Spontaneous Intracranial Hypotension Research Articles

Volumetric Changes in the Choroid Plexus Associated with Spontaneous Intracranial Hypotension in Patients with Spinal CSF Leak.

The choroid plexus contains specialized ependymal cells responsible for CSF production. Recent studies have demonstrated volumetric and perfusion changes in the choroid plexus with age and neurodegenerative disorders, however, volumetric changes in the choroid plexus in low pressure states is not known. The purpose of this study is to evaluate volumetric differences in choroid plexus size in patients with spontaneous intracranial hypotension (SIH) resultant from spinal CSF leaks compared with healthy controls. This was a retrospective, institutional review board-approved study. Patients with MRI evidence of SIH and a spinal CSF leak diagnosed on myelography and subsequently confirmed at surgery were included in this study. All patients included in this study including age-matched healthy controls had a brain MRI performed on a either a 1.5 or 3T scanner with acquisition of 3D T1 postcontrast (eg, BRAVO, MPRAGE, etc). In all patients, the trigonum ventriculi volume, in the atria of the lateral ventricles, was contoured by using Visage-7 segmentation tools on the volumetric postcontrast T1 sequence. A basic 2-tailed t test was used to compare choroid plexus volumes between the 2 groups. Thirty-four patients were included with 17 patients with SIH with spinal CSF leak and 17 healthy control patients who were age- and sex-matched. The mean age of patients was 45 years, standard deviation 14 years. The mean volume of the choroid plexus for patients with SIH with spinal CSF leak was 1.2 cm3 (standard deviation = 0.26) compared with 0.63 cm3 (standard deviation = 0.31) in the control group (P < .0001). Results of this study demonstrate a higher choroid plexus volume in patients with SIH with spinal CSF leak compared with age- and sex-matched healthy controls. This likely reflects compensatory mechanisms to counteract intracranial hypotension by increasing CSF production as well as increased vascularity of the choroid plexus through expansion of the intracranial blood pool.

Spontaneous Intracranial Hypotension: Role of Computed Tomography-guided Cervical Epidural Blood Patch.

Lumbar epidural blood patch (EBP) is not successful in all cases of cerebrospinal fluid (CSF) leak, particularly in the cervicothoracic region. The present study is a retrospective analysis of a cohort of patients who had undergone computed tomography (CT)-guided cervical EBP for spontaneous intracranial hypotension (SIH) due to CSF leak in the cervical region. We retrospectively collected data from March 2009 to 2020. Our inclusion criteria were (1) clinical syndrome associated with CSF leak, (2) headache coming on shortly after assuming the erect position and relief achieved by lying down, (3) CT myelography evidence of CSF leak in the cervical region, and (4) patient not responding to conservative management for 7 days. Exclusion criteria were patients with bleeding diathesis and infection. There were six females and four males. Ages ranged from 32 to 57, with an average of 42 years. On presentation, all patients underwent contrast-enhanced magnetic resonance imaging (MRI) and CT myelography. Cervical EBP was done under CT scan guidance. Assessment of headache was done on a 10-point numerical rating scale (NRS) before and after the procedure. Results are categorized into complete relief, partial relief, and no relief categories. Nine out of 10 patients were completely recovered. One patient was partially recovered. The average NRS scale was 9.6 before treatment, which became 0.4 after EBP. No neurological or vascular complications were seen. Computed tomography-guided cervical EBP is an optimum and effective way of treating SIH due to a leak in the cervical region. It has a higher success rate than lumbar EBP. However, prospective randomized controlled trials of cervical vs lumbar EBP will further validate our observation.

Clinical Findings, Radiological Characteristics, and Treatment Options of Spontaneous and Secondary Intracranial Hypotension: A Single-Center Experience in Turkey.

Introduction Intracranial hypotension can occur for many reasons, including trauma, surgery, congenital defects, or spontaneous rupture of the dura mater. Symptoms appear long before cerebrospinal fluid (CSF) leaks are diagnosed. Treatment procedures include a variety of conservative and invasive techniques appropriate to the nature of the etiological cause and the severity of the disease. In this cross-sectional study, we aimed to investigate the clinical and imaging features and treatment options of intracranial hypotension patients and to compare them in terms of different etiologies. Methods The data from intracranial hypotension patients were analyzed retrospectively. Symptomatology, neurological findings, and radiological features were compared between patients with spontaneous intracranial hypotension (SIH) and those with secondary causes. Radiological outcomes of conservative treatment and epidural blood patch (EBP) were also evaluated for both groups. Results Of the 30 patients, 23 were female. In 14 of the patients (46.6%), a possible cause of CSF leakage was detected. Compared to intracranial hypotension patients with a secondary cause, SIH patients complained of posterior neck and shoulder pain more frequently(p=0.014, p=0.006). MRI features did not differ significantly when the two groups were compared (p>0.05). The first and sixth-month follow-up MRIs of patients treated with EBP or a conservative approach showed similar improvement rates (p=0.788). Conclusions There was no significant difference in radiological recovery time between conservative treatment and EBP in patients with intracranial hypotension. Radiological recovery times are similar in patients with secondary intracranial hypotension and SIH.

Cerebrospinal fluid-venous fistula visualisation by intrathecal pressurization: A technical note and illustrative case.

CSF-venous fistulas (CVFs) are an important cause of spontaneous intracranial hypotension and challenging to diagnose. Lateral decubitus positioning during myelography and a technique called "resisted inspiration" has shown to improve CVF detection. However, the impact of intrathecal pressurization to improve visualization of CVF has mostly been speculated on. In this brief report, we demonstrate how a CVF became progressively more visible only after stepwise intrathecal pressurization: An indication of the importance of pressurization for CVF detection.

Spontaneous intracranial hypotension in a patient without corpus callosum: A case report.

Spontaneous intracranial hypotension (SIH) is a well-established condition typically presenting with disabling orthostatic headache. Corpus callosum agenesis (CCA) is one of the most common human brain malformations with a wide spectrum of associated malformations, chromosomal abnormalities, and clinical syndromes. A 53-year-old woman presented with recurrent orthostatic headache for about 1 month. The head computed tomography examination of the patient showed CCA and the initial pressure of subsequent lumbar puncture was only 5 centimeters cerebrospinal fluid. Magnetic resonance imaging examination confirmed CCA with gray matter heterotopia. The final diagnose was SIH related headache with CCA. The patient's symptom improved after oral hydration, intravenous fluids, and bed rest. Favorable outcome was observed. Although this co-occurrence of SIH and CCA is probably coincidental, this finding suggests that the developmental malformations of the brain may lead to structural changes in brain tissue or disturbances in cerebrospinal fluid production and reflux, resulting in pathological changes over time.

Prevalence, clinical presentation, and treatment-management of cerebral venous thrombosis associated with spontaneous intracranial hypotension: A combined case-series and systematic literature review approach.

Cerebral venous thrombosis (CVT) is a rare complication of spontaneous intracranial hypotension (SIH). Therefore, its correct diagnosis and the corresponding optimal treatment-management identification remains challenging. Over the last 10 years, 300 patients received a definite SIH diagnosis at our stroke center. Through thorough review of the database, we identified all patients with SIH-related CVT. In addition, we performed a systematic literature review including all publications on SIH-related CVT. Five out of our 300 SIH patients showed CVT (F/M:2/3, mean age: 51.8 ± 15.7). Through the literature search, 72 additional cases were identified. Overall, the prevalence was 1.3% and main clinical presentations were orthostatic headache, nausea, and vomiting. The CVT was predominantly located at the superior sagittal sinus. Treatment strategies included anticoagulants (ACs) (43%), epidural blood patch (EBP) (19.4%), and combined AC + EBP (33.3%). In our cohort, all but one patient received combined EBP and AC. The mean clinical and radiological follow-up were 2 years and 1.5 years, respectively. Complete clinical recovery was reported in 96% of the cases, whereas 56% showed complete radiological CVT resolution. Among patients without radiological resolution (26.4%), 57% received AC-only, while 43% received combined AC + EBP. Of our five cases, all but one patient received combined AC + EBP. The overall prevalence of SIH-related CVT was 1.3%. AC and combined AC + EBP were the most used treatment-management strategies. CVT resolution was more commonly achieved after the combined strategy. Overall, the rate of complete clinical recovery was 96%.

Epidural Blood Patch for the Treatment of Spontaneous Intracranial Hypotension: A Case Series.

Epidural blood patch (EBP) is frequently used for the treatment of spontaneous intracranial hypotension (SIH) and anesthesiologists are often involved in performing such procedures. However, the optimal technique and approach of EBP remains uncertain. This case series included adult patients with SIH who underwent EBPs at London Health Science Centre, Ontario, Canada between 2010 and 2022. Demographics, clinical presentations, investigations, and EBP treatment details were collected and analyzed. Univariate analysis was used to investigate the association of the variables with the likelihood of EBP 1-month efficacy and the efficacy duration of EBP. The study included 36 patients with SIH who received at least 1 EBP. EBPs provided immediate relief in almost all patients, albeit with diminishing effects over time. The 1-month efficacy improved with increasing number of EBP attempts (P=0.032, Fisher exact test), though no particular EBP technique or volume of injectate was associated with better efficacy (P=0.38, Fisher exact test). Though permanent resolution of symptoms was observed in only 24 of 82 EBPs (29%), 24 of 36 patients (67%) had permanent symptom resolution following repeated EBPs. EBP is a promising treatment and symptomatic relief option in patients suffering from the debilitating symptoms of SIH. Tailored EBP techniques, including use of targeted higher volume EBP and a multi-level catheter guided technique for refractory cases, showed efficacy in our institutional setting. Despite its limitations, this study contributes valuable insights and experiences into the use of EBP for treatment of SIH.

Hypermobile Ehlers-Danlos syndrome and spontaneous CSF leaks: the connective tissue conundrum.

Collagen, the most abundant protein in the body, is a key component of the extracellular matrix (ECM), which plays a crucial role in the structure and support of connective tissues. Abnormalities in collagen associated with connective tissue disorders (CTD) can lead to neuroinflammation and weaken the integrity of the blood-brain barrier (BBB), a semi-permeable membrane that separates the brain's extracellular fluid from the bloodstream. This compromise in the BBB can result from disruptions in ECM components, leading to neuroinflammatory responses, neuronal damage, and increased risks of neurological disorders. These changes impact central nervous system homeostasis and may exacerbate neurological conditions linked to CTD, manifesting as cognitive impairment, sensory disturbances, headaches, sleep issues, and psychiatric symptoms. The Ehlers-Danlos syndromes (EDS) are a group of heritable CTDs that result from varying defects in collagen and the ECM. The most prevalent subtype, hypermobile EDS (hEDS), involves clinical manifestations that include joint hypermobility, skin hyperextensibility, autonomic dysfunction, mast cell activation, chronic pain, as well as neurological manifestations like chronic headaches and cerebrospinal fluid (CSF) leaks. Understanding the connections between collagen, CSF, inflammation, and the BBB could provide insights into neurological diseases associated with connective tissue abnormalities and guide future research.

Spinal dementia: Don’t miss it, it’s treatable

Background & purposeAround 5% of dementia patients have a treatable cause. To estimate the prevalence of two rare diseases, in which the treatable cause is at the spinal level.MethodsA radiology information system was searched using the terms CT myelography and the operation and classification system (OPS) code 3-241. The clinical charts of these patients were reviewed to identify patients with a significant cognitive decline.ResultsAmong 205 patients with spontaneous intracranial hypotension (SIH) and proven CSF leaks we identified five patients with a so-called frontotemporal brain sagging syndrome: Four of those had CSF venous fistulas and significantly improved by occluding them either by surgery or transvenous embolization. Another 11 patients had infratentorial hemosiderosis and hearing problems and ataxia as guiding symptoms. Some cognitive decline was present in at least two of them. Ten patients had ventral dural tears in the thoracic spine and one patient a lateral dural tear at C2/3 respectively. Eight patients showed some improvement after surgery.DiscussionIt is mandatory to study the (thoracic) spine in cognitively impaired patients with brain sagging and/ or infratentorial hemosiderosis on MRI. We propose the term spinal dementia to draw attention to this region, which in turn is evaluated with dynamic digital subtraction and CT myelography.

Spontaneous intracranial hypotension - a spinal disease.

Spontaneous intracranial hypotension (SIH) remains an underdiagnosed condition despite increasing awareness due to recent scientific advances. Diagnosis can be delayed by the broad clinical presentation and imaging pitfalls. This results in a high degree of physical impairment for patients, including social and psychological sequelae as well as long-term damage in the case of delayed diagnosis and treatment.The study is based on a selective literature search on PubMed including articles from 1990 to 2023 and the authors' clinical experience from working in a CSF center.SIH mostly affects middle-aged women, with the primary symptom being position-dependent orthostatic headache. In addition, there is a broad spectrum of possible symptoms that can overlap with other clinical conditions and therefore complicate the diagnosis. The causative spinal CSF loss can be divided into three main types: ventral (type 1) or lateral (type 2) dural leak and CSF-venous fistula (type 3). The diagnosis can be made using a two-stage workup. As a first step, noninvasive MRI of the head and spine provides indicators of the presence of SIH. The second step using focused myelography can identify the exact location of the cerebrospinal fluid leak and enable targeted therapy (surgical or interventional). Intrathecal pressure measurement or intrathecal injection of gadolinium is no longer necessary for primary diagnosis. Serious complications in the course of the disease can include space-occupying subdural hematomas, superficial siderosis, and symptoms of brain sagging, which can lead to misinterpretations. Treatment consists of closing the dural leak or the cerebrospinal fluid fistula. Despite successful treatment, a relapse can occur, which highlights the importance of follow-up MRI examinations and emphasizes the chronic nature of the disease. This paper provides an overview of the diagnostic workup of patients with suspected SIH and new developments in imaging and therapy. · SIH is an underdiagnosed condition with a wide range of possible symptoms.. · The first diagnostic step using MRI provides indications of the presence of SIH.. · The second diagnostic step using (dynamic) myelography can identify the CSF leak.. · Collaboration with a CSF center is advisable for further diagnosis and treatment.. · Prompt detection and treatment of SIH improves the outcome.. · Zander C, Wolf K, El Rahal A et al. Spontaneous intracranial hypotension - a spinal disease. Fortschr Röntgenstr 2024; DOI 10.1055/a-2318-8994.

Spontaneous Intracranial Hypotension

Spontaneous Intracranial Hypotension

An Insidious Complication of Spontaneous Spinal CSF Leak: Superficial Siderosis

Superficial siderosis of the CNS can result in long-term morbidity for patients with spontaneous intracranial hypotension, even after successful treatment of the causative spinal CSF leak. Spontaneous spinal CSF leaks are categorized by causative mechanisms, which include dural tears, meningeal diverticula, and CSF-venous fistulas. Our case series demonstrates superficial siderosis preferentially involving the cerebellar folia, typically resulting in symptoms of classic infratentorial superficial siderosis, and mainly occurring secondary to type 1A spinal CSF leaks.Learning Objectives: &lt;list list-type="order"&gt; &lt;list-item&gt;Superficial siderosis due to spontaneous spinal CSF leaks preferentially involves the cerebellar folia.&lt;/list-item&gt; &lt;list-item&gt;Symptoms are typically those of classic infratentorial superficial siderosis, consisting of hearing and vestibular impairment, gait ataxia, myelopathy and/or cognitive dysfunction.&lt;/list-item&gt; &lt;list-item&gt;Superficial siderosis typically occurs in setting of type 1A spinal CSF leaks (ventral dural tears).&lt;/list-item&gt; &lt;/list&gt;

Cerebrospinal Fluid Leaks: Challenges in Localizing Spontaneous Spinal Leak Sites and Minimally Invasive Treatment

The confirmation of cerebrospinal fluid (CSF) leaks in the setting of spontaneous intracranial hypotension (SIH) by imaging involves a growing toolset of multimodal advanced spinal and skull base imaging techniques, for which exists a unique set of challenges for each CSF leak type. Furthermore, the repertoire of minimally invasive CSF leak treatment beyond nontargeted epidural blood patch administration has grown widely, with varied practices across institutions. This review describes current diagnostic imaging and treatment modalities as they apply to the challenges of CSF leak localization and management.

Surgical Management of Spontaneous Lateral Skull Base Cerebrospinal Fluid Leaks: The Middle Cranial Fossa Approach

Surgical Management of Spontaneous Lateral Skull Base Cerebrospinal Fluid Leaks: The Middle Cranial Fossa Approach

Innovations in the Treatment of Spinal Cerebrospinal Fluid Leaks

Spontaneous spinal cerebrospinal fluid (CSF) leaks are uncommon but can be neurologically debilitating. When initial treatments fail, definitive repair or closure of the leak is indicated. Depending upon the type of leak present, innovative strategies for their treatment have been developed. Among them are open surgical techniques using a transdural approach for the closure of ventral CSF leaks, minimally invasive tubular techniques for the reduction and repair of lateral meningeal diverticula, and endovascular embolization of CSF-venous fistulas. Illustrative cases demonstrating the indications for and implementation of these techniques are provided.

An algorithm for the surgical approach to spontaneous temporal bone CSF leak

ObjectiveTo suggest a comprehensive algorithm for the surgical approach for correcting of sources of temporal bone CSF leaks. MethodsA case series for patients operated in a single academic tertiary referral center between 2011 and 4.2022. Included in the study were 46 patients, 5 of whom had a bilateral problem, resulting in 51 pathologic temporal bones. The presentation was an active CSF leak (38 patients) or bacterial otogenic meningitis (8 patients). Follow up ranged from 8 months to 5 years. ResultsOf the 42 ears operated via the default middle fossa approach, 37 were successful (88 %) in controlling CSF leak. None had intracranial complications or sensorineural hearing loss. Location, number and size of the defects, hearing status, associated superior semicircular canal dehiscence, additional intra-temporal or intra-cranial pathologies may indicate a transmastoid approach. Of the six ears that had a canal wall up mastoidectomy as a primary procedure, one required revision due to ongoing CSF leak. Five revision cases and three primary cases were effectively sealed with a subtotal petrosectomy and obliteration. One was lost to follow-up. Hearing was reconstructed with bone-anchored hearing implants in 6 out of these 8 ears. ConclusionsThe middle fossa approach could be used as a default approach for sealing TD. There are a number of indications for transmastoid approaches in both primary and revision cases. Obliteration of the ear was used in all revision cases. The suggested algorithm can help in planning surgery for temporal bone CSF leaks or a history of otogenic meningitis.

Epidural Blood Patch for Atypical Headache due to Spontaneous Intracranial Hypotension Associated with Meningeal Diverticulum

Epidural Blood Patch for Atypical Headache due to Spontaneous Intracranial Hypotension Associated with Meningeal Diverticulum

Long-Term Epidural Patching Outcomes and Predictors of Benefit in Patients With Suspected CSF Leak Nonconforming to ICHD-3 Criteria.

Spinal CSF leaks lead to spontaneous intracranial hypotension (SIH). While International Classification of Headache Disorders, Third Edition (ICHD-3) criteria necessitate imaging confirmation or low opening pressure (OP) for SIH diagnosis, their sensitivity may be limited. We offered epidural blood patches (EBPs) to patients with symptoms suggestive of SIH, with and without a documented low OP or confirmed leak on imaging. This study evaluates the efficacy of this strategy. We conducted a prospective cohort study with a nested case-control design including all patients who presented to a tertiary headache clinic with clinical symptoms of SIH who completed study measures both before and after receiving an EBP between August 2016 and November 2018. The mean duration of symptoms was 8.7 ± 8.1 years. Of 85 patients assessed, 69 did not meet ICHD-3 criteria for SIH. At an average of 521 days after the initial EBP, this ICHD-3-negative subgroup experienced significant improvements in Patient-Reported Outcomes Measurement Information System (PROMIS) Global Physical Health score of +3.3 (95% CI 1.5-5.1), PROMIS Global Mental Health score of +1.8 (95% CI 0.0-3.5), Headache Impact Test (HIT)-6 head pain score of -3.8 (95% CI -5.7 to -1.8), Neck Disability Index of -4.8 (95% CI -9.0 to -0.6) and PROMIS Fatigue of -2.3 (95% CI -4.1 to -0.6). Fifty-four percent of ICHD-3-negative patients achieved clinically meaningful improvements in PROMIS Global Physical Health and 45% in HIT-6 scores. Pain relief following lying flat prior to treatment was strongly associated with sustained clinically meaningful improvement in global physical health at an average of 521 days (odds ratio 1.39, 95% CI 1.1-1.79; p < 0.003). ICHD-3-positive patients showed high rates of response and previously unreported, treatable levels of fatigue and cognitive deficits. Patients who did not conform to the ICHD-3 criteria for SIH showed moderate rates of sustained, clinically meaningful improvements in global physical health, global mental health, neck pain, fatigue, and head pain after EBP therapy. Pre-treatment improvement in head pain when flat was associated with later, sustained improvement after EBP therapy among patients who did not meet the ICHD-3 criteria. This study provides Class IV evidence that epidural blood patch is an effective treatment of suspected CSF leak not conforming to ICHD-3 criteria for SIH.

Epidural Blood Patch in Subdural Hematoma Due to Spontaneous Intracranial Hypotention: A Case Report and Literature Review

Spontaneous intracranial hypotension (SIH) is an orthostatic headache syndrome with variable symptoms and complications which is often misdiagnosed at initial manifestations. SIH results from spontaneous CSF leakage leading to brain sag. The typical findings on cranial MR imaging consist of subdural fluid collections, enhancement of the pachymeninges, engorgement of venous structures, pituitary hyperemia, and sagging of the brain. Subdural hematoma may occur as a result of tearing of bridging veins and usually develop into chronic subdural hematoma. The majority of patients with SDH due to SIH have chronic DSH and, therefore, rarely present with neurological deficits. Evacuation of SDH may be performed for large SDH with ME (mass effects), or when dilated or asymmetric pupil is present. However in most cases, evacuation of the hematoma is not necessary and may result in worsened outcomes. The epidural blood patch (EBP) is a treatment of choice. Fortunately, most of these subdural hematomas can be handled with treatment directed at the underlying spinal CSF leak without the need for surgery. We report the case of 42-year-old man with the chief complaint of orthostatic headache. He was admitted to neurology ward and after imaging studies, it was found that he has bilateral subdural hematoma. Due to the lack of history of trauma, underlying disease, and coagulation disorder, and considering the imaging findings, the patient was referred to the pain department to perform an epidural blood patch. After performing the epidural blood patch, the patient's pain was relieved immediately, and during a three-month follow-up period, the epidural hematoma was completely absorbed. Spontaneous intracranial hypotension (SIH) is a highly misdiagnosed and underdiagnosed disorder and requires a high index of suspicion for diagnosis. During the last decades, a much larger number of spontaneous cases are identified. Literature is a bit confusing, with some authors recommending evacuation of subdural fluid in cases of deteriorating consciousness and few others recommending EBP first even in patients with comatose state but epidural patch is often an important part of treatment.

Lateral Spinal CSF Leaks in Patients with Spontaneous Intracranial Hypotension: Radiologic-Anatomic Study of Different Variants.

Spinal CSF leaks cause spontaneous intracranial hypotension. Several types of leaks have been identified, and one of these types is the lateral dural tear. Performing myelography with the patient in the decubitus position allows precise characterization of these leaks. The purpose of the current study was to describe the different variants of spontaneous lateral CSF leaks. This retrospective cohort study included a consecutive group of patients with spontaneous intracranial hypotension and lateral CSF leaks who underwent digital subtraction myelography in the decubitus position and underwent surgery to repair the CSF leak between July 2018 and June 2023. The mean age of the 53 patients (37 women and 16 men) was 35.5 years. Three different variants of lateral CSF leak could be identified. Forty-nine patients (92.5%) had a lateral dural tear associated with the nerve root sleeve. The dural tear was at the axilla of the nerve root sleeve in 36 patients (67.9%) and at the shoulder in 13 patients (24.5%). Four patients (7.5%) had a lateral dural tear at the level of the pedicle that was not associated with the nerve root sleeve. Findings on digital subtraction myelography were concordant with intraoperative findings in all patients. An extradural CSF collection was seen in all patients with a lateral dural tear associated with the nerve root sleeve but in only 2 of the 4 patients with the pedicular variant of a lateral dural tear. We identified 3 variants of spontaneous lateral dural tears. Most lateral dural tears are associated with extradural CSF collections and arise from either the axilla (67.9%) or the shoulder (24.5%) of the nerve root sleeve. Lateral dural tears at the level of the pedicle (7.5%) not associated with the nerve root sleeve are uncommon and may require specialized imaging for their detection.