Rare diseases · Sign or symptom
Increased intracranial pressure
Rise in pressure inside skull
HP:0002516
What it means
An increase of the pressure inside the cranium (skull) and thereby in the brain tissue and cerebrospinal fluid.
Normal values of intracranial pressure in adults are around 7-15 mm Hg in the supine position. The term pseudotumor cerebri (also: idiopathic intracranial hypertension or benign intracranial hypertension) is the presence of increased intracranial pressure in the absence of a brain tumor or other identifiable cause. It should be coded using this term and negations of appropriate other HPO terms. Increased intracranial pressure can damage tissue, contribute to hydrocephalus, cause brain herniation, and restrict blood supply to the brain.
Rare diseases that can present with this58
Very common80–99%
7Common30–79%
14- Angiostrongyliasis
- Arnold-Chiari malformation type I
- Central neurocytoma
- Crouzon syndrome
- Crouzon syndrome-acanthosis nigricans syndrome
- Familial cerebral cavernous malformation
- Familial colorectal cancer Type X
- Japanese encephalitis
- Lynch syndrome
- Medulloblastoma
- Muenke syndrome
- Non-syndromic bicoronal craniosynostosis
- Pineoblastoma
- X-linked adrenoleukodystrophy
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Intracranial hypertension · Intracranial pressure elevation · Pseudotumor cerebri
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.