated that no pets had died with clinical signs consistent with rabies or disappeared during the preceding 6 months.

As a result of close contact with the patient and/or her secretions, rabies postexposure prophylaxis was administered to 55 persons, including eight family members, three friends, 35 health-care workers, five members of the autopsy team, three transport personnel, and one mortician. Reported by: JG Debbie, DVM, S Frantz, PhD, LF Novick, MD, CV Trimarchi, MS, GS Birkhead, MD, Acting State Epidemiologist, New York State Dept of Health; M Baker, MD, D Hill, MD, R Fuchs, MD, Pediatric Associates, Middletown; M Valsamis, MD, C Vallejo, MD, B Roseman, MD, S Schroeder, MD, Westchester County Medical Center, Valhalla. Viral and Rickettsial Zoonoses Br, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: Human rabies in the United States is uncommon, primarily because of canine rabies-control programs and access to improved human rabies biologicals. Since 1980, 16 human rabies cases have been reported in the United States. Of these, seven were acquired from exposure outside the United States; for nine of the 16 cases, no definitive history of exposure was identified. Potential reasons for the failure of public health authorities to establish definitive exposures include unrecognized exposure, communication (i.e., language) barriers, and memory loss and impaired speech because of encephalitis at presentation. Rabies is not usually diagnosed when patients initially receive medical evaluation. Since 1980, of the 16 persons with rabies diagnosed in the United States, rabies was diagnosed postmortem in nine. In addition, six cases of human-to-human transmission were diagnosed postmortem among recipients of transplanted corneas, whose donors died of an illness unrecognized as rabies (1).

Although rabies occurs rarely in the United States, it should be considered in the differential diagnosis of any acute progressive encephalitis of unknown etiology. In the absence of a clear history of animal exposure, the diagnosis of rabies may be difficult because of the nonspecific nature of initial clinical presentation. In addition to encephalitis, other manifestations suggestive of rabies in the case described in this report included paresthesia, hydrophobia, and copious salivation. Antemortem diagnosis of human rabies is possible through laboratory analysis of CSF, serum, saliva, and biopsy of nuchal skin or brain tissue. Although an early suspicion of rabies does not alter the prognosis, it may permit both institution of measures to reduce the number of persons exposed to rabies during patient care and identification of persons who are candidates for postexposure prophylaxis. Consultation with state and federal health officials is recommended for human rabies evaluation.

The case in this report is the sixth since 1980 in which insectivorous bats were implicated. A definite history of exposure through a bat's bite was identified for only one of the six cases, while contact with a bat was associated with two additional cases; for three cases, the nature of exposure was not determined, but bat rabies variants were identified by molecular typing.

Bat rabies is enzootic in the United States, and cases have been reported from all of the 48 contiguous states (2). The rabies virus variant identified in this case, and in three of the other five occurring since 1980, is associated with the silver-haired bat (Lasionycteris noctivagans), a solitary, migratory species, with a preferred habitat of old-growth forest. This species is infrequently submitted for rabies diagnosis. For example, of 7047 bats submitted for rabies diagnosis and identified to species in New York from 1988 through 1992, 25 (0.4%) were L. noctivagans; of these, two were rabid (C. Trimarchi, New York State Department of Health, unpublished data, 1993). The rabies virus variant associated with this species (identified in 11 of 12 isolates from silver-haired bats) was rarely found in other bats (five {2.1%} of 238 samples tested) or in terrestrial mammals (five {0.7%} of 700 samples).

Exposure to potentially rabid animals (e.g., paralyzed bats) should be avoided. Postexposure prophylaxis is recommended for all persons bitten or scratched by such animals and for nonbite exposures involving contamination of lesions or mucous membranes with saliva or other potentially infectious materials (3). Bat bites may be more difficult to recognize than those inflicted by terrestrial animals. Treatment should be considered for any physical contact with bats when bite or mucous membrane contact cannot be excluded. Because reduction of bat populations is neither feasible nor desirable as a means for controlling rabies in bats, efforts to prevent this problem should be directed toward the exclusion of bats from human dwellings to minimize direct contact with humans and companion animals. In addition, all dogs and cats in the 48 contiguous states and Alaska should have a current rabies vaccination (4).

References

1. Gode GR, Bhide NK. Two rabies deaths after corneal grafts from one donor. Lancet 1988;2:791.

2. Krebs JW, Holman RC, Hines U, Strine TW, Mandel EJ, Childs JE. Rabies surveillance in the United States during 1991. J Am Vet Med Assoc 1992;201:1836-48.

3. ACIP. Rabies prevention -- United States, 1991: recommendations of the Immunization Practices Advisory Committee (ACIP). MMWR 1991;40(no. RR-3).

4. CDC. Compendium of animal rabies control, 1993: recommendations of the National Association of State Public Health Veterinarians, Inc. MMWR 1993;42(no. RR-3).

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