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Intracranial hypotension

MedGen UID:
141851
•Concept ID:
C0524812
•
Disease or Syndrome
Synonyms: Hypotension, Intracranial; Intracranial Hypotension
SNOMED CT: Intracranial hypotension (433691000124104)
 
Monarch Initiative: MONDO:0006811

Definition

Reduction of cerebrospinal fluid pressure characterized clinically by headache which is maximal in an upright posture and occasionally by an abducens nerve palsy (see abducens nerve diseases), neck stiffness, hearing loss (see deafness); nausea; and other symptoms. This condition may be spontaneous or secondary to spinal puncture; neurosurgical procedures; dehydration; uremia; trauma (see also craniocerebral trauma); and other processes. Chronic hypotension may be associated with subdural hematomas (see hematoma, subdural) or hygromas. (From Semin Neurol 1996 Mar;16(1):5-10; Adams et al., Principles of Neurology, 6th ed, pp637-8) [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIntracranial hypotension

Professional guidelines

PubMed

Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.
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
Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome: A Systematic Review and Meta-analysis.
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
Spontaneous Intracranial Hypotension: Pathogenesis, Diagnosis, and Treatment.
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

Spontaneous intracranial hypotension.
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
Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.
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
Spontaneous Intracranial Hypotension.
Wang SJ
Continuum (Minneap Minn) 2021 Jun 1;27(3):746-766. doi: 10.1212/CON.0000000000000979. PMID: 34048402
Headache Emergencies.
Kopel D, Peeler C, Zhu S
Neurol Clin 2021 May;39(2):355-372. doi: 10.1016/j.ncl.2021.01.003. PMID: 33896523
Assessing Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension With a Scoring System Based on Brain Magnetic Resonance Imaging Findings.
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

Epidural blood patch: A narrative review.
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
Spontaneous intracranial hypotension: searching for the CSF leak.
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
Spontaneous Intracranial Hypotension.
Schievink WI
N Engl J Med 2021 Dec 2;385(23):2173-2178. doi: 10.1056/NEJMra2101561. PMID: 34874632
Spontaneous intracranial hypotension: diagnostic and therapeutic workup.
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
Spontaneous Intracranial Hypotension: Pathogenesis, Diagnosis, and Treatment.
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

Spontaneous intracranial hypotension.
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
Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.
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
Epidural blood patch: A narrative review.
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
Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome: A Systematic Review and Meta-analysis.
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
Spontaneous Intracranial Hypotension: Pathogenesis, Diagnosis, and Treatment.
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

Cerebrospinal Fluid Analysis.
Shahan B, Choi EY, Nieves G
Am Fam Physician 2021 Apr 1;103(7):422-428. PMID: 33788511
Spontaneous intracranial hypotension: review and expert opinion.
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
Assessing Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension With a Scoring System Based on Brain Magnetic Resonance Imaging Findings.
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
Intracranial hypotension and hypertension in children and adolescents.
Ozge A, Bolay H
Curr Pain Headache Rep 2014 Jul;18(7):430. doi: 10.1007/s11916-014-0430-7. PMID: 24880802
Thunderclap headache.
Dilli E
Curr Neurol Neurosci Rep 2014 Apr;14(4):437. doi: 10.1007/s11910-014-0437-9. PMID: 24643327

Clinical prediction guides

Diffuse Calvarial Hyperostosis and Spontaneous Intracranial Hypotension: A Case-Control Study.
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
Parkinsonism and cerebrospinal fluid disorders.
Youn J, Todisco M, Zappia M, Pacchetti C, Fasano A
J Neurol Sci 2022 Feb 15;433:120019. Epub 2021 Oct 1 doi: 10.1016/j.jns.2021.120019. PMID: 34674853
Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome: A Systematic Review and Meta-analysis.
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
Assessing Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension With a Scoring System Based on Brain Magnetic Resonance Imaging Findings.
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
Update on intracranial hypertension and hypotension.
Hoffmann J, Goadsby PJ
Curr Opin Neurol 2013 Jun;26(3):240-7. doi: 10.1097/WCO.0b013e328360eccc. PMID: 23594732

Recent systematic reviews

Efficacy of Epidural Blood Patching or Surgery in Spontaneous Intracranial Hypotension: A Systematic Review and Evidence Map.
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
Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.
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
Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome: A Systematic Review and Meta-analysis.
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
The status of diagnosis and treatment to intracranial hypotension, including SIH.
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
A systematic literature review of intracranial hypotension following chiropractic.
Tuchin P
Int J Clin Pract 2014 Mar;68(3):396-402. Epub 2013 Dec 22 doi: 10.1111/ijcp.12247. PMID: 24372942

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