Acta Neuropathologica Acta Neuropathol. (Berl.) 49, 7-12 (1980) 9 Springer-Verlag 1980 Granulomatous Encephalitis, lntracranial Arteritis, and Mycotic Aneurysm Due to a Free-living Ameba A. Julio Martinez 1, Cirilo Sotelo-Avila 2, Hilda Alcal~ 3, and Eddy Willaert 4 1 PathologyDepartment (Neuropathology),Presbyterian-UniversityHospital, Universityof Pittburgh, Pittsburgh, PA 15213, USA 2 NorthwesternUniversitySchool of Medicine, Head, ClinicalPathology,The Children's Memorial Hospital, Chicago, IL 60614, USA 3 Director of Pathology (Neuropathologist),Hospital Infantil de Mexico, Mexico 7, D.F. 4 MedicalResearch, 151, Veteran's AdministrationMedical Center, Gainesville,FL 32602, USA Summary. Primary amebic meningoencephalitis and granulomatous amebic encephalitis are well recognized clinicopathological entities caused by free-living amebas. Associated arteritis and "mycotic aneurysms" with infiltration of intracranial arteries by lymphocytes, amebic trophozoites and cysts have not been previously reported. A 26-month-old girl had a 3-week history of encephalitis, characterized, initially, by vomiting and lowgrade fever. Subsequently, she developed ataxia, generalized weakness, lethargy, and esotropia. The first CSF showed 490RBC/gl, 705WBC/gl with 90% mononuclears. Her pupils reacted briskly to light. Moderate nuchal rigidity, nystagmus, fixed downward gaze, anisocoria, bilateral 6th nerve palsy, left arm monoparesis and left Babinski were present. CAT scan revealed slight symmetrical dilatation of anterior horns of lateral ventricles and an area of abnormal enhancement above the 3rd ventricle. She died 14 days after admission, 5 weeks after onset of symptoms. The brain showed focal necrotizing encephalopathy, involving thalami, cerebellum, brain stem, and cervical and upper thoracic spinal cord. Numerous free-living amebic trophozoites and cysts were present within a chronic granulomatous encephalitis. There were thrombosis of basilar, posterior cerebral, and vertebral arteries with profuse chronic panarteritis, fibrinoid necrosis, and mycotic aneurysms. Key words: Free-living amebas - Encephalitis Granuloma - Acanthamoeba - Naegleria - "Mycotic aneurysm" Offprint requests to." A. Julio Martinez, M.D. (address see above) Increasing interest in the pathogenicity and the biological behavior of free-living amebas belonging to the genera Naegleria and Acanthamoeba, have been noted recently in the medical literature. However, the disease may be very old and unrecognized cases may have occurred [8]. The most frequent etiologic agents responsible for primary amebic meningoencephalitis (PAM) and granulomatous amebic encephalitis (GAE) are freeliving amebas of the genera Naegleria [4, 5, 9, 20] and Acanthamoeba [2, 10, 11, 13, 18,27,28] or closely related genus [7]. To date, more than 100 cases of PAM and nearly 20 cases of GAE have been reported. Some "extracerebral" infections have also been found due to Acanthamoeba sp. [3, 12, 16, 25]. PAM usually occurs in healthy, young individuals with a recent history of swimming in man-made lakes, heated swimming pools, and warm springs. Apparently the infecting cysts or trophozoites might be transported by the air [15]. Free-living amebas have been isolated from the air, [14] from drinking water [22] from the nasal passages and throats of clinically normal individuals [30, 33], or as contaminant in bacterial [29] and tissue cultures [24]. Naegleria fowleri produces an acute, fulminant, necrotizing hemorrhagic meningoencephalitis affecting the olfactory bulbs, base of the frontal and temporal lobes, and cerebellum. The portal of entry is through the olfactory neuroepithelium by direct penetration of sustentacular cells and invasion of the amyelinic submucosal nervous plexuses by the trophozoites. No cysts are found in the lesions. GAE due to Acanthamoeba sp. is probably an opportunistic infection [21] of the CNS which has occurred in chronically ill and immunosuppressed patients without a history of recent swimming. The portal of entry into the CNS may be from the lower 0001-6322/80/0049/0007/$1.20 8 respiratory tract or f r o m a skin lesion, reaching the CNS t h r o u g h the h e m a t o g e n o u s route. The predom i n a n t host reaction is a s u b a c u t e or chronic g r a n u l o m a t o u s encephalitis c o m p o s e d of lymphocytes, p l a s m a cells, monocytes, a n d m u l t i n u c l e a t e d giant cells, hence the n a m e g r a n u l o m a t o u s amebic encephalitis. Cysts, as well as trophozoites, m a y be f o u n d within the CNS lesions. The most affected areas are the m i d b r a i n , basal ganglia, a n d posterior fossa structures. Because p a t h o g e n i c free-living a m e b a s are wide= spread in nature, a n d m a n is frequently exposed to them, p u b l i c health implications are obvious. We report a case of g r a n u l o m a t o u s amebic encephalomyelitis in a 2 6 - m o n t h - o l d girl, which was n o t caused by Naegleria a n d p r o b a b l y n o t by Acan- thamoeba. Report of a Case A 26-montfl-old girl was admitted to the hospital with abrupt vomiting and low-grade fever. Two days before her illness began, she waded in Nachez river, Texas. Three weeks before admission, her coordination was impaired and she became less active. Eight days before admission, she was admitted to a hospital for a day because of vomiting, lethargy, weakness, and esotropia of the left eye. She was given Donnatal and discharged. Four days later she was readmitted with persistent signs and symptoms, and generalized, red skin welts. Two days after admission the skin lesions disappeared. The cerebrospinal fluid (CSF) examination showed 490 RBC/gl and 705 WBC/~tl with 90 % mononuclears. On the second hospital day the CSF revealed 97 RBCAd, 122 WBC/lalwith 99 ~ mononuclears; protein, 322 mg/dl; and glucose, 20 mg/dl. She failed to improve after administration of Doxycyclineand was transferred to another hospital. Examination at the children's hospital revealed a temperature of 38.4~ pulse ll6/min, respirations, 36 rain, and blood pressure, 9'0 mm/Hg, systolic. Moderate nuchal rigidity was noted. The left pupil was 1 mm and the right was 2 mm; they reacted briskly to light. There was downward nystagmus, downward gaze deviation, bilateral 6th nerve palsy and a Babinski sign on the left. She was obtunded. The hematocrit was 33%; WBC, 8,200/gl with 61% segmented neutrophils; electrolytes, glucose, and BUN were normal. The CSF had an opening pressure of 225 mm/HzO; 62 RBC/gl, 280 WBC/pl with 94% mononuclears; protein 350mg/dl and glucose, 24mg/dl. Gram stain and india ink preparation were negative. Amebas were searched for, bm not identified in a wet preparation of CSF. Counter immunoelectroptloresis on sermn and CSF was negative for Haemophilus influenzae b; Neisseria meningitidis A, C, and D; and PneurnococcusOmni A through F. Fluorescent staining of smears of sediment of urine, CSF and gastric aspirate were negative for acid fast bacilli. Complement fixation titers for cytomegalovirus, herpes, and mycoplasma were 1:32, 1:8, and 1:8, respectively. The spot screen and presumptive tests for infectious mononucleosis were negative. Blood and CSF cultures were subsequently negative. Chloromycetin, 100 mg/kg in four daily doses, Isoniazid, 20 mg/kg/day in two daily doses, and Rifampin, 20 mg/kg/day in two daily doses were administered. An EEG 1 day after admission showed diffuse slow wave activity without focality. Computerized axial tomography (CAT) showed asymmetricalventricnlar dilatation and, with the addition of contrast material, a midline area of enhancement was present above the third ventricle consistent with an inflammatory or vascular lesion. Acta Neuropathol. (Berl.) 49 (i980) She continued to deteriorate, responded only to deep painful stimuli and required assisted ventilation on the third day of hospitalization. Decadron, 3 mg, four times a day, was administered without relief. Chloramphenicol was discontinued. The following day, the serum electrolytes were as follows: sodium, 125 mEq/l; potassium, 3.8 mEq/l; chloride, 88 mEq/1,and CO2, 24 mEqfl. Serum osmolality was 262 mOsm/1; urine electrolytes and osmolality were compatible with SIADH, which persisted for 5 days. The CSF, 6 days after admission revealed an opening and closing pressure of 100, 72 mm H20, respectively;WBC, 1t0 with 100 % mononuclears, red cells, i ; glucose, 17 mg % (simultaneous blood glucose 91); and protein, too high to read. Indirect immunofluorescent antibodies for Herpes simplex were negative. Chloramphenicol was reinstated on the 7th hospital day. A second CAT scan showed an area of low density which did not enhance in the temporoparietal area. The right lateral ventricle was effaced. The midline abnormal enhancement above the third ventricle remained unchanged. On the 10th hospital day, she remained unresponsive without brain stem reflexes. Rifampin was discontinued, and Streptomycin, 20 mg/kg/day was administered. On the 13th hospital day, no evidence of cerebral electrical activity was detected on the EEG. She died on the 14th hospital day, 5 weeks after the onset of fever and vomiting. Autopsy Findings The m a j o r pathological findings were limited to the central n e r v o u s system (CNS) a n d lungs. The b r a i n weighed 1,310g. The external appearance of the cerebral hemispheres show large areas o f encephalomalacia. The leptomeninges were transp a r e n t on the convexities a n d thick a n d o p a q u e at the base. B o t h olfactory nerves were necrotic at their tips. The d i e n c e p h a l o n a n d walls o f the third ventricle were necrotic. The t h a l a m u s showed two abscesses, the largest measured 1.5 cm in diameter. H o r i z o n t a l sections of the b r a i n stem reveals e n c e p h a l o m a l a c i a of the superior a n d inferior colliculi. The cerebral a q u e d u c t c o n t a i n e d necrotic debris. The lower p o r t i o n of the b r a i n stem exhibited h e m o r r h a g i c necrosis o f the e n t i r e , floor of the f o u r t h ventricle, the latter being occupied by necrotic tissue from the cerebellar vermis. The cerebellum shows diffuse encephalomalacia, more prom i n e n t in the anterior p o r t i o n with p r o t r u s i o n into the fourth ventricle. H o r i z o n t a l sections o f the spinal cord showed some foci of m y e l o m a l a c i a at the cervical a n d u p p e r thoracic levels, Histopathological Findings There was a necrotizing " g r a n u l o m a t o u s " encephalitis involving the t e g m e n t u m , associated with perivascular cuffing by lymphocytes a n d occasional p l a s m a cell. There was m i n i m a l chronic leptomeningitis. The basilar, posterior cerebral, a n d p o r t i o n s of the vertebral arteries revealed ~ a n e u r y s m s " with panarteritis (Fig. 1). I n addition, there were segmental A. J. Martinez et al. : Granulomatous Amebic Encephalitis 9 Fig'. 1. Portion of the wall of the basilar artery. Two multi-nucleated giant cells are seen in the lumen (arrowheads). Amebic trophozoites are seen (arrows)between spaces of the media. The interna elastica lamina is intact in this segment. H.-E. • 490 Fig. 2. t~hrastructmal features of an amebic trophozoite. • 5,400. lnset: Amebic trophozoites and modest lymphocytic infiltration in thalamic lesion. H.-E. x 360 organizing thrombosis with multinucleated giant cells and numerous amebic trophozoites within the arterial wall, mainly in the adventitia and muscularis. There were numerous multinucleated giant cells in the thrombus and in the adventitia (Fig. 1). Foci ofnecrotizing granulomatous encephalitis, on the posterior and lateral portions of the medulla oblongata and in one inferior olivary nucleus were identified. There was "fibrinoid" necrosis of blood vessels. Clusters of trophozoites around blood vessels and within neuropil with virtual absence of inflammatory reaction was also a striking feature. Well presm'ved amebic trophozoites were seen measuring 1 5 - 20 l~m in diameter. Occasional spherical amebic cysts were seen with slight wrinkled walls, measuring 10 btm in average diameter. The roots of the 12th cranial nerve were surrounded by lymphocytes and few plasma cells. The cerebellar peduncles and the central white matter o f the cerebellum, revealed a necrotizing granulomatous encephalitis with multinucleated giant cells, occasional amebic trophozoites, and a few amebic cysts. Fibrinoid necrosis of medium-sized arteries was also seen. The thalamus showed a large area of necrotizing "chronic" granulomatous encephalitis with clusters of amebic trophozoites (Fig. 2, Inset). The choroid plexus was undergoing necrosis. Amebic trophozoites were seen in the deeper portions of the Virchow-Robin spaces. The cervical and upper thoracic spinal cord disclosed focal necrotizing granulomatous myelitis with mild leptomeningitis. Trophozoites were seen in these regions. Perivascular cuffing by lymphocytes was prominent. The roots of the cauda equina showed mild chronic inflammation. General Histopathological Findings The lungs showed foci of atelectasis and occasional loci of chronic pneumonitis, one of them containing an amebic trophozoite. The rest of the organs showed no significant pathology. Special Pathology Studies Despite p o s t m o r t e m autolysis, trophozoites and cysts were adequately preserved, permitting ultrastructural 10 evaluation. The technique used was described elsewhere [7]. Ultrastructurally, the trophozoites possessed the characteristic dense nucleolus surrounded by granular chromatin and abundant cytoplasm containing short cytoplasmic processes bound by a thin, well-defined membrane (Fig. 2). The cytoplasm contained round mitochondria, free ribosomes, and different-sized vesicles and vacuoles. Some trophozoites revealed abundant, parallel stalks of endoplasmic reticulum, lysosomes, and glycogen-like granules. Golgi apparatus was not detected. Their nuclei were centrally or eccentrically located, and contained a dense nucleolus. "Vacuoles" and "dense" bodies were seen within the nuclear chromatin. The nuclear membrane was double, but was absent in the dividing trophozoites. In some instances, the nucleus contained a dividing nucleolus without nuclear membrane and at other times, two or three well defined nucleoli were seen. The nuclear divisions appear to be mesomitotic or at the end of the telophase. Some trophozoites which may be in a preencystment stage disclosed a condensation of cytoplasm at the periphery and the presence of numerous, small electron-dense vacuoles, perhaps representing fragments of cytoplasmic membranes. No interzonal bodies or nuclear caps were detected. Cysts were rounded or slightly ovoid, with a prominent, thick, slightly wrinkled poreless wall often composed of partially "split" concentrical layers of electron dense material. In between the splitting there was finely granular material. Smooth and rough endoplasmic reticula were barely visible. Abundant electron-dense lysosomal-like bodies were seen in the cysts. Microbiologic Studies The sediment of the CSF collected on the patient's sixth hospital day was placed into bacterized agar plates at 37~ and 25~ no growth was obtained after 14 days. Fragments of brain taken at autopsy were placed in Fletcher's medium and then into bacterized agar plates at 37~ and 25 ~C. No growth was obtained in either case. Indirect Imrnunofluorescent Antibody Analyses ( IFA) The histologic Sections of the patient's brain were deparaffinized before application of the diluted antisera. The following antisera, which were produced in rabbits, were used in this case: (1) Whole cell antisera [35]; anti-N.fowIeri (ITMAP 359), anti-A.polyphaga (P 23), anti-A, astronyxis (Ray), anti-A.palestinensis (Reich), anti-A, royreba (Oak Ridge), anti-A, rhysodes (CCAP 1534), anti-A, griffini (CCAP 1501/4), anti- Acta Neuropathol. (Berl.) 49 (1980) Table 1. Results of indirect immunofluorescent antibody test on cerebral tissue Antisera Dilution ResulP Homologous end-point titer 0 0 0 0 0 0 0 0 0 0 1/512 1/128 1/64 1/128 1/128 1/128 1/64 1/128 1/128 1/512 0 0 0 1/512 1/512 1/1024 1. Whole cell antisera: anti-N.fowleri anti-A.polyphaga anti-A, astronyxis anti-A.palestinensis anti-A, royreba anti-A, rhysodes anti-A.griffini anti-H, vermiformis anti- V. avara anti-E, histolytica 1/32 1/16 1/8 1/16 1/16 1/16 1/16 1/32 1/32 1/64 2. Plasma membrane antisera: anti-A, eulbertsoni 1/32 anti-A, eastellannii 1/64 anti-A, rhysodes 1/64 a 0 = no fluorescence H. verrniformis (CCAP 1534/7), anti-V, avara (CCAP 1588/A), and anti-E, histolytica (HK 9); (2)Plasma membrane antisera [31]; anti-A, culbertsoni (A-l), antiA. castellanii (Neff), and anti-A, rhysodes (CCAP 1534/3). The histologic sections were incubated and the immune complexes were visualized after incubation of the sections with fluorescein-conjugated rabbit antiimmunoglobulin (1:50) (Institute Pasteur, France). The sections were read with a Leitz Ortholux microscope equipped for fluorescence with a ploemopak 2 and a super-pressure mercury lamp HBO 6, 100 W. The filter system for FITC combined with two interference filters KP, 490, K 510, and K 445 were used. The results of the indirect immunofluorescence analyses are given in the Table 1. None of the antisera used stained the ameba in the brain sections. Discussion The clinical picture and the histopathological reaction in CNS resembled those of Acanthamoeba, i.e., chronic granulomatous inflammation with multinucleated giant cells, lymphocytes, and plasma cells. Such pathologic findings have not been described in proven cases of Naegleria. Human and animal experiments are consistent with these findings [6, 17, 19]. Also, the presence of cysts has not yet been described in PAM due to Naegleria; however, the absence of such may be related to the lack of an identified chronic stage of this disease. A very important feature of this case is the arterial involvement. In some places "organizing" thrombi A. J. Martinez et al. : Granulomatous Amebic Encephalitis were seen with a moderately severe panarteritis associated with lymphocytic infiltration and the presence of foreign body giant cells, There were at least two places (basilar artery and posterior cerebral artery) in which "mycotic" aneurysms were present. Molinari [23] stated that infection of the arterial wall in cases of "mycotic" aneurysms is mediated through stasis and sepsis in the vasavasorum. In addition, the inflammation appears to proceed from the adventitial surface toward the media. Bell and Butler [1] believe that embolic material, usually from the heart, occludes the vasavasorum allowing bacterial growth within the wall of the artery. The inflammatory response weakens the vessel wall resulting in aneurysmal dilatation and possible rupture. Some intracranial "mycotic" aneurysms, usually of extravascular origin, may become thrombosed and may decrease in size or spontaneously disappear [32]. It appears that free-living amebas, in particular Acanthamoeba sp., have a predilection for attacking vascular walls, thus leading to necrotizing arteritis with thrombosis and aneurysm. The nuclear division of the amebas in this case appeared to be mesomitotic; that is, the nuclear membrane disappears throughout mitosis and polar caps are absent. Mesomitosis division is consistent with Acanthamoeba or Hartmannetta. The absence of interzonal bodies during mitosis serves to exclude the Naegleria, as well as Vahlkarnpfia as the causative organisms. The presence of the cyst in the patient's CNS also points against Naegleria since, characteristically, this ameba does not form cysts in tissues. Furthermore, the cysts present in the patient's CNS had wrinkled, concentrically laminated walls without identifiable pores. Naegleria cysts are smooth and contain pores in their double wall. However, such cysts do not completely exclude Naegleria, as Page [26] has pointed out that the morphology of cysts of Naegleria can vary considerably, from smooth to rough to angular and may even be wrinkled. The large dark karyosome excludes, with the exception of Iodamoeba, members of the family Entamoebidae. If the organism identified belonged to the Entamoebidae family, ultrastructural evidence of peripherally located chromatin within the nuclei, as well as an absence of mitochondria, should have been noted. Such was not the case [26]. The genus Iodamoeba cannot be completely excluded, but the cysts were .not typical of that organism. The results of the immunological studies gave further evidence that the unknown pathogenic ameba was neither Naegleria nor Acantharnoeba. Other genera belonging to the family of Vahlkampfiidae, such as tetramitus and paratetramitus, might be considered also, as they have the same mitotic pattern. Organisms in the tissues of the patient reported herein did not fluoresce when exposed to anti-N.fowleri fluorescent 11 tagged antisera. Tissues containing N.Jowleri obtained from autopsy specimens of culturally proven Naegleria PAM cases stained well with anti-N.fowleri fluorescent antisera. One still cannot exclude the possibility that some alteration of antigens occurred, possibly related to fixation of tissues, deparaffinization, or time from death until autopsy. To date, all isolates of Naegleria obtained from cases of PAM throughout the world have shown remarkable antigenic homology. Since cross reactivity between non-pathogenic N. gruberi and pathogenic N.fowleri is incomplete, it is certainly possible, at least on an immunologic basis, that the organism still could be a species of Naegleria other than N.fowleri, either N. gruberi or an as yet undescribed species [34, 36]. Thus, the organism responsible for this patient's death was atypical of both Naegleria and Acanthamoeba, but was a free-living ameba belonging to an unidentified genus. It may be another species of Vahlkarnpfia (other than V. avara) as suggested by the most reliable characteristics available for study, viz., the frequent mitosis and division patterns. Although the responsible pathogen was not precisely identified in this instance, this case is important because it illustrates that serious diseases may be produced in humans (and probably in many other animals) by free-living ameba other than Naegleria, Hartmannella, Acanthamoeba, and Vahlkampfia. It emphasizes that the variety of free-living ameba capable of producing disease is as yet undefined, and that it may be considerable [5, 6]. These organisms are numerous, varied and ubiquitous in nature [15, 22]; they are distributed world-wide; they multiply rapidly and appear in a variety of forms; and some species either prefer or readily invade mammallian tissues. Thus, the public health implications are obvious, and the scope of this problem needs to be defined. Acknowledgements.The authors wish to give special thanks to Dr. John Moossy,and Dr. Ann Stevensfor their suggestionsand criticism during the preparation of this manuscript; and Mss. Janet Morrow and Agnes Zachoszczfor their secretarial assistance. References 1~ Bell,W. E., Butler, C. : Cerebralmycotic aneurysms in children. Two case reports. Neurology (Minneap.) 18, 81- 86 (1968) 2. Bhagwandeen,S. B., Carter, R. F., Naik, K. G., Levitt, D. : A caseofhartmanelIidamebicmeningoencephalitisin zambia.Am. J. Clin. Pathol. 63, 483-492 (I975) 3. Borochovitz,D., Martinez,A. J., Patterson,G. T. : Osteomyelitis of bone graft of mandible with Acanthamoebacastellaniiinfection. Hum. Pathol. (in press) 4. Callicott0J. H., Nelson, E. C., Jones, M. M., dos Santos, J. G., Utz, J. P., Duma, R. J., Morrison, J. V. : Meningoencephalitis due to pathogenic freeqiving amoeba. JAMA 206, 579-582 0968) 12 5. Carter, R. F. : Primary amoebic meningoencephalitis : Clinical, pathological, and epidemiologicaI features of six fatal cases. J. Path. Bact. 96, 1 - 2 5 (1968) 6. Culbertson, C. G.: Pathogenic Acanthamoeba (Hartmannella). Am. J. Clin. Pathol. 35, 195-202 (1961) 7. Duma, R. J., Helwig, W. B., Martinez, A. J. : Meningoencephalitis and brain abscess due to free-living amoeba. Ann. Intern. Med. 88, 468-473 (1978) 8. Flexner, S. : Amoebae in an abscess of the jaw. Johns Hopkins Hosp. Bull. 3, 104 106 (1892) 9. Fowler, M., Carter, R. F. : Acute pyogenic meningitis probably due to Acanthamoeba sp. Br. Med. J. 2, 740-742 (1965) 10. Hoffmann, E. O., Garcia, C., Lunseth, J., McGary, P., Coover, J. : A case of primary amebic meningoencephalitis. Light and electron microscopy and immunohistologic studies. Am. J. Trop. Med. Hyg. 27, 2 9 - 3 8 (1978) 11. Jager, B. V., Stature, W. P. : Brain abscesses caused by fi'ee-living amoeba probably of the genus Hartmannella in a patient with Hodgkin's disease. Lancet II, 1343-i345 (1972) 12. Jones, D. B., Visvesvara, G. S., Robinson, N. M. : Aeanthamoeba Polyphaga keratitis and Aeanthamoeba uveitis associated with fatal meningoencephalitis. Trans. Ophthalmol. Soc. UK 95, 221-232 (1975) 13. Kernohan, J. W., Magath, T. B., Schloss, G. T. : Granuloma of brain probably due to Endolimax Williamsi (Iodamoeba biitschlii). Arch. Pathol. 70, 576-580 (1960) 14. Kingston, D., Warhurst, D. C. : Isolation of amoeba from the air. J. Med. Microbioi. 2, 27--36 (1969) 15. Lawande, R. V., Abraham, S. N., John, I., Egler, L. J. : Recovery of soil amebas from the nasal passages of children during the dusty harmattan period in zaria. Am. J. Clin. Pathol. 71, 201-203 (1979) 16. Lund, O. E., Stefani, F. H., Dechant, W. : Amoebic keratitis: A clinicopathological case report. Br. J. Ophthalmol. 62, 373 - 375 (1978) 17. Markowitz, S. M., Sobieski, T., Martinez, A. J., Duma, R. J.: Experimental Acanthamoeba infections in mice pretreated with methylprednisolone or tetracycline. Am. J. Pathol. 92, 733 741 (1978) 18. Marinez, A. J., Sotelo-Avila, C., Carcia-Tamayo, J., TakanoMor6n, J., Willaert, E., Stamm, W. P. : Meningoencephalitis due to acanthamoeba sp.: Pathogenesis and clinicopatbological study. Acta Nenropathol. (Berl.) 37, 183-191 (1977) 19. Martinez, A. J., Markowitz, S. M., Duma, R. J. : Experimental pneumonitis and encephalitis caused by Acanthamoeba in mice: Pathogenesis and ultrastructural features. J. Infect. Dis. 1313, 692-699 (1975) 20. Martinez, A. J., dos Santos, J. G., Nelson, E. C., Stamm, W. P., Willaert, E.: Primary amebic meningoencephalitis. Path. Annual, Vol. 12, 1977, Part 2, pp. 225-255. Sommers, S. C., Rosen, P. P., (eds.). New York: Appleton-Century-Crofts 1977 Acta NeuropathoL (Bed.) 49 (1980) 21. Martlnez, A. J. : Is Acanthamoebic encephalitis an opportunistic infection? J. Neuropathol. Exp. Neurol. 38, 331 (1979) 22. Molet, B., Derr-Harf, C., Schreiber, J. E., Kremer, M. : 1Etudedes amibes libres dans les eaux de Strasbourg. Ann. Parasitol. Hum. Comp. 51, 401-406 (1976) 23. Molinari, G. F.: Septic cerebral embolism. Stroke 3, 117-122 (1972) 24. Moore, A. E., Hlinka, J. : Hartmannella sp. (Acanthamoeba) as a tissue culture contaminant. J. Natl. Cancer Inst. 40, 569--581 (1968) 25. Nagington, J., Watson, P. G., Playfair, T. J., McGill, J., Jones, B. R., McG. Steele, A. D. : Amoebic infection of the eye. Lancet II, 1537-1540 (1974) 26. Page, F. C. : An illustrated key to freshwater and soil amoebae. Freshwater Biol. 34, 1 - 1 5 5 (1976) 27. Ringsted, J., Jager, B. V., Suk, D., Visvesvara, G. S. : Probable Acanthamoeba meningoencephalitis in a Korean child. Am. J. Clin. Pathol. 66, 723-730 (1976) 28. Robert, V. B., Rorke, L. B. : Primary amebic encephalitis probably from Aeanthamoeba. Ann. Intern. Med. 79, 174- 179 (1973) 29. Shin, L. E., Hadley, P. B.: Note on the spontaneous contamination of a bacterial culture by an organism resembling Hartmannella castellanii. J. Infect. Dis. 58, 23 27 (1936) 30. Sko~il, V., Cerva, L., Serbus, C.: Epidemiological study of amoebas of the limax group in military communities. First Report. J. Hyg. Epidemiol. Microbiol. ImmunoL (Praha) 14, 61 - 66 (1970) 31. Stevens, A. R., Kilpatrick, T., Willaert, E., Capron, A.: Serological anaIyses of cell surface antigens of Acanthamoeba species with plasma membrane antisera. J. Protozool. 24, 3 1 6 324 (1977) 32. Suwanwela, C., Suwanwela, N., Charuchinda, S., Hongsaprobhas, C. : Intracranial mycotic aneurysms of extravascular origin. J. Neurosurg. 36, 552-559 (1972) 33. Wang, S. S., Feldman, H. A. : Isolation of Hartmannella species from human throats. N. Engl. J. Med. 277, 1174-1179 (1967) 34. Willaert, E. : Isolement et culture in vitro des amibes du genre Naegleria. Ann. Soc. Belg. Med. Trop. 51, 701-708 (1971) 35. Willaert, E. : l~tude immunotaxonomique des genres Naegleriaet Acanthamoeba. Acta Zool. Pathol. Antverp. 59, 1--239 (1976) 36. Willaert, E., Stevens, A. R., Healy, G. R.: Retrospective identification of Acanthamoeba culbertsoni in a case of amoebic meningoencephalitis. J. Clin. Pathol. 31, 717-720 (1978) Received September 7, 1979/Accepted November 8, 1979