The Freiburg Registry on SpontanEous IntercrAnial Hypotension (SIH) & Post-duraL Puncture Headache (PDPH)
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 2,000
- 试验地点
- 1
- 主要终点
- Primary Diagnosis given after primary workup (yes/no)
研究概览
简要总结
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.
详细描述
Spinal CSF leaks have a severe impact on quality of life and health. Symptoms vary from chronic headache syndrome to intracranial bleeding, cognitive decline, and coma. Crucially, yet often disregarded, the disease holds the potential for cure.
Lumbar dural leaks can arise from commonly performed medical interventions such as diagnostic lumbar punctures, spinal anesthesia, spinal infiltrations, or incidental dural punctures during epidural analgesia in obstetric care, resulting in post-dural puncture headache (PDPH).
Additionally, a notable fraction of spinal CSF leaks manifests as spontaneous intracranial hypotension (SIH), which derives from different types of spontaneous leaks along the Spine and remains broadly under-recognized.
The main clinical symptoms of spinal CSF leaks are orthostatic headaches with a most often defined beginning, typically accompanied by hearing impairment and dizziness, worsened by exercise and movement. Additionally, other manifestations are known, such as cognitive decline, bilateral brachial amyotrophy, paradox headache, fatigue, and many more. In patients presenting with spinal CSF leaks, the accurate diagnosis often eludes clinicians, leading to frequent misdiagnoses of chronic migraine, fatigue, or psychiatric conditions. Many patients endure months, if not years, before a diagnosis of a treatable condition is finally established. The heterogeneity of the symptoms can appear inconsistent or even paradoxical, posing diagnostic challenges. The extent of possible long-term deterioration is not recognized.
The reported incidence of PDPH fluctuates considerably, ranging from 2 to 40 per 100 procedures performed. This variance is influenced by patient-related factors (e.g., age, gender, pregnancy status) and procedural factors (e.g., needle size and type). PDPH, according to current criteria, occurs within 5 days after a dural puncture. Nevertheless, there are widely underestimated pitfalls: a dural puncture is not always recognized by the person performing an epidural procedure, symptoms can occur after more than 5 days, and disease courses can be chronic. These facts are widely unknown, leading to largely hidden figures of patients being under or misdiagnosed and, thus, not treated. Moreover, PDPH is primarily observed following unintentional dural puncture during the administration of obstetric anaesthesia and analgesia to parturients.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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
排除标准
- •a) Symptoms beeing conclusively explained by another known diagnosis
结局指标
主要结局
Primary Diagnosis given after primary workup (yes/no)
时间窗: up to 4 weeks after inclusion
* 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
次要结局
- SIH/PDPH - Experience of the local team(at time of inclusion)
- SIH/PDPH - Patients' demographics(at time of inclusion)
- SIH/PDPH - weight(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)
- SIH/PDPH - height(at time of inclusion)
- SIH/PDPH - modified Ranking scale(at time of inclusion)
- SIH/PDPH - pre-existing Neurological deficits (yes/no)(at time of inclusion)
- SIH/PDPH - Neurological deficits in routine clinical testing(at time of inclusion)
- SIH - Montreal cognitive Assessment(at time of inclusion)
- SIH - Trail-making test part B(at time of inclusion)
- PDPH -BMI(at time of inclusion)
- SIH/PDPH - Regular use of stimuli > 6 months (yes/no)(at time of inclusion)
- SIH/PDPH - Headache-Impact-Test (HIT)-6(at time of inclusion)
- SIH/PDPH - 5 dimensions /5 levels European Quality of life questionaire (EQ-5D-5L) Index(at time of inclusion)
- SIH/PDPH - Self-Administered Comorbidity Questionaire (SCQ)(at time of inclusion)
- PDPH - Patient'S Global Impression of Change (PIGC)(at time of inclusion)
- SIH - Opening pressure on lumbar puncture if performed (not recommended in routine), or myelogram(up to 4 weeks)
- SIH/PDPH - Total Bern-Score MRI head according to Dobrocky et al.(up to 4 weeks)
- SIH/PDPH - Subitems Bern-Score MRI head according to Dobrocky et al.(up to 4 weeks)
- SIH - MRI head - Superficial Siderosis(up to 4 weeks)
- SIH - MRI head - Layered calvarial hyperostosis(up to 4 weeks)
- SIH - Sinus vein thrombosis(up to 4 weeks)
- SIH/PDPH - Volumetry of the CSF-space MRI head & spine(up to 4 weeks)
- SIH/PDPH - Volumetry of the CNS MRI head & spine(up to 4 weeks)
- SIH/PDPH - Imaging Density score(up to 4 weeks)
- SIH/PDPH - Total radiation dose applied per diagnostic procedure with radiation performed(at primary workup)
- SIH - Myelography number(up to 24 weeks)
- SIH - Type of myelography technique applied (yes/no)(up to 4 weeks)
- SIH - Adverse events during myelography (yes/no)(up to 4 weeks)
- SIH - Headache before and after myelogram(up to 4 weeks)
- SIH - Positioning during index myelography that proofs the leak (yes/no)(up to 4 weeks)
- SIH - myelography that proofs the leak performed applying (yes/no)(up to 4 weeks)
- SIH/PDPH - Lumbar Infusiontest - Pressure at baseline(up to 4 weeks)
- SIH/PDPH - Lumbar Infusiontest - Resistence to CSF outflow (Rcsf)(up to 4 Weeks)
- SIH/PDPH - Lumbar Infusiontest - Elastance(up to 4 weeks)
- SIH/PDPH - Lumbar Infusiontest - Pressure-Volume-Index(up to 4 weeks)
- SIH/PDPH - Lumbar Infusiontest - Needle resistance(up to 4 weeks)
- SIH/PDPH - PET-CT - Evidence of CSF-loss (yes/no)(up to 4 weeks)
- SIH/PDPH - PET-CT if performed -(up tp 4 weeks)
- SIH/PDPH - Laboratory parameters at diagnosis (normal/abnormal)(up to 4 weeks after inclusion)
- SIH - CSF leak types identified by dynamic myelography (yes/no)(up to 4 weeks)
- SIH - Multiples leaks (yes/no)(up to 4 weeks)
- SIH - Level of spinal CSF leak (spinal segment) and side in myelogram(up to 4 weeks)
- SIH - In case of surgery: spinal segment and side confirmed? (yes/no)(up to 4 weeks)
- SIH/PDPH - Experience of leading interventionalist in SIH-diagnostics/year(up to 4 weeks)
- SIH/PDPH - Disease duration at time of therapy(up to 4 weeks)
- SIH/PDPH - Therapy performed after CSF leak diagnosis (yes/no)(up to 4 weeks)
- SIH/PDPH - Experience of leading surgeon in SIH/PDPH-surgeries/year(up to 4 weeks or longer, depending on the number of surgeries)
- SIH/PDPH - Interventions needed addressing secondary complications of spinal CSF leaks, especially chronic subdural hematoma(up to 4 weeks)
- PDPH - Event of putative dural puncture(up to 4 weeks)
- PDPH - Event of putative dural puncture (yes/ no)(up to 4 weeks)
- SIH/PDPH - adverse events related to current treatment at discharge (yes/no)(up to 4 weeks)
- PDPH - Lumbar segment of putative dural puncture known (yes/no)(up to 4 weeks)
- PDPH - Puncture under imaging guidance(up to 4 weeks)
- PDPH - Duration between (putative) dural puncture until the onset of symptoms(up to 4 weeks)
- PDPH - Time to first blood patch(up to 4 weeks)
- PDPH - Number of bloodpatches received before admission(up to 4 weeks)
- PDPH - Prior treatments at the time of admission (yes/no)(up to 4 weeks)
- PDPH - Post-dural puncture pseudomeningocele ("arachnoid bleb")(up to 4 weeks)
- PDPH - Spinal Segment location of the "arachnoid bleb"(up to 4 weeks)
- PDPH - Identified CSF leak types by dynamics myelography and/or intraoperatively:(up to 4 weeks)
- PDPH - Intraoperatively Identified membranes (yes/no)(up to 4 weeks)
- PDPH - Dinosaur-tail sign (yes/no)(at time of inclusion)
- PDPH - specific segment of identified intraoperative membranes (descriptive)(up to 4 weeks)
- PDPH - Diagnostic procedures performed (yes/no, number)(uo to 4 weeks)
- PDPH - CSF leak types identified by dynamic myelography and/or intraoperatively(up to 4 weeks)
- PDPH - Volume lumbar bloodpatch(up to 4 weeks)
- SIH/PDPH - work capacity(at time of inclusion)
- SIH/PDPH -complaints(at time of inclusion)
- SIH/PDPH - headache severity(at time of inclusion)
- SIH/PDPH - maximum duration being continuously upright(at time of inclusion)
- SIH/PDPH - Severeness of shoulder- and neck pain(at time of inclusion)
- SIH/PDPH - severeness of nausea(at time of inclusion)
- SIH/PDPH - severeness of hearing disturbances and tinnitus(at time of inclusion)
- SIH/PDPH - severeness of cognitive deficits(at time of inclusion)
- SIH/PDPH - severeness of visual disturbances(at time of inclusion)
- SIH/PDPH - . ability to focus and concentrate(at time of inclusion)
- SIH/PDPH - MRI spine: Spinal longitudinal extradural fluid collection (SLEC) (yes/no)(up to 4 weeks)
- SIH/PDPH - MRI-spine: DiverTICula (TIC) ≥8mm (yes/no)(up to 4 weeks)
- SIH/PDPH - Volumetry of epidural spinal veins at C2(up to 4 weeks)
- SIH/PDPH - MRI spine: Enlarged cervical epidural spinal veins at C2(up to 4 weeks)
- SIH/PDPH - Days within the last month(at time of inclusion)
- SIH/PDPH - severeness of dizziness(at time of inclusion)
- SIH/PDPH - lumbar infusion test - pressure at tilt down(up to 4 weeks after inclusion)
- SIH/PDPH - lumbar infusion test - pressure at tilt up 10°(up to 4 weeks after inclusion)
- SIH/PDPH - lumbar infusion test - pressure at tilt up 30°(up to 4 weeks after inclusion)
- SIH/PDPH - lumbar infusion test - pressure at plateau(up to 4 weeks after inclusion)
- SIH/PDPH - Laboratory parameters at diagnosis (decriptive)(up to 4 weeks after inclusion)
- SIH/PDPH - Lumbar Infusiontest - amplitude at baseline(up to 4 weeks)
- SIH/PDPH - lumbar infusion test - amplitude at tilt down(up to 4 weeks after inclusion)
- SIH/PDPH - lumbar infusion test -amplitude at tilt up 10°(up to 4 weeks after inclusion)
- SIH/PDPH - lumbar infusion test - amplitude at tilt up 30°(up to 4 weeks after inclusion)
- SIH/PDPH - lumbar infusion test - amplitude at plateau(up to 4 weeks after inclusion)
- SIH/PDPH - Results of neuropathological tissue analysis if assessed (descriptive)(up to 6 weeks)
- SIH/PDPH - phase-contrast MRI : CSF-velocity(up to 4 weeks)
- SIH/PDPH - phase-contrast MRI: Stroke-volume CSF(up to 4 weeks)
- SIH/PDPH - phase-contrast MRI: Spinal cord motion(up to 4 weeks)
- SIH/PDPH - phase-contrast MRI: Spinal cord velocities(up to 4 weeks)
- SIH/PDPH - MRI spine: Bud-on-branching sign (yes/no)(up to 4 weeks)
- SIH/PDPH - MRI spine: Localized flow-voids in sagittal spine T2 images (yes/no)(up to 4 weeks)
研究者
Myriam Peters
Scientist
University Hospital Freiburg