pseudotumor cerebri; idiopathic intracranial hypertension
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Introduction
A disorder simulating the presence of an intracranial tumor.
Etiology
- impaired absorption of CSF by arachnoid villi
- dural sinus thrombosis
- mastoiditis
- Behcet disease
- renal failure
- obstructive sleep apnea
- pharmacologic causes:
- obesity is risk factor
Epidemiology
- commonly associated with obesity in young females
- may occur during pregnancy or in the peripartum
Pathology
Clinical manifestations
- papilledema may be observed on funduscopy
- often asymmetric, rarely unilateral
- headache (> 90%)
- pulsatile tinnitus (60%)
- transient visual disturbances
- neck pain in common
- ophthalmoplegia
- cranial nerve 6 palsy is a false localizing sign associated with intracranial hypertension
- obesity common
Diagnostic procedures
- lumbar puncture (elevated opening pressure) > 250 mm Hg
- CSF examination is normal
- slit lamp examination
Radiology
- contrast-enhanced brain MRI may show
- widening of optic nerve sheaths
- partially empty sella
- flattening of the posterior optic globes[4]
- small ventricles
- magnetic resonance venography to exclude dural sinus venous thrombosis[4]
Management
- consult neurology & ophthalmology
- goals: preserve vision, alleviate symptoms
- carbonic anhydrase inhibitor (acetazolamide, topiramate)[4]
- avoid glucocorticoids due to weight gain & fluid retention
- if medical management fails & visual changes are progressive
- optic nerve sheath fenestration
- lumboperitoneal shunting has high rate of failure[4]
- long-term
More general terms
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 829-39
- ↑ Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 72, 167
- ↑ 4.0 4.1 4.2 4.3 4.4 Medical Knowledge Self Assessment Program (MKSAP) 15,16,17,18,19. American College of Physicians, Philadelphia 2009,2012,2015,2018,2021
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ Wall M. Idiopathic intracranial hypertension. Neurol Clin. 2010 Aug;28(3):593-617 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20637991
- ↑ Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol. 2016 Jan;15(1):78-91. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26700907