SI! Refer to: Carmalt JE, Theis J, Goldstein E: Spinal cysticercosis. West J Med 123:311-313, Oct 1975
Spinal Cysticercosis JANICE E. CARMALT, MD JEROLD THEIS, DVM ELLIOT GOLDSTEIN, MD Davis, California
INFECTION OF HUMANS with the larval form of the tapeworm, Taenia solium, is common in some parts of the world including Mexico. Sites of infection commonly include the eyes, subcutaneous tissues, muscles, and the brain; less commonly involved are the heart, liver, lungs, abdominal cavity and quite rarely, the spinal cord.' In our patient, features consistent with either a spinal cord tumor or tuberculous meningitis were seen. In fact, however, cysticercosis of the spinal cord was present.
Report of a Case A 24-year-old Mexican farm laborer was transferred to the Neurosurgical Service of the University of California, Davis-Sacramento Medical Center in July 1974 after findings on a myelogram done at an outside hospital showed multiple filling defectg. While in Mexico in 1961 the patient had had an episode of "complete paralysis" which resolved after six months. He was told he had recovered from poliomyelitis. In 1969 he was From the Section of Infectious and Immunologic Diseases, Department of Internal Medicine (Drs. Carmalt and Goldstein); and Department of Medical Microbiology (Dr. Theis); University of California, Davis, School of Medicine. Submitted February 3, 1975. Reprint requests to: J. E. Carmalt, MD, Department of Internal Medicine, Section of Infectious and Immunologic Diseases, University of California, Davis, School of Medicine, Davis, CA 95616.
OR
TS
admitted to a hospital in California with fever and meningismus. A specimen of cerebrospinal fluid (CSF) showed the following on testing: leukocytes 18,000 per cu mm with 82 percent polymorphonuclear leukocytes; protein 196 mg per 100 ml; glucose 6 mg per 100 ml. Because tuberculous meningitis was considered likely, antituberculous therapy was instituted; however, cultures of the lumbar CSF for acid-fast bacilli, fungi and bacteria were sterile. The patient evidently recovered and was well until the present episode occurred. In early 1974, the patient noted progressive weakness and muscle fatigue of both legs with cramping in the left leg, and mild, evanescent headaches. In April 1974, he experienced eight days of fever and stiff neck; in May 1974, severe headaches, nausea, vomiting and low back pain which radiated to both legs developed. The medical history was otherwise unremarkable. The only -familial disease was epilepsy in a brother living in Mexico. On physical examination, mild paraspinous tenderness in the region of the fourth to fifth lumbar vertebrae was noted, with no sciatic notch tenderness. Straight leg raising to 300 bilaterally produced back pain with lower extremity radiation. Cranial nerves, deep tendon reflexes, gait and sensory examination findings were normal. The hemoglobin was 13.4 grams per 100 ml, the hematocrit was 39.7 percent, the leukocyte count was 8,700 per cu mm with 60 percent polymorphonuclear leukocytes, 1 percent bands, 21 percent lymphocytes, 5 percent eosinophiles, 4 percent basophiles and 8 percent monocytes. Liver function tests gave findings within normal limits except for lactic dehydrogenase of 250 mu per ml (normal 100 to 225 mu per ml) and total protein 8.2 grams per 100 ml (normal 6 to 8 grams per 100 ml). Results of urinalysis, and prothrombin time and partial thromboplastin time were normal. By serum immunoelectrophoresis, the immunoglobulin M (IgM) was 170 mg per 100 ml (normal 50 to 110 mg per 100 ml) with normal concentrations of immunoglobulin G (IgG) and immunoglobulin A (IgA). Roentgenograms of the chest, lumbosacral spine, skull and THE WESTERN JOURNAL OF MEDICINE
311
CASE REPORTS
right hip were interpreted as normal; no calcifications were seen. The day after admission, multiple filling defects were seen on myelography in the lumbar and thoracic regions and were radiologically consistent in appearance with neurofibromas (Figure 1). Cerebrospinal fluid obtained at myelography con-
Figure 1.-Lateral and anteroposterior views of gram showing tumor-like defects.
myelo-
Figure 2.-Saggital section of cysticercus of Taenia solium removed at surgical operation. Expanded from X9.6 using hematoxylin-eosin stain.
312
OCTOBER 1975
-
123
* 4
tained 154 mg of protein per 100 ml; 10 mg of glucose per 100 ml; 196 leukocytes per cu mm with 5 percent polymorphonuclear and 95 percent mononuclear cells. India ink stain, acid-fast stain, Gram stain, acid-fast culture, bacterial and fungal cultures gave negative results. By cytologic examination, sheets of cells were noted which were thought to be ependymal in origin. A Gallium-67 scan showed increased uptake in the area of the fourth to fifth lumbar vertebrae. Eleven days after admission, papilledema was noted but the patient was alert, oriented and otherwise unchanged neurologically. There were no abnormalities found on electroencephalography, brain scan and orbital venography. Thirteen days after admission, the lumbar cerebrospinal fluid was under a pressure of 90 mm of mercury; protein, 216 mg per 100 ml; glucose, 3 mg per 100 ml; leukocytes, 27 per cu mm with 18 percent polymorphonuclear and 82 percent mononuclear cells. Again search for microorganisms was unrewarding. Fourteen days after admission, somnolence, meningismus and a temperature of 39°C (103°F) developed. Antituberculous therapy was begun, but the patient failed to improve. Ventriculography and cerebral arteriography documented the presence of communicating hydrocephalus, and raised the possibility of a lesion in the region of the left cerebellopontine angle. On the seventeenth hospital day lumbar laminectomy was carried out. Multiple subarachnoid cysts were removed and a lumboperitoneal shunt- was established. By histopathologic examination, the cysts were found to be cysticerci of Taenia solium
Figure 3.-Higher magnification (x 320) of cuticle from cysticercus of Taenia solium characteristic wart-like projections of cuticle.
CASE REPORTS
(Figures 2 and 3). The cysticercal indirect hemagglutination titer of the patient's serum was subsequently reported as positive at a dilution of 1: 2,048. Cerebrospinal fluid titers were not run. The patient and immediate relatives were advised of the definitive diagnosis. The importance of medical observations following the initial discharge from the hospital was stressed. In spite of the use of an interpreter and several sessions of explanation, the family refused to believe the diagnosis, and the patient was lost to follow-up.
Discussion Pigs are the normal intermediate hosts of Taenia solium; human involvement may occur by two means: (1) larvae may be ingested in infected, undercooked pork resulting in the growth of an adult tapeworm in the human gut and (2) ova may enter by way of food or drink contaminated with human feces, or autoinfection may occur in the person harboring Taenia solium by regurgitation of intestinal contents into the stomach.5 By the second method humans may become infected with the larval stages, which can be found in muscles, as well as brain, skin and eyes. Clinical manifestations vary with the location of the parasites; when infection involves the central nervous system, epilepsy, transient pareses, intermittent obstructive hydrocephalus, dysequilibrium, meningoencephalitis, involuntary movements, failing vision and mental disturbances have been reported.4 Reports of spinal cysticercosis are exceedingly rare;9"°0 thus, of 450 cases of cysticercosis in British soldiers, only two cases involved the spinal cord.7 Hesketh reported cysticercosis of the dorsal cord mimicking tumor and diagnosed only at surgical operation.9 Laboratory studies are of little help in making a diagnosis; cerebrospinal fluid and peripheral blood eosinophilia may be present. On roentgenography, calcified cysts in the soft tissues and brain may be noted.5 The intradermal and complement fixation tests for cysticercosis have not received wide acceptance because of their reported unreliability in proven cases.6'7 Two immunological tests, somewhat more recently developed, appear to be more reliable.2'3 These are the precipitin ring test and indirect hemagglutination test. Findings on both have been reported positive even at high dilutions of serum, although selection of the antigen source may be critical in determining the sensitivity.2
Although positive reactions using cerebrospinal fluid have been reported with the precipitin ring test in proven cases,2 the indirect hemagglutination reaction has been reported less satisfactory using cerebrospinal fluid.2 The techniques for the. serological tests are reported elsewhere.2 3 In our patient, cerebrospinal fluid had all of the features of tuberculous meningitis including low glucose, high pressure and protein, and mononuclear pleocytosis. There also was a history suggestive of previous tuberculous disease and during the course of the illness, papilledema and mental changes developed suggesting cerebrospinal fluid block. Myelographic findings indicated spinal cord tumors, thought to be neurofibromas. Although epilepsy is a common manifestation of central nervous system cysticercosis,"4-6' 7" there was no history of seizures in this patient. Possibly the strange episode of paralysis and the unusual meningitis several years earlier represent symptoms of parasitic infection. Epilepsy has been reported as a sequela to cerebral cysticercosis.8 Although proof is lacking, epilepsy reported in the brother of our patient may have been due to cysticercosis. In summary, our patient has an extremely rare form of a disease which is common in the country of his origin. The diagnosis was made at surgical operation, after an exhaustive battery of investigations was unhelpful. Physicians confronted with perplexing central nervous system disorders in persons from parts of the world in which T. solium infection is endemic would be well advised to include cysticercal serologic testing early in the evaluation of such patients. REFERENCES 1. Faust EC, Russell PF, Jung RC: Craig and Faust's Clinical Parasitology, 8th Ed. Philadelphia, Lea & Febiger, 1970, p 529 2. Biagi FF, Tay J: A precipitation reaction for the diagnosis of cysticercosis. Am J Trop Med Hyg 7:63-65, Jan 1958 3. Biagi FF, Navarrete F, Pifia A, et al: Estudio de tres reacciones serologicas en el diagnostico de la cisticercosis. Rev Med Hosp Gen (Mexico City) 24:501-508, Nov-Dec 1961 4. Bickerstaff ER: Cerebral cysticercosis: Common but unfamiliar manifestations. Br Med J 1: 1055-1058, Apr 30, 1955 5. Brailsford F: Cysticercus cellulosae-Its radiographic detection in the musculature and the central nervous system. Br J Radiol 14:79-93, Mar 1941 6. Dixon HBF, Hardgreaves WH: Cysticercosis (Taenia solium) -A further ten years' clinical study, covering 284 cases. Q J Med 13:107-121, Oct 1944 7. Dixon HBF, Lipscomb FM: Cysticercosis-An analysis and followup of 450 cases. Med Res Counc Spec Rep Ser (Lond), Ser 299, 1961, pp 58 8. Heinz HJ, MacNab GM: Cysticercosis in the Banta of Southern Africa. S Afr J Med Sci 30:19-31, Jul 1965 9. Hesketh KT: Cysticercosis of the dorsal cord. J Neurol Neurosurg Psychiatry 28:445-448, Oct 1965 10. Kahn P: Cysticercosis of the central nervous system with amyotrophic lateral sclerosis: Case report and review of the literature. J Neurol Neurosurg Psychiatry 35:81-87, Feb 1972 11. White JC, Sweet WH, Richardson EP: Cysticercosis cerebri -A diagnostic and therapeutic problem of increasing importance. N Engl J Med 256:479-486, Mar 14, 1957
THE WESTERN JOURNAL OF MEDICINE
313
Spinal Cysticercosis JANICE E. CARMALT, MD JEROLD THEIS, DVM ELLIOT GOLDSTEIN, MD Davis, California
INFECTION OF HUMANS with the larval form of the tapeworm, Taenia solium, is common in some parts of the world including Mexico. Sites of infection commonly include the eyes, subcutaneous tissues, muscles, and the brain; less commonly involved are the heart, liver, lungs, abdominal cavity and quite rarely, the spinal cord.' In our patient, features consistent with either a spinal cord tumor or tuberculous meningitis were seen. In fact, however, cysticercosis of the spinal cord was present.
Report of a Case A 24-year-old Mexican farm laborer was transferred to the Neurosurgical Service of the University of California, Davis-Sacramento Medical Center in July 1974 after findings on a myelogram done at an outside hospital showed multiple filling defectg. While in Mexico in 1961 the patient had had an episode of "complete paralysis" which resolved after six months. He was told he had recovered from poliomyelitis. In 1969 he was From the Section of Infectious and Immunologic Diseases, Department of Internal Medicine (Drs. Carmalt and Goldstein); and Department of Medical Microbiology (Dr. Theis); University of California, Davis, School of Medicine. Submitted February 3, 1975. Reprint requests to: J. E. Carmalt, MD, Department of Internal Medicine, Section of Infectious and Immunologic Diseases, University of California, Davis, School of Medicine, Davis, CA 95616.
OR
TS
admitted to a hospital in California with fever and meningismus. A specimen of cerebrospinal fluid (CSF) showed the following on testing: leukocytes 18,000 per cu mm with 82 percent polymorphonuclear leukocytes; protein 196 mg per 100 ml; glucose 6 mg per 100 ml. Because tuberculous meningitis was considered likely, antituberculous therapy was instituted; however, cultures of the lumbar CSF for acid-fast bacilli, fungi and bacteria were sterile. The patient evidently recovered and was well until the present episode occurred. In early 1974, the patient noted progressive weakness and muscle fatigue of both legs with cramping in the left leg, and mild, evanescent headaches. In April 1974, he experienced eight days of fever and stiff neck; in May 1974, severe headaches, nausea, vomiting and low back pain which radiated to both legs developed. The medical history was otherwise unremarkable. The only -familial disease was epilepsy in a brother living in Mexico. On physical examination, mild paraspinous tenderness in the region of the fourth to fifth lumbar vertebrae was noted, with no sciatic notch tenderness. Straight leg raising to 300 bilaterally produced back pain with lower extremity radiation. Cranial nerves, deep tendon reflexes, gait and sensory examination findings were normal. The hemoglobin was 13.4 grams per 100 ml, the hematocrit was 39.7 percent, the leukocyte count was 8,700 per cu mm with 60 percent polymorphonuclear leukocytes, 1 percent bands, 21 percent lymphocytes, 5 percent eosinophiles, 4 percent basophiles and 8 percent monocytes. Liver function tests gave findings within normal limits except for lactic dehydrogenase of 250 mu per ml (normal 100 to 225 mu per ml) and total protein 8.2 grams per 100 ml (normal 6 to 8 grams per 100 ml). Results of urinalysis, and prothrombin time and partial thromboplastin time were normal. By serum immunoelectrophoresis, the immunoglobulin M (IgM) was 170 mg per 100 ml (normal 50 to 110 mg per 100 ml) with normal concentrations of immunoglobulin G (IgG) and immunoglobulin A (IgA). Roentgenograms of the chest, lumbosacral spine, skull and THE WESTERN JOURNAL OF MEDICINE
311
CASE REPORTS
right hip were interpreted as normal; no calcifications were seen. The day after admission, multiple filling defects were seen on myelography in the lumbar and thoracic regions and were radiologically consistent in appearance with neurofibromas (Figure 1). Cerebrospinal fluid obtained at myelography con-
Figure 1.-Lateral and anteroposterior views of gram showing tumor-like defects.
myelo-
Figure 2.-Saggital section of cysticercus of Taenia solium removed at surgical operation. Expanded from X9.6 using hematoxylin-eosin stain.
312
OCTOBER 1975
-
123
* 4
tained 154 mg of protein per 100 ml; 10 mg of glucose per 100 ml; 196 leukocytes per cu mm with 5 percent polymorphonuclear and 95 percent mononuclear cells. India ink stain, acid-fast stain, Gram stain, acid-fast culture, bacterial and fungal cultures gave negative results. By cytologic examination, sheets of cells were noted which were thought to be ependymal in origin. A Gallium-67 scan showed increased uptake in the area of the fourth to fifth lumbar vertebrae. Eleven days after admission, papilledema was noted but the patient was alert, oriented and otherwise unchanged neurologically. There were no abnormalities found on electroencephalography, brain scan and orbital venography. Thirteen days after admission, the lumbar cerebrospinal fluid was under a pressure of 90 mm of mercury; protein, 216 mg per 100 ml; glucose, 3 mg per 100 ml; leukocytes, 27 per cu mm with 18 percent polymorphonuclear and 82 percent mononuclear cells. Again search for microorganisms was unrewarding. Fourteen days after admission, somnolence, meningismus and a temperature of 39°C (103°F) developed. Antituberculous therapy was begun, but the patient failed to improve. Ventriculography and cerebral arteriography documented the presence of communicating hydrocephalus, and raised the possibility of a lesion in the region of the left cerebellopontine angle. On the seventeenth hospital day lumbar laminectomy was carried out. Multiple subarachnoid cysts were removed and a lumboperitoneal shunt- was established. By histopathologic examination, the cysts were found to be cysticerci of Taenia solium
Figure 3.-Higher magnification (x 320) of cuticle from cysticercus of Taenia solium characteristic wart-like projections of cuticle.
CASE REPORTS
(Figures 2 and 3). The cysticercal indirect hemagglutination titer of the patient's serum was subsequently reported as positive at a dilution of 1: 2,048. Cerebrospinal fluid titers were not run. The patient and immediate relatives were advised of the definitive diagnosis. The importance of medical observations following the initial discharge from the hospital was stressed. In spite of the use of an interpreter and several sessions of explanation, the family refused to believe the diagnosis, and the patient was lost to follow-up.
Discussion Pigs are the normal intermediate hosts of Taenia solium; human involvement may occur by two means: (1) larvae may be ingested in infected, undercooked pork resulting in the growth of an adult tapeworm in the human gut and (2) ova may enter by way of food or drink contaminated with human feces, or autoinfection may occur in the person harboring Taenia solium by regurgitation of intestinal contents into the stomach.5 By the second method humans may become infected with the larval stages, which can be found in muscles, as well as brain, skin and eyes. Clinical manifestations vary with the location of the parasites; when infection involves the central nervous system, epilepsy, transient pareses, intermittent obstructive hydrocephalus, dysequilibrium, meningoencephalitis, involuntary movements, failing vision and mental disturbances have been reported.4 Reports of spinal cysticercosis are exceedingly rare;9"°0 thus, of 450 cases of cysticercosis in British soldiers, only two cases involved the spinal cord.7 Hesketh reported cysticercosis of the dorsal cord mimicking tumor and diagnosed only at surgical operation.9 Laboratory studies are of little help in making a diagnosis; cerebrospinal fluid and peripheral blood eosinophilia may be present. On roentgenography, calcified cysts in the soft tissues and brain may be noted.5 The intradermal and complement fixation tests for cysticercosis have not received wide acceptance because of their reported unreliability in proven cases.6'7 Two immunological tests, somewhat more recently developed, appear to be more reliable.2'3 These are the precipitin ring test and indirect hemagglutination test. Findings on both have been reported positive even at high dilutions of serum, although selection of the antigen source may be critical in determining the sensitivity.2
Although positive reactions using cerebrospinal fluid have been reported with the precipitin ring test in proven cases,2 the indirect hemagglutination reaction has been reported less satisfactory using cerebrospinal fluid.2 The techniques for the. serological tests are reported elsewhere.2 3 In our patient, cerebrospinal fluid had all of the features of tuberculous meningitis including low glucose, high pressure and protein, and mononuclear pleocytosis. There also was a history suggestive of previous tuberculous disease and during the course of the illness, papilledema and mental changes developed suggesting cerebrospinal fluid block. Myelographic findings indicated spinal cord tumors, thought to be neurofibromas. Although epilepsy is a common manifestation of central nervous system cysticercosis,"4-6' 7" there was no history of seizures in this patient. Possibly the strange episode of paralysis and the unusual meningitis several years earlier represent symptoms of parasitic infection. Epilepsy has been reported as a sequela to cerebral cysticercosis.8 Although proof is lacking, epilepsy reported in the brother of our patient may have been due to cysticercosis. In summary, our patient has an extremely rare form of a disease which is common in the country of his origin. The diagnosis was made at surgical operation, after an exhaustive battery of investigations was unhelpful. Physicians confronted with perplexing central nervous system disorders in persons from parts of the world in which T. solium infection is endemic would be well advised to include cysticercal serologic testing early in the evaluation of such patients. REFERENCES 1. Faust EC, Russell PF, Jung RC: Craig and Faust's Clinical Parasitology, 8th Ed. Philadelphia, Lea & Febiger, 1970, p 529 2. Biagi FF, Tay J: A precipitation reaction for the diagnosis of cysticercosis. Am J Trop Med Hyg 7:63-65, Jan 1958 3. Biagi FF, Navarrete F, Pifia A, et al: Estudio de tres reacciones serologicas en el diagnostico de la cisticercosis. Rev Med Hosp Gen (Mexico City) 24:501-508, Nov-Dec 1961 4. Bickerstaff ER: Cerebral cysticercosis: Common but unfamiliar manifestations. Br Med J 1: 1055-1058, Apr 30, 1955 5. Brailsford F: Cysticercus cellulosae-Its radiographic detection in the musculature and the central nervous system. Br J Radiol 14:79-93, Mar 1941 6. Dixon HBF, Hardgreaves WH: Cysticercosis (Taenia solium) -A further ten years' clinical study, covering 284 cases. Q J Med 13:107-121, Oct 1944 7. Dixon HBF, Lipscomb FM: Cysticercosis-An analysis and followup of 450 cases. Med Res Counc Spec Rep Ser (Lond), Ser 299, 1961, pp 58 8. Heinz HJ, MacNab GM: Cysticercosis in the Banta of Southern Africa. S Afr J Med Sci 30:19-31, Jul 1965 9. Hesketh KT: Cysticercosis of the dorsal cord. J Neurol Neurosurg Psychiatry 28:445-448, Oct 1965 10. Kahn P: Cysticercosis of the central nervous system with amyotrophic lateral sclerosis: Case report and review of the literature. J Neurol Neurosurg Psychiatry 35:81-87, Feb 1972 11. White JC, Sweet WH, Richardson EP: Cysticercosis cerebri -A diagnostic and therapeutic problem of increasing importance. N Engl J Med 256:479-486, Mar 14, 1957
THE WESTERN JOURNAL OF MEDICINE
313
