Emergency department presentation of idiopathic intracranial hypertension

Idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is a syndrome characterized by an elevated intracranial pressure in the absence of a focal lesion, infective process, or hydrocephalus. New onset IIH may present to the emergency department in a variety of ways. To describe the etiologic associations and clinical features in this disorder, we performed a retrospective analysis of consecutive emergency department patients with new onset IIH during the calendar years 1987-1996. A total of 52 patients met all study criteria. The mean patient age was 27+/-8.9 years; the female-to-male ratio was 7:1. An etiologic association could be identified in 85% of cases and included obesity, hypertension, drugs, endocrine, and systemic disorders. Headache was a dominant complaint in most patients (48/52) and associated with dizziness, nausea, and/or visual complaints. Fourteen patients (27%) were not diagnosed on their initial ED visit and were more likely to have atypical clinical features (71% vs. 24%; P = .004). Atypical features included paraesthesias, neck/back pain, unilateral headache, vertigo, and nystagmus. Papilledema, the ophthalmoscopic hallmark of IIH, was not detected initially in 11 patients (21%). These results suggest that IIH is a relatively uncommon neurological illness that may have a variety of causes. The emergency department diagnosis may be complicated by atypical clinical features and a lack of detectable papilledema.