Abstract
Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial
pressure in the setting of normal brain parenchyma and normal cytologic and chemical
analyses of the cerebrospinal fluid. Pathophysiology of this syndrome remains unclear,
while its incidence may be potentially rising in children. The presenting signs and
symptoms of PTCS are heterogeneous to usually include headache, papilledema, and visual
disturbances (i.e., vision loss or double vision). The clinical features of PTCS in
children have changed over the years, making the diagnosis challenging in some cases.
Young children may present with atypical symptoms, such as those mimicking a posterior
fossa tumor. There are reports of PTCS in children in the absence of headache. Papilledema,
considered the acute hallmark of PTCS at onset, can also be absent. It is thus important
for pediatricians to recognize atypical presentations especially as previous studies
have suggested children presenting with headache in the absence of papilledema or
those with papilledema in the absence of any other clinical signs may have increased
risk of developing visual constriction deficits and permanent loss of vision, respectively.
Moreover, many conditions in the pediatric age group may mimic PTCS, such as migraine
and tension headaches. Cerebral venous sinus thrombosis and some neoplastic processes
such as gliomatosis cerebri and leptomeningeal spread of hematologic malignancies
are known to augment intracranial pressure and have to be carefully excluded. To avoid
misdiagnosis and potential long-term sequel, a thorough clinical assessment is crucial
to promptly and correctly recognize PTCS.
Keywords
idiopathic intracranial hypertension - pseudotumor cerebri - children - differential
diagnosis
