Clinical features of late-onset pseudotumor cerebri fulfilling the Modified Dandy Criteria

Objectives: To characterize the clinical features of patients with pseudotumor cerebri (PTC) fulfilling the Modified Dandy Criteria who were diagnosed at or after the age of 44 years. Methods: We reviewed the medical records between 1987 and 1999 of 14 patients at a single institution who were diagnosed as having PTC at 44 years of age or older according to the Modified Dandy Criteria: neurologic manifestations attributable to generalized increased intracranial pressure, elevated cerebrospinal fluid pressure with normal cerebrospinal fluid composition demonstrated by lumbar puncture, and normal or small ventricles demonstrated by neuroimaging. We documented presenting symptoms and signs, significant medical conditions, and visual field follow-up. Results: There were nine women and five men. Nine patients (64%) were obese. Five patients (36%) were asymptomatic. None presented with headache alone. Four patients (29%) had an identifiable cause of intracranial hypertension, including two with transverse sinus thrombosis, one with severe chronic obstructive pulmonary disease and cor pulmonale, and one with corticosteroid withdrawal after prolonged administration. During a median follow-up of 2 years of 12 patients, visual fields remained stable in 8, improved in 3, and worsened in 1. Conclusions: In comparison with patients who have idiopathic intracranial hypertension, our small series of 14 patients diagnosed after the age of 44 years were more often men, were less often obese, were less symptomatic, and had identifiable causes of intracranial hypertension in a substantial minority (29%). The visual prognosis in this age group is generally good. Because the nonidiopathic causes of PTC would be overlooked by adhering to the Modified Dandy Criteria, we propose a modification that excludes patients who have dural venous sinus disease demonstrated on magnetic resonance imaging and those who may be exposed to medications or toxins or have systemic disorders that are known to increase intracranial pressure.