Neurnrodiolngy
Neuroradiology 13, 1-5 (1977)
@ by Springer-Verlag 1977
ORIGINALS
Jmgiographic Findings Mth an Intracranial Gumma F. Y. Tsai 1, A. O. Schilp 2, and J. S. Leo 1 Section of Neuroradiology 1 and Division of Neurosurgery2, Albany Medical Center Hospital, Albany, N. Y., USA
Summary. The case of a syphilitic gumma in the cerebellopontine angle is reported. The angiographic appearance of neurosyphilis is similar to that of cerebral inflammatory processes of all varieties. The hypervascularity of the blush shown on angiography is due to hyperemia surrounding the focal necrosis. Vasoconstriction and dilatation may be seen with meningovascular syphilis and gummata. This case showed a focal hypervascularized lesion unlike the avascular lesions described in the textbooks.
ture and findings can mimic inflammatory disorders or neoplasms and many patients with cerebral gummata have been operated upon because of a suspected intracranial tumor. The angiographic appearance of cerebral syphilitic gumma has not been documented specifically in textbooks of angiography [9]. We have found only one report [4] in which the angiographic findings in a patient with a parietal gumma have been described. The following case report is an example of the angiographic findings in such a disorder.
Key words: Syphilis - Gumma - Angiography Cerebellopontine gumma.
Case Report
Cerebral syphilitic gummatous disease was seen, not infrequently before, and even during, the first half of this century. The incidence declined remarkably after the discovery of penicillin [4, 8, 12]. The clinical pic-
a
A 39 year old woman experienced sudden loss of hearing with tinnitus in the right ear while speaking on the phone four weeks before admission. She also complained about some incoordination of gait and double
iJ
Fig. 1. a and b. AP tomograms of the righ~ and left petrous bones show normal internal auditory canals and petrous tips
F. Y. Tsai et al.: Angiographic Findings with an Intracranial Gumma
Fig. 2. Magnification AP view of vertebral angiogram shows a small hypervascular lesion in the right CPA
vision which had disappeared by the time of admission. Two days prior to admission she felt sharp, stabbing pain behind the right ear. Neurological examination on admission revealed nystagmus on left lateral gaze with the fast component to the left and a diminished right corneal response due to a profound peripheral facial weakness on the right. Tone was lateralized to the left with the Weber test and no sound was recognized on the right side. Audiometry and cold caloric testing showed no response from the right ear. Routine laboratory studies were normal. VDRL and FFA-ABS were positive. The CSF contained protein of 38 rag%, glucose of 70/110 mg%, and 125 WBC
with 80% lymphocytes. The CSF cytology was class II and the serology was positive. Skull radiograms and tomograms of the petrous bones were normal (Fig. 1). Vertebral angiography showed a small focal hypervascular mass in the right cerebellopontine angle (CPA) (Fig. 2). Air encephalography and pantopaque cisternography demonstrated a mass measuring approximately 1 X 1.5 cm in the right CPA (Fig. 3). The meninges of the posterior fossa appeared normal on exploration of the right CPA. A 1 cm sized mass was firmly adherent to the acoustic nerve. With microdissection, utilizing the operating microscope, the lesion, greyish in color, was removed from the acoustic nerve at the internal acoustic meatus. A small portion of the mass which extended into the meatus had to be brought forward with an angled grasping forceps. Some fascicles of the eighth nerve had been spread apart by the mass. The seventh nerve was immediately inferior to the eighth nerve and both nerves seemed to be flattened and compressed by the mass at the meatus. No other abnormalities were seen. The postoperative recovery was uneventful. Histological examination of the lesion showed macrophages and neuronal fibrils with necrosis and acute inflammatory cell infiltration (Fig. 4). Discussion
According to Anderson [1], the evidence of syphilitic infection of the central nervous system may occur in the primary, secondary or tertiary stages. In the primary stage it is self limited and may disappear spontaneously. Clinical evidence of neurosyphilis most commonly occurs during the secondary stage with
Fig. 3. a Air encephalogram showed a small mass effect in the right CPA. b Pantopaque cisternogram showed a small mass at the right porus acousticus
F. Y. Tsai et al.: Angiographic Findings with an Intracranial Gumma
3
Fig. 4. a Epithelial hyperplasia of vascular wall on the left (arrowhead); plasma cells and macrophages in the right lower corner (H.-E. x 47,5). b Plasma cells are in the field (arrowheads), a macrophage in right lower corner (H.-E. x 152). Levaditi stain for spirochetes did not reveal any organisms
F. Y. Tsai et al.: Angiographic Findings with an Intracranial Gumma
prominent symptoms of meningeal irritation. Only 5 to 10% of neurosyphilis reappears in an active form after remaining latent for a period of several years. Active neurosyphilis occurs as two separate clinical entities, meningovascular and parenchymatous syphilis. Meningovascular syphilis is associated predominantly with arteritis, leptomeningitis and pachymeningitis. Parenchymatous syphilis manifests itself by paresis, tabes or myelitis. The cerebral manifestations of neurosyphilis may rarely be in the form of a gumma [1], which is a focal, nonsuppurative, inflammatory process with an area of central necrosis. Gummas usually originate in a subcortical area and may grow large enough to invade the dura via the vessels [1, 3, 16]. Bagdasar [2] reported 8 cases of cerebral gummata in his series observed up to 1927. Cushing [5] found 5 cases of cerebral gummata in 194 intracranial tumors in his Baltimore series, observed from 1908 to 1912. Subsequently in his Boston series of 1500 surgical operations of the brain from 1913 to 1927, he found 7 cerebral gummata. Dandy [7] did not mention the exact incidence, but he concluded that the surgical findings of cerebral gumma were exceedingly rare. Olivecrona [17] found only one case of cerebral gumma in his series of neoplasms. Obrador [16] described cerebral gumma as an avascular lesion similar to that of cerebral tuberculoma, but there were no illustrations of cerebral angiograms in his chapter. Bianchi and Frera [4] reported a cerebral gumma in a 35 year old man with carotid angiograms which showed irregular and slender lumina of the anterior cerebral arteries toward the left. Rabinov [18] subsequently described the angiographic findings of meningovascular syphilis in a 46 year old male in which the carotid angiogram demonstrated small lumina of the anterior cerebral arteries. A localized blush with narrowing of the superficial veins was also shown. He concluded that the absence of a mass effect in his case was the major point in differentiating the angiographic findings of meningovascular syphilis from cerebral gumma. Ferris et al. [9, 10] considered that the hypervascularity of cerebral infection was probably secondary to focal arterial occlusion. Leeds and Goldberg [14] described vasoconstriction or dilatation as a frequent occurrence with cerebral inflammatory disease of all varieties. The arterial narrowing was considered to be due to encroachment by inflammatory exudate as a result of cellular infiltration, vasospasm, or a combination of both. The angiographic findings described by Bianchi and Frera [4] and Rabinov [18] were probably related to an inflammatory response of the perivascular structures, showing a focal hyperemia surrounding the central necrosis [13]. an inflamma-
tory mass might also show narrowing of the adjacent vessels associated with hypervascularity and a blush. In the present case, the carotid and vertebral angiograms failed to demonstrate any irregularity or narrowing of the intracranial vessels. A small focal vascular mass was seen in the right cerebellopontine angle. The venous structures appeared to be unremarkable. The angiographic findings were not quite similar to the reports of the authors mentioned above. The focal vascular lesion could be due to a small arterial occlusion secondary to central necrosis of the lesion. The angiographic appearance is somewhat like the hypervascular neurinoma described by Moscow and Newton [15]. The findings did not suggest inflammation and were interpreted as representing a neurinoma or meningioma. Other tumors less frequently found in the CPA are epidermoids, gliomas, arachnoid cysts, occasional aneurysms and metastatic carcinomas [6, 11]. Although neurosyphilis is rare today, the disease remains endemic. The surge in the incidence of syphilis in the 1960s will probably lead to an increase of neurosyphilis in the future. Since the clinical symptoms and signs of cerebral gummata will mimic those of intracranial neoplasms, cerebral angiography will still inevitably be performed on some cases of neurosyphilis. The presence of a focal area of hypervascularity might then, in such cases, represent gumma formation. Correlation with "other aspects of this disease and the positive serology in both blood and CSF will confirm the diagnosis. In cases of neurosyphilis, where the progress of the disease is slow and the patient's deterioration not critical, consideration should be given to the use of penicillin therapy during an appropriate observation period. Whether this will alter the pathogenesis of cerebral syphilitic gumma is not exactly known [16].
References 1. Anderson, W. ed.: Pathology. pp. 1323-1325 St. Louis: Mosby 1953 2. Bagdasar, D.: Le traitement chirurgical des gommes cerebrales. Rev. neurol. 2, 1-30 (1929) 3. Bailey, P.: Intracranial tumors. Springfield, IlL: Thomas 1933 4. Bianchi, M., Frera, C.: A case of brain gumma. J. Neurol. Neurosurg. Psychiat. 20, 133-135 (1957) 5. Cushing, H.: Intracranial tumors. Springfield, II1.: Thomas 1932 6. Dale, A. J. D.: The cerebellopontine angle syndrome. Med. C1. NA. 52, 789-795 (1968) 7. Dandy, W. E.: Surgery of the brain, pp. 367-369. Hagerstown, Md.: Prior 1945 8. Fabing, H.: Neurosyphilis -- a review. Lancet 75, 346-347 (1955)
F. Y. Tsai et al.: Angiographic Findings with an Intracranial Gumma 9. Ferris, E. J.: Arteritis. Radiology of the skull and brain, VoIl. II, Bk 4, 2566-2577. Newton, T. H., Potts, D. G., eds. St. Louis: Mosby 1974 10. Ferris, E. J.: Rudikoff, J. C. Shapiro, J. H.: Cerebral angiography of cerebral infection. Radiology 90, 727-737 (1968) 11. Histselberger, W. E.: Other tumors of the cerebellopontine angle. Arch Otolaryng. 88, 712-714 (1968) 12. Joffe, R., Black, M. M., Floyd, M.: Changing clinical picture of neurosyphilis - report of seven unusual cases. Brit. med. J. 1, 211-212 (1968) 13. Lagerren, C., Lindbom, A., Soderberg, G.: Hypervascularization in chronic inflammation demonstrated by angiography angiographic, histopathologic and micro-angiographic studies. Acta radiol. 49, 441-452 (1958) 14. Leeds, N. E., Goldberg, H. I.: Angiographic manifestation in cerebral inflammatory disease. Radiology 98, 595-604 (1971) 15. Moscow, N. P., Newton, T. H.: Angiographic features of hypervascular neuromas of the head and neck. Radiology 114, 635-640 (1975)
5 16. Obrador, S.: Tuberculoma and syphilitic gumma. Textbook of neurological surgery. Youmans, J. R., ed. pp. 1569-1573. Philadelphia: Saunders 1973 17. Olivercrona, H.: The surgical treatment of intracranial tumor. Handbuch der Neurochirurgie, Vol. 4, Part 4. Berlin, Heidelberg, New York: Springer 1967 18. Rabinov, K. R.: Angiographic findings in a case of brain syphilis. Radiology 80, 622-624 (1963) Received: November 4, 1976
Feng. Y. Tsai, M. D. Department of Radiology Section of Neuroradiology Albany Medical Center Hospital Albany, NY 12208, USA
Neuroradiology 13, 1-5 (1977)
@ by Springer-Verlag 1977
ORIGINALS
Jmgiographic Findings Mth an Intracranial Gumma F. Y. Tsai 1, A. O. Schilp 2, and J. S. Leo 1 Section of Neuroradiology 1 and Division of Neurosurgery2, Albany Medical Center Hospital, Albany, N. Y., USA
Summary. The case of a syphilitic gumma in the cerebellopontine angle is reported. The angiographic appearance of neurosyphilis is similar to that of cerebral inflammatory processes of all varieties. The hypervascularity of the blush shown on angiography is due to hyperemia surrounding the focal necrosis. Vasoconstriction and dilatation may be seen with meningovascular syphilis and gummata. This case showed a focal hypervascularized lesion unlike the avascular lesions described in the textbooks.
ture and findings can mimic inflammatory disorders or neoplasms and many patients with cerebral gummata have been operated upon because of a suspected intracranial tumor. The angiographic appearance of cerebral syphilitic gumma has not been documented specifically in textbooks of angiography [9]. We have found only one report [4] in which the angiographic findings in a patient with a parietal gumma have been described. The following case report is an example of the angiographic findings in such a disorder.
Key words: Syphilis - Gumma - Angiography Cerebellopontine gumma.
Case Report
Cerebral syphilitic gummatous disease was seen, not infrequently before, and even during, the first half of this century. The incidence declined remarkably after the discovery of penicillin [4, 8, 12]. The clinical pic-
a
A 39 year old woman experienced sudden loss of hearing with tinnitus in the right ear while speaking on the phone four weeks before admission. She also complained about some incoordination of gait and double
iJ
Fig. 1. a and b. AP tomograms of the righ~ and left petrous bones show normal internal auditory canals and petrous tips
F. Y. Tsai et al.: Angiographic Findings with an Intracranial Gumma
Fig. 2. Magnification AP view of vertebral angiogram shows a small hypervascular lesion in the right CPA
vision which had disappeared by the time of admission. Two days prior to admission she felt sharp, stabbing pain behind the right ear. Neurological examination on admission revealed nystagmus on left lateral gaze with the fast component to the left and a diminished right corneal response due to a profound peripheral facial weakness on the right. Tone was lateralized to the left with the Weber test and no sound was recognized on the right side. Audiometry and cold caloric testing showed no response from the right ear. Routine laboratory studies were normal. VDRL and FFA-ABS were positive. The CSF contained protein of 38 rag%, glucose of 70/110 mg%, and 125 WBC
with 80% lymphocytes. The CSF cytology was class II and the serology was positive. Skull radiograms and tomograms of the petrous bones were normal (Fig. 1). Vertebral angiography showed a small focal hypervascular mass in the right cerebellopontine angle (CPA) (Fig. 2). Air encephalography and pantopaque cisternography demonstrated a mass measuring approximately 1 X 1.5 cm in the right CPA (Fig. 3). The meninges of the posterior fossa appeared normal on exploration of the right CPA. A 1 cm sized mass was firmly adherent to the acoustic nerve. With microdissection, utilizing the operating microscope, the lesion, greyish in color, was removed from the acoustic nerve at the internal acoustic meatus. A small portion of the mass which extended into the meatus had to be brought forward with an angled grasping forceps. Some fascicles of the eighth nerve had been spread apart by the mass. The seventh nerve was immediately inferior to the eighth nerve and both nerves seemed to be flattened and compressed by the mass at the meatus. No other abnormalities were seen. The postoperative recovery was uneventful. Histological examination of the lesion showed macrophages and neuronal fibrils with necrosis and acute inflammatory cell infiltration (Fig. 4). Discussion
According to Anderson [1], the evidence of syphilitic infection of the central nervous system may occur in the primary, secondary or tertiary stages. In the primary stage it is self limited and may disappear spontaneously. Clinical evidence of neurosyphilis most commonly occurs during the secondary stage with
Fig. 3. a Air encephalogram showed a small mass effect in the right CPA. b Pantopaque cisternogram showed a small mass at the right porus acousticus
F. Y. Tsai et al.: Angiographic Findings with an Intracranial Gumma
3
Fig. 4. a Epithelial hyperplasia of vascular wall on the left (arrowhead); plasma cells and macrophages in the right lower corner (H.-E. x 47,5). b Plasma cells are in the field (arrowheads), a macrophage in right lower corner (H.-E. x 152). Levaditi stain for spirochetes did not reveal any organisms
F. Y. Tsai et al.: Angiographic Findings with an Intracranial Gumma
prominent symptoms of meningeal irritation. Only 5 to 10% of neurosyphilis reappears in an active form after remaining latent for a period of several years. Active neurosyphilis occurs as two separate clinical entities, meningovascular and parenchymatous syphilis. Meningovascular syphilis is associated predominantly with arteritis, leptomeningitis and pachymeningitis. Parenchymatous syphilis manifests itself by paresis, tabes or myelitis. The cerebral manifestations of neurosyphilis may rarely be in the form of a gumma [1], which is a focal, nonsuppurative, inflammatory process with an area of central necrosis. Gummas usually originate in a subcortical area and may grow large enough to invade the dura via the vessels [1, 3, 16]. Bagdasar [2] reported 8 cases of cerebral gummata in his series observed up to 1927. Cushing [5] found 5 cases of cerebral gummata in 194 intracranial tumors in his Baltimore series, observed from 1908 to 1912. Subsequently in his Boston series of 1500 surgical operations of the brain from 1913 to 1927, he found 7 cerebral gummata. Dandy [7] did not mention the exact incidence, but he concluded that the surgical findings of cerebral gumma were exceedingly rare. Olivecrona [17] found only one case of cerebral gumma in his series of neoplasms. Obrador [16] described cerebral gumma as an avascular lesion similar to that of cerebral tuberculoma, but there were no illustrations of cerebral angiograms in his chapter. Bianchi and Frera [4] reported a cerebral gumma in a 35 year old man with carotid angiograms which showed irregular and slender lumina of the anterior cerebral arteries toward the left. Rabinov [18] subsequently described the angiographic findings of meningovascular syphilis in a 46 year old male in which the carotid angiogram demonstrated small lumina of the anterior cerebral arteries. A localized blush with narrowing of the superficial veins was also shown. He concluded that the absence of a mass effect in his case was the major point in differentiating the angiographic findings of meningovascular syphilis from cerebral gumma. Ferris et al. [9, 10] considered that the hypervascularity of cerebral infection was probably secondary to focal arterial occlusion. Leeds and Goldberg [14] described vasoconstriction or dilatation as a frequent occurrence with cerebral inflammatory disease of all varieties. The arterial narrowing was considered to be due to encroachment by inflammatory exudate as a result of cellular infiltration, vasospasm, or a combination of both. The angiographic findings described by Bianchi and Frera [4] and Rabinov [18] were probably related to an inflammatory response of the perivascular structures, showing a focal hyperemia surrounding the central necrosis [13]. an inflamma-
tory mass might also show narrowing of the adjacent vessels associated with hypervascularity and a blush. In the present case, the carotid and vertebral angiograms failed to demonstrate any irregularity or narrowing of the intracranial vessels. A small focal vascular mass was seen in the right cerebellopontine angle. The venous structures appeared to be unremarkable. The angiographic findings were not quite similar to the reports of the authors mentioned above. The focal vascular lesion could be due to a small arterial occlusion secondary to central necrosis of the lesion. The angiographic appearance is somewhat like the hypervascular neurinoma described by Moscow and Newton [15]. The findings did not suggest inflammation and were interpreted as representing a neurinoma or meningioma. Other tumors less frequently found in the CPA are epidermoids, gliomas, arachnoid cysts, occasional aneurysms and metastatic carcinomas [6, 11]. Although neurosyphilis is rare today, the disease remains endemic. The surge in the incidence of syphilis in the 1960s will probably lead to an increase of neurosyphilis in the future. Since the clinical symptoms and signs of cerebral gummata will mimic those of intracranial neoplasms, cerebral angiography will still inevitably be performed on some cases of neurosyphilis. The presence of a focal area of hypervascularity might then, in such cases, represent gumma formation. Correlation with "other aspects of this disease and the positive serology in both blood and CSF will confirm the diagnosis. In cases of neurosyphilis, where the progress of the disease is slow and the patient's deterioration not critical, consideration should be given to the use of penicillin therapy during an appropriate observation period. Whether this will alter the pathogenesis of cerebral syphilitic gumma is not exactly known [16].
References 1. Anderson, W. ed.: Pathology. pp. 1323-1325 St. Louis: Mosby 1953 2. Bagdasar, D.: Le traitement chirurgical des gommes cerebrales. Rev. neurol. 2, 1-30 (1929) 3. Bailey, P.: Intracranial tumors. Springfield, IlL: Thomas 1933 4. Bianchi, M., Frera, C.: A case of brain gumma. J. Neurol. Neurosurg. Psychiat. 20, 133-135 (1957) 5. Cushing, H.: Intracranial tumors. Springfield, II1.: Thomas 1932 6. Dale, A. J. D.: The cerebellopontine angle syndrome. Med. C1. NA. 52, 789-795 (1968) 7. Dandy, W. E.: Surgery of the brain, pp. 367-369. Hagerstown, Md.: Prior 1945 8. Fabing, H.: Neurosyphilis -- a review. Lancet 75, 346-347 (1955)
F. Y. Tsai et al.: Angiographic Findings with an Intracranial Gumma 9. Ferris, E. J.: Arteritis. Radiology of the skull and brain, VoIl. II, Bk 4, 2566-2577. Newton, T. H., Potts, D. G., eds. St. Louis: Mosby 1974 10. Ferris, E. J.: Rudikoff, J. C. Shapiro, J. H.: Cerebral angiography of cerebral infection. Radiology 90, 727-737 (1968) 11. Histselberger, W. E.: Other tumors of the cerebellopontine angle. Arch Otolaryng. 88, 712-714 (1968) 12. Joffe, R., Black, M. M., Floyd, M.: Changing clinical picture of neurosyphilis - report of seven unusual cases. Brit. med. J. 1, 211-212 (1968) 13. Lagerren, C., Lindbom, A., Soderberg, G.: Hypervascularization in chronic inflammation demonstrated by angiography angiographic, histopathologic and micro-angiographic studies. Acta radiol. 49, 441-452 (1958) 14. Leeds, N. E., Goldberg, H. I.: Angiographic manifestation in cerebral inflammatory disease. Radiology 98, 595-604 (1971) 15. Moscow, N. P., Newton, T. H.: Angiographic features of hypervascular neuromas of the head and neck. Radiology 114, 635-640 (1975)
5 16. Obrador, S.: Tuberculoma and syphilitic gumma. Textbook of neurological surgery. Youmans, J. R., ed. pp. 1569-1573. Philadelphia: Saunders 1973 17. Olivercrona, H.: The surgical treatment of intracranial tumor. Handbuch der Neurochirurgie, Vol. 4, Part 4. Berlin, Heidelberg, New York: Springer 1967 18. Rabinov, K. R.: Angiographic findings in a case of brain syphilis. Radiology 80, 622-624 (1963) Received: November 4, 1976
Feng. Y. Tsai, M. D. Department of Radiology Section of Neuroradiology Albany Medical Center Hospital Albany, NY 12208, USA
