Investigators at Erciyes University, Kayseri, Turkey, studied the etiological and clinical features, treatment, and prognosis of pseudotumor cerebri (PTC) in 42 consecutive patients (average age at symptom onset 10 years; range 12 months to 17 years). Girls outnumbered boys, 27 (64%) to 15 (36%). Obesity was associated in 11 (26.2%) patients. Headache in 32 (76%) was the most common presenting symptom. Headache was acute in 13 (31%), chronic daily in 12 (28.8%), acute recurrent in 4 (9.5%), and chronic relapsing in 3 (7.1%). Diplopia occurred in 18 (42.9%), visual loss in 14 (33.3%), vomiting in 15 (35.7%). Papilledema was present in all patients, and VIth cranial nerve paralysis in 8 (19.1%), one bilateral. Mean CSF opening pressure was 350 +/- 96 mm water. One had venous sinus thrombosis on MR venography.

Etiology was unidentified in 30 patients (71%) and termed primary PTC-HH. Secondary causes were detected in 12 (28.6%), defined as secondary PTC, and these included familial Mediterranean fever in 2, preceding trauma (2), and one of each of the following: mycophenolate mofetil-induced PTC, hypervitaminosis A, corticosteroid withdrawal with nephrotic syndrome, oral contraceptives, Guillain-Barre syndrome, urinary tract infection, varicella-zoster virus infection and dural venous sinus thrombosis with otitis media. Treatment included LP, acetazolamide (effective in 14 (37.8%)), and topiramate (effective in 13 of 17 patients (82.4%)). Mean duration of medical treatment was 9 months (range 1-48 months). Ventricular peritoneal shunt was beneficial in 3 patients with impaired visual fields, and visual acuity was normal in all patients at follow-up. [1]

COMMENT. Criteria for the diagnosis of idiopathic intracranial hypertension (HH) or pseudotumor cerebri (PTC) are as follows: 1) symptoms and signs of increased intracranial pressure or papilledema, 2) elevated CSF pressure at LP, 3) normal CSF composition, and 4) normal brain imaging [1]. Treatable associated disorders should be excluded or treated. Topiramate appeared more effective than acetazolamide in this study and may be used as the drug of choice. Prompt diagnosis and management are important to prevent loss of visual field and acuity.