Pseudotumor cerebri, also known as idiopathic intracranial hypertension, is an increase in intracranial pressure without an explainable cause such as a mass lesion or hydrocephalus.
Epidemiology
Pseudotumor cerebri occurs 2-8 times more frequently in women than in men1. Obesity is present in 11-90% of cases, although it is not as prevalent in men as in women2. The incidence among women of childbearing age is 19-21/100.0003 4, whereas the incidence in the general population is 1-2/100.0005. Peak incidence occurs in the third decade of life.
Diagnostic criteria
The diagnosis of pseudotumor cerebri is one of exclusion and can be established based on the following four criteria6 7:
- Opening pressure during a lumbar puncture > 20 cmH20.
- Cerebrospinal fluid composition shows normal glucose and cell count. Protein levels are normal but may be decreased in ~ 2/3 of cases.
- Symptoms and signs of increased intracranial pressure alone, without focal neurological deficits. An accepted exception is palsy of the abducens nerve, which may result from the increased intracranial pressure8.
- Normal brain imaging, although slit ventricles and an empty sella may occur.
References
Handbook of Neurosurgery. Greenberg M, ed. 9th Edition. New York: Thieme; 2019 ↩ ↩ ↩ ↩ ↩ ↩
Digre, Kathleen B., and James J. Corbett. "Pseudotumor cerebri in men." Archives of neurology 45.8 (1988): 866-872. ↩ ↩
Radhakrishnan, Kurupath, et al. "Idiopathic intracranial hypertension (pseudotumor cerebri): descriptive epidemiology in Rochester, Minn, 1976 to 1990." Archives of Neurology 50.1 (1993): 78-80. ↩ ↩
Durcan, F. Jane, James J. Corbett, and Michael Wall. "The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana." Archives of Neurology 45.8 (1988): 875-877. ↩ ↩
Radhakrishnan, Kurupath, et al. "Idiopathic intracranial hypertension." Mayo Clinic Proceedings. Vol. 69. No. 2. Elsevier, 1994. ↩ ↩
Ahlskog JE, ONeill BP. Pseudotumor Cerebri. Ann Int Med. 1982;97:249-256. ↩ ↩