Pediatric Pseudotumor Cerebri Syndrome: Diagnostic Criteria and Classification

Abstract

Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the context of normal brain parenchyma and normal cytologic and chemical analyses of the cerebrospinal fluid. The presenting signs and symptoms of PTCS are various and usually include headache, papilledema, and visual disturbances (e.g., vision loss, double vision). Idiopathic intracranial hypertension, which is the primary form of PTCS, generally affects postpubertal overweight women of childbearing age. Secondary PTCS refers to a clinical diagnosis of PTCS attributable to one or more of a variety of identifiable causes, including venous sinus thrombosis, medications, and medical conditions other than obesity alone. The diverse clinical spectrum of pediatric PTCS is becoming increasingly recognized and recent research in this field is aiming to identify a common pathophysiology. We hereby review the recent diagnostic criteria and classification of pediatric PTCS.