Pseudotumor cerebri (PTC) is a perplexing syndrome of increased intracranial pressure
(ICP) without a space-occupying lesion. The terminology for the disorder has changed
over the years and the diagnostic criteria revised to reflect advances in diagnostic
technology and insights into the disease process [
[1]
]. The classification and nomenclature depend on the presence or absence of an underlying
cause. When the diagnostic criteria are followed, a secondary etiology is unlikely
(Box 1[
[1]
]). When no secondary cause is identified, the syndrome is termed “idiopathic intracranial
hypertension (IIH).”
Box 1
Diagnostic criteria for pseudotumor cerebri
-
Symptoms, if present, reflect only those of increased intracranial pressure or papilledema.
-
Signs are attributable only to increased intracranial pressure or papilledema.
-
Documented elevated intracranial pressure during lumbar puncture is measured in the lateral decubitus position.
-
Cerebrospinal fluid composition is normal.
-
There is no ventriculomegaly, mass, structural, or vascular lesion on neuroimaging studies (ie, MRI or contrast-enhanced CT for typical patients, and MRI and magnetic resonance venography for all others).
-
If no other cause (including medication) of intracranial hypertension is identified, the syndrome is termed “idiopathic intracranial hypertension.”
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