Ezl
Movement Disorders Vol. 5 , No. I , 1990, pp. 4W3 0 1990 Movement Disorder Society
PP
MENT
A Case of Rabies in North America Mohit Bhatt, Erik Wolters, Joseph Tsui, Barry J. Snow, and Donald B. Calne Belzherg Laboratory of Clinical Neuroscience, Department of Medicine, Division of Neurology, University Hospital-U.B.C., Vancouver, British Columbia, Canada
Summary: A 25-year-old man developed classical hydrophobic rabies 3 months after being bitten on the face. We present his involuntary movements on videotape and discuss the clinical and epidemiological features of rabies. Though rabies is seen in tropical countries commonly and in North America occasionally, recording of the clinical features is rare. Key Words: RabiesEpidemiology.
of July 1985, he was bitten on the face by a bat. Antirabies prophylaxis was not administered to him at that time. He remained well until October 25, 1985, when he experienced facial paraesthesia, neck pains, cervical lymphadenopathy, profuse sweating, high fever, a heavy sensation in his head, and abdominal muscle spasms on drinking. On examination (videotape segment), he had an anxious facial expression and risus sardonicus. He had recurrent involuntary muscle spasms involving his pharynx and abdominal wall. Tone was increased in all four extremities; there was ataxia with some weakness in the legs. Tendon reflexes were normal; plantar responses were flexor, and sensation was normal. General examination revealed priapism, but no other abnormality. Rabies was diagnosed and antirabies immunoglobulin, human diploid cell vaccine, and interferon were administered. When the spasms became frequent, he was ventilated artificially and given diazepam and later pancuronium bromide. He had to be sedated with morphine, because the spasms became intractable and his general condition deteriorated. He developed pneumonia, acute respiratory distress syndrome, diabetes insipidis, and hypopituitarism. Despite all supporting measures, he died. The serum and cerebrospinal fluid (CSF) showed the presence of rabies antibodies. Rabies fluorescent antibody test was positive from the brainstem, hippocampus, cerebellum, and the cerebrum. The
Rabies is an acute viral encephalitis, transmitted by infected secretions, usually saliva. Most human rabies is transmitted through the bite of an infected dog. Other animals that transmit the disease include the wolf (Arctic circle, Eastern Europe), the mongoose (South Africa, Caribbean), the fox (Western Europe), the vampire bat (Latin America), and occasionally, the cat ( 1 4 ) . Rarely, virus may be inhaled in caves that harbor bats. The role of the alternative carrier becomes important in developed countries where the population of stray dogs is negligible and pets are vaccinated. Human to human transmission is very uncommon. After an animal bite, there is a variable incubation period. The patient develops prodromal symptoms, followed by encephalitis. During the early phase, the patient remains conscious and is often aware of his fate. We present such a patient who permitted videotape recording of his involuntary muscle spasms. CASE REPORT
D’S’ was a 25-year-01d student working as a cook in a camp in Alberta, Canada. During the third week A videotape segment accompanies this article. Address correspondence and reprint requests to Dr. D. B. Calne at Belzberg Laboratory of Clinical Neuroscience, Department of Medicine, Division of Neurology, University HospitalU.B.C., 2211 Wesbrook Mall, Vancouver, B.C. V6T 1W5,Canada.
40
41
virus was typed using a monoclonal antibody test and was similar to a bat strain of the rabies virus. DISCUSSION
A true global estimate of the incidence of rabies is difficult to obtain, as the disease is most common in underdeveloped countries. Only 21 cases of rabies have been recorded in Canada since 1924, and most (71%) before 1960 (5). In 1985, 2,495 people received postexposure prophylaxis in Canada, an increase of 11% over 1984. Nationally, 1 in every 10,000 Canadians has received antirabies treatment. The WHO estimates that more than 1 million persons were immunized in India during 1973 (6). This is considered an underestimate by Turner (7), who claims more than 3 million people receive prophylactic vaccination each year in India. In one Indian center (JJ Hospital, Bombay) there were 36 fatal cases of rabies in 5 years (8). The government of India reported 61 1 deaths due to rabies in 1980, and this is considered a gross underestimate (9). According to Sehgal and Bhatia, rabies causes 45,00050,000 deaths annually in India; this includes urban and rural rabies (10). A large number of these patients are children. The subcontinent, comprising India, Pakistan, and Bangladesh, has the largest rabies problem in the world. In Thailand, 200-300 and in China 5,000-7,000 deaths due to rabies are reported annually (11). A large number of cases go either undiagnosed or unreported in countries where the disease is prevalent. In a pathological study in Cali, Columbia between 1962 and 1966, 1.7% of patients were found to have died of undiagnosed rabies out of a total of 1,596 deaths (11). Rabies remains an endemic disease in Latin America. One case was reported from the U.S.A. in 1985 (12). Not everyone bitten by a rabid animal will develop the disease. If a rabid wolf bites more than one person and one of them dies, the chances are that 60% of those remaining will develop the disease (13). Thirty-two percent of patients bitten by rabid dogs develop the disease. The figure can be markedly reduced by early treatment. In tropical America and Asia, 97.5% of animal rabies is reported in domestic animals like dogs and cats. In developed nations, 81.3% of animal rabies is seen in wild animals like skunks, bats, raccoons, and foxes (14). The economic impact of animal rabies is unclear, except for cattle rabies transmitted by vampire bat
in South America, where more than 100,000 cattle are estimated to die of rabies per year (15). We have presented a patient with typical hydrophobic rabies. The case is unique due to the recordings of the patient’s symptoms and signs on videotape. The rabies virus enters the wound at the time of the bite by the rabid animal and replicates in the muscle for days to months, explaining the variable incubation period (16). This period ranges from 10 days to more than 1 year, the average being 1-2 months. Once the muscle spindles become involved, the virus enters the peripheral nervous system and ascends by retrograde axoplsmic flow to the corresponding dorsal root ganglion, the spinal cord, and then to the brainstem (17). It replicates rapidly in the central nervous system (CNS), and the glycoprotein excrescences on the viral surface become attached to the acetylcholine receptors. The rabies virus continues to travel and spread along the nerves to involve the salivary glands, the cornea, and other visceral organs. This explains human to human transmission following corneal transplant. There are five reported cases of rabies following corneal grafting, and each recipient developed similar disease to that of the donor (18). It is usually taught that the incubation is shorter for wounds near the CNS, but this is not reliable, as in our case, the incubation was 3 months even with a face bite. Rabies presents in two forms, the more common hydrophobic or furious variety and the rarer paralytic or dumb type, where paralysis predominates. Both types are invariably fatal. Recent studies using monoclonal antibody typing have revealed six antigenic types, and only two of them, namely the Mokola and the Duvenhage viruses, are known to cause human disease. Monoclonal typing could resolve whether different strains of rabies virus are responsible for the two clinical forms of human rabies-the furious and the paralytic. Classical or hydrophobic rabies usually begins with a prodromal period, when patients have fever, anorexia, photophobia, and body aches. Fifty to 80% of patients complain of paraesthesiae and/or fasciculations at the site of the bite. This period lasts for 1 4 days. In Thailand, itching was the earliest symptom in more than 40% of cases: itching was either localized to the site of the bite or to the whole limb, and in some cases, it was intense enough to provoke frenzied scratching and excoriation of skin (19). The reason for localized pares-
Movement Disorders, Voi. 5 , No. I , 1990
42
M . BHATT ET AL.
thesiae could be involvement of the dorsal root ganglion of the nerve supplying the area of the animal bite. Priapism with spontaneous emission is a recognized symptom at this stage of the disease. The prodromal phase is followed by the brainstem phase, characterized by lower cranial nerve involvement with violent, involuntary contractions of the respiratory muscles, producing excessive salivation and difficulty swallowing. Hydrophobia is a distinctive feature. It is associated with terror, excitement, cardiac arrhythmias, and generalized convulsions. The spasms are generally expected to be painful, but in our patient, there was little pain associated with the spasm, as in other studies (19). Hydrophobia is caused by particular sensitivity of bulbar muscles to the stimulus of swallowing fluids, and ultimately to the mere sight of drinks. Even the thought of water can produce laryngeal spasm and choking. The generalized encephalitic phase follows the brainstem phase. The patient hallucinates, develops excessive motor activity, confusion, agitation, focal weakness, and high fever. There are periods of calm and lucidity, but as the disease progresses, these phases shorten and the patient lapses into coma. Signs related to autonomic dysfunction are obvious. After this phase, death soon follows, unless the patient is kept alive by assisted ventilation. Under these circumstances, other problems ensue, such as diabetes insipidis, cardiac arrhythmias, acute respiratory distress syndrome, inappropriate ADH secretion, thrombocytopenia, and paralytic ileus. U1timately, death follows. Rabies virus has a predilection for the limbic system, reticular formation, pontine tegmentum, and the cranial nerve nuclei in the floor of the forth ventricle. The inspiratory neurons near the nucleus ambiguous may undergo dam-
age, and this may explain the inspiratory spasms (20). Although the usual pattern is for 80% of cases to develop the classical hydrophobic disease, in the Trinidad epidemic, transmitted by the vampire bat, all patients had dumb rabies (21). Paralytic rabies presents with rapidly progressive ascending weakness, simulating the Landry-Guillain-Barre syndrome (21,22). In some, there may be a sensory level on the trunk, as seen in transverse myelitis. In countries where the sheep brain vaccine is still in use, dumb rabies needs to be distinguished from postvaccinial demylination. If the incubation period is more than 6 weeks, rabies is more likely. The history of local paresthesiae at the site of animal bite is also suggestive of rabies. In countries where rabies is prevalent, it is best to inquire about animal bite in all patients admitted with acute motor peripheral neuropathy , especially when the CSF shows unexpected pleocytosis. The most useful laboratory study is the fluorescent antibody test. Titers >1:10,000 in serum, saliva, or CSF is diagnostic of rabies (23,24). Negri bodies are pathognomonic of rabies. They are usually located in the Ammon’s horn and the cerebellum as intracytoplasmic inclusions, 1-10 Fm in size. A mild perivascular leukocytic infiltration is seen in the cerebral cortex. The measures to be taken after an animal bite is thorough wound cleaning by scrubbing with soap and water. For passive immunity, human rabies immunoglobulin, 20 mg/kg body weight, should be given intramuscularly, with 50% of this dose infiltrated locally. To produce active immunity, human diploid cell vaccine is the treatment of choice. The recommended regimen is 1 ml of the vaccine to be injected intramuscularly on days 0, 3, 7 , 14, 28, and
TABLE 1. Rabies: postexposure prophylaxis Type of animal Domestic Ilomestic Stray dogs, cats Wild (bat, skunk, bobcat, fox, jackal, raccoon, coyote), other carnivores
Condition of animal Vaccinated, healthy, can be observed Ill, suspected rabies Unknown, cannot be observed Regarded as rabid unless negative by laboratory test
Treatment of exposed persons None RIG, HDCV RIG, HDCV RIG, HDCV
RIG, rabies immune globulins; HDCV, human diploid cell vaccine. Adopted from recommendation of the Center for Disease Control, Atlanta, GA, 1984.
Movement Disorders, Vnl. 5 , No. 1. 1990
RABIES #lowing the bite. Given the high cost of the ‘I diploid cell vaccine, sheep brain vaccine is commonly used for prophylactic measures in countries. An alternative means of cost rez on is to inject 0.1 ml of the human diploid cell ne intradermally at eight sites on day 0, four * on day 7, and a single site on days 28 and 91. efficacy of this regimen can be judged from its a in 78 patients bitten by animals with proven ,hies; all were alive after 2 years (19). Table 1 provides guidelines on postexposure prophylaxis. j
1
8
l_l
LEGEND TO THE VIDEOTAPE Video record of involuntary muscular spasms with the onset of brainstem encephalitis in a case of hydrophobic rabies. These spasms were painless, and the patient’s sensorium was clear at the time of the recording. The patient could answer appropriately; only the involuntary spasms interrupted his speech. REFERENCES I . Winkler WC. Rabies in the United States. J Infect Dzs 1972;125:674-5. 2. Parker RL, Sikes RK. Development of rabies inhibiting substance in skunks infected with rabies virus. Pub Health Rep 1966;91:941. 3. McLean RG. Rabies in raccoons in United States. J Infect Dis 1972;123:680-1. 4. WHO Expert Committee on Rabies Report. WHO Tech Rep Series 1973; no. 523. 5. Varughese PV. Rabies in Canada in 1985. Can Med Assoc J 19873 36:1277-80. 6. Rabies Zoonosis surveillance. Center for Disease Control, US Department of Health, Education, and Welfare, US Public Health Service, August 1976. 7. Turner GS. A review of the world epidemiology of rabies. Trans R SOC Trop Med Hyg 1976;70:175-8.
43
8. Wadia NH, Bhatt MH. Acute viral encephalitis commonly prevalent in India. In: Sainani GS, Joshi VR, Mebita PJ, eds. Advances in Medecine. Bombay: The Association of Physicians of India, in press. 9. Ministry of Health and Family Welfare, Government of India Health Statistics of India, Central Bureau of Health Intelligence, New Delhi, 1981. 10. Sehgal S, Bhatia R. Rabies-urrent status and proposed control programs in India. Proceedings of Workshop on Rabies Surveillance and Control, National Institute of Communicable Diseases, Delhi, India: NICD. 11. Baer GM. Research towards rabies prevention: overview. Rev Infec Dis 1988;10:S576-7. 12. Weekly Epidemiology Record, no. 26. June 26 1987:192-3. 13. Hattwick MAW, Gregg MB. The disease in man, vol 11. In: Baer GM, ed. The natural history of rabies. Philadelphia: Academic Press, 1975:281-305. 14. Acha PN, Arambulo PV 111. Rabies in the tropics-history and current status. In: Kuwert E, Merieux C, Koprowski H, Bogel K, eds. Rabies in the tropics. Berlin: Springer-Verlag, 1985:343-59. 15. Baer GM. Bovine paralytic rabies and rabies in vampire bats. In: Baer GM, ed. The natural history of rabies, vol 11. Philadelphia: Academic Press, 1975:155-75. 16. Miller A, Nathanson N. Rabies: recent advances in pathogenesis and control. Ann Neurol 1977;2:511-9. 17. Murphy FA, Bauer SP, Harrison AK, Winn WC. Comparative pathogenesis of rabies and rabies like virus. Viral infection and transit from inolulation site to the central nervous system. Lab Invest 1973;28:361-76. 18. Sureau P, Portnoi D, Rollin P, Lapresle C, Lapresle C, Chaouni-Berbich A. Prevention de la transmission interhumaine de la rage apres greffe de cornee. CR Acad Sci (Paris) 1981 ;293:689-92. 19. Warrell DA, Warrell MJ. Human rabies and its prevention: an overview. Rev Infect Dis 1988;10:S726-31. 20. Warrell DA, Davidson NMc D, Pope HM, et al. Pathophysiologic studies in human rabies. A m J Med 1976;60:180-90. 21. Love SV. Paralytic rabies. Review of literature and report of a case. J Pediatr 1944;24:312. 22. Hattwick MAW, Weis TT, Stechschulte JC, Baer GM, Gregg MB. Recovery from rabies. A case report. Ann Intern Med 1972;76:931 4 2 . 23. Dumb rabies (editorial). Lancet 1978;ii: 1031-2. 24. Bhatt DR, Hattwick MAW, Gardsen R, Emmons RW, Johnson HN. Human rabies. A m J Dis Child 1974;127:862.
Movement Disorders, Vol. 5 , No. 1 , 1990
Movement Disorders Vol. 5 , No. I , 1990, pp. 4W3 0 1990 Movement Disorder Society
PP
MENT
A Case of Rabies in North America Mohit Bhatt, Erik Wolters, Joseph Tsui, Barry J. Snow, and Donald B. Calne Belzherg Laboratory of Clinical Neuroscience, Department of Medicine, Division of Neurology, University Hospital-U.B.C., Vancouver, British Columbia, Canada
Summary: A 25-year-old man developed classical hydrophobic rabies 3 months after being bitten on the face. We present his involuntary movements on videotape and discuss the clinical and epidemiological features of rabies. Though rabies is seen in tropical countries commonly and in North America occasionally, recording of the clinical features is rare. Key Words: RabiesEpidemiology.
of July 1985, he was bitten on the face by a bat. Antirabies prophylaxis was not administered to him at that time. He remained well until October 25, 1985, when he experienced facial paraesthesia, neck pains, cervical lymphadenopathy, profuse sweating, high fever, a heavy sensation in his head, and abdominal muscle spasms on drinking. On examination (videotape segment), he had an anxious facial expression and risus sardonicus. He had recurrent involuntary muscle spasms involving his pharynx and abdominal wall. Tone was increased in all four extremities; there was ataxia with some weakness in the legs. Tendon reflexes were normal; plantar responses were flexor, and sensation was normal. General examination revealed priapism, but no other abnormality. Rabies was diagnosed and antirabies immunoglobulin, human diploid cell vaccine, and interferon were administered. When the spasms became frequent, he was ventilated artificially and given diazepam and later pancuronium bromide. He had to be sedated with morphine, because the spasms became intractable and his general condition deteriorated. He developed pneumonia, acute respiratory distress syndrome, diabetes insipidis, and hypopituitarism. Despite all supporting measures, he died. The serum and cerebrospinal fluid (CSF) showed the presence of rabies antibodies. Rabies fluorescent antibody test was positive from the brainstem, hippocampus, cerebellum, and the cerebrum. The
Rabies is an acute viral encephalitis, transmitted by infected secretions, usually saliva. Most human rabies is transmitted through the bite of an infected dog. Other animals that transmit the disease include the wolf (Arctic circle, Eastern Europe), the mongoose (South Africa, Caribbean), the fox (Western Europe), the vampire bat (Latin America), and occasionally, the cat ( 1 4 ) . Rarely, virus may be inhaled in caves that harbor bats. The role of the alternative carrier becomes important in developed countries where the population of stray dogs is negligible and pets are vaccinated. Human to human transmission is very uncommon. After an animal bite, there is a variable incubation period. The patient develops prodromal symptoms, followed by encephalitis. During the early phase, the patient remains conscious and is often aware of his fate. We present such a patient who permitted videotape recording of his involuntary muscle spasms. CASE REPORT
D’S’ was a 25-year-01d student working as a cook in a camp in Alberta, Canada. During the third week A videotape segment accompanies this article. Address correspondence and reprint requests to Dr. D. B. Calne at Belzberg Laboratory of Clinical Neuroscience, Department of Medicine, Division of Neurology, University HospitalU.B.C., 2211 Wesbrook Mall, Vancouver, B.C. V6T 1W5,Canada.
40
41
virus was typed using a monoclonal antibody test and was similar to a bat strain of the rabies virus. DISCUSSION
A true global estimate of the incidence of rabies is difficult to obtain, as the disease is most common in underdeveloped countries. Only 21 cases of rabies have been recorded in Canada since 1924, and most (71%) before 1960 (5). In 1985, 2,495 people received postexposure prophylaxis in Canada, an increase of 11% over 1984. Nationally, 1 in every 10,000 Canadians has received antirabies treatment. The WHO estimates that more than 1 million persons were immunized in India during 1973 (6). This is considered an underestimate by Turner (7), who claims more than 3 million people receive prophylactic vaccination each year in India. In one Indian center (JJ Hospital, Bombay) there were 36 fatal cases of rabies in 5 years (8). The government of India reported 61 1 deaths due to rabies in 1980, and this is considered a gross underestimate (9). According to Sehgal and Bhatia, rabies causes 45,00050,000 deaths annually in India; this includes urban and rural rabies (10). A large number of these patients are children. The subcontinent, comprising India, Pakistan, and Bangladesh, has the largest rabies problem in the world. In Thailand, 200-300 and in China 5,000-7,000 deaths due to rabies are reported annually (11). A large number of cases go either undiagnosed or unreported in countries where the disease is prevalent. In a pathological study in Cali, Columbia between 1962 and 1966, 1.7% of patients were found to have died of undiagnosed rabies out of a total of 1,596 deaths (11). Rabies remains an endemic disease in Latin America. One case was reported from the U.S.A. in 1985 (12). Not everyone bitten by a rabid animal will develop the disease. If a rabid wolf bites more than one person and one of them dies, the chances are that 60% of those remaining will develop the disease (13). Thirty-two percent of patients bitten by rabid dogs develop the disease. The figure can be markedly reduced by early treatment. In tropical America and Asia, 97.5% of animal rabies is reported in domestic animals like dogs and cats. In developed nations, 81.3% of animal rabies is seen in wild animals like skunks, bats, raccoons, and foxes (14). The economic impact of animal rabies is unclear, except for cattle rabies transmitted by vampire bat
in South America, where more than 100,000 cattle are estimated to die of rabies per year (15). We have presented a patient with typical hydrophobic rabies. The case is unique due to the recordings of the patient’s symptoms and signs on videotape. The rabies virus enters the wound at the time of the bite by the rabid animal and replicates in the muscle for days to months, explaining the variable incubation period (16). This period ranges from 10 days to more than 1 year, the average being 1-2 months. Once the muscle spindles become involved, the virus enters the peripheral nervous system and ascends by retrograde axoplsmic flow to the corresponding dorsal root ganglion, the spinal cord, and then to the brainstem (17). It replicates rapidly in the central nervous system (CNS), and the glycoprotein excrescences on the viral surface become attached to the acetylcholine receptors. The rabies virus continues to travel and spread along the nerves to involve the salivary glands, the cornea, and other visceral organs. This explains human to human transmission following corneal transplant. There are five reported cases of rabies following corneal grafting, and each recipient developed similar disease to that of the donor (18). It is usually taught that the incubation is shorter for wounds near the CNS, but this is not reliable, as in our case, the incubation was 3 months even with a face bite. Rabies presents in two forms, the more common hydrophobic or furious variety and the rarer paralytic or dumb type, where paralysis predominates. Both types are invariably fatal. Recent studies using monoclonal antibody typing have revealed six antigenic types, and only two of them, namely the Mokola and the Duvenhage viruses, are known to cause human disease. Monoclonal typing could resolve whether different strains of rabies virus are responsible for the two clinical forms of human rabies-the furious and the paralytic. Classical or hydrophobic rabies usually begins with a prodromal period, when patients have fever, anorexia, photophobia, and body aches. Fifty to 80% of patients complain of paraesthesiae and/or fasciculations at the site of the bite. This period lasts for 1 4 days. In Thailand, itching was the earliest symptom in more than 40% of cases: itching was either localized to the site of the bite or to the whole limb, and in some cases, it was intense enough to provoke frenzied scratching and excoriation of skin (19). The reason for localized pares-
Movement Disorders, Voi. 5 , No. I , 1990
42
M . BHATT ET AL.
thesiae could be involvement of the dorsal root ganglion of the nerve supplying the area of the animal bite. Priapism with spontaneous emission is a recognized symptom at this stage of the disease. The prodromal phase is followed by the brainstem phase, characterized by lower cranial nerve involvement with violent, involuntary contractions of the respiratory muscles, producing excessive salivation and difficulty swallowing. Hydrophobia is a distinctive feature. It is associated with terror, excitement, cardiac arrhythmias, and generalized convulsions. The spasms are generally expected to be painful, but in our patient, there was little pain associated with the spasm, as in other studies (19). Hydrophobia is caused by particular sensitivity of bulbar muscles to the stimulus of swallowing fluids, and ultimately to the mere sight of drinks. Even the thought of water can produce laryngeal spasm and choking. The generalized encephalitic phase follows the brainstem phase. The patient hallucinates, develops excessive motor activity, confusion, agitation, focal weakness, and high fever. There are periods of calm and lucidity, but as the disease progresses, these phases shorten and the patient lapses into coma. Signs related to autonomic dysfunction are obvious. After this phase, death soon follows, unless the patient is kept alive by assisted ventilation. Under these circumstances, other problems ensue, such as diabetes insipidis, cardiac arrhythmias, acute respiratory distress syndrome, inappropriate ADH secretion, thrombocytopenia, and paralytic ileus. U1timately, death follows. Rabies virus has a predilection for the limbic system, reticular formation, pontine tegmentum, and the cranial nerve nuclei in the floor of the forth ventricle. The inspiratory neurons near the nucleus ambiguous may undergo dam-
age, and this may explain the inspiratory spasms (20). Although the usual pattern is for 80% of cases to develop the classical hydrophobic disease, in the Trinidad epidemic, transmitted by the vampire bat, all patients had dumb rabies (21). Paralytic rabies presents with rapidly progressive ascending weakness, simulating the Landry-Guillain-Barre syndrome (21,22). In some, there may be a sensory level on the trunk, as seen in transverse myelitis. In countries where the sheep brain vaccine is still in use, dumb rabies needs to be distinguished from postvaccinial demylination. If the incubation period is more than 6 weeks, rabies is more likely. The history of local paresthesiae at the site of animal bite is also suggestive of rabies. In countries where rabies is prevalent, it is best to inquire about animal bite in all patients admitted with acute motor peripheral neuropathy , especially when the CSF shows unexpected pleocytosis. The most useful laboratory study is the fluorescent antibody test. Titers >1:10,000 in serum, saliva, or CSF is diagnostic of rabies (23,24). Negri bodies are pathognomonic of rabies. They are usually located in the Ammon’s horn and the cerebellum as intracytoplasmic inclusions, 1-10 Fm in size. A mild perivascular leukocytic infiltration is seen in the cerebral cortex. The measures to be taken after an animal bite is thorough wound cleaning by scrubbing with soap and water. For passive immunity, human rabies immunoglobulin, 20 mg/kg body weight, should be given intramuscularly, with 50% of this dose infiltrated locally. To produce active immunity, human diploid cell vaccine is the treatment of choice. The recommended regimen is 1 ml of the vaccine to be injected intramuscularly on days 0, 3, 7 , 14, 28, and
TABLE 1. Rabies: postexposure prophylaxis Type of animal Domestic Ilomestic Stray dogs, cats Wild (bat, skunk, bobcat, fox, jackal, raccoon, coyote), other carnivores
Condition of animal Vaccinated, healthy, can be observed Ill, suspected rabies Unknown, cannot be observed Regarded as rabid unless negative by laboratory test
Treatment of exposed persons None RIG, HDCV RIG, HDCV RIG, HDCV
RIG, rabies immune globulins; HDCV, human diploid cell vaccine. Adopted from recommendation of the Center for Disease Control, Atlanta, GA, 1984.
Movement Disorders, Vnl. 5 , No. 1. 1990
RABIES #lowing the bite. Given the high cost of the ‘I diploid cell vaccine, sheep brain vaccine is commonly used for prophylactic measures in countries. An alternative means of cost rez on is to inject 0.1 ml of the human diploid cell ne intradermally at eight sites on day 0, four * on day 7, and a single site on days 28 and 91. efficacy of this regimen can be judged from its a in 78 patients bitten by animals with proven ,hies; all were alive after 2 years (19). Table 1 provides guidelines on postexposure prophylaxis. j
1
8
l_l
LEGEND TO THE VIDEOTAPE Video record of involuntary muscular spasms with the onset of brainstem encephalitis in a case of hydrophobic rabies. These spasms were painless, and the patient’s sensorium was clear at the time of the recording. The patient could answer appropriately; only the involuntary spasms interrupted his speech. REFERENCES I . Winkler WC. Rabies in the United States. J Infect Dzs 1972;125:674-5. 2. Parker RL, Sikes RK. Development of rabies inhibiting substance in skunks infected with rabies virus. Pub Health Rep 1966;91:941. 3. McLean RG. Rabies in raccoons in United States. J Infect Dis 1972;123:680-1. 4. WHO Expert Committee on Rabies Report. WHO Tech Rep Series 1973; no. 523. 5. Varughese PV. Rabies in Canada in 1985. Can Med Assoc J 19873 36:1277-80. 6. Rabies Zoonosis surveillance. Center for Disease Control, US Department of Health, Education, and Welfare, US Public Health Service, August 1976. 7. Turner GS. A review of the world epidemiology of rabies. Trans R SOC Trop Med Hyg 1976;70:175-8.
43
8. Wadia NH, Bhatt MH. Acute viral encephalitis commonly prevalent in India. In: Sainani GS, Joshi VR, Mebita PJ, eds. Advances in Medecine. Bombay: The Association of Physicians of India, in press. 9. Ministry of Health and Family Welfare, Government of India Health Statistics of India, Central Bureau of Health Intelligence, New Delhi, 1981. 10. Sehgal S, Bhatia R. Rabies-urrent status and proposed control programs in India. Proceedings of Workshop on Rabies Surveillance and Control, National Institute of Communicable Diseases, Delhi, India: NICD. 11. Baer GM. Research towards rabies prevention: overview. Rev Infec Dis 1988;10:S576-7. 12. Weekly Epidemiology Record, no. 26. June 26 1987:192-3. 13. Hattwick MAW, Gregg MB. The disease in man, vol 11. In: Baer GM, ed. The natural history of rabies. Philadelphia: Academic Press, 1975:281-305. 14. Acha PN, Arambulo PV 111. Rabies in the tropics-history and current status. In: Kuwert E, Merieux C, Koprowski H, Bogel K, eds. Rabies in the tropics. Berlin: Springer-Verlag, 1985:343-59. 15. Baer GM. Bovine paralytic rabies and rabies in vampire bats. In: Baer GM, ed. The natural history of rabies, vol 11. Philadelphia: Academic Press, 1975:155-75. 16. Miller A, Nathanson N. Rabies: recent advances in pathogenesis and control. Ann Neurol 1977;2:511-9. 17. Murphy FA, Bauer SP, Harrison AK, Winn WC. Comparative pathogenesis of rabies and rabies like virus. Viral infection and transit from inolulation site to the central nervous system. Lab Invest 1973;28:361-76. 18. Sureau P, Portnoi D, Rollin P, Lapresle C, Lapresle C, Chaouni-Berbich A. Prevention de la transmission interhumaine de la rage apres greffe de cornee. CR Acad Sci (Paris) 1981 ;293:689-92. 19. Warrell DA, Warrell MJ. Human rabies and its prevention: an overview. Rev Infect Dis 1988;10:S726-31. 20. Warrell DA, Davidson NMc D, Pope HM, et al. Pathophysiologic studies in human rabies. A m J Med 1976;60:180-90. 21. Love SV. Paralytic rabies. Review of literature and report of a case. J Pediatr 1944;24:312. 22. Hattwick MAW, Weis TT, Stechschulte JC, Baer GM, Gregg MB. Recovery from rabies. A case report. Ann Intern Med 1972;76:931 4 2 . 23. Dumb rabies (editorial). Lancet 1978;ii: 1031-2. 24. Bhatt DR, Hattwick MAW, Gardsen R, Emmons RW, Johnson HN. Human rabies. A m J Dis Child 1974;127:862.
Movement Disorders, Vol. 5 , No. 1 , 1990
