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Objective: To identify the most frequent causes of secondary pseudotumor cerebri syndrome and compare phenotype, clinical presentation, and symptoms of secondary pseudotumor cerebri syndrome to the primary form of pseudotumor cerebri syndrome, idiopathic intracranial hypertension., Methods: The study was a prospective cohort study including patients with new-onset pseudotumor cerebri syndrome. Diagnostic work up was standardized. Patients were diagnosed with secondary pseudotumor cerebri syndrome or idiopathic intracranial hypertension according to the revised Friedman criteria. Secondary pseudotumor cerebri syndrome patients were categorized into five causes: medication, systemic causes, sleep apnea, cerebrovascular causes, and several competing causes. Phenotype, clinical presentation, symptoms and neuroimaging were compared between groups., Results: Out of 278 cases, 28 secondary pseudotumor cerebri syndrome and 120 idiopathic intracranial hypertension patients were included. The most frequent causes of secondary pseudotumor cerebri syndrome were medication (n  =  8, 28.6%) and systemic causes (n = 8, 28.6%), followed by sleep apnea (n = 5, 17.9%), cerebrovascular causes (n = 4, 14.3%) and several competing causes (n = 3, 10.7%). Secondary pseudotumor cerebri syndrome and idiopathic intracranial hypertension patients were phenotypically alike and predominately female, premenopausal, and obese. Symptoms and objective findings at disease onset were similar between groups., Conclusion: Secondary pseudotumor cerebri syndrome should be considered in all patients with suspected pseudotumor cerebri syndrome as secondary pseudotumor cerebri syndrome and idiopathic intracranial hypertension patients are phenotypically and clinically alike. A thorough diagnostic workup is needed as treatment of idiopathic intracranial hypertension and secondary pseudotumor cerebri syndrome is markedly different.

Background: Our objective was to assess optic nerve sheath diameter (a marker of elevated intracranial pressure) and optic disc elevation (a marker of papilledema) in pseudotumor cerebri syndrome using transorbital sonography., Methods: The study was a prospective case-control study. We included patients with new-onset pseudotumor cerebri syndrome and matched healthy controls. All had fundoscopy, lumbar puncture with opening pressure and transorbital sonography. Sonography was assessed by a blinded observer., Results: We evaluated 45 patients and included 23 cases. We recruited 35 controls. Optic nerve sheath diameter was larger in pseudotumor cerebri syndrome compared to controls (6.3 ± 0.9 mm versus 5.0 ± 0.5 mm, p < 0.001) and so was optic disc elevation (0.9 ± 0.4 mm versus 0.4 ± 0.1 mm, p < 0.001). The optimal cut-off point for optic nerve sheath diameter was 6 mm with a sensitivity of 74% for prediction of pseudotumor cerebri syndrome and 68% for prediction of elevated opening pressure. Specificity was 94%. The optimal cut-off point for optic disc elevation was 0.6 mm. Sensitivity was 100% and specificity 83% for prediction of pseudotumor cerebri syndrome., Conclusion: Optic disc elevation and optic nerve sheath diameter are increased in new-onset pseudotumor cerebri syndrome. Optic disc elevation achieved high specificity and excellent sensitivity for diagnosis of pseudotumor cerebri syndrome. Transorbital sonography (TOS) is a potential, non-invasive screening tool for pseudotumor cerebri syndrome in headache clinics.

Background: The aim of the study was to evaluate the presenting symptoms and signs of idiopathic intracranial hypertension (IIH) in a large cohort of patients and to estimate their possible role in establishing the diagnosis of IIH., Methods: This prospective cohort study in two tertiary centers, the Danish Headache Center in Rigshospitalet-Glostrup and the Neurology Clinic of the Clinical Center of Serbia, included 286 patients referred by attending specialists for possible IIH evaluation. Patients were divided into two groups: one with confirmed IIH diagnosis and one with rejected IIH diagnosis., Results: The diagnosis of IIH was confirmed in 219 (76.6%) patients. It was more often confirmed if the patient was referred by an ophthalmologist than if the referral was from a neurologist (83.6% vs. 69.8%, p = .029) and in patients with higher body mass index (BMI) (p = .032). Transient visual obscurations (p = .006), double vision (p = .033), neck pain (p = .025), and tinnitus (p = .013) were presenting symptoms more frequently reported by patients with IIH diagnosis. In the same group of patients, papilledema (p < .001) and sixth nerve palsy (p = .010) were noted significantly more often. Papilledema was extracted by multivariate analysis as an independent predictor of IIH diagnosis (p < .001)., Conclusion: Although studies investigating IIH report an abundance of presenting symptoms, our results indicate that these symptoms are not diagnostic for IIH. Papilledema is the most reliable clinical sign predicting the correct IIH diagnosis in patients with suspected IIH., (Copyright © 2019 Elsevier B.V. All rights reserved.)