Pseudotumor Cerebri

Basics

  • Most common in pre-menopausal obese females
  • Most common symptom is headache
  • An important preventable cause of blindness
  • Normal imaging studies and a lumbar puncture only demonstrates increased pressure
  • Most cases are treatable with medication though refractory cases often require an operation

Symptoms and Signs

  • Headache
  • Decreased visual acuity, double vision, and blindness
  • Nausea
  • Other symptoms: stiff neck, ringing ears, difficulty walking, eye pain on movement, dizziness
  • Worsening of symptoms with bending over or valsalva

Demographics

  • Female : Male ratio 2-8:1
  • Increased in obesity and women of childbearing age: Peaks in 30s

Diagnosis

  • Diagnosis is made on the combination of normal imaging studies (CT and MRI) with increased opening pressure during a lumbar puncture
  • Ophthalmologic exam

Treatment

  • Weight loss
  • Stop offending drugs
  • Fluid and salt restriction
  • Diuretics- Lasix, Diamox or Neptazane
  • Surgery- Optic nerve sheath fenestration or a lumboperitoneal/ventriculoperitoneal shunt

Prognosis

  • Spontaneous resolution is common within 1 year
  • Permanent visual loss occurs in 2-24%
  • Recurs in about 10%

Differential Diagnosis

  • Drugs: Keprone, lindane, excess vitamin A, oral contraceptives
  • Tumor
  • Dural sinus thrombosis
  • Chiari malformation
  • Infection
  • Neurosarcoidosis
  • Pseudopapilledema
  • Malignant hypertension
  • Guillain-Barre syndrome
  • head trauma