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Stroke in a Patient with Hemolytic-Uremic Syndrome with a Good' Outcome Abraham Steinberg, MD, Miriam Ish·Horowitcz, MD, Orly EI·Peleg, MD, Joseph Mor, MD and David Branski, MD We report a child with HUS complicated by stupor, hemiparesis, expressive aphasia and focal seizures. CAT scan of the brain demonstrated 2 large cerebral non-hemorrhagic infarcts. Despite the severe neurological involvement the child eventually made a good recovery. Steinberg A, Ish-Horowitcz M, El-Peleg 0, Mor J, Branski D. Stroke in a patient with hemolytic-uremic syndrome with a good outcome. Brain Dev 1986;8:70-72 From the Division of Pediatric Neurology and Department of Pediatrics, Bikur Cholim General Hospital, Jerusalem, Israel. Received for pUblication: August 2,1985. Accepted for pUblication: November 25, 1985. Key words: CATscan. Hemolytic·uremic syndrome, stroke, Correspondence address: D. Branski, MD, Department of Pediatrics, Bikur Cholim Hospital, 3-5 Strauss St Jerusalem, Israel. Neurological complications occur frequently in the hemolytic-uremic syndrome (HUS) and may constitute a major clinical manifestation [1, 2]. Those patients whose complications include coma or hemiparesis have been reported to have a worse prognosis [I, 3] . We report the case of a patient with HUS complicated by stupor and hemiparesis due to a large cerebral infarction, who nevertheless made a good recovery. Table 1 Reported CAT scan findings in the hemolytic uremic syndrome CA T scan findings No of patients Bilateral small areas of infarctions in the basal ganglia [8] 3 Intracerebral hemorrhage [8] Fig 1 Initial non-contrast CAT scan showing a wedgeshaped left parietal hypodense area and a smaller left frontal hypodense region consistent with distribution of the middle and anterior cerebral arteries, respectively. Fig 2 5 months later non-contrast CAT scan showing an old left parietal infarct with left hemispheric atrophy, and a residual left frontal infarct. Case Report A 2~-year-old white female was admitted to our department, with a 2-day history of bloody diarrhea, vomiting and abdominal pain. Her past medical and family history were unremarkable . On admission her general physical and neurological examination were normal apart from signs of mild dehydration and increased bowel sounds. Initial laboratory investigations showed a hemoglobin of 14.2 g/dl, MCV 75 fl, white cell count 15,100/mm3 with neutrophils of 70%. Urinary acetone was +4. Serum electrolytes, liver function tests and blood gases were all within normal limits. Urea was 13.2 mmol/L. All stool cultures were negative. On the sixth day of her admission she became anuric, and had hematemesis. On examination she had gained 2.5 kg. She was pale, edematous, dyspneic and tachycardic. Blood pressure was 150/1 00 mmHg; her liver was palpable 6 cm below the right costal margin. Laboratory investigations showed a hemoglobin of 5.1 g/dl, reticulocytes 17%, thrombocytes 20,000/mm 3 • The peripheral blood smear demonstrated many burr cells, fragmented Hypodense cortical infarcts with delayed hemorrhage [9] 2 Hypodense non-hemorrhagic cortical infarcts (present case) cells and acanthocytes consistent with microangiopathic hemolytic anemia. Serum sodium was 120 mmol/L, potassium 4.3 mmol/L, urea 25.6 mmol/L, creatinine 566 mmol/L, uric acid 634 mmol/L, calcium 1.4 mmol/L, phosphate 0.8 mmol/L, SGOT 58 units/L, SGPT 64 units I L, serum protein 51 giL, albumin 27 giL, PTT - normal, fibrinogen 3.6 giL. Arterial Astrup was pH 7.26, PCO z 28 mmHg, base excess -13, actual bicarbonate 12 mmol/L, POz 76 mmHg. Ci1est X-ray showed congestion in both lungs. ECG and echocardiogram were normal. A diagnosis of HUS was made. Peritoneal dialysis was started with a good clinical and biochemical response. The rise in blood pressure was controlled with hydralazine. She also received 5 transfusions of packed cells, fresh frozen plasma, digoxin and cimetidine. On the twelfth day of her admission she appeared confused disoriented and became stuporous. At that time blood pressure and biochemical data were normal. EEG showed hemispheral asymmetry with delta slowing in the left parieto-temponil region, as well as paroxysmal epileptiform generalized discharge. The following day she had several episodes of right focal seizures, following which she developed a complete right sided hemiplegia and an expressive aphasia. Computerized axial tomography (CAT) scan showed moderate cortical atrophy with two large hypo dense areas of infarction in the left parietal and frontal areas (Fig 1). There was no evidence of hemorrhage. Treatment with phenobarbital was started and she had no further convulsions. One year after discharge she remains well. Her renal function is normal. Her language has gradually returned to normal. Her intellectual functioning is normal and she is well-adjusted in a kindergarten. She has only a residual mild Steinberg et al: Stroke in hemolytic-uremic syndrome 71 right hemiparesis. Repeat CAT scan shows a left parietal old infarct with left hemispheral atrophy. The left frontal infarct seen on the initital scan has almost completely resolved (Fig 2). Discussion Major neurological involvement in the hemolytic-uremic syndrome (HUS) includes seizures, coma, hemiparesis, aphasia and decerebrate posturing [2,3] . Bale et al [1] and Gianantonio et al [2] found neurological manifestations in 41% and 50% of their patients with HUS, respectively. Others [4, 5] have encountered serious complications of the central nervous system (CNS) less frequently. Several pathogenic mechanisms have been postulated: a) Metabolic derangements, i.e. volume overload, hyponatremia, hypocalcemia, hypoglycemia, acid-base disturbances or changes in serum osmolarity [1, 2, 4] . b) Hypertension [6] . c) Cerebral microvascular thrombi causing changes in the CNS similar to those occuring in the kidney [7]. Some workers, however, have not been able to demonstrate such changes on neuropathological evaluation [1, 3] . Since the advent of the CAT scan, 6 case reports have substantiated the existence of structural CNS changes in patients with HUS. There were either multiple areas of infarction in the basal ganglia, or hemorrhagic cerebral infarcts (Table 1). We add a seventh case with CAT scan evidence of large non-hemorrhagic cerebral infarcts. Since our patient had no 72 Brain & Development, Vol 8, No 1,1986 predisposing factors for stroke, other than the HUS, we conclude that the stroke was secondary to cerebral microangiopathy and associated microthrombi. In contrast to previous reports of a poor prognosis in cases of HUS with neurological complications [1, 3] , our case report supports the conclusion of Steele et al [8] that even with severe CNS involvement the prognosis in HUS may still be good. References 1. Bale JF, Brasher C, Siegler RL. CNS manifestations of the hemolytic-uremic syndrome. Am. J Dis Child 1980;134:869-72. 2. Gianantonio CA, Vitacco M, Mendilarzu F, Gallo J, Sojo ET. Hemolytic-uremic syndrome. Nephron 1973;11:174-92. 3. Rooney JC, Anderson RM, Hopkins IJ. Clinical and pathological aspects of central nervous system involvement in the haemolytic uremic syndrome. Aust PediatrJ 1981;7:28-33. 4. Lieberman E. Hemolytic uremic syndrome. J Pediatr 1972;80:1-16. 5. Tune BM,Leavitt TJ, Gribble TJ. The hemolytic uremic syndrome in California: a review of 28 non-heparinized cases with long-term fOllow-up. J Pediatr 1973;82:304-10. 6. Kaplan BS, Thomson PO, Chadareuian JP. The hemolytic-uremic syndrome. Pediatr Clin North Am 1976;23:761-77. 7. Upadhyaya K, Barwick K, Fishaut M, Kashgarian M, Siegel NJ. The importance of non-renal involvement in hemolytic-uremic syndrome. Pediatrics 1980;65: 115-20. 8. Steele BT, Murphy N, Chuong SH, McGreal 0, Arbus GS. Recovery from prolonged coma in hemolytic uremic syndrome. J Pediatr 1983; 102:402-4. 9. Crisp DE, Siegler RL, Bale JF, Thompson JA. Hemorrhagic cerebral infarction in the hemolyticuremIc syndrome. J Pediatr 1981;99:273-6.