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Anthrax meningitis: Case report
Case Report
Anthrax Meningitis:
Case Report
BirdalYorgancigil, MD;* Mustafa Demirci, MD;+ Mehmet Unlu, MD:* Erdogan Setik, MD;§ and Mehmet Doganay, MD” Anthrax occurs in three clinical forms in humans: cutaneous, gastrointestinal, and pulmonary Meningitis is rarely seen and is always fatal. It may be a complication of any of the three forms as a result of lymphohematogenous spread from the primary lesions or may develop as primary infection, where anthrax is endemic. It is noted that about 100 cases with anthrax meningitis have been reported in the world literature.‘-* This report presents a case of hemorrhagic meningitis due to Bacillus anthracis. This is believed to be the second article about anthrax meningitis in Turkey. The first case was reported 12 years ago.5 CASE
REPORT
A 64-year-old man was admitted to hospital with high fever, shortness of breath, and unconsciousness. His complaints had begun 3 days before his admission with malaise, headache, and abdominal pain. On examination, the patient was comatose and pupils were dilated. His temperature was 40°C pulse rate lbO/min, and respiratory rate 40/min. His blood pressure was loo/60 mmHg. Neurologic examination revealed indefinite meningeal signs without neck rigidity, negative Kernig and Brudzinski signs and Babinski reflex. Cornea1 and deep tendon reflexes were both negative, and the patient did not respond to painful stimuli. His right retroauricular region and neck were slightly swollen and hyperemic, suggesting parotitis. There were no cutaneous lesions. Radiologic examination of the chest revealed enlargement of the mediastinal lymph nodes, linear atelectasis, bronchiectasis, and minimal pleural effusion. Subamchnoid hemorrhage was found in cerebral computed tomography
*Assistant Professor, ‘Specialist in Clinical Microbiology, *Assistant Professor, Siileyman Demirel University, Medical Faculty, Department of Chest Diseases, Isparta, Turkey; %peciaiist in of Infectious Diseases, Isparta State Hospital, Isparta, Turkey; and qProfessor, Erciyes University, Medical Faculty, Department of Infectious Diseases, Kayseri, Turkey. Address correspondence to Dr. Mustafa Demirci, H?z+rbey Sk. No. 5D: 32100, Isparta,Turkey. E-mail: mdemircMdr.com.
220
Mah. 1546
White blood cell count was 18,500/mm3, and erythrocyte sedimentation rate (ESR) was 21 mm/h. On lumbar puncture, cerebrospinal fluid (CSF) was projectile and hemorrhagic. There were 200 white blood cells/mm3 with 90% polymorphonuclear leukocytes, 240 mg protein, and 5 mg/dL glucose in CSESmear of CSF revealed encapsulated gram-positive bacilli. Bacillus anthracis was isolated from cultures of CSF,blood, and the specimen aspirated from the parotid. The isolate was susceptible to penicillin, chloramphenicol, tetracycline, erythromycin, and ciprofloxacin. It was also sensitive to specific gamma phage. The isolate was inoculated into fresh blood and incubated at 37°C for 6 hours, and encapsulation was observed. The patient, who was medicated empirically with penicillin, chloramphenicol, mannitol, and corticosteroids, did not respond to treatment and died 20 hours after admission. Autopsy was not performed. An epidemiologic investigation revealed that the patient was a wool and hide trader. All his family members were well after 9 months, and no other death attributable to anthrax occurred in the village where he lived. DISCUSSION
Anthrax is an endemic disease in Turkey, and between 1990 and 1994,1779 human anthrax cases were recorded. Annual incidence of human anthrax cases is about 350. The majority of cases recorded were from the northeastern, eastern, and central parts of Turkey Most patients were agricultural workers. The notified human anthrax cases were cutaneous. A few cases of gastrointestinal anthrax are known to have been reported in Turkey, but inhalation anthrax has not been reported in Turkey yet.’ A case with anthrax meningitis developing from a cutaneous lesion was reported in 1985.5 The present case is the second report of anthrax meningitis in Turkey. Anthrax is a sporadically occurring disease in Isparta, which is located in southwestern Turkey Only 13 cases have been reported in this region since 1985, all of which were cutaneous anthrax with agricultural origin6 Human cases of anthrax in an agricultural environment result from direct contact with animals that are sick or have died from anthrax. It is reported that 25 workers in one textile factory contracted the disease, and the source was found to be goat hair imported from Pakistan.’
Anthrax
Anthrax spores have several characteristics suitable for a biologic weapon, such as low visibility, high potency, accessibility, and relatively easy delivery, and they could be used not only in war but also for terrorist activities. Some countries have considered anthrax as a biologic warfare agent.* An epidemic of anthrax (77 cases and 66 deaths) was caused by accidental release of anthrax spores in a narrow zone downwind of a Soviet military microbiology facility in Sverdlovsk in the Soviet Union.” The wool and hide trader described in this case may have become infected by inhalation or through direct contact with anthrax-contaminated animal wool or hide. This case is also a typical example of occupational exposure to the organism. Clinical and radiologic findings together with the history suggest that this case was inhalation anthrax complicated with meningitis. Autopsy could not be performed because of refusal by the family. The diagnosis was confirmed by isolation of 8. anthrucis from CSF,blood, and parotid aspirate cultures. Anthrax meningitis is an extremely rare entity. The most common portals of entry are skin (52.8%) and lungs (22.9%). About 11.5% of the cases are reported to be primary anthrax meningitis. I0 In the case presented here, there were no external signs of injury or lesions, and the onset of the disease, as if a cold with cough and fever, might point to a respiratory entry. Sepsis was proved in this patient by isolation of the organism from blood. Pulmonary anthrax commonly follows a biphasic clinical pattern, with a benign initial phase followed by an acute, severe second phase that is almost always fatal. The initial phase begins as a nonspecific illness with malaise, low-grade fever, and dry cough, or it may resemble a mild upper respiratory tract infection as in the present case.” However, there is a sudden onset of respiratory distress and deterioration of the patient’s general status in pulmonary anthrax. Radiologic changes are of great sign& cance in establishing the diagnosis of inhalation anthrax. There is usually pleural effusion associated with marked widening of the mediastinal shadow owing to hemorrhagic lymphadenitis.* The chest radiograph in the present case showed enlargement of the mediastinum, linear atelectasia, and pleural effusion. The average duration of the acute stage is reported to be less than 24 hours, almost always ending in death.” Several reports have described the development of an inflammatory lesion resembling a cutaneous lesion in the oral cavity, involving the posterior wall, the hard palate, or the tonsils, but the authors did not come across any reports showing that anthrax involves the parotid.‘2,1” In the present case, no lesion was noted in the oral cavity. Ingestion of contaminated meat or hematogenous spread of respiratory anthrax might cause oropharyngeal anthrax and parotitis; however, exclusive involvement of the parotid is unusual. Meningeal anthrax is described as a hemorrhagic meningitis in which intracranial arteritis is a prominent
Meningitis
/ Yorgencigil et al
221
feature.14 Other findings, as exemplified by this case, include subarachnoid hemorrhage and hemorrhage. Definitive diagnosis requires ex amining CSE Cerebrospinal fluid is commonly hemorrhagic and has contained polymorphonuclear leukocytes and anthrax bacilli. Protein and glucose levels are like those in the other bacterial meningitides. Few patients have survived anthrax meningitis, despite use of antibiotics to which the organism is sensitive in vitro.’ The patient described here did not respond to antibiotic and supportive therapy and died in a short period. The drug of choice in the treatment of anthrax is still penicillin, although isolates of penicillin-resistant B. m&rack have been reported. No penicillin-resistant B. antbruc-k infection has been observed in Turkey.15 It has been suggested that the ideal therapy in meningitis and inhalation anthrax should include a specific antitoxic serum.2~3 Control of the disease in humans depends on control of the disease in animals. Effective animal vaccines are available. A human anthrax vaccine is also available. In industry and agriculture, methods for protecting humans from contact with infectious material should be the primary concern.
REFERENCES 1. Dixon TC, Meselson M, Guillemin J, Hanna PC. Anthrax. N Engl J Med 1999; 341:815-826. 2. Tahernia AC, Hashemi C. Survival in anthrax meningitis. Pediatrics 1972; 50:329-333. 3. Tabatabaie P,Syadati A. Bacillus antbracis as a cause of bacterial meningitis. Pediatr Infect Dis J 1993; 12:1035-1037. 4. George S, Mathai D, Balraj V, LaIitha MK, John TJ. An outbreak of anthrax meningoencephalitis. Trans R Sot Trop Med Hyg 1994; 88:206-207. 5. Kiliqturgay K, GGkirmak F, TGre 0, Giiml G, Helvaci S. An anthrax meningitis case [Turkish]. J Uludag University Medical Faculty 1985; 12:73-76. 6. Doganay M. Human anthrax in Turkey. Salisbury Med Bull 1996; 87(Spec Suppl):8-10. 7. Pfisterer RM. Eine Milzbrandepidemie in der Schweiz. Schweiz Med Wochenschr 1991; 121:813-825. 8. Lew DP. Bacillus antbracis (anthrax). In: Mandell GL, Bennett JE, DoIin R, eds. Principles and practice of infectious diseases. 5th ed. New York: Churchill Livingstone, 2000: 2215-2220. 9. Abramova FA, Grinberg LM,Yampolskaya OV, Walker DH. Pathology of inhalational anthrax in 42 cases from the Sverdlovsk outbreak of 1979. Proc Nat1 Acad Sci U S A 1993; 90:2291-2294. 10. Doganay M. Anthrax. In: Proceedings of the 22nd Turkish Microbiology Congress. Turkish Microbiology Society 1986:
7:25-40. 11. Penn CC, Klotz SA. Anthrax pneumonia. Semin Respir Infect 1997; 12:28-30. 12. Onerci M, Ergin NT. Oropharyngeal anthrax. Laryngorhinootologie 1993; 72:350-351. 13. Navacharoen N, Sirisanthana T, Navacharoen W, Ruckphaopunt K. Oropharyngeal anthrax. J Laryngol Otol 1985; 99:1293-1295. 14. Range1 RA, Gonzalez DA. Bacillus anthracic meningitis. Neurology 1975; 25:525-530. 15. Doganay M, Aydin N. Antimicrobial susceptibility of Bacillus anthracis. Stand J Infect Dis 1991; 23:333-335.
Anthrax Meningitis:
Case Report
BirdalYorgancigil, MD;* Mustafa Demirci, MD;+ Mehmet Unlu, MD:* Erdogan Setik, MD;§ and Mehmet Doganay, MD” Anthrax occurs in three clinical forms in humans: cutaneous, gastrointestinal, and pulmonary Meningitis is rarely seen and is always fatal. It may be a complication of any of the three forms as a result of lymphohematogenous spread from the primary lesions or may develop as primary infection, where anthrax is endemic. It is noted that about 100 cases with anthrax meningitis have been reported in the world literature.‘-* This report presents a case of hemorrhagic meningitis due to Bacillus anthracis. This is believed to be the second article about anthrax meningitis in Turkey. The first case was reported 12 years ago.5 CASE
REPORT
A 64-year-old man was admitted to hospital with high fever, shortness of breath, and unconsciousness. His complaints had begun 3 days before his admission with malaise, headache, and abdominal pain. On examination, the patient was comatose and pupils were dilated. His temperature was 40°C pulse rate lbO/min, and respiratory rate 40/min. His blood pressure was loo/60 mmHg. Neurologic examination revealed indefinite meningeal signs without neck rigidity, negative Kernig and Brudzinski signs and Babinski reflex. Cornea1 and deep tendon reflexes were both negative, and the patient did not respond to painful stimuli. His right retroauricular region and neck were slightly swollen and hyperemic, suggesting parotitis. There were no cutaneous lesions. Radiologic examination of the chest revealed enlargement of the mediastinal lymph nodes, linear atelectasis, bronchiectasis, and minimal pleural effusion. Subamchnoid hemorrhage was found in cerebral computed tomography
*Assistant Professor, ‘Specialist in Clinical Microbiology, *Assistant Professor, Siileyman Demirel University, Medical Faculty, Department of Chest Diseases, Isparta, Turkey; %peciaiist in of Infectious Diseases, Isparta State Hospital, Isparta, Turkey; and qProfessor, Erciyes University, Medical Faculty, Department of Infectious Diseases, Kayseri, Turkey. Address correspondence to Dr. Mustafa Demirci, H?z+rbey Sk. No. 5D: 32100, Isparta,Turkey. E-mail: mdemircMdr.com.
220
Mah. 1546
White blood cell count was 18,500/mm3, and erythrocyte sedimentation rate (ESR) was 21 mm/h. On lumbar puncture, cerebrospinal fluid (CSF) was projectile and hemorrhagic. There were 200 white blood cells/mm3 with 90% polymorphonuclear leukocytes, 240 mg protein, and 5 mg/dL glucose in CSESmear of CSF revealed encapsulated gram-positive bacilli. Bacillus anthracis was isolated from cultures of CSF,blood, and the specimen aspirated from the parotid. The isolate was susceptible to penicillin, chloramphenicol, tetracycline, erythromycin, and ciprofloxacin. It was also sensitive to specific gamma phage. The isolate was inoculated into fresh blood and incubated at 37°C for 6 hours, and encapsulation was observed. The patient, who was medicated empirically with penicillin, chloramphenicol, mannitol, and corticosteroids, did not respond to treatment and died 20 hours after admission. Autopsy was not performed. An epidemiologic investigation revealed that the patient was a wool and hide trader. All his family members were well after 9 months, and no other death attributable to anthrax occurred in the village where he lived. DISCUSSION
Anthrax is an endemic disease in Turkey, and between 1990 and 1994,1779 human anthrax cases were recorded. Annual incidence of human anthrax cases is about 350. The majority of cases recorded were from the northeastern, eastern, and central parts of Turkey Most patients were agricultural workers. The notified human anthrax cases were cutaneous. A few cases of gastrointestinal anthrax are known to have been reported in Turkey, but inhalation anthrax has not been reported in Turkey yet.’ A case with anthrax meningitis developing from a cutaneous lesion was reported in 1985.5 The present case is the second report of anthrax meningitis in Turkey. Anthrax is a sporadically occurring disease in Isparta, which is located in southwestern Turkey Only 13 cases have been reported in this region since 1985, all of which were cutaneous anthrax with agricultural origin6 Human cases of anthrax in an agricultural environment result from direct contact with animals that are sick or have died from anthrax. It is reported that 25 workers in one textile factory contracted the disease, and the source was found to be goat hair imported from Pakistan.’
Anthrax
Anthrax spores have several characteristics suitable for a biologic weapon, such as low visibility, high potency, accessibility, and relatively easy delivery, and they could be used not only in war but also for terrorist activities. Some countries have considered anthrax as a biologic warfare agent.* An epidemic of anthrax (77 cases and 66 deaths) was caused by accidental release of anthrax spores in a narrow zone downwind of a Soviet military microbiology facility in Sverdlovsk in the Soviet Union.” The wool and hide trader described in this case may have become infected by inhalation or through direct contact with anthrax-contaminated animal wool or hide. This case is also a typical example of occupational exposure to the organism. Clinical and radiologic findings together with the history suggest that this case was inhalation anthrax complicated with meningitis. Autopsy could not be performed because of refusal by the family. The diagnosis was confirmed by isolation of 8. anthrucis from CSF,blood, and parotid aspirate cultures. Anthrax meningitis is an extremely rare entity. The most common portals of entry are skin (52.8%) and lungs (22.9%). About 11.5% of the cases are reported to be primary anthrax meningitis. I0 In the case presented here, there were no external signs of injury or lesions, and the onset of the disease, as if a cold with cough and fever, might point to a respiratory entry. Sepsis was proved in this patient by isolation of the organism from blood. Pulmonary anthrax commonly follows a biphasic clinical pattern, with a benign initial phase followed by an acute, severe second phase that is almost always fatal. The initial phase begins as a nonspecific illness with malaise, low-grade fever, and dry cough, or it may resemble a mild upper respiratory tract infection as in the present case.” However, there is a sudden onset of respiratory distress and deterioration of the patient’s general status in pulmonary anthrax. Radiologic changes are of great sign& cance in establishing the diagnosis of inhalation anthrax. There is usually pleural effusion associated with marked widening of the mediastinal shadow owing to hemorrhagic lymphadenitis.* The chest radiograph in the present case showed enlargement of the mediastinum, linear atelectasia, and pleural effusion. The average duration of the acute stage is reported to be less than 24 hours, almost always ending in death.” Several reports have described the development of an inflammatory lesion resembling a cutaneous lesion in the oral cavity, involving the posterior wall, the hard palate, or the tonsils, but the authors did not come across any reports showing that anthrax involves the parotid.‘2,1” In the present case, no lesion was noted in the oral cavity. Ingestion of contaminated meat or hematogenous spread of respiratory anthrax might cause oropharyngeal anthrax and parotitis; however, exclusive involvement of the parotid is unusual. Meningeal anthrax is described as a hemorrhagic meningitis in which intracranial arteritis is a prominent
Meningitis
/ Yorgencigil et al
221
feature.14 Other findings, as exemplified by this case, include subarachnoid hemorrhage and hemorrhage. Definitive diagnosis requires ex amining CSE Cerebrospinal fluid is commonly hemorrhagic and has contained polymorphonuclear leukocytes and anthrax bacilli. Protein and glucose levels are like those in the other bacterial meningitides. Few patients have survived anthrax meningitis, despite use of antibiotics to which the organism is sensitive in vitro.’ The patient described here did not respond to antibiotic and supportive therapy and died in a short period. The drug of choice in the treatment of anthrax is still penicillin, although isolates of penicillin-resistant B. m&rack have been reported. No penicillin-resistant B. antbruc-k infection has been observed in Turkey.15 It has been suggested that the ideal therapy in meningitis and inhalation anthrax should include a specific antitoxic serum.2~3 Control of the disease in humans depends on control of the disease in animals. Effective animal vaccines are available. A human anthrax vaccine is also available. In industry and agriculture, methods for protecting humans from contact with infectious material should be the primary concern.
REFERENCES 1. Dixon TC, Meselson M, Guillemin J, Hanna PC. Anthrax. N Engl J Med 1999; 341:815-826. 2. Tahernia AC, Hashemi C. Survival in anthrax meningitis. Pediatrics 1972; 50:329-333. 3. Tabatabaie P,Syadati A. Bacillus antbracis as a cause of bacterial meningitis. Pediatr Infect Dis J 1993; 12:1035-1037. 4. George S, Mathai D, Balraj V, LaIitha MK, John TJ. An outbreak of anthrax meningoencephalitis. Trans R Sot Trop Med Hyg 1994; 88:206-207. 5. Kiliqturgay K, GGkirmak F, TGre 0, Giiml G, Helvaci S. An anthrax meningitis case [Turkish]. J Uludag University Medical Faculty 1985; 12:73-76. 6. Doganay M. Human anthrax in Turkey. Salisbury Med Bull 1996; 87(Spec Suppl):8-10. 7. Pfisterer RM. Eine Milzbrandepidemie in der Schweiz. Schweiz Med Wochenschr 1991; 121:813-825. 8. Lew DP. Bacillus antbracis (anthrax). In: Mandell GL, Bennett JE, DoIin R, eds. Principles and practice of infectious diseases. 5th ed. New York: Churchill Livingstone, 2000: 2215-2220. 9. Abramova FA, Grinberg LM,Yampolskaya OV, Walker DH. Pathology of inhalational anthrax in 42 cases from the Sverdlovsk outbreak of 1979. Proc Nat1 Acad Sci U S A 1993; 90:2291-2294. 10. Doganay M. Anthrax. In: Proceedings of the 22nd Turkish Microbiology Congress. Turkish Microbiology Society 1986:
7:25-40. 11. Penn CC, Klotz SA. Anthrax pneumonia. Semin Respir Infect 1997; 12:28-30. 12. Onerci M, Ergin NT. Oropharyngeal anthrax. Laryngorhinootologie 1993; 72:350-351. 13. Navacharoen N, Sirisanthana T, Navacharoen W, Ruckphaopunt K. Oropharyngeal anthrax. J Laryngol Otol 1985; 99:1293-1295. 14. Range1 RA, Gonzalez DA. Bacillus anthracic meningitis. Neurology 1975; 25:525-530. 15. Doganay M, Aydin N. Antimicrobial susceptibility of Bacillus anthracis. Stand J Infect Dis 1991; 23:333-335.