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Benign intracranial hypertension

MedGen UID:
18732
•Concept ID:
C0033845
•
Disease or Syndrome
Synonyms: Idiopathic intracranial hypertension; Pseudotumor Cerebri
SNOMED CT: Benign intracranial hypertension (68267002); Pseudotumor cerebri (68267002); Nonne's syndrome (68267002); Otitic hydrocephalus syndrome (68267002); Noninfective serous meningitis (68267002); BIH - Benign intracranial hypertension (68267002); IIH - Idiopathic intracranial hypertension (68267002); Idiopathic intracranial hypertension (68267002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0009468
OMIM®: 243200
Orphanet: ORPHA238624

Definition

Idiopathic intracranial hypertension is a neurological disorder characterized by isolated increased intracranial pressure manifesting with recurrent and persistent headaches, nausea, vomiting, progressive and transient obstruction of the visual field, papilledema. Visual loss can be irreversible. [from ORDO]

Clinical features

From HPO
Hypertensive disorder
MedGen UID:
6969
•Concept ID:
C0020538
•
Disease or Syndrome
The presence of chronic increased pressure in the systemic arterial system.
See: Feature record | Search on this feature
Increased intracranial pressure
MedGen UID:
56241
•Concept ID:
C0151740
•
Finding
An increase of the pressure inside the cranium (skull) and thereby in the brain tissue and cerebrospinal fluid.
See: Feature record | Search on this feature
Optic papillitis
MedGen UID:
10565
•Concept ID:
C0030353
•
Finding
Papilledema refers to edema (swelling) of the optic disc secondary to any factor which increases cerebral spinal fluid pressure.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBenign intracranial hypertension
Follow this link to review classifications for Benign intracranial hypertension in Orphanet.

Professional guidelines

PubMed

An update on the management of pseudotumor cerebri.
Galgano MA, Deshaies EM
Clin Neurol Neurosurg 2013 Mar;115(3):252-9. Epub 2012 Dec 21 doi: 10.1016/j.clineuro.2012.11.018. PMID: 23265564
Management of idiopathic intracranial hypertension in parturients: anesthetic considerations.
Karmaniolou I, Petropoulos G, Theodoraki K
Can J Anaesth 2011 Jul;58(7):650. Epub 2011 Apr 26 doi: 10.1007/s12630-011-9508-4. PMID: 21519980
Otologic manifestations of benign intracranial hypertension syndrome: diagnosis and management.
Sismanis A
Laryngoscope 1987 Aug;97(S42):1-17. doi: 10.1288/00005537-198708001-00001. PMID: 3302575

Recent clinical studies

Etiology

Idiopathic intracranial hypertension: Contemporary review and implications for the otolaryngologist.
Stevens SM, Rizk HG, Golnik K, Andaluz N, Samy RN, Meyer TA, Lambert PR
Laryngoscope 2018 Jan;128(1):248-256. Epub 2017 Mar 27 doi: 10.1002/lary.26581. PMID: 28349571
Idiopathic intracranial hypertension.
Wakerley BR, Tan MH, Ting EY
Cephalalgia 2015 Mar;35(3):248-61. Epub 2014 May 20 doi: 10.1177/0333102414534329. PMID: 24847166
Headache caused by raised intracranial pressure and intracranial hypotension.
Ramadan NM
Curr Opin Neurol 1996 Jun;9(3):214-8. doi: 10.1097/00019052-199606000-00011. PMID: 8839614
Benign intracranial hypertension.
Cody CM
Am Fam Physician 1992 Apr;45(4):1671-8. PMID: 1558043
Benign intracranial hypertension.
Weisberg LA
Medicine (Baltimore) 1975 May;54(3):197-207. doi: 10.1097/00005792-197505000-00002. PMID: 1143085

Diagnosis

An Up to Date Review of Pseudotumor Cerebri Syndrome.
Burkett JG, Ailani J
Curr Neurol Neurosci Rep 2018 May 2;18(6):33. doi: 10.1007/s11910-018-0839-1. PMID: 29721718
Idiopathic intracranial hypertension.
Wakerley BR, Tan MH, Ting EY
Cephalalgia 2015 Mar;35(3):248-61. Epub 2014 May 20 doi: 10.1177/0333102414534329. PMID: 24847166
Pulsatile tinnitus.
Liyanage SH, Singh A, Savundra P, Kalan A
J Laryngol Otol 2006 Feb;120(2):93-7. Epub 2005 Nov 25 doi: 10.1017/S0022215105001714. PMID: 16359136
Pseudotumor cerebri.
Bandyopadhyay S
Arch Neurol 2001 Oct;58(10):1699-701. doi: 10.1001/archneur.58.10.1699. PMID: 11594936
Benign intracranial hypertension.
Lancet 1976 Nov 6;2(7993):1007. PMID: 62221

Therapy

Idiopathic intracranial hypertension.
Wakerley BR, Tan MH, Ting EY
Cephalalgia 2015 Mar;35(3):248-61. Epub 2014 May 20 doi: 10.1177/0333102414534329. PMID: 24847166
Not so benign intracranial hypertension.
Digre KB
BMJ 2003 Mar 22;326(7390):613-4. doi: 10.1136/bmj.326.7390.613. PMID: 12649214Free PMC Article
Pseudotumor cerebri.
Bandyopadhyay S
Arch Neurol 2001 Oct;58(10):1699-701. doi: 10.1001/archneur.58.10.1699. PMID: 11594936
Lithium-induced headache.
Alvarez-Cermeño JC, Fernández JM, O'Neill A, Moral L, Saiz-Ruiz J
Headache 1989 Apr;29(4):246-7. doi: 10.1111/j.1526-4610.1989.hed22904246.x. PMID: 2497083
Benign intracranial hypertension (pseudotumor cerebri).
Green M
Pediatr Clin North Am 1967 Nov;14(4):819-30. PMID: 4875464

Prognosis

Intracranial Hypertension in Children: Etiologies, Clinical Features, and Outcome.
Masri A, Jaafar A, Noman R, Gharaibeh A, Ababneh OH
J Child Neurol 2015 Oct;30(12):1562-8. Epub 2015 Mar 11 doi: 10.1177/0883073815574332. PMID: 25762586
Association of benign intracranial hypertension and spontaneous encephalocele with cerebrospinal fluid leak.
Brainard L, Chen DA, Aziz KM, Hillman TA
Otol Neurotol 2012 Dec;33(9):1621-4. doi: 10.1097/MAO.0b013e318271c312. PMID: 23150096
Benign intracranial hypertension.
Cody CM
Am Fam Physician 1992 Apr;45(4):1671-8. PMID: 1558043
Benign intracranial hypertension.
Weisberg LA
Medicine (Baltimore) 1975 May;54(3):197-207. doi: 10.1097/00005792-197505000-00002. PMID: 1143085
Benign intracranial hypertension (pseudotumor cerebri).
Green M
Pediatr Clin North Am 1967 Nov;14(4):819-30. PMID: 4875464

Clinical prediction guides

Cortical astrogliosis and increased perivascular aquaporin-4 in idiopathic intracranial hypertension.
Eide PK, Eidsvaag VA, Nagelhus EA, Hansson HA
Brain Res 2016 Aug 1;1644:161-75. Epub 2016 May 14 doi: 10.1016/j.brainres.2016.05.024. PMID: 27188961
Cerebral venous thrombosis--clinical presentations.
Mehndiratta MM, Garg S, Gurnani M
J Pak Med Assoc 2006 Nov;56(11):513-6. PMID: 17183979
Monitoring and interpretation of intracranial pressure.
Czosnyka M, Pickard JD
J Neurol Neurosurg Psychiatry 2004 Jun;75(6):813-21. doi: 10.1136/jnnp.2003.033126. PMID: 15145991Free PMC Article
Lithium-induced headache.
Alvarez-Cermeño JC, Fernández JM, O'Neill A, Moral L, Saiz-Ruiz J
Headache 1989 Apr;29(4):246-7. doi: 10.1111/j.1526-4610.1989.hed22904246.x. PMID: 2497083
Benign intracranial hypertension.
Weisberg LA
Medicine (Baltimore) 1975 May;54(3):197-207. doi: 10.1097/00005792-197505000-00002. PMID: 1143085

Recent systematic reviews

Effectiveness of optic nerve sheath fenestration in preserving vision in idiopathic intracranial hypertension: an updated meta-analysis and systematic review.
Prokop K, Opęchowska A, Sieśkiewicz A, Lisowski Ł, Mariak Z, Łysoń T
Acta Neurochir (Wien) 2024 Nov 25;166(1):476. doi: 10.1007/s00701-024-06345-y. PMID: 39585430Free PMC Article
Idiopathic intracranial hypertension: Proposal of a stratification strategy for monitoring risk of disease progression.
Onyia CU, Ogunbameru IO, Dada OA, Owagbemi OF, Ige-Orhionkpaibima FS, Olarewaju OA, Komolafe EO
Clin Neurol Neurosurg 2019 Apr;179:35-41. Epub 2019 Feb 15 doi: 10.1016/j.clineuro.2019.02.013. PMID: 30818126
Bariatric Surgery or Non-surgical Weight Loss for Idiopathic Intracranial Hypertension? A Systematic Review and Comparison of Meta-analyses.
Manfield JH, Yu KK, Efthimiou E, Darzi A, Athanasiou T, Ashrafian H
Obes Surg 2017 Feb;27(2):513-521. doi: 10.1007/s11695-016-2467-7. PMID: 27981458Free PMC Article
Bariatric surgery as a treatment for idiopathic intracranial hypertension: a systematic review.
Handley JD, Baruah BP, Williams DM, Horner M, Barry J, Stephens JW
Surg Obes Relat Dis 2015 Nov-Dec;11(6):1396-403. Epub 2015 Aug 12 doi: 10.1016/j.soard.2015.08.497. PMID: 26499350
Growth hormone for children with chronic kidney disease.
Hodson EM, Willis NS, Craig JC
Cochrane Database Syst Rev 2012 Feb 15;2012(2):CD003264. doi: 10.1002/14651858.CD003264.pub3. PMID: 22336787Free PMC Article

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