Neonatal Polycythemia Causing Multiple Cerebral Infarcts Minoli Amit, MD, Peter R. Camfield, MD \s=b\A child had neonatal polycythemia, REPORT OF A CASE severe seizures beginning at 6 weeks of a computerized tomography showing multiple cerebral infarcts. Follow-up showed substantial developmental delay with spastic quadriplegia. This case illustrates that neonatal polycy- age, and scan themia is a rare but preventable cause of multiple cerebral infarctions, subsequent seizures, and developmental delay. (Arch Neurol 37:109-110, 1980) "M"eurologic manifestations of neonatal polycythemia have been well described.12 Convulsions may occur in the neonatal period and subsequently. Gross et al2 reported 18 polycythemic neonates who were lethargic and "jit¬ tery," with five showing generalized or focal seizures. Four of the 14 of these children who were followed up had significant motor or mental retar¬ dation. The mechanism by which poly¬ cythemia causes neurologic and other symptoms is thought to be sludging of hyperviscous blood in small vessels.1 We describe a baby with neonatal polycythemia who had severe convul¬ sions at 6 weeks of age and who was found to have multiple cerebral infarcts on computerized tomography ' (CT) scan. Accepted for publication Feb 20, 1979. From the Department of Pediatrics, I. W. Killam Hospital for Children and Dalhousie University, Halifax, Nova Scotia, Canada. Reprint requests to Department of Pediatrics, I. W. Killam Hospital for Children, PO Box 070, Halifax, Nova Scotia, B3J 3G9, Canada (Dr Camfield). A 6-week-old infant was admitted to the I. W. Killam Hospital for Children, Hali¬ fax, Nova Scotia, with a five-day history of generalized seizures. He was born after a normal pregnancy and vaginal delivery to a gravida 3, para 1, abortus 2 mother. The gestation was 40 weeks and birth weight was 3,290 g. There was no perinatal asphyxia. Details of cord clamping were not recorded. Within six hours of delivery, he was tremorous, fed poorly, and vomited several times. The hemoglobin level of blood obtained by heel prick was 26.4 g/dL and the hematocrit reading was 72%. Val¬ ues for WBCs, blood glucose, serum electro¬ lytes, calcium, bilirubin, urinalysis, and abdominal x-ray films and EEG were normal on the first day of life. Symptoms continued, and on the third day the baby was plethoric, irritable, hypertonic, and mildly dehydrated. A diagnosis of neonatal hyperviscosity syndrome was made, and treatment included immediate phlebotomy of 15 mL of blood replaced with 10 mL of saline and vigorous oral rehydration. With¬ in 24 hours, there was a marked improve¬ ment in symptoms and the hemoglobin level fell to 19 g/dL. Following discharge on the seventh day, he seemed well until the onset of seizures. On admission, he was afebrile. Height and weight were at the 50th percentile, and head circumference was at the 75th percentile. Initially, the baby was hypotonic and difficult to arouse. He had frequent, brief, generalized, tonic-clonic seizures, each lasting a few seconds. There were no focal findings, and results of the general examination were normal. The family history was unremarkable. He was treated with phénobarbital and phenytoin with a prompt and substantial decrease in the frequency of seizures. As the seizures came under control, his overall Downloaded From: http://archneur.jamanetwork.com/ by a University of Manitoba User on 06/21/2015 muscle tone was thought to be markedly increased. The hemoglobin level was 12.5 g/dL, with a hematocrit reading of 35%. Values for the following were normal: WBC count; platelet count; clotting time; IgM; serum electrolytes, calcium, phosphorus, and al¬ kaline phosphatase; blood gases; BUN; serum creatinine; urinalysis; urine amino acids; roentgenograms of skull and chest; and lumbar puncture. An EEG the day after admission showed nearly continuous, multifocal, moderate-voltage spikes and sharp waves with no response to intrave¬ nous pyridoxine hydrochloride. One week later, an EEG showed moderate improve¬ ment. A CT scan without enhancement showed Multiple infarctions from polycythemia. multiple symmetrical areas of low density that were interpreted as infarctions (Fig¬ ure). The baby was treated with phénobarbi¬ tal and phenytoin, and there were no fur¬ ther seizures until 10 weeks of age, when a brief cluster occurred and anticonvulsant drug dosage was increased. Neurologic examination then showed him to be alert with mildly increased muscle tone through¬ out. There was a low-amplitude rotatory nystagmus. At 4 months of age, there were no fur¬ ther seizures and his height and weight continued at the 90th percentile; the head circumference had fallen to the 25th percentile. He was active and smiled appro¬ priately; however, examination showed mild head lag and moderately increased muscle tone in his legs. An EEG showed infrequent, moderate-voltage sharp waves and spikes, which was an improvement from the previous tracing. Another CT scan showed the areas of low density to have decreased in size. At 9 months of age, he had definite spastic quadriplegia with sustained clonus at the ankles. His social development appeared normal. COMMENT This case points out a preventable cause of neurologic disease. The assumption that severe polycythemia in the first few days of life was the cause of both infarctions and resul¬ tant convulsions at 6 weeks requires justification. The hemoglobin and hematocrit values were in a range that has been associated with cere¬ bral, cardiac, and renal disorders in the newborn.2 A hematocrit reading of more than 65% may be sufficient to increase viscosity to the point of impairing blood flow in small ves¬ sels.2-4 This child's neonatal symptoms of poor feeding, "jitteriness," vomit¬ ing, and plethora are typical of the neonatal hyperviscosity syndrome. The onset of seizures six weeks later is consistent with the usual latency from cerebral infarction to seizures in older patients.' The findings on CT scan at 6 weeks of age suggest an earlier insult, and the gradual resolution at 4 months of age is in keeping with infarction.'· For these reasons, we consider that multiple small-vessel occlusions from neonatal polycythe¬ mia were the cause of the neurologic findings and seizures. Polycythemia in the newborn has several causes, some of which are preventable. Delayed umbilical cord clamping or cord stripping that markedly increases the hematocrit reading can be avoided.7 Early inter¬ vention to prevent chronic intrauter¬ ine hypoxia from placental dysfunc¬ tion in preeclampsia or diabetes may prevent additional polycythemic cases. Prompt recognition of severe polycythemia should lead to early phlebotomy or partial-exchange transfusion, restoring the baby's blood viscosity to safe levels. Our patient's polycythemia was not treated until 3 days of age. Perhaps the neonatal symptoms and later neurologic problems might have been avoided by earlier treatment. Whether or not all newborns should be screened for polycythemia is uncer¬ tain. In one large study, 0.1% of con¬ secutively studied newborns had a peripheral hematocrit reading of more than 77%.s Others have reported elevated blood viscosity in 5% of consecutively studied newborns." Un¬ til it can be established how many of these children will have neonatal symptoms and which ones will be Downloaded From: http://archneur.jamanetwork.com/ by a University of Manitoba User on 06/21/2015 among the few individuals with neu¬ rologic sequelae, it would seem rea¬ sonable to promptly reduce the hema¬ tocrit reading of those with neonatal symptoms to the range of 60% and to observe most asymptomatic infants very closely.4 As our information about this syndrome increases, a more aggressive approach to asymptomatic newborns may become justified.10 Dorothy Barnard, MD; Brian Byrne, MD; Carol Camfield, MD; and J.A.R. Tibbies, MD; and Robin Walker, MD, reviewed the manuscript. R. Walker referred the patient. References 1. Wood JL: Plethora in the newborn infant associated with cyanosis and convulsion. J Pediatr 54:143-151, 1959. 2. Gross G, Hathaway W, McGaughey H: Hyperviscosity in the neonate. J Pediatr 82:1004\x=req-\ 1112, 1973. 3. Dintenfass L: Blood viscosity, internal fluidity of the red cell, dynamic coagulation and the critical capillary radius as factors in the physiology and pathology of the circulation and microcirculation (review). Med J Aust 1:688-696, 1968. 4. Nathan D, Oski F: Hematology of Infancy and Childhood. Philadelphia, WB Saunders Co, 1974. 5. Fischer CM, in Thorn GW, Adams RD, et al (eds): Harrison's Principles of Internal Medicine, ed 8. New York, McGraw-Hill Book Co Inc, 1977, p 1762. 6. Lukin R, Chambers A, Tomsick T: Cerebral vascular lesions: Infarction, hemorrhage, aneurysm and arterlovenous malformation. Semin Roentgenol 12:77-89, 1977. 7. Usher R, Shepard M, Lind J: The blood volume of the newborn infant and placental transfusion. Acta Paediatr 52:497-512, 1963. 8. Weinberger M, Oleinick A: Neonatal polycythemia. Clin Res 19:209, 1971. 9. Goldberg K, Lubchenco L: Neonatal hyperviscosity incidence and effect of partial plasma exchange transfusion. Pediatr Res 9:372, 1975. 10. Goldberg K, Lubchenco L: Sequelae of neonatal hyperviscosity. Pediatr Res 10:448, 1976.