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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