Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)
- Increased intracranial pressure with no clear pathophysiology
- Many have suggested calling it pseudotumor cerebri syndrome emphasizing the variety of subtypes (1).
- Subtypes include true idiopathic intracranial hypertension, idiopathic intracranial hypertension with underlying cause, and idiopathic intracranial hypertension without papilledema.
- 0.9/100,000 in general population (2)
- 4 to 11.9/100,000 in obese women
- 19/100,000 in women >20% over ideal body weight and reported 323/100,000 in those seeking bariatric surgery (3)
- Lower incidence in Scandinavia in adults of 0.65/100,000 (4)
- In children age <7 years, males and females equally affected
- Children age >7 years, obese girls have doubled incidence of obese boys and more than doubled for age 12 to 15 years (5).
- Rare in children <3 years or adults >60 years
- Men potential bimodal distribution, prepubertal and middle-aged (6)
- Hypothesized increasing incidence due to increased rates of obesity but not studied
Etiology and Pathophysiology
- Unknown certain etiology
- Associated with obesity; fewer cases in countries with less obesity (4)
- Questionable association with increased intra-abdominal pressure (10)
- May be associated with increased vitamin A levels and corticosteroid treatment (10)
- Possible new cause with elevated aldosterone
- Related to sex hormones; noted to be associated with polycystic ovarian syndrome and low testosterone in men (8); theorized related to female hormones because significant difference in incidence in females regardless of obesity during reproductive years
- Questions concerning whether venous sinus stenosis is the cause of or the consequence of idiopathic intracranial hypertension (10)
No known hereditary factors
Because correlated with weight gain, weight management is the only known prevention, with potential support in studies of children (5).
Commonly Associated Conditions
- Polycystic ovarian syndrome often comorbid; uncertain if sex hormones have causal relationship or both diagnoses exist in overweight and obese women of reproductive age (10)
- Obstructive sleep apnea more common in men with pseudotumor cerebri (24% vs. 4% of women) (8)
- Other potential links are with tetracycline use (more in adolescents for acne) (10).
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Domino, Frank J., et al., editors. "Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688631/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_.
Pseudotumor Cerebri (Idiopathic Intracranial Hypertension). In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688631/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_. Accessed May 30, 2023.
Pseudotumor Cerebri (Idiopathic Intracranial Hypertension). (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688631/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_
Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 May 30]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688631/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) ID - 1688631 ED - Domino,Frank J, ED - Baldor,Robert A, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688631/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_ PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -