The Freiburg Registry on SpontanEous IntercrAnial Hypotension (SIH) & Post-duraL Puncture Headache (PDPH)

NCT06711731 | Recruiting

• 1 other identifier: 24-1357-S1
• Study Type: observational
• Target: 2,000
• Locations: 1 country
• Sites: 1

Brief Summary

Spinal CSF leaks are considered as rare disease. They cause a variety of symptoms, mainly culminating in a chronic headache syndrome. Crucially, yet often disregarded, the disease holds the potential for cure. The multitude of symptoms, and their inconsistency over time are just two of many challenges preventing timely diagnosis and treatment in many patients. Spinal CSF leaks can occur after intentional or accidental dural puncture (post-dural puncture headache - PDPH) or spontaneously (spontaneous intracranial hypotension - SIH). Awareness is steadily increasing with simultaneous increase of recognized patients. Yet, research and diagnostic is mainly provided by few specialized centers, as e.g. Freiburg. Thus, many observations point towards a large non-diagnosed and non-recognized number of patients, most likely being misdiagnosed and mistreated. Objective: The aim of the registry is to collect structured information on the frequency, cause, symptoms, diagnostic procedures, treatment options and long-term outcome. With the help of the registry, we would like to contribute to a better understanding and treatment of the diseases. Methods: Prospective, longitudinal registry on patients with suspected SIH or PDPH, including data on demographics, clinical presentation, diagnostic findings, treatment, at treatment outcome.

Conditions

CerebroSpinal Fluid (CSF) Leak

Keywords

SIH; PDPH; Spinal leaks; CSF leak; SLEC; Spontaneouse Intercranial Hypotension; CSF-venous fistula; Post Dural Puncture Headache; Cerebrospinal Fluid Leak; Rare disease

Outcome Measures

Primary Outcomes (1)

• Primary Diagnosis given after primary workup (yes/no)

  * SIH (fulfilling ICHD-3 criteria) - yes/no * atypical SIH - yes/on * Asymptomatic SIH (positive imaging signs only) - yes/no * PDPH (fulfilling ICHD-3-criteria) - yes/no * persistentPDPH - yes/no

  up to 4 weeks after inclusion

Secondary Outcomes (103)

• SIH/PDPH - Experience of the local team

  at time of inclusion

• SIH/PDPH - Patients' demographics

  at time of inclusion

• SIH - Previously received treatment at time of inclusion (yes/no)

  at time of inclusion

• SIH/PDPH - age

  at time of inclusion

• SIH/PDPH - sex

  at time of inclusion

Other Outcomes (27)

• SIH/PDPH - Follow up-Reported adverse events related to treatment (yes/no)

  4 weeks and 3 month after treatment

• SIH/PDPH - Follow up-Duration of treatment for rebound hypertension after therapy of the CSF leak

  4 weeks after treatment

• SIH/PDPH - Follow up-Average dosage of acetazolamide per day

  4 weeks after treatment

Study Arms (1)

spinal CSF leak

Patients with suspected spinal CSF leak based on one of the following: 1. History of new orthostatic symptoms with or without prior spinal procedure 2. Imaging suggestive for spinal CSF leak

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The registry is an explorative study on patients suffering from a rare disease - therefor it is aimed to include all eligible patients for a time-limit of 10 years. All patients with suspected spinal CSF leak will be approached and informed about the registry. In case of legal incapacity, the caregivers will be approached. The inclusion of this vulnerable group is considerable as they represent a small but very important subgroup of patients suffering from demntia stemming from a traetable cause.

You may qualify if:

• Patients with suspected spinal CSF leak based on one of the following
• History of new orthostatic symptomes with or without prior spinal procedure
• Imaging suggestive for spinal CSF leak
• Informed consent

You may not qualify if:

• a) Symptoms beeing conclusively explained by another known diagnosis

Sponsors & Collaborators

• University Hospital Freiburg: lead

Study Sites (1)

Medical Center - University of Freiburg, Germany

Freiburg im Breisgau, 79106, Germany

RECRUITING

Related Publications (25)

• Schievink WI, Maya MM, Moser FG, Simon P, Nuno M. Incidence of spontaneous intracranial hypotension in a community: Beverly Hills, California, 2006-2020. Cephalalgia. 2022 Apr;42(4-5):312-316. doi: 10.1177/03331024211048510. Epub 2021 Sep 23.

  PMID: 34553617 BACKGROUND

• Lashkarivand A, Eide PK. Brain Sagging Dementia-Diagnosis, Treatment, and Outcome: A Review. Neurology. 2022 May 10;98(19):798-805. doi: 10.1212/WNL.0000000000200511. Epub 2022 Mar 25.

  PMID: 35338080 BACKGROUND

• Schievink WI, Maya M, Barnard Z, Tache RB, Prasad RS, Wadhwa VS, Moser FG, Nuno M. The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities. Alzheimers Dement (N Y). 2022 Dec 18;8(1):e12367. doi: 10.1002/trc2.12367. eCollection 2022.

  PMID: 36544987 BACKGROUND

• Schievink WI, Maya MM, Harris J, Galvan J, Tache RB, Nuno M. Infratentorial Superficial Siderosis and Spontaneous Intracranial Hypotension. Ann Neurol. 2023 Jan;93(1):64-75. doi: 10.1002/ana.26521. Epub 2022 Oct 22.

  PMID: 36200700 BACKGROUND

• Sprave T, Gkika E, Verma V, Grosu AL, Stoian R. Patient reported outcomes based on EQ-5D-5L questionnaires in head and neck cancer patients: a real-world study. BMC Cancer. 2022 Nov 29;22(1):1236. doi: 10.1186/s12885-022-10346-4.

  PMID: 36447175 BACKGROUND

• Cella D, Grunwald V, Escudier B, Hammers HJ, George S, Nathan P, Grimm MO, Rini BI, Doan J, Ivanescu C, Paty J, Mekan S, Motzer RJ. Patient-reported outcomes of patients with advanced renal cell carcinoma treated with nivolumab plus ipilimumab versus sunitinib (CheckMate 214): a randomised, phase 3 trial. Lancet Oncol. 2019 Feb;20(2):297-310. doi: 10.1016/S1470-2045(18)30778-2. Epub 2019 Jan 15.

  PMID: 30658932 BACKGROUND

• Niraj G, Mushambi M, Gauthama P, Patil A, Kelkar A, Hart E, Nurmikko T; Accidental Dural Puncture Outcome Study Collaborative Group. Persistent headache and low back pain after accidental dural puncture in the obstetric population: a prospective, observational, multicentre cohort study. Anaesthesia. 2021 Aug;76(8):1068-1076. doi: 10.1111/anae.15491. Epub 2021 Apr 23.

  PMID: 33891312 BACKGROUND

• Ranganathan P, Golfeiz C, Phelps AL, Singh S, Shnol H, Paul N, Attaallah AF, Vallejo MC. Chronic headache and backache are long-term sequelae of unintentional dural puncture in the obstetric population. J Clin Anesth. 2015 May;27(3):201-6. doi: 10.1016/j.jclinane.2014.07.008. Epub 2014 Dec 4.

  PMID: 25483233 BACKGROUND

• MacArthur C, Lewis M, Knox EG. Accidental dural puncture in obstetric patients and long term symptoms. BMJ. 1993 Apr 3;306(6882):883-5. doi: 10.1136/bmj.306.6882.883.

  PMID: 8490410 BACKGROUND

• Piechowiak EI, Aeschimann B, Hani L, Kaesmacher J, Mordasini P, Jesse CM, Schankin CJ, Raabe A, Schar RT, Gralla J, Beck J, Dobrocky T. Epidural Blood Patching in Spontaneous Intracranial Hypotension-Do we Really Seal the Leak? Clin Neuroradiol. 2023 Mar;33(1):211-218. doi: 10.1007/s00062-022-01205-7. Epub 2022 Aug 26.

  PMID: 36028627 BACKGROUND

• Beck J, Hubbe U, Klingler JH, Roelz R, Kraus LM, Volz F, Lutzen N, Urbach H, Kieselbach K, Fung C. Minimally invasive surgery for spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension. J Neurosurg Spine. 2022 Sep 9;38(1):147-152. doi: 10.3171/2022.7.SPINE2252. Print 2023 Jan 1.

  PMID: 36087332 BACKGROUND

• Carlton Jones L, Butteriss D, Scoffings D. Spontaneous intracranial hypotension: the role of radiology in diagnosis and management. Clin Radiol. 2022 Mar;77(3):e181-e194. doi: 10.1016/j.crad.2021.11.007. Epub 2021 Dec 20.

  PMID: 34949452 BACKGROUND

• 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. Assessing Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension With a Scoring System Based on Brain Magnetic Resonance Imaging Findings. JAMA Neurol. 2019 May 1;76(5):580-587. doi: 10.1001/jamaneurol.2018.4921.

  PMID: 30776059 BACKGROUND

• Pradeep A, Madhavan AA, Brinjikji W, Cutsforth-Gregory JK. Incidence of spontaneous intracranial hypotension in Olmsted County, Minnesota: 2019-2021. Interv Neuroradiol. 2025 Jun;31(3):364-368. doi: 10.1177/15910199231165429. Epub 2023 Mar 22.

  PMID: 36945844 BACKGROUND

• Farb RI, Nicholson PJ, Peng PW, Massicotte EM, Lay C, Krings T, terBrugge KG. Spontaneous Intracranial Hypotension: A Systematic Imaging Approach for CSF Leak Localization and Management Based on MRI and Digital Subtraction Myelography. AJNR Am J Neuroradiol. 2019 Apr;40(4):745-753. doi: 10.3174/ajnr.A6016. Epub 2019 Mar 28.

  PMID: 30923083 BACKGROUND

• Hani L, Fung C, El Rahal A, Volz F, Kraus LM, Schnell O, Ferrarese R, Erny D, Schwabenland M, Urbach H, Lutzen N, Beck J. Distinct Pattern of Membrane Formation With Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension. Oper Neurosurg. 2024 Jan 1;26(1):71-77. doi: 10.1227/ons.0000000000000914. Epub 2023 Sep 25.

  PMID: 37747369 BACKGROUND

• Uppal V, Russell R, Sondekoppam RV, Ansari J, Baber Z, Chen Y, DelPizzo K, Dirzu DS, Kalagara H, Kissoon NR, Kranz PG, Leffert L, Lim G, Lobo C, Lucas DN, Moka E, Rodriguez SE, Sehmbi H, Vallejo MC, Volk T, Narouze S. Evidence-based clinical practice guidelines on postdural puncture headache: a consensus report from a multisociety international working group. Reg Anesth Pain Med. 2024 Jul 8;49(7):471-501. doi: 10.1136/rapm-2023-104817.

  PMID: 37582578 BACKGROUND

• Jesse CM, Hani L, Fung C, Ulrich CT, Schar RT, Dobrocky T, Piechowiak EI, Goldberg J, Schankin C, Sintonen H, Beck J, Raabe A. The impact of spontaneous intracranial hypotension on social life and health-related quality of life. J Neurol. 2022 Oct;269(10):5466-5473. doi: 10.1007/s00415-022-11207-7. Epub 2022 Jun 14.

  PMID: 35701531 BACKGROUND

• Beck J, Ulrich CT, Fung C, Fichtner J, Seidel K, Fiechter M, Hsieh K, Murek M, Bervini D, Meier N, Mono ML, Mordasini P, Hewer E, Z'Graggen WJ, Gralla J, Raabe A. Diskogenic microspurs as a major cause of intractable spontaneous intracranial hypotension. Neurology. 2016 Sep 20;87(12):1220-6. doi: 10.1212/WNL.0000000000003122. Epub 2016 Aug 26.

  PMID: 27566748 BACKGROUND

• Kilic K, Korsbaek JJ, Jensen RH, Cvetkovic VV. Diagnosis of idiopathic intracranial hypertension - the importance of excluding secondary causes: A systematic review. Cephalalgia. 2022 May;42(6):524-541. doi: 10.1177/03331024211056580. Epub 2021 Nov 25.

  PMID: 34822742 BACKGROUND

• Ducros A, Biousse V. Headache arising from idiopathic changes in CSF pressure. Lancet Neurol. 2015 Jun;14(6):655-68. doi: 10.1016/S1474-4422(15)00015-0.

  PMID: 25987284 BACKGROUND

• Cheema S, Anderson J, Duncan C, Davagnanam I, Armstrong P, Redfern N, Ordman A, D'Antona L, Nissen J, Sayal P, Vaughan-Huxley E, Lagrata S, Iodice V, Snape-Burns J, Joy C, Matharu M. Survey of healthcare professionals' knowledge, attitudes and practices regarding spontaneous intracranial hypotension. BMJ Neurol Open. 2022 Sep 5;4(2):e000347. doi: 10.1136/bmjno-2022-000347. eCollection 2022.

  PMID: 36110926 BACKGROUND

• Cheema S, Joy C, Pople J, Snape-Burns J, Trevarthen T, Matharu M. Patient experience of diagnosis and management of spontaneous intracranial hypotension: a cross-sectional online survey. BMJ Open. 2022 Jan 20;12(1):e057438. doi: 10.1136/bmjopen-2021-057438.

  PMID: 35058269 BACKGROUND

• Hani L, Fung C, Jesse CM, Ulrich CT, Piechowiak EI, Gralla J, Raabe A, Dobrocky T, Beck J. Outcome after surgical treatment of cerebrospinal fluid leaks in spontaneous intracranial hypotension-a matter of time. J Neurol. 2022 Mar;269(3):1439-1446. doi: 10.1007/s00415-021-10710-7. Epub 2021 Jul 18.

  PMID: 34274993 BACKGROUND

• Volz F, Fung C, Wolf K, Lutzen N, Urbach H, Kraus LM, Omer M, Beck J, El Rahal A. Recovery and long-term outcome after neurosurgical closure of spinal CSF leaks in patients with spontaneous intracranial hypotension. Cephalalgia. 2023 Aug;43(8):3331024231196808. doi: 10.1177/03331024231195830.

  PMID: 37652456 BACKGROUND

Related Links

• Spinal CSF Leak Foundation - Home - Spinal CSF Leak Foundation. Accessed September 5, 2023
• Homepage. CSF Leak Association. Accessed September 5, 2023
• SPINAL CSF LEAK CANADA. SPINAL CSF LEAK CANADA. Accessed September 5, 2023

MeSH Terms

Conditions

Cerebrospinal Fluid Otorrhea; Post-Dural Puncture Headache; Cerebrospinal Fluid Leak; Rare Diseases

Condition Hierarchy (Ancestors)

Neurologic Manifestations; Nervous System Diseases; Craniocerebral Trauma; Trauma, Nervous System; Signs and Symptoms; Pathological Conditions, Signs and Symptoms; Wounds and Injuries; Headache Disorders, Secondary; Headache Disorders; Brain Diseases; Central Nervous System Diseases; Disease Attributes; Pathologic Processes

Central Study Contacts

Katharina Wolf, Dr. med.

CONTACT

+49 761 270-50010; katharina.wolf@uniklinik-freiburg.de

Florian Volz, Dr. med.

CONTACT

florian.volz@uniklinik-freiburg.de

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 24 Months
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Scientist

Study Record Dates

• First Submitted: November 15, 2024
• First Posted: December 2, 2024
• Study Start: November 4, 2024
• Primary Completion (Estimated): December 4, 2034
• Study Completion (Estimated): December 4, 2036
• Last Updated: December 2, 2024

Record last verified: 2024-11

Locations

DE (1)