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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
Show allHide all

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

Cerebral angiography as a tool for diagnosis and management of idiopathic intracranial hypertension syndrome.
Montoya-Casella A, Vargas-Escamilla WR, Gómez-Martínez A, Herrera-Trujillo A
Clin Imaging 2022 Aug;88:53-58. Epub 2022 May 2 doi: 10.1016/j.clinimag.2022.04.014. PMID: 35643043
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

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
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
Neurological disorders in pregnancy.
Shehata HA, Okosun H
Curr Opin Obstet Gynecol 2004 Apr;16(2):117-22. doi: 10.1097/00001703-200404000-00004. PMID: 15017339
Benign intracranial hypertension.
Weisberg LA
Medicine (Baltimore) 1975 May;54(3):197-207. doi: 10.1097/00005792-197505000-00002. PMID: 1143085

Diagnosis

Treatment of Pseudotumor Cerebri (Sinus Stenosis).
Thanki S, Guerrero W, Mokin M
Neurosurg Clin N Am 2022 Apr;33(2):207-214. Epub 2022 Mar 2 doi: 10.1016/j.nec.2021.11.002. PMID: 35346452
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
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

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
Neurological disorders in pregnancy.
Shehata HA, Okosun H
Curr Opin Obstet Gynecol 2004 Apr;16(2):117-22. doi: 10.1097/00001703-200404000-00004. PMID: 15017339
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
Benign intracranial hypertension (pseudotumor cerebri).
Green M
Pediatr Clin North Am 1967 Nov;14(4):819-30. PMID: 4875464

Prognosis

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
Cerebral venous thrombosis--clinical presentations.
Mehndiratta MM, Garg S, Gurnani M
J Pak Med Assoc 2006 Nov;56(11):513-6. PMID: 17183979
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

Alitretinoin reduces erythema in inherited ichthyosis.
Onnis G, Chiaverini C, Hickman G, Dreyfus I, Fischer J, Bourrat E, Mazereeuw-Hautier J
Orphanet J Rare Dis 2018 Apr 4;13(1):46. doi: 10.1186/s13023-018-0783-9. PMID: 29618363Free PMC Article
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
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

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
Growth hormone for children with chronic kidney disease.
Vimalachandra D, Hodson EM, Willis NS, Craig JC, Cowell C, Knight JF
Cochrane Database Syst Rev 2006 Jul 19;(3):CD003264. doi: 10.1002/14651858.CD003264.pub2. PMID: 16856001

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