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Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • 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.

Epidemiology

Incidence
  • 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

Prevalence
  • Prospective studies show 90–98% are women (7). Others show 9% prevalence in males considering all age groups (8).
  • 2–12% of pregnant females (9)
  • Female to male ratio ranges in adults from 4:1 to 6.1:1 (4)

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)

Genetics
No known hereditary factors

Risk Factors

  • Rapidly increasing weight gain (2)
  • Prepubertal children do not have associations with obesity (3,5).
  • Female sex during reproductive years

General Prevention

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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Citation

Stephens, Mark B., et al., editors. "Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816550/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_.
Pseudotumor Cerebri (Idiopathic Intracranial Hypertension). In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816550/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_. Accessed May 22, 2019.
Pseudotumor Cerebri (Idiopathic Intracranial Hypertension). (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816550/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_
Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 May 22]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816550/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 - 816550 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816550/all/Pseudotumor_Cerebri__Idiopathic_Intracranial_Hypertension_ PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -