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Show J. C/in. Neuro-ophthalmol. 4:47-48, 1984. Papilledema in Paroxysmal Nocturnal Hemoglobinuria SEVINC AKTAN, M.D. TULAY KANSU, M.D. EMIN KANSU, M.D. TURCUT ZILELI, M.D. Abstract A 26-year-old man with the diagnosis of paroxysmal nocturnal hemoglobinuria presented with papilledema due to increased intracranial pressure without localizing neurological signs. It is concluded that papilledema and benign intracranial hypertension could result from a cerebral vein thrombosis which can complicate the course of paroxysmal nocturnal hemoglobinuria. This has not been a well-recognized feature of the disease. Introduction Paroxysmal nocturnal hemoglobinuria is a rare and acquired form of hemolytic anemia of unknown etiology, which is characterized by chronic intravascular hemolysis with exacerbations associated with hemoglobinuria and a marked thrombotic tendency. The underlying abnormality which affects red blood cells, granulocytes, and platelets in this disease is an inordinate sensitivity of membrane to the hemolytic action of serum complement.! We have encountered a patient who had papilledema and increased intracranial pressure without focal neurological signs complicating paroxysmal nocturnal hemoglobinUria. Although cerebral vein thrombosis has been reported in patients with paroxysmal nocturnal hemoglobinuria,2-spapilledema and increased intracranial pressure has, to our knowledge, not been reported previously. Case Report A 26-year-old man, a medical student, went to a local institution in October 1981, with chief complaints of fatigue and dizziness. He was diagnosed as having iron deficiency anemia, for which he was treated with an iron supplement 4 From the Departments of Neurology (SA, TK, Tl) and Hematology (EK), Hacettepe University Hospitals, Ankara, Turkey. March 1984 months prior to admission. In February 1982, he developed jaundice. The diagnosis of chronic persistent hepatitis was made, and he was treated with prednisolone 30 mg daily. The jaundice subsided to some degree, but fatigability and malaise continued, and in September 1982, he was admitted to our institution because of recurrent jaundice and abdominal swelling. The diagnosis of paroxysmal nocturnal hemoglobinuria was made on the basis of positive acid-ham, sucrose hemolysis, and Crosby thrombin tests. Low levels of leucocyte alkaline phosphatase and a hemolytic crisis during iron supplementation supported the diagnosis of paroxysmal nocturnal hemoglobinuria. Liver biopsy showed venous congestion, and hepatosplenomegaly was detected on radionuclide scanning. Venous angiography revealed thrombosis of the hepatic vein. The inferior vena cava was found to be patent. The diagnosis of Budd-Chiari syndrome complicating the paroxysmal noctural hemoglobinuria was made. Halotestin (flusoxymesterone) 10 mg daily and prednisolone 20 mg daily were started, and dipyridamole and aspirin were added to the treatment regimen to prevent thrombotic complications. In addition, he was given aldactone and a low-salt diet for the ascites. He did well until April 1983, when he was readmitted with a history of loss of appetite, fatigue, dizziness, jaundice, and dark urine. Laboratory studies at that time revealed serum electrolytes within normal limits, SGOT 170 U, SGPT 384 U, total protein 7 mg%, albumin 3.8 mg%, direct bilirubin 7.5 mg%, and indirect 4.5 mg%. Hemoglobin was 10 g% and complete blood count revealed 70 polymorpholeucocytes, seven atypical lymphocytes, four monocytes and 11 lymphocytes. Thrombocytes were normal. Prothrombin time was 17 seconds (control 11 seconds), partial thromboplastin time was 94 seconds (normal 45-90), fibrinogen was 710 mgjdl (normal 200-500), and fibrin-fibrinogen degradation products were 40 J.l.g. Sucrose hemolysis test, acid-ham test, and Crosby thrombin tests were all strongly positive. Liver and spleen scan- 47 Papilledema in Hemoglobinuria ning was compatible with chronic parenchymal dysfunction. In May 1983, he had a generalized convulsion, and neurological examination revealed bilateral papilledema. Visual acuity was 20/20 in both eyes, and the remainder of the neurological examination was within nonnallimits. Skull x-rays, electroencephalography, and computerized tomography of the head showed no abnonnalities. Lumbar puncture was perfonned because of the suspicion of benign intracranial hypertension, and the opening pressure was found to be above 300 mm H20. There were no cells in the cerebrospinal fluid, and the protein was 14 mg%. Considering the diagnosis of benign intracranial hypertension due to cortical vein thrombosis, dexamethasone 16 mg daily was started, and repeat lumbar punctures were perfonned. One week after the beginning of the treatment, there was a mild resolution of the papilledema, and the cerebrospinal fluid pressure was within nonnallimits. Administration of oral corticosteroids was continued in gradually decreasing dosage as an inpatient. His last ophthalmoscopic examination 1 month after his initial examination showed only slight blurring of the disc margins and mild elevation of the optic discs bilaterally. Discussion Paroxysmal nocturnal hemoglobinuria is a disease in which the red cells are abnonnally sensitive to the lytic action of serum complement. 1 Patients with paroxysmal nocturnal hemoglobinuria are prone to venous thrombosis, and vein thrombosis with resultant Budd-Chiari syndrome is particularly common.6 . 7 Neurologic findings of paroxysmal nocturnal hemoglobinuria have received scant attention in the literature, and the nervous system manifestations are usually thought to be due to small venous occlusions, although the morphologic evidence is often lacking. 2- 5 The clinical and laboratory findings of our patient were compatible with paroxysmal nocturnal hemoglobinuria, Budd-Chiari syndrome, and benign intracranial hypertension. The latter may be associated with vitamin or drug therapy, including corticosteroids, endocrine disorders, cranial venous sinus thrombosis or, as in the majority of cases, it may be without demonstrable cause.s It is possible that benign intracranial hypertension was produced by the steroid treatment which this patient had been taking for 8 months prior to the manifestations of increased intracranial pressure. However, the prompt decrease in intracranial pressure accompanying treatment with increased dosage of steroids suggest that there was no direct relationship between the low-dose steroid treatment and benign intracranial hypertension in our patient. Systemic illnesses, including iron deficiency anemia and blood dyscrasias, have also been implicated as causative factors in benign intracranial hypertension.s The level of anemia in the present patient was not as severe as that recorded in the literature, and the elevated intracranial pressure probably should not be ascribed to the level of anemia alone. We believe that highly suspected cortical vein thrombosis played a significant role in the pathogenesis of the increased intracranial pressure in this patient. In conclusion, papilledema and increased intracranial pressure can be a manifestation of paroxysmal nocturnal hemoglobinuria that has not been reported previously, and paroxysmal nocturnal hemoglobinuria should be added to the list of etiologic possibilities that must be considered in evaluating a patient with increased intracranial pressure of unknown cause. References 1. Rosse, W.F.: Paroxysmal nocturnal hemoglobinuria. In Hematology, W. J. Williams, E. Beutler, A J. Erslev, and R Rundles, Eds. McGraw Hill, New York, 1977, p. 562. 2. Johnson, R.V., Kaplan, S.R, and Blailock, Z.B.: Cerebral vein thrombosis in paroxysmal nocturnal hemoglobinuria. Marchiafava Micheli syndrome. Neurology 20: 681, 1970. 3. Mliftiioglu, E., Kansu, E., Kansu, T., and Ariogul, S.: Norolojik semptomlarla kendisini gOsteren bir paroksismal noktiirnal hemoglobinUri olgusu. Hacettepe TIP jCerrahi Bulteni (Turk) 15: 246, 1982. 4. Scott, R.B., Robb-Smith, AH.T., and Scowen, F.F.: The Marchiafava Micheli syndrome of nocturnal hemoglobinuria. Q.f. Med. 7: 95, 1938. 5. Crosby, W.H.: Paroxysmal nocturnal hemoglobinuria: Relation of the clinical manifestations to underlying pathogenic mechanisms. Blood 8: 769, 1953. 6. Leibowitz, AL., and Hartmann, RC.: The BuddChiari syndrome and paroxysmal nocturnal hemoglobinuria. Hr. J. Hematol. 48: 1, 1981. 7. Hartman, R.C., Luther, AB., and Jenkins, D.E.: Fulminant hepatic venous thrombosis in paroxysmal nocturnal hemoglobinuria: Definition of a medical emergency. Johns Hopkins Med. f. 146: 247, 1980. 8. Miller, N.R: Papilledema: A sign of increased intracranial pressure. In Walsh and Hoyt's Clinical Neuro-ophthalmology, Vol. 1 (4th ed.), N.R Miller, Ed. Williams & Wilkins, Baltimore, 1982, p. 201. Write for reprints to: Turgut Zileli, M.D., Department of Neurology, Hacettepe University Hospitals, Ankara, Turkey. Journal of Clinical Neuro-ophthalmology |
