Intracranial Complications of Leukemia in Children

Pathologic findings in the brain and meninges in untreated patients with leukemia have been reviewed. Involvement of the meninges and cerebral parenchyma were not unusual and occurred in acute and chronic disease regardless of cell type. Despite the frequency of intracranial involvement in untreated patients with leukemia, resultant clinical symptoms were infrequently noted. In the English literature only nine published reports suggestive of increased intracranial pressure in the patient with untreated disease could be found. A number of leukemic children who have received adrenal steroid hormones and chemotherapy have developed signs of increased intracranial pressure. Approximately one-fourth of the children with leukemia followed at this hospital in the last 18 months have developed this complication. Symptoms and signs are variable but usually include irritability, headache, vomiting, papilledema, separation of sutures, increase in circumference of the head, increased pressure and concentration of protein in the spinal fluid and normal or low concentration of sugar. In rare instances children with this sydrome have also developed hypertension, increased appetite with resultant obesity and various neurologic abnormalities. The pathogenesis of this syndrome is unknown. The available evidence suggests that such agents as 6-mercaptopurine and Methotrexate® which are known to cross the blood-brain barrier in greatly reduced amounts fail to control the intracranial progression of leukemia even though the disease may be under fair control elsewhere in the body. The most effective treatment for this complication at the present time is therapy with roentgen rays to the entire skull. Usually a total of 250 to 500 r given in 7 to 10 days will cause symptoms to disappear and spinal fluid findings to improve without adversely affecting the course of the disease in general. There is also some evidence that oral therapy with adrenal steroid hormones is also effective if resistance to this type of therapy has not already developed.