Aspergillosis of the fourth ventricle Case report AMILCAR J. E. CORREA, M.D., RAUL BRINCKHAUS, M.D., SAUL KESLER, M.D., AND EUGENIO MARTINEZ,M.D. Centro de Investigaciones Neurologicas, Santa Fe, Argentina The authors present a case of Aspergillus infection of the brain; this is believed to be the second case of its kind in Argentina, and probably the only reported case involving an intracranial mycetoma. KEvWoRDS mycetoma
9 fungus 9 Aspergillus 9 hydrocephalus
I
NVECT~ON of the brain by Aspergillus has recently become more common, probably as a consequence of the increasing use of antibacterial or suppression therapy. We are presenting such a case, in which a mycetoma blocked the cerebrospinal pathways. Case Report
This 49-year-old woman was admitted to our hospital with a 1-month history of increasing generalized headaches and vomiting. There was no personal or family history of diabetes mellitus or any debilitating disease. Examination. The patient was stuporous and slow in response, with a stiff neck and positive Kernig and Brudzinski's signs; no Babinski's sign could be elicited. There was bilateral papilledema without other focal signs. Laboratory data, including fasting and 2 hours postprandial blood sugar, were normal. Prior to her referral a lumbar puncture had been performed by her family physician, who reported increased cerebrospinal fluid 236
9
(CSF) pressure, protein content 120 mg%, glucose 50 mg%, low chlorides, and 4 lymphocytes/mm 3. A brain scan with 113In was considered normal; carotid angiography showed bowing of both anterior cerebral arteries compatible with hydrocephalus. A ventriculogram performed through a right frontal burr hole demonstrated definite evidence of obstructive hydrocephalus with dilatation of all four ventricles. The obstruction appeared to be at the level of the foramina of Magendie and Luschka. Operation. A posterior fossa exploration was carried out with the patient in the sitting position. There was definite herniation of both tonsils. After careful retraction of the tonsils a white cotton ball-like mass, 1 • 1 • 1 cm in size, was seen obstructing the opening of the foramina of Magendie (Fig. 1). The mass was hard on palpation, and with the help of the operating microscope, it was carefully dissected and totally removed (Fig. 2). It was then possible to see a vein running on the dorsal aspect of the medulla; its walls appeared indurated and apparently J. Neurosurg. / Volume 43 / August, 1975
Aspergillosis of the fourth ventricle
FIG. 1. Operative photograph showing mycetoma blocking the foramina of Magendie, and thickening of the arachnoid covering the cisterna magna. thrombosed as a consequence of the invasive nature of the same process. A section of this vein was also obtained for pathological examination. Pathology. Microscopic examination of histological sections with silver methenamine and periodic acid-Schiff stain showed an almost solid mass of septate hyphae associated with acute and chronic inflammatory cells, red blood cells, and necrotic tissue (Fig. 3). The vein was also infiltrated with the same type of septate hyphae resembling those of the genus AspergiHus. Postoperative Course. P o s t o p e r a t i v e recovery was uneventful and the patient was completely relieved of the symptoms caused by her increased intracranial pressure. On the basis of the pathology report, further investigation was done for possible concealed diabetes mellitus or invasion of other organs with Aspergillus, but all tests were negative. One week after surgery the patient was having breakfast alone in her room, when she suddenly died. P o s t m o r t e m examination was denied by the family. Discussion
Aspergillus species have a world-wide distribution, predominantly in warm, damp regions, and are notorious laboratory contaminants. The lungs are most frequently the site of primary involvement. Infection of the J. Neurosurg. / Volume 43 / August, 1975
FIG. 2. Microdissection of the mycetoma. Lesion adhered to the vein running on the dorsal aspect of the medulla.
FIG. 3. Photomicrograph of Aspergillus. Silver methenamine, • 1450. central nervous system probably takes place by hematogenous dissemination, by direct extension, or even inoculation? ,9 There was no evidence of the disease on any other organ that might have been responsible for dissemination in our patient. The use of steroids, antibiotic therapy, or chemotherapy, as well as debilitating conditions such as tuberculosis, uncontrolled diabetes, bronchiectasis, sepsis, dysentery, syphilis, and liver cirrhosis have been suggested as possible predisposing fac237
A. J. E. Correa, R. Brinckhaus, S. Kesler and E. Martinez t o r s ? 5.1s However, none of these conditions was present in our case. The pathological reaction may simulate an encephalitic e or meningitic picture, mainly mimicking tuberculosis or an abscess, o-H or g r a n u l o m a t o u s solitary or multiple lesions.l-a,8-8.12.14 The occasional occurrence of a cavity containing a "branching mass of hyphae, so-called m y c e t o m a or fungus ball," has been mentioned with regard to pulmonary aspergillosis,' but in no reported case involving the central nervous system were we able to find a description similar to the findings in our case, which we believe represented a " m y c e t o m a " in the subarachnoid space. The adjacent vessel invaded with the same type of h y p h a e is evidence of possible hematogenous dissemination, despite the lack of a p r i m a r y source. Invasion of the vessel walls and thrombosis had been observed as a distinctive feature of Aspergillus. 8,8a3 Cerebrospinal fluid findings in all cases reviewed, as well as in our own, showed increased protein content; C S F culture was not helpful in the diagnosis in these cases. In most cases, as in this one, the diagnosis was based on the morphology and staining properties, a l t h o u g h cultures might secure better histopathological diagnosis and species identification. M u k o y a m a , et al., ~ and McKee s described three cases of aspergillosis in which the immediate cause of death was attributed to the rupture of mycotic aneurysms or rupture of the internal carotid artery after involvement of the arterial wall. McKee s writes of his patient that "while walking on the ward he suddenly collapsed and died." A similar mechanism m a y have been responsible for the sudden death of our patient. References 1. Banaim J, Parisi A: Aspergillosis. Cerebral Rev Neurol (Buenos Aires) 17:247-256, 1959
238
2. Burnston J, Blackwood W: A case of aspergillus infection of the brain. J Pathol Bacteriol 86:225-229, 1963 3. Cawley EP: Aspergillosis and the aspergilli. Report of a unique case of the disease. Arch Intern Med 80:423-434, 1947 4. Finegold SM, Will D, Murray JF: Aspergillosis. A review and report of twelve cases. Am J Meal 27:463-482, 1959 5. Grceviv N, Matthews WF: Pathologic changes in acute disseminated aspergillosis: particularly involvement of the central nervous system. Am J Clin Pathol 32:536-551, 1959 6. Iyer S, Dodge PR, Adams RD: Two cases of aspergillus infection of the central nervous system. J Neurol Neurosurg Psychiatry 15:152-163, 1952 7. Hackson I J, Eaerl K, Kuri J: Solitary aspergillus granuloma of the brain. Report of 2 cases. J Neurosurg 12:53-61, 1955 8. McKee EE: Mycotic infection of brain with arteritis and subarachnoid hemorrhage. Report of a case. Am J Clin Pathol 20:381-384, 1950 9. Mukoyama M, Gimple K, Poser CM: Aspergillosis of the central nervous system. Report of a brain abscess due to A. fumigatus and review of the literature. Neurology (Minneap) 19:967-974, 1969 10. Olsen FS, Eriksen KR, Stenderup A, et al: (A case of Aspergillosis cerebri.) Ugeskr Laeger 124:1881-1884, 1962 (Dan) 11. Peet MM: Aspergillus fumigatus infection of the cerebellum. Trans Am Neurol Assoc 71:165, 1946 12. Tveten L, Lr AC, Hauge T: Aspergillosis cerebri. Report of a case. Acta Chir Scand 130:149-156, 1956 13. Welsh RA, McClinton LT: Aspergillosis of lungs and duodenum with fatal intestinal hemorrhage. Arch Pathol 57:379-382, 1954 14. Ziskind J, Pizzolato P, Buff EE: Aspergillosis of the brain. Report of a case. Am J Clin Pathol 29:554-559, 1958 Address reprint requests to: Amilcar J. E. Correa, M.D., Centro de Investigaciones Neurologicas, Urquiza 3077, Santa Fe, Argentina.
J. Neurosurg. / Volume 43 / August, 1975
9 fungus 9 Aspergillus 9 hydrocephalus
I
NVECT~ON of the brain by Aspergillus has recently become more common, probably as a consequence of the increasing use of antibacterial or suppression therapy. We are presenting such a case, in which a mycetoma blocked the cerebrospinal pathways. Case Report
This 49-year-old woman was admitted to our hospital with a 1-month history of increasing generalized headaches and vomiting. There was no personal or family history of diabetes mellitus or any debilitating disease. Examination. The patient was stuporous and slow in response, with a stiff neck and positive Kernig and Brudzinski's signs; no Babinski's sign could be elicited. There was bilateral papilledema without other focal signs. Laboratory data, including fasting and 2 hours postprandial blood sugar, were normal. Prior to her referral a lumbar puncture had been performed by her family physician, who reported increased cerebrospinal fluid 236
9
(CSF) pressure, protein content 120 mg%, glucose 50 mg%, low chlorides, and 4 lymphocytes/mm 3. A brain scan with 113In was considered normal; carotid angiography showed bowing of both anterior cerebral arteries compatible with hydrocephalus. A ventriculogram performed through a right frontal burr hole demonstrated definite evidence of obstructive hydrocephalus with dilatation of all four ventricles. The obstruction appeared to be at the level of the foramina of Magendie and Luschka. Operation. A posterior fossa exploration was carried out with the patient in the sitting position. There was definite herniation of both tonsils. After careful retraction of the tonsils a white cotton ball-like mass, 1 • 1 • 1 cm in size, was seen obstructing the opening of the foramina of Magendie (Fig. 1). The mass was hard on palpation, and with the help of the operating microscope, it was carefully dissected and totally removed (Fig. 2). It was then possible to see a vein running on the dorsal aspect of the medulla; its walls appeared indurated and apparently J. Neurosurg. / Volume 43 / August, 1975
Aspergillosis of the fourth ventricle
FIG. 1. Operative photograph showing mycetoma blocking the foramina of Magendie, and thickening of the arachnoid covering the cisterna magna. thrombosed as a consequence of the invasive nature of the same process. A section of this vein was also obtained for pathological examination. Pathology. Microscopic examination of histological sections with silver methenamine and periodic acid-Schiff stain showed an almost solid mass of septate hyphae associated with acute and chronic inflammatory cells, red blood cells, and necrotic tissue (Fig. 3). The vein was also infiltrated with the same type of septate hyphae resembling those of the genus AspergiHus. Postoperative Course. P o s t o p e r a t i v e recovery was uneventful and the patient was completely relieved of the symptoms caused by her increased intracranial pressure. On the basis of the pathology report, further investigation was done for possible concealed diabetes mellitus or invasion of other organs with Aspergillus, but all tests were negative. One week after surgery the patient was having breakfast alone in her room, when she suddenly died. P o s t m o r t e m examination was denied by the family. Discussion
Aspergillus species have a world-wide distribution, predominantly in warm, damp regions, and are notorious laboratory contaminants. The lungs are most frequently the site of primary involvement. Infection of the J. Neurosurg. / Volume 43 / August, 1975
FIG. 2. Microdissection of the mycetoma. Lesion adhered to the vein running on the dorsal aspect of the medulla.
FIG. 3. Photomicrograph of Aspergillus. Silver methenamine, • 1450. central nervous system probably takes place by hematogenous dissemination, by direct extension, or even inoculation? ,9 There was no evidence of the disease on any other organ that might have been responsible for dissemination in our patient. The use of steroids, antibiotic therapy, or chemotherapy, as well as debilitating conditions such as tuberculosis, uncontrolled diabetes, bronchiectasis, sepsis, dysentery, syphilis, and liver cirrhosis have been suggested as possible predisposing fac237
A. J. E. Correa, R. Brinckhaus, S. Kesler and E. Martinez t o r s ? 5.1s However, none of these conditions was present in our case. The pathological reaction may simulate an encephalitic e or meningitic picture, mainly mimicking tuberculosis or an abscess, o-H or g r a n u l o m a t o u s solitary or multiple lesions.l-a,8-8.12.14 The occasional occurrence of a cavity containing a "branching mass of hyphae, so-called m y c e t o m a or fungus ball," has been mentioned with regard to pulmonary aspergillosis,' but in no reported case involving the central nervous system were we able to find a description similar to the findings in our case, which we believe represented a " m y c e t o m a " in the subarachnoid space. The adjacent vessel invaded with the same type of h y p h a e is evidence of possible hematogenous dissemination, despite the lack of a p r i m a r y source. Invasion of the vessel walls and thrombosis had been observed as a distinctive feature of Aspergillus. 8,8a3 Cerebrospinal fluid findings in all cases reviewed, as well as in our own, showed increased protein content; C S F culture was not helpful in the diagnosis in these cases. In most cases, as in this one, the diagnosis was based on the morphology and staining properties, a l t h o u g h cultures might secure better histopathological diagnosis and species identification. M u k o y a m a , et al., ~ and McKee s described three cases of aspergillosis in which the immediate cause of death was attributed to the rupture of mycotic aneurysms or rupture of the internal carotid artery after involvement of the arterial wall. McKee s writes of his patient that "while walking on the ward he suddenly collapsed and died." A similar mechanism m a y have been responsible for the sudden death of our patient. References 1. Banaim J, Parisi A: Aspergillosis. Cerebral Rev Neurol (Buenos Aires) 17:247-256, 1959
238
2. Burnston J, Blackwood W: A case of aspergillus infection of the brain. J Pathol Bacteriol 86:225-229, 1963 3. Cawley EP: Aspergillosis and the aspergilli. Report of a unique case of the disease. Arch Intern Med 80:423-434, 1947 4. Finegold SM, Will D, Murray JF: Aspergillosis. A review and report of twelve cases. Am J Meal 27:463-482, 1959 5. Grceviv N, Matthews WF: Pathologic changes in acute disseminated aspergillosis: particularly involvement of the central nervous system. Am J Clin Pathol 32:536-551, 1959 6. Iyer S, Dodge PR, Adams RD: Two cases of aspergillus infection of the central nervous system. J Neurol Neurosurg Psychiatry 15:152-163, 1952 7. Hackson I J, Eaerl K, Kuri J: Solitary aspergillus granuloma of the brain. Report of 2 cases. J Neurosurg 12:53-61, 1955 8. McKee EE: Mycotic infection of brain with arteritis and subarachnoid hemorrhage. Report of a case. Am J Clin Pathol 20:381-384, 1950 9. Mukoyama M, Gimple K, Poser CM: Aspergillosis of the central nervous system. Report of a brain abscess due to A. fumigatus and review of the literature. Neurology (Minneap) 19:967-974, 1969 10. Olsen FS, Eriksen KR, Stenderup A, et al: (A case of Aspergillosis cerebri.) Ugeskr Laeger 124:1881-1884, 1962 (Dan) 11. Peet MM: Aspergillus fumigatus infection of the cerebellum. Trans Am Neurol Assoc 71:165, 1946 12. Tveten L, Lr AC, Hauge T: Aspergillosis cerebri. Report of a case. Acta Chir Scand 130:149-156, 1956 13. Welsh RA, McClinton LT: Aspergillosis of lungs and duodenum with fatal intestinal hemorrhage. Arch Pathol 57:379-382, 1954 14. Ziskind J, Pizzolato P, Buff EE: Aspergillosis of the brain. Report of a case. Am J Clin Pathol 29:554-559, 1958 Address reprint requests to: Amilcar J. E. Correa, M.D., Centro de Investigaciones Neurologicas, Urquiza 3077, Santa Fe, Argentina.
J. Neurosurg. / Volume 43 / August, 1975
