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Pseudotumor Cerebri

Pseudotumor Cerebri 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)
  • In prepubertal children, males and females equally affected
  • Rare in children <3 years or adults >60 years
  • Men potential bimodal distribution, prepubertal and middle-aged (4)
  • Hypothesized increasing incidence due to increased rates of obesity but not studied

Prevalence
  • Prospective studies show 90–98% are women (5). Uncertain whether women are more likely to be in trials. Others show 9% prevalence in males considering all age groups (6).
  • 2–12% of pregnant females (7)

Etiology and Pathophysiology

  • Unknown certain etiology
  • Associated with obesity and questionable increased intra-abdominal pressure (8)
  • Possible new cause with elevated aldosterone
  • Related to sex hormones; noted to be associated with polycystic ovarian syndrome and low testosterone in men
  • Questions concerning whether venous sinus stenosis is the cause of or the consequence of idiopathic intracranial hypertension (9)

Genetics
No known hereditary factors

Risk Factors

  • Rapidly increasing weight gain (2)
  • Prepubertal children do not have associations with obesity (3).
  • Questionable role of sex hormones, including low testosterone in men (6)

General Prevention

Because correlated with weight gain, weight management is the only known prevention although not specifically stated.

Commonly Associated Conditions

  • Polycystic ovarian syndrome often comorbid; uncertain if sex hormones have causal relationship or both diagnoses occur in overweight and obese women of reproductive age (8)
  • Obstructive sleep apnea more common in men with pseudotumor cerebri (24% vs. 4% of women) (6)

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Citation

Stephens, Mark B., et al., editors. "Pseudotumor Cerebri." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816550/all/Pseudotumor_Cerebri.
Pseudotumor Cerebri. 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. Accessed March 22, 2019.
Pseudotumor Cerebri. (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
Pseudotumor Cerebri [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 March 22]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816550/all/Pseudotumor_Cerebri.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Pseudotumor Cerebri 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 PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -