. 2023 Oct;94(10):835-843.

doi: 10.1136/jnnp-2023-331166. Epub 2023 May 5.

Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension

Sanjay Cheema^{1

2}, Jane Anderson³, Heather Angus-Leppan⁴, Paul Armstrong⁵, David Butteriss⁶, Lalani Carlton Jones^{7

8}, David Choi^{1

9}, Amar Chotai⁶, Linda D'Antona^{1

9}, Indran Davagnanam^{1

10}, Brendan Davies¹¹, Paul J Dorman¹², Callum Duncan¹³, Simon Ellis¹¹, Valeria Iodice^{1

14}, Clare Joy¹⁵, Susie Lagrata², Sarah Mead¹⁵, Danny Morland¹⁶, Justin Nissen¹⁷, Jenny Pople¹⁵, Nancy Redfern¹⁶, Parag P Sayal⁹, Daniel Scoffings¹⁸, Russell Secker¹⁵, Ahmed K Toma^{1

9}, Tamsin Trevarthen¹⁵, James Walkden¹⁹, Jürgen Beck²⁰, Peter George Kranz²¹, Wouter Schievink²², Shuu-Jiun Wang^{23

24}, Manjit Singh Matharu^{25

2}

Affiliations

¹ Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London, UK.
² Headache and Facial Pain Group, National Hospital for Neurology and Neurosurgery, London, UK.
³ Neurology Department, Addenbrooke's Hospital, Cambridge, UK.
⁴ Neurology Department, Royal Free London NHS Foundation Trust, London, UK.
⁵ Neuroradiology Department, Institute of Neurological Sciences, Glasgow, UK.
⁶ Department of Neuroradiology, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
⁷ Neuroradiology Department, Guy's and St Thomas' Hospitals NHS Trust, London, UK.
⁸ Neuroradiology Department, King's College Hospital NHS Foundation Trust, London, UK.
⁹ Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, London, UK.
¹⁰ Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, UK.
¹¹ Neurology Department, University Hospitals of North Midlands NHS Trust, Stoke-on-Trent, UK.
¹² Department of Neurology, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
¹³ Department of Neurology, NHS Grampian, Aberdeen, UK.
¹⁴ Autonomic Unit, National Hospital for Neurology and Neurosurgery, London, UK.
¹⁵ CSF Leak Association, Strathpeffer, UK.
¹⁶ Department of Anaesthesia, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
¹⁷ Neurosurgery Department, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
¹⁸ Department of Radiology, Addenbrooke's Hospital, Cambridge, UK.
¹⁹ Neurosurgery Department, NHS Grampian, Aberdeen, UK.
²⁰ Department of Neurosurgery, Medical Center-University of Freiburg, Freiburg, Germany.
²¹ Department of Radiology, Duke University Medical Center, Durham, North Carolina, USA.
²² Neurosurgery Department, Cedars-Sinai Medical Center, Los Angeles, California, USA.
²³ Neurology Department, Taipei Veterans General Hospital, Taipei, Taiwan.
²⁴ Brain Research Center, National Yang Ming Chiao Tung University School of Medicine, Taipei, Taiwan.
²⁵ Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London, UK manjit.matharu@ucl.ac.uk.

PMID: 37147116
PMCID: PMC10511987
DOI: 10.1136/jnnp-2023-331166

Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension

Sanjay Cheema et al. J Neurol Neurosurg Psychiatry. 2023 Oct.

. 2023 Oct;94(10):835-843.

doi: 10.1136/jnnp-2023-331166. Epub 2023 May 5.

Authors

Affiliations

¹ Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London, UK.
² Headache and Facial Pain Group, National Hospital for Neurology and Neurosurgery, London, UK.
³ Neurology Department, Addenbrooke's Hospital, Cambridge, UK.
⁴ Neurology Department, Royal Free London NHS Foundation Trust, London, UK.
⁵ Neuroradiology Department, Institute of Neurological Sciences, Glasgow, UK.
⁶ Department of Neuroradiology, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
⁷ Neuroradiology Department, Guy's and St Thomas' Hospitals NHS Trust, London, UK.
⁸ Neuroradiology Department, King's College Hospital NHS Foundation Trust, London, UK.
⁹ Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, London, UK.
¹⁰ Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, UK.
¹¹ Neurology Department, University Hospitals of North Midlands NHS Trust, Stoke-on-Trent, UK.
¹² Department of Neurology, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
¹³ Department of Neurology, NHS Grampian, Aberdeen, UK.
¹⁴ Autonomic Unit, National Hospital for Neurology and Neurosurgery, London, UK.
¹⁵ CSF Leak Association, Strathpeffer, UK.
¹⁶ Department of Anaesthesia, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
¹⁷ Neurosurgery Department, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
¹⁸ Department of Radiology, Addenbrooke's Hospital, Cambridge, UK.
¹⁹ Neurosurgery Department, NHS Grampian, Aberdeen, UK.
²⁰ Department of Neurosurgery, Medical Center-University of Freiburg, Freiburg, Germany.
²¹ Department of Radiology, Duke University Medical Center, Durham, North Carolina, USA.
²² Neurosurgery Department, Cedars-Sinai Medical Center, Los Angeles, California, USA.
²³ Neurology Department, Taipei Veterans General Hospital, Taipei, Taiwan.
²⁴ Brain Research Center, National Yang Ming Chiao Tung University School of Medicine, Taipei, Taiwan.
²⁵ Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London, UK manjit.matharu@ucl.ac.uk.

PMID: 37147116
PMCID: PMC10511987
DOI: 10.1136/jnnp-2023-331166

Abstract

Background: We aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG).

Methods: A 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH.

Results: SIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided.

Conclusions: This multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH.

Keywords: CSF dynamics; headache; interventional; neuroradiology; neurosurgery.

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Conflict of interest statement

Competing interests: JA: remuneration for consultancy advice and education provision from Allergan/AbbVie and TEVA. HA-L: lectures and education paid by International Medical Press, Sanofi and Eisai. LCJ: lecture fees received from Radiopaedia. SC: research fellowship sponsored by Abbott. LD'A: supported by an NIHR Academic Clinical Fellowship and was the recipient of a research fellowship sponsored by B Braun. BD: remuneration for consultancy advice and education provision from TEVA, Allergan and Lilly. PJD: shareholding in BMS, Regeneron and Ionis Pharma. SE: owns the North Midlands Neurosciences. VI: reports speaker fees and honoraria from Theravance Biopharma and Jensen, outside of the present work; supported by the National Institute for Health and Care Research University College London Hospitals Biomedical Research Centre. SL: received fees for attending advisory meetings, presentations and preparing presentation materials from Allergan, TEVA, Eli Lilly and Novartis. MSM: chair of the medical advisory board of the CSF Leak Association, serves on the advisory board for Abbott, Allergan, Novartis, Eli Lilly, Medtronic, Autonomic Technologies and TEVA, and has received payment for the development of educational presentations from Allergan, electroCore, Eli Lilly, Novartis and TEVA. CJ, SM, JP, RS, TT: members of CSF Leak Association. S-JW: received honoraria as a moderator from AbbVie, Pfizer, Eli Lilly and Biogen, and has been the PI in trials sponsored by AbbVie, Novartis and Lundbeck. He has received research grants from the Taiwan Minister of Technology and Science (MOST), Brain Research Center, National Yang Ming Chiao Tung University from The Featured Areas Research Center Program within the framework of the Higher Education Sprout Project by the Ministry of Education (MOE) in Taiwan, Taipei Veterans General Hospital, Taiwan Headache Society and Taiwan branches of Eli Lilly and Novartis.

Figures

**Figure 1**
Flow diagram of guideline development process. SIG, specialist interest group.

**Figure 2**
Typical MRI findings of spontaneous intracranial hypotension (SIH). (A) Sagittal T1 image showing enlargement of the pituitary, decreased mamillopontine distance, sagging of the brainstem and cerebellar tonsillar descent. (B) Axial T1 postcontrast image showing diffuse smooth dural thickening and pachymeningeal contrast enhancement. (C) Coronal T2 image showing distension of the dural venous sinuses. (D) Sagittal T2 image showing extensive ventral spinal longitudinal epidural collection (SLEC) extending from the upper cervical to thoracic regions. (E) Axial T2 image showing ventral SLEC.

**Figure 3**
Algorithm for MRI-positive patients. This algorithm is designed to show the recommended pathway for most patients rather than capture every single possible situation which may occur in the management of a patient with spontaneous intracranial hypotension (SIH). CSF, cerebrospinal fluid; CTM, CT myelography; CVF, CSF-venous fistula; DSM, digital subtraction myelography; EBP, epidural blood patch; LD-CTM, lateral decubitus CT myelography; LD-DSM, lateral decubitus digital subtraction myelography; MDT, multidisciplinary team; SLEC, spinal longitudinal epidural collection; UFCTM, ultrafast CT myelography.

**Figure 4**
Algorithm for MRI-negative patients. CSF, cerebrospinal fluid; CVF, CSF-venous fistula; EBP, epidural blood patch; LD-CTM, lateral decubitus CT myelography; LD-DSM, lateral decubitus digital subtraction myelography; MDT, multidisciplinary team; SIH, spontaneous intracranial hypotension.

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Comment in

Comment on: multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.
Esposito A, Ugga L, Tedeschi E, Iodice R, Moccia M. Esposito A, et al. J Neurol Neurosurg Psychiatry. 2024 Jan 11;95(2):196-197. doi: 10.1136/jnnp-2023-331981. J Neurol Neurosurg Psychiatry. 2024. PMID: 37591690 No abstract available.

References

1. Schievink WI, Maya MM, Jean-Pierre S, et al. A classification system of spontaneous spinal CSF leaks. Neurology 2016;87:673–9. 10.1212/WNL.0000000000002986 - DOI - PubMed
1. Schievink WI, Maya MM, Moser FG, et al. Incidence of spontaneous intracranial hypotension in a community: Beverly Hills, California, 2006-2020. Cephalalgia 2022;42:312–6. 10.1177/03331024211048510 - DOI - PubMed
1. D’Antona L, Jaime Merchan MA, Vassiliou A, et al. Clinical presentation, investigation findings, and treatment outcomes of spontaneous intracranial hypotension syndrome: a systematic review and meta-analysis. JAMA Neurol 2021;78:329–37. 10.1001/jamaneurol.2020.4799 - DOI - PMC - PubMed
1. Schievink WI. Spontaneous intracranial hypotension. N Engl J Med 2021;385:2173–8. 10.1056/NEJMra2101561 - DOI - PubMed
1. Wang SJ. Spontaneous intracranial hypotension. CONTINUUM 2021;27:746–66. 10.1212/CON.0000000000000979 - DOI - PubMed

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Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension

Affiliations

Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension

Authors

Affiliations

Abstract

Conflict of interest statement

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