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Journal of Clinical Neuro-ophthalmology 8(2): 87-91, 1988. Neurocysticercosis Simulating Pseudotumor Cerebri (Pseudopseudotumor) Oscar H. Del Brutto, M.D., and Julio Sotelo, M.D. <91988 Raven Press, Ltd., New York We report two patients who presented with intracranial hypertension without localizing signs and normal computed tomography (CT) scans. In both cases, the diagnosis of pseudotumor cerebri was erroneously made until the results of cerebrospinal fluid (CSF) analysis demonstrated cysticercosis. Our findings reinforce the concept that the diagnosis of pseudotumor cerebri should not be made without study of CSF, and add another cause for the syndrome of pseudopseudotumor. Key Words: Cysticercosis-Intracranial hypertensionPseudotumor cerebri - Pseudopseudotumor. From the Division de Investigacion, Instituto Nacional de Neurologia y Neurocirugia, Mexico, D.F. Address correspondence and reprint requests to Dr. J. Sotelo at Research Division, Instituto Nacional de Neurologia y Neurocirugia, Insurgentes sur 3877, 14410, Mexico 22, D.F. 87 Pseudotumor cerebri is a syndrome of obscure cause in which increased intracranial pressure is present without evidence of an expanding lesion or hydrocephalus (1-4). A lumbar puncture is of paramount importance for the correct diagnosis of this condition, since the cerebrospinal fluid (CSF) must be normal except for increased opening pressure (1,2). In contrast, there are some patients with an apparently characteristic pseudotumor cerebri in whom CSF examination may detect conditions not otherwise evident; the term pseudopseudotumor has been used to denote these cases (5,6). Etiologic considerations for the syndrome of pseudopseudotumor include sarcoidosis, viral encephalitis, and syphilitic, tuberculous, or carcinomatous meningitis (2,5,6). Parasitic diseases of the central nervous system (CNS) can conceivably produce this syndrome, but they are not considered in the differential diagnosis of pseudopseudotumor. Here we report two patients with neurocysticercosis (NCC) whose diagnosis would have been pseudotumor cerebri if not for the CSF findings. CASE REPORTS Case 1 A 36-year-old obese woman was evaluated because of a IS-day history of progressive headache, vomiting, and an ill-defined visual complaint. On admission, the patient was alert and oriented. Neuro-ophthalmologic exam demonstrated bilateral papilledema, concentric reduction of visual fields, and bilateral enlargement of the blind spot; visual acuity was 20/20 in each eye. The other results of physical examination were unremarkable. Computed tomography (CT) scan showed small lateral ventricles but was otherwise normal (Fig. lA). Lumbar puncture yielded a clear CSF with an opening pressure of 350 mm H20; cyto- 88 / Clin Neuro-ophthalrnol. Vol. 8. Nu. 2. 1988 O. H. DEL BRUTTO AND J. SOTELO FIG. 1. Contrasted CT scans showing the progression of the disease in patient 1. (A) At the time of admission, CT shows small lateral ventricles but is otherwise normal. (B) Five months later, acute hydrocephalus appears, and (C) 1 year after admission, CT scan shows two ventricular shunts placed because of asymmetric hydrocephalus, and multiple cysticerci in brain parenchyma and subarachnoid space. NEUROCYSTICERCOSIS SIMULATING PSEUDOTUMOR CEREBRI 89 chemical analysis showed 23 mononuclear cells per mm3 with normal glucose and protein content. Immunological reactions to cysticercus antigens [enzyme linked immunosorbent assay (ELISA) and complement fixation test] were positive in eSF. The patient was treated with steroids with progressive improvement and was released asymptomatic after 20 days. Five months later, she was readmitted because of headache and gait disturbance. CT scan showed acute hydrocephalus (Fig. 18). A ventriculoperitoneal shunt was placed and the patient was discharged with marked clinical improvement; at that time, a ventricular CSF analysis showed 45 mononuclear cells per mm3, 120 mgldl protein, and 55 mgldl glucose. Further evolution was torpid; she was readmitted on three occasions because of recurrent intracranial hypertension associated with shunt dysfunction, which required replacements of the valve. On her last admission, 1 year after the first evaluation, CT scan showed asymmetric hydrocephalus and multiple cysts throughout the brain parenchyma and subarachnoidal cisterns. A contralateral ventriculoperitoneal shunt was placed (Fig. lC) and a therapeutic course with praziquantel was planned; however, the patient presented systemic complications after surgery, and died 15 days later. Case 2 A 17-year-old obese woman complained of intermittent headache and vomiting for 7 months. On admission to the hospitaL she was alert and oriented. Neuro-ophthalmologic exam showed bilateral papilledema with enlargement of the blind spot. Visual acuity was 20/20 in each eye and visual fields were normal. CT scan showed smalllateral ventricles without midline shift or other abnormalities (Fig. 2A). Lumbar puncture yielded a clear CSF with an opening pressure of 250 mm H20; cytochemical analysis showed 20 mononuclear cells per mm3, 90 mgldl protein, and 33 mgldl glucose. Immunological reactions to cysticercus antigens were positive in CSF. The patient was managed with steroids with clinical improvement and was discharged after 10 days. Ten months later, CT scan showed small lateral ventricles but was otherwise normal (Fig. 28). Repeated CSF examination showed persistence of positive immunological reactions to cysticercus antigens. With the exception of mild, transient headache, which resolved with common analgesics, the patient was asymptomatic. A therapeutic course with albendazole was proposed; however, she refused the trial. DISCUSSION Cysticercosis is the most common parasitic disease of the central nervous system (CNS) (7); it is endemic in developing countries (8,9) and in industrialized nations with a high immigrant population (10-12). Cysticercosis occurs when humans become the intermediate host in the life cycle of the tapeworm Taenia solium by ingesting its eggs from contaminated food. Eggs hatch into oncospheres in the human intestinal tract; oncospheres cross the intestinal wall, enter the bloodstream, and are carried into the tissues of the host where, after a period of 2 months, the larvae (cysticerci) develop (13). Main target organs of cysticerci are the eye, subcutaneous tissue, skeletal muscles, and CNS. In the latter, cysticerci can lodge in the brain parenchyma, subarachnoid space, ventricular system, or spinal cord (14,15). Clinical manifestations of neurocysticercosis are varied, nonspecific, and largely depend on the number and site of the lesions, the host's immune response to the parasite, and sequelae of previous infestations (16-18). Diagnosis of neurocysticercosis is difficult on clinical grounds, but proper integration of CT (19) and CSF (20,21) data permits an accurate diagnosis for most patients. Therapy of neurocysticercosis is also varied and must be chosen according to the pleomorphism of the disease (22,23); as a general rule, two main factors determine the therapeutic approach: the activity of the disease and the location of cysticerci (22). Intracranial hypertension is a frequent fonn of presentation of neurocysticercosis (7,16). This syndrome is often induced by hydrocephalus secondary to adhesive arachnoiditis (24), intraventricular cysts (25), large clumps of cysts in the subarachnoid space (26), or cysticercotic encephalitis (27). In those cases, CT scan usually shows characteristic abnormalities (24-27) that facilitate the diagnosis of neurocysticercosis. In our two patients, CT scans taken at the initial evaluation were normal (Figs. lA and 2A), so that the diagnosis of neurocysticercosis would have been missed if not for the CSF findings. Since an initial diagnosis of pseudotumor cerebri was made in both patients, a lumbar puncture was performed as part of routine evaluation. Abnormal findings in the cytochemical analysis of CSF discarded the diagnosis of pseudotumor cerebri (1,2) and enabled us to suspect the diagnosis of neurocysticercosis, which was subsequently documented by immunological reactions in CSF. As we have previously noted, ELISA and complement fixation tests are highly reliable and specific tests for neur- J Clill Neuro·oplJthalrnol. Vol. 8, No.2, 1988 NEUROCYSTICERCOSIS SIMULATING PSEUDOTUMOR CEREBRI 91 2. Corbett JJ. Problems in the diagnosis and treatment of pseudotumor cerebri. 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Rev Neural (Paris) 1986;143:635-7. 10. Keane JR. Cysticercosis acquired in the United States. Ann Neural 1980;8:643 (letter). 11. Richards Fa, Schantz PM, Ruiz-Tiben E, Sorvillo FJ. Cysticercosis in Los Angeles county. lAMA 1985;254:3444-8. 12. Schultz TS, Ascher! GF. Cerebral cysticercosis: occurrence in the immigrant population. Neurosurgery 1978;3:164-9. 13. Faust EC, Russell PF, Jung RC. Cysticercosis. In: Faust EC, ed. Clinical parasitology. Philadelphia: Lea & Febiger, 1970:529-35. 14. Escobar A, Nieto D. Parasitic diseases. In: Minckler J, ed. Pathology of the nervous system, Vol. 3. New York: McGrawHill, 1972:2503-21. 15. Sotelo J, Del Brutto OH. Neurocysticercosis. In: Roman GC, ed. Tropical neurology: an overview. Boca Raton: CRC Press Inc., 1988 (in press). 16. McCormick GF, Zee CS, Heiden J. Cysticercosis cerebri: review of 127 cases. Arch NeuroI1982;39:534-9. 17. Keane JR. Neuro-ophthalmologic signs and symptoms of cysticercosis. Arch OphthalmoI1982;100:1445-8. 18. Del Brutto OH, Garcia E, Talamas 0, Sotelo J. Sex-related severity of inflammation in parenchymal brain cysticercosis. Arch Intern Med 1988 (in press). 19. Rodriguez-Carbajal J, Palacios E, Zee CS. Neuroradiology of cysticercosis of the central nervous system. In: Palacios E, Rodriguez-Carbajal J, Taveras JM, eds. Cysticercosis of the central nervous system. Springfield, IL: Charles C. Thomas, 1983:101-43. 20. Rosas N, Sotelo J, Nieto D. ELISA in the diagnosis of neurocysticercosis. Arch NeuroI1986;43:353-6. 21. Nieto D. Cysticercosis of the central nervous system: diagnosis by means of the spinal fluid complement fixation test. Neurology 1956;6:725-38. 22. Sotelo J, Del Brutto OH. Therapy of neurocysticercosis. Child's Nerv Syst 1987;3:208-11. 23. Sotelo J. Cysticercosis. In: Johnson RT, ed. Current therapy in neurologic diseases-2. Philadelphia: Decker, 1987:114-7. 24. Sotelo J, Marin C. Hydrocephalus secondary to cysticercotic arachnoiditis: long-term follow-up review of 92 cases. I Neurosurg 1987;66:686-9. 25. Madrazo L Garcia JA, Sandoval M, Lopez FJ. Intraventricular cysticercosis. Neurosurgery 1983;12:148-52. 26. Ramina R, Hunhevicz CS. Cerebral cysticercosis presenting as mass lesion. Surg Neural 1986;25:89-93. 27. Rangel R, Torres B, Del Brutto 0, Sotelo J. Cysticercotic encephalitis: a severe form in young females. Am I Trap Med Hyg 1987;36:387-92. 28. Sotelo J, Escobedo F, Rodriguez-Carbajal J, Torres B, Rubio-Donnadieu F. Therapy of parenchymal brain cysticercosis with praziquantel. N Engl J Med 1984;310:1001-7. 29. Sotelo J, Torres B, Rubio-Donnadieu F, Escobedo F, Rodriguez- Carbajal J. Praziquantel in the treatment: long-term follow-up. Neurology 1985;35:752-5. 30. Escobedo F, Penagos P, Rodriguez-Carbajal J, Sotelo J. AIbendazole therapy for neurocysticercosis. Arch hJtem Med 1987;147:738-41. 31. Sotelo J, Escobedo F, Penagos P. A1bendazole versus praziquantel for therapy of neurocysticercosis; a controlled trial. Arch Neural 1988 (in press). 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