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Spontaneous intracranial hypotension

MedGen UID:
155914
Concept ID:
C0751731
Disease or Syndrome
Synonyms: Essential Intracranial Hypotension; Hypotension, Essential Intracranial; Hypotension, Spontaneous Intracranial; Intracranial Hypotension, Essential; Intracranial Hypotension, Spontaneous; Spontaneous Intracranial Hypotension
SNOMED CT: Spontaneous intracranial hypotension (16476681000119105)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018624
Orphanet: ORPHA443180

Definition

A rare headache resulting from a cerebrospinal fluid (CSF) leak with subsequent lowered CSF pressure, characterized clinically by severe headaches which typically worsen upon standing up and get better when lying down. Additional features may include neck stiffness, nausea, vomiting, vertigo, tinnitus, visual disturbances, and cognitive abnormalities, among others, as sagging and displacement of the brain can lead to a variety of lesions and symptoms. [from ORDO]

Professional guidelines

PubMed

Cheema S, Anderson J, Angus-Leppan H, Armstrong P, Butteriss D, Carlton Jones L, Choi D, Chotai A, D'Antona L, Davagnanam I, Davies B, Dorman PJ, Duncan C, Ellis S, Iodice V, Joy C, Lagrata S, Mead S, Morland D, Nissen J, Pople J, Redfern N, Sayal PP, Scoffings D, Secker R, Toma AK, Trevarthen T, Walkden J, Beck J, Kranz PG, Schievink W, Wang SJ, Matharu MS
J Neurol Neurosurg Psychiatry 2023 Oct;94(10):835-843. Epub 2023 May 5 doi: 10.1136/jnnp-2023-331166. PMID: 37147116Free PMC Article
D'Antona L, Jaime Merchan MA, Vassiliou A, Watkins LD, Davagnanam I, Toma AK, Matharu MS
JAMA Neurol 2021 Mar 1;78(3):329-337. doi: 10.1001/jamaneurol.2020.4799. PMID: 33393980Free PMC Article
Kranz PG, Gray L, Malinzak MD, Amrhein TJ
Neuroimaging Clin N Am 2019 Nov;29(4):581-594. Epub 2019 Aug 26 doi: 10.1016/j.nic.2019.07.006. PMID: 31677732

Recent clinical studies

Etiology

Parikh SK
Curr Pain Headache Rep 2024 May;28(5):395-401. Epub 2024 Mar 2 doi: 10.1007/s11916-024-01231-9. PMID: 38430310Free PMC Article
Cheema S, Mehta D, Qureshi A, Sayal P, Kamourieh S, Davagnanam I, Matharu M
Pract Neurol 2024 Mar 19;24(2):98-105. doi: 10.1136/pn-2023-003986. PMID: 38135500Free PMC Article
Cheema S, Anderson J, Angus-Leppan H, Armstrong P, Butteriss D, Carlton Jones L, Choi D, Chotai A, D'Antona L, Davagnanam I, Davies B, Dorman PJ, Duncan C, Ellis S, Iodice V, Joy C, Lagrata S, Mead S, Morland D, Nissen J, Pople J, Redfern N, Sayal PP, Scoffings D, Secker R, Toma AK, Trevarthen T, Walkden J, Beck J, Kranz PG, Schievink W, Wang SJ, Matharu MS
J Neurol Neurosurg Psychiatry 2023 Oct;94(10):835-843. Epub 2023 May 5 doi: 10.1136/jnnp-2023-331166. PMID: 37147116Free PMC Article
Wang SJ
Continuum (Minneap Minn) 2021 Jun 1;27(3):746-766. doi: 10.1212/CON.0000000000000979. PMID: 34048402
Dobrocky T, Grunder L, Breiding PS, Branca M, Limacher A, Mosimann PJ, Mordasini P, Zibold F, Haeni L, Jesse CM, Fung C, Raabe A, Ulrich CT, Gralla J, Beck J, Piechowiak EI
JAMA Neurol 2019 May 1;76(5):580-587. doi: 10.1001/jamaneurol.2018.4921. PMID: 30776059Free PMC Article

Diagnosis

Zetlaoui PJ, Buchheit T, Benhamou D
Anaesth Crit Care Pain Med 2022 Oct;41(5):101138. Epub 2022 Aug 2 doi: 10.1016/j.accpm.2022.101138. PMID: 35952552
Dobrocky T, Nicholson P, Häni L, Mordasini P, Krings T, Brinjikji W, Cutsforth-Gregory JK, Schär R, Schankin C, Gralla J, Pereira VM, Raabe A, Farb R, Beck J, Piechowiak EI
Lancet Neurol 2022 Apr;21(4):369-380. Epub 2022 Feb 25 doi: 10.1016/S1474-4422(21)00423-3. PMID: 35227413
Schievink WI
N Engl J Med 2021 Dec 2;385(23):2173-2178. doi: 10.1056/NEJMra2101561. PMID: 34874632
Luetzen N, Dovi-Akue P, Fung C, Beck J, Urbach H
Neuroradiology 2021 Nov;63(11):1765-1772. Epub 2021 Jul 23 doi: 10.1007/s00234-021-02766-z. PMID: 34297176Free PMC Article
Kranz PG, Gray L, Malinzak MD, Amrhein TJ
Neuroimaging Clin N Am 2019 Nov;29(4):581-594. Epub 2019 Aug 26 doi: 10.1016/j.nic.2019.07.006. PMID: 31677732

Therapy

Zduńska A, Cegielska J, Zduński S, Domitrz I
Nutrients 2023 Jul 17;15(14) doi: 10.3390/nu15143170. PMID: 37513588Free PMC Article
Cheema S, Anderson J, Angus-Leppan H, Armstrong P, Butteriss D, Carlton Jones L, Choi D, Chotai A, D'Antona L, Davagnanam I, Davies B, Dorman PJ, Duncan C, Ellis S, Iodice V, Joy C, Lagrata S, Mead S, Morland D, Nissen J, Pople J, Redfern N, Sayal PP, Scoffings D, Secker R, Toma AK, Trevarthen T, Walkden J, Beck J, Kranz PG, Schievink W, Wang SJ, Matharu MS
J Neurol Neurosurg Psychiatry 2023 Oct;94(10):835-843. Epub 2023 May 5 doi: 10.1136/jnnp-2023-331166. PMID: 37147116Free PMC Article
Zetlaoui PJ, Buchheit T, Benhamou D
Anaesth Crit Care Pain Med 2022 Oct;41(5):101138. Epub 2022 Aug 2 doi: 10.1016/j.accpm.2022.101138. PMID: 35952552
D'Antona L, Jaime Merchan MA, Vassiliou A, Watkins LD, Davagnanam I, Toma AK, Matharu MS
JAMA Neurol 2021 Mar 1;78(3):329-337. doi: 10.1001/jamaneurol.2020.4799. PMID: 33393980Free PMC Article
Kranz PG, Gray L, Malinzak MD, Amrhein TJ
Neuroimaging Clin N Am 2019 Nov;29(4):581-594. Epub 2019 Aug 26 doi: 10.1016/j.nic.2019.07.006. PMID: 31677732

Prognosis

Shahan B, Choi EY, Nieves G
Am Fam Physician 2021 Apr 1;103(7):422-428. PMID: 33788511
Ferrante E, Trimboli M, Rubino F
Acta Neurol Belg 2020 Feb;120(1):9-18. Epub 2019 Jun 18 doi: 10.1007/s13760-019-01166-8. PMID: 31215003
Dobrocky T, Grunder L, Breiding PS, Branca M, Limacher A, Mosimann PJ, Mordasini P, Zibold F, Haeni L, Jesse CM, Fung C, Raabe A, Ulrich CT, Gralla J, Beck J, Piechowiak EI
JAMA Neurol 2019 May 1;76(5):580-587. doi: 10.1001/jamaneurol.2018.4921. PMID: 30776059Free PMC Article
Ozge A, Bolay H
Curr Pain Headache Rep 2014 Jul;18(7):430. doi: 10.1007/s11916-014-0430-7. PMID: 24880802
Dilli E
Curr Neurol Neurosci Rep 2014 Apr;14(4):437. doi: 10.1007/s11910-014-0437-9. PMID: 24643327

Clinical prediction guides

Jagiasi K, Barvalia PP
Neurol India 2022 Nov-Dec;70(6):2422-2426. doi: 10.4103/0028-3886.364052. PMID: 36537427
Babcock JC, Johnson DR, Benson JC, Kim DK, Luetmer PH, Shlapak DP, Cross CP, Johnson MP, Cutsforth-Gregory JK, Carr CM
AJNR Am J Neuroradiol 2022 Jul;43(7):978-983. Epub 2022 Jun 30 doi: 10.3174/ajnr.A7557. PMID: 35772803Free PMC Article
D'Antona L, Jaime Merchan MA, Vassiliou A, Watkins LD, Davagnanam I, Toma AK, Matharu MS
JAMA Neurol 2021 Mar 1;78(3):329-337. doi: 10.1001/jamaneurol.2020.4799. PMID: 33393980Free PMC Article
Dobrocky T, Grunder L, Breiding PS, Branca M, Limacher A, Mosimann PJ, Mordasini P, Zibold F, Haeni L, Jesse CM, Fung C, Raabe A, Ulrich CT, Gralla J, Beck J, Piechowiak EI
JAMA Neurol 2019 May 1;76(5):580-587. doi: 10.1001/jamaneurol.2018.4921. PMID: 30776059Free PMC Article
Hoffmann J, Goadsby PJ
Curr Opin Neurol 2013 Jun;26(3):240-7. doi: 10.1097/WCO.0b013e328360eccc. PMID: 23594732

Recent systematic reviews

Amrhein TJ, Williams JW Jr, Gray L, Malinzak MD, Cantrell S, Deline CR, Carr CM, Kim DK, Goldstein KM, Kranz PG
AJNR Am J Neuroradiol 2023 Jun;44(6):730-739. Epub 2023 May 18 doi: 10.3174/ajnr.A7880. PMID: 37202114Free PMC Article
Cheema S, Anderson J, Angus-Leppan H, Armstrong P, Butteriss D, Carlton Jones L, Choi D, Chotai A, D'Antona L, Davagnanam I, Davies B, Dorman PJ, Duncan C, Ellis S, Iodice V, Joy C, Lagrata S, Mead S, Morland D, Nissen J, Pople J, Redfern N, Sayal PP, Scoffings D, Secker R, Toma AK, Trevarthen T, Walkden J, Beck J, Kranz PG, Schievink W, Wang SJ, Matharu MS
J Neurol Neurosurg Psychiatry 2023 Oct;94(10):835-843. Epub 2023 May 5 doi: 10.1136/jnnp-2023-331166. PMID: 37147116Free PMC Article
Konovalov A, Gadzhiagaev V, Vinogradov E, Nikitin N, Eliava S, Konovalov N
World Neurosurg 2022 May;161:91-96. Epub 2022 Feb 15 doi: 10.1016/j.wneu.2022.02.036. PMID: 35176526
D'Antona L, Jaime Merchan MA, Vassiliou A, Watkins LD, Davagnanam I, Toma AK, Matharu MS
JAMA Neurol 2021 Mar 1;78(3):329-337. doi: 10.1001/jamaneurol.2020.4799. PMID: 33393980Free PMC Article
Lin JP, Zhang SD, He FF, Liu MJ, Ma XX
J Headache Pain 2017 Dec;18(1):4. Epub 2017 Jan 13 doi: 10.1186/s10194-016-0708-8. PMID: 28091819Free PMC Article

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