Diagnosis of idiopathic intracranial hypertension - the importance of excluding secondary causes: A systematic review

Abstract

Background Idiopathic intracranial hypertension is characterized by increased intracranial pressure without any pathological findings on neuroimaging, except for signs of high intracranial pressure. Before diagnosing idiopathic intracranial hypertension secondary causes of increased intracranial pressure should be excluded. Objective to characterize the phenotype of patients with secondary intracranial hypertension and to identify possible risk factors for secondary intracranial hypertension. Methods We have systematically searched the PubMed database. The publications were analyzed according to the patient phenotype, age, gender, comorbidities, body mass index/weight status, and additional medication. The results are summarized in four categories: medication, infection, hormonal induced intracranial hypertension and miscellaneous groups of diseases related to sIH. Results We identified 105 eligible papers which included 272 cases. There were 49.6% pediatric cases. Among the adult group,70.9% were women. A total of 40.4% of all cases were obese or overweight, 27% among adults and 13.4% among pediatric cases. Increased BMI and recent weight gain, anemia, renal diseases and hypertension were the most frequent comorbidities related to sIH. Conclusion Among sIH patients, 40.4% were obese or overweight; two thirds were women. We recommend that even patients with a typical IIH phenotype should be screened for secondary causes.

Phenotyping non-idiopathic pseudotumor cerebri syndrome – A prospective cohort study

“…In a systematic review tetracycline class antibiotics and retinoids had the highest number of reported cases of medication induced sPTCS with !20 cases (12). Prednisolone has mostly been associated with sPTCS in children in previous studies (12,18). For two patients categorized as 'several competing causes known to cause sPTCS' debut of sPTCS was associated with both prednisolone treatment and renal failure.…”

“…males, post-menopausal females or BMI <30. Previous studies investigating sPTCS have primarily been case studies or retrospective studies and only one recent literature review has reviewed various causes of sPTCS (18). Interestingly this study found that among sPTCS cases, 70.9% were female and 40.4% had a BMI >25 (18).…”

“…Previous studies investigating sPTCS have primarily been case studies or retrospective studies and only one recent literature review has reviewed various causes of sPTCS (18). Interestingly this study found that among sPTCS cases, 70.9% were female and 40.4% had a BMI >25 (18). This begs the question whether clinicians should consider sPTCS in patients who display the typical IIH phenotype or whether this should primarily be a concern in atypical patients.…”

Idiopathic Intracranial Hypertension: An Unusual Cause of Headache in a Patient With Sickle Cell Disease

“… 3 Many risk factors for IIH have been established, including medication use (tetracycline antibiotics, retinoids) and medical disease (endocrinopathies, leukemia, iron deficiency anemia), but the link between SCD or hydroxyurea use and IIH remains unclear. 9 Among all previously published cases of IIH in patients with SCD, the patients have been either prepubertal or obese. 5 - 8 …”

Guidelines of the International Headache Society for Controlled Clinical Trials in Idiopathic Intracranial Hypertension

“…Given that IIH is rarer in prepubertal children, and potentially has a different underlying pathogenesis (32), the present guidelines will primarily focus on those postpuberty and adults. The present guidelines will only address IIH and not secondary pseudotumor cerebri syndrome nor Idiopathic Intracranial Hypertension without Papilledema (IIHWOP) as these are rare conditions with likely alternative underlying pathophysiology (33)(34)(35).…”