Spontaneous Intracranial Hypotension: Controversies in Treatment.

Spontaneous intracranial hypotension (SIH) is a disorder of low cerebrospinal fluid (CSF) volume secondary to CSF leakage through a dural defect along the neuraxis with an estimated incidence of 5 people per 100,000.Great debate persists on the optimal treatment of this pathology, and clinical results are often contradictory. SIH may be initially approached via conservative measures, such as bed rest that is often supplemented with hydration, caffeine, and theophylline, which overall relieve symptoms in a small subset of patients at 6 months. Epidural blood patching (EBP) is generally the next consideration in symptom management. It is the most commonly performed intervention for spinal CSF leaks, as the first option or following the failure of conservative treatment. Further procedures may be performed in the case of EBP partial or temporary response and if the spinal CSF leak has been definitively localized (Beck et al., Neurology 87:1220-26, 2016). In those cases, if the CSF leak is well localized, the surgical closure of the spinal CSF leak may be considered.SIH is a complex but treatable CSF disorder. Despite recent advances in the field of neuroimaging and the different therapeutic options available, the appropriate management remains controversial and should be tailored to the patient.

A systematic review and meta-analysis of factors affecting the outcome of the epidural blood patching in spontaneous intracranial hypotension.

Spontaneous intracranial hypotension (SIH) is an often misdiagnosed condition resulting from non-iatrogenic cerebrospinal fluid (CSF) hypovolemia, typically secondary to spinal CSF leakage. Patients commonly present with posture-related headache, nausea, and vomiting. Following failure of conservative measures, epidural blood patching (EBP) is the most commonly performed intervention for spinal CSF leaks. The authors performed a systematic review and meta-analysis of existing literature to evaluate the role of different factors possibly affecting the efficacy of the EBP procedure. In accordance with the PRISMA guidelines, PubMed/Medline and SCOPUS databases were searched. Six eligible articles were retrieved. Five hundred patients were treated for SIH with EBP, of which 300 reported good response defined as complete remission of symptoms within 48 h after the first EBP requiring no further invasive treatment. Among the factors available for meta-analysis, none was found to be statistically significant in affecting the efficacy of the EBP procedure. A largely symmetrical funnel plot is reported for all the variables evaluated, indicating that publication bias did not play a significant role in the observed effects. The current knowledge about SIH and the EBP is scarce. The existing literature is contradictory and insufficient to aid in clinical practice. More studies are needed to draw significant conclusions that may help in the identification of patients at higher risk of EBP failure, who may benefit from different approaches.

Contribution of Otoacoustic Emissions for Diagnosis of Atypical or Recurrent Intracranial Hypotension. A Cases Series.

BACKGROUND: The diagnosis of spontaneous or post-traumatic intracranial hypotension mainly rely on clinical features and neuroimaging. In atypical presentations, other non-invasive tests are missing to support the diagnosis of intracranial hypotension. The assessment of otoacoustic emissions (OAE) shown interest to monitor intracranial pressure mainly in cases of intracranial hypertension. This non-invasive method was also assessed in response to pressure change after lumbar puncture. A few reports showed abnormal results of distortion product otoacoustic emissions (DPOAE) in cases of spontaneous or post-traumatic intracranial hypotension. We describe additional results in a series of intracranial hypotension cases. We discuss the interest of DPOAE assessment in atypical presentations of intracranial hypotension. We review the other non-invasive tests reported in literature. METHODS: We studied 4 atypical or recurrent cases of spontaneous or post-traumatic intracranial hypotension by recording OAE in sitting then supine position. RESULTS: Unilateral or bilateral abnormal changes of DPOAE were recorded in all cases, in response to postural test. These changes were present even in the absence of vestibular symptoms. CONCLUSION: The study of OAE may be a non-invasive tool for the diagnosis of spontaneous intracranial hypotension.

A Review of Spontaneous Intracranial Hypotension.

PURPOSE OF REVIEW: Spontaneous intracranial hypotension (SIH) is an underdiagnosed phenomenon predominantly presenting with low cerebrospinal fluid (CSF) pressure and postural headache in setting of CSF leak. The goal of this paper is to provide updates on the pathology, diagnosis, and management of SIH. The utility of multiple imaging modalities and the use of epidural blood patches and fibrin glue polymers are explored. RECENT FINDINGS: In regard to diagnosis, new non-invasive modalities in detection of SIH including transorbital ultrasound and serum biomarkers are found. In addition, increased efficacy of large volume and repeated placement of multiple epidural blood patches (EBP) are seen. In addition, the management of refractory SIH using fibrin glue polymers has proved efficacious in recent case series. While the diagnosis may be challenging for clinicians, future research in SIH is leading to more rapid detection methods. Future studies may target optimal use of EBP in comparison to fibrin glue polymers, in addition to new developments in increased understanding of SIH physiology and phenotype.

Postural Headaches Due to Cerebrospinal Fluid Leakage Through Subarachnoid-Pleural Fistula: A Case Report.

BACKGROUND: Postural headaches are commonly associated with spontaneous intracranial hypotension and cerebrospinal fluid (CSF) leakage from the spine. A subarachnoid-pleural fistula (SPF) is a very rare and serious type of CSF fistula that has mostly been reported following traumatic causes. CASE REPORT: Here, the case of a 36-year-old woman who suffered from postural headaches after chiropractic manipulation of her neck was reported. Brain and spinal magnetic resonance (MR) imaging showed brain sagging, a C7-T1 dural defect, and overt CSF leakage. Heavy T2-weighted MR myelography revealed paravertebral fluid collections, communicating with pleural effusions through bilateral SPFs. The postural headaches were relieved by 3 courses of epidural blood patches. DISCUSSIONS: From the patient's history, her severe SPFs were attributed to rupture of the spinal leptomeninges during vigorous chiropractic manipulation of her neck. Heavy T2-weighted MR myelography could delineate the route of SPFs and CSF leakages.

The frequency and severity of intracranial hypotension post-intraoperative lumbar drainage using a Tuohy needle and the traditional needle.

Background Intraoperative lumbar cerebrospinal fluid (CSF) drainage is a well-recognised technique in cranial and vascular surgery. The goal of the study was to assess the frequency and severity of intracranial hypotension post-intraoperative lumbar drainage performed using two different techniques, a 14G Tuohy needle versus an 18G traditional needle. Methods The medical records and imaging studies of 94 patients who had undergone open cranial operation were retrospectively studied: 47 patients had intraoperative lumbar drainage and 47 patients did not. A 14G Tuohy needle was employed in 27 (57.4%) patients and an 18G traditional needle was employed in 20 (42.6%) patients. Results There were signs of intracranial hypotension on MR images in nine (19.1%) patients who had intraoperative lumbar CSF drainage; none of the patients in the control group exhibited the MR signs of intracranial hypotension. A 14G needle was used in 6/9 patients and resulted in severe complications: one patient developed a delayed intracranial epidural hematoma that required emergency evacuation and a blood patch. Another patient developed somnolence that required two epidural blood patches and a third patient had protracted headaches. The 18G needle was used in the remaining 3/9 patients who were asymptomatic or presented with mild headaches. Conclusion The use of the smaller 18G traditional needle was associated with better outcomes with regards to intracranial hypotension, and the frequency of severe complications was higher with the use of the 14G Tuohy needle.

Headache due to spontaneous intracranial hypotension and subsequent cerebral vein thrombosis.

Cerebral vein thrombosis (CVT) is a rare complication of spontaneous intracranial hypotension (SIH). When to suspect a thrombotic disorder during the course of intracranial hypotension is not fully elucidated. A 48-year-old woman was admitted because of SIH with no signs of CVT on neuroimaging. The occurrence of diplopia and blurred vision 12 days later led to the performance of further investigations, which revealed thrombosis of the left lateral sinus, in the absence of variations in the headache characteristics. Among the other 4 cases of SIH clearly preceding the occurrence of CVT reported so far, only one had a change in the headache pattern related to CVT development. Although a change in the characteristics of headache is considered a marker of CVT in patients with SIH, this is not invariably part of the clinical scenario. Any new neurologic finding on exam in the disease course should raise a suspicion of venous thrombosis, thus prompting further specific investigations.

Lumbar epidural blood patch: An effective treatment for intracranial hypotension.

Background: The literature has demonstrated the efficacy of lumbar epidural blood patch (LEBP) in the management of spontaneous intracranial hypotension (SIH). However, the underlying pathophysiology of such management remains unclear. In this study, we aim to evaluate the utility of LEBP injections in the management of SIH and develop a potential management algorithm used in the triage and management of SIH patients. Methods: We retrospectively examined the clinical case notes of 14 patients with SIH (age: 25-69 years) who were managed with LEBP injections during the year of 2016-2021. We evaluated the presenting symptoms of each selected patient and radiological findings as well as treatment outcomes. Our aim is to evaluate the effectiveness of LEBP in the treatment of SIH patients through follow-up clinical and imaging assessment. Results: About 93% of patients describe the presence of headache at presentation, while 43% describe it as being of an orthostatic nature. All patients demonstrated typical findings on magnetic resonance imaging brain. Treatment success assessed through symptomatic improvement and radiological resolution was found in 85% of our patients at a 2-month interval. Conclusion: LEBP injection is an effective method of management in patients with a diagnosis of SIH. It should be considered in all SIH patients irrespective of whether a "dural leak" can be localized through radiological investigations.

Epidural Contrast Volume on Post-Myelogram Lumbar CT Predicts the Rate of Subsequent Blood Patch Treatment.

BACKGROUND AND PURPOSE: Headaches due to cerebrospinal fluid (CSF) leakage are a well-known complication of dural puncture. The purpose of this study was to determine whether the presence and volume of epidural contrast on postmyelogram CTs of the lumbar spine were associated with post-dural puncture headaches (PDPHs) requiring epidural blood patch (EBP) treatment. METHODS: A retrospective case control study of all fluoroscopically guided lumbar myelograms performed over a 5-year period by a single radiology practitioner assistant was performed. Ten patients who underwent EBP treatment after their myelograms were identified. Forty-six patients with similar demographics who did not receive blood patches were then selected. CT-lumbar myelogram images of patients and controls were reviewed. The volume of epidural contrast was then quantified as "severe" or "mild." Severe epidural contrast was defined as contrast detected in the ventral epidural space, the extra-foraminal space, or extending greater than or equal to the length of two vertebral bodies from the level of dural puncture. RESULTS: Some amount of epidural contrast was seen in all patients. However, a severe volume of epidural contrast was associated with increased risk for PDPH requiring an EBP (odds ratio = 37.00; 95% CI = 4.1-330.8, p = 0.0012). CONCLUSION: Severe epidural contrast on postmyelogram CTs of the lumbar spine was associated with an increased risk of PDPH requiring EBP treatment. When present, this finding can alert the proceduralist that the patient may require closer observation and follow up with earlier intervention.

Treatment of spontaneous intracranial hypotension: experiences in a UK regional neurosciences Centre.

A robust treatment paradigm for spontaneous intracranial hypotension has yet to be agreed upon. We present retrospective data from the patient cohort at our UK regional neurosciences centre from 2010-2020 and describe our locally developed treatment pathway.Seventy-three patients were identified: 31 men and 42 women; mean age was 42 years. The majority presented with a headache of variable duration, and most had positive imaging. Very few patients (7%) responded to conservative treatment. Sixty-six underwent epidural blood patching, with 39 (59%) having a good response. Twenty-three patients underwent myelography and targeted treatment (injection of fibrin sealant at the leak site), with 13 (57%) showing a good response. One patient had successful surgery. The relapse rate after response to epidural blood patching was 10%, and after response to targeted treatment was 23%. Most patients who relapsed responded to repeated treatments.The outcome data for our diverse patient cohort shows the success of a staged approach to treatment. Relapse rates are low, and surgery is only rarely required. We use these data to inform our discussions with patients, and present them here to enable other centres to develop robust investigation and treatment paradigms of their own.

Orthostatic Enlargement of a Supracerebellar Arachnoid Cyst With Cerebellar Descent Visualized by Upright Computed Tomography.

BACKGROUND: Posterior fossa arachnoid cysts are often asymptomatic, but can rarely cause postural headache, the mechanism of which remains unknown. CASE DESCRIPTION: We present a 40-year-old woman with an asymptomatic supracerebellar arachnoid cyst. Upright computed tomography (CT) showed enlargement of the arachnoid cyst and caudal descent of the cerebellum compared with supine CT with narrowing of the craniocervical junction cerebrospinal fluid space. CONCLUSIONS: This finding aids in understanding the association of posterior fossa arachnoid cysts with orthostatic headache. Clinicians should be aware of possible posture-related changes in intracranial structures.

Orthostatic Headaches Associated With Spontaneous Intracranial Hypotension and Autonomic Dysfunction-A Case Series in Young Patients.

Background: Orthostatic headaches can be noted in spontaneous intracranial hypotension and orthostatic intolerance. We present a case series of young patients diagnosed with spontaneous intracranial hypotension and were treated for the same but subsequently developed orthostatic intolerance. Methods: We retrospectively reviewed charts for seven young patients with orthostatic headaches related to spontaneous intracranial hypotension and orthostatic intolerance. Results: Patients were diagnosed with spontaneous intracranial hypotension. Diagnosis was confirmed by identifying epidural contrast leakage and three of seven patients were noted to have early renal contrast excretion on computerized tomography myelography. Patients were treated with epidural blood patches. All patients showed persistent symptoms of autonomic dysfunction after treatment of spontaneous intracranial hypotension and orthostatic intolerance was confirmed with head-up tilt table test. Conclusions: Patients with spontaneous intracranial hypotension failing to improve following epidural blood patching should be evaluated for orthostatic intolerance.

Stroke after spontaneous intracranial hypotension: Not a single mechanism. Case report and review of literature.

Spontaneous intracranial hypotension (SIH) is a rare syndrome, typically manifests as orthostatic headache. Sometimes considered asbenignillness, neurological complications are well described, in particular subdural hematoma and cerebral venous sinus thrombosis. Brain infarction as complication of SIH is rarely reported. The main mechanism supported in the literature is the stretching of arteries due to the sagging of the brain. We report a case of SIH followed with brain infarction, with a distinct presentation from previous literature, suggesting a different mechanism. A 35 year-old had severe orthostatic headache, responsible for prolonged bed rest. One month later, he had acute left hemiparesis secondary to stroke and right posterior cerebral artery occlusion. Stroke MRI showed arguments for intracranial hypotension (thickened meninges). He was successfully treated with intravenous rtPA thrombolysis. Headache were resolved after an epidural blood patch. A patent foramen ovale was detected. Clinical features of this description were compared with previous literature. This case suggest a different mechanism for cerebral infarction after intracranial hypotension. In case of prolonged lying down due to intracranial hypotension, the presence of patent foramen ovale could be a risk factor for embolic stroke.

An unusual postural headache: a case report.

BACKGROUND: This paper presents a case of an evolving unusual thunderclap headache that presented to a chiropractor. CASE PRESENTATION: The intense "migraine-like" headache was aggravated by standing up and relieved substantially when lying down. This low pressure, orthostatic headache was diagnosed as a spontaneous intracranial hypotension (SIH) secondary to a spontaneous tear of the dura. It was referred to the local hospital for management with autogolous blood injection to form an epidural blood patch of the defect. It resolved substantially within 3 days. CONCLUSIONS: The significance of key features in the history and examination and how if not recognised and subsequently treated with manual therapy, the dural tear could be attributed to the treatment of the chiropractor, a treatment that would typically involve cervical manipulation. Discussion is provided of the implications of a missed diagnosis and possible subsequent chiropractic management with the evolving SIH being attributed to the chiropractic intervention rather than its true "spontaneous" nature.

Penetrating Osseous Spicules Causing High-Flow Ventral CSF Leaks in the Setting of Relatively Low BMI : A Preliminary Study.

OBJECTIVE/BACKGROUND: We have anecdotally observed patients with high-flow ventral cerebrospinal fluid (CSF) leaks resulting from penetrating osseous spicules or calcified discs to be relatively thin. The purpose of this study was to explore the validity of this observation and determine if a potential association exists between low body mass index (BMI) and high-flow spinal ventral CSF leaks resulting from such dura-penetrating lesions. METHODS: Sixteen consecutive patients with precisely localized high-flow ventral spinal CSF leaks on dynamic myelography were identified. The cause of the CSF leak was determined. The BMI on the date nearest to and within 2 weeks of myelography was recorded. Utilizing exact sign test, the body mass index was compared to the average BMI from the National Health and Nutrition Examination Survey (Centers for Disease Control), matched to sex and age-range. RESULTS: The cohort consisted of 10 males (63%) and 6 females with a mean age of 54 years (range 37-72 years). In all patients, a spiculated osteophyte/calcified disc was identified at the site of the leak. Fourteen patients (88%) had a BMI below the matched national average, while only two patients (13%) had values above the national average (p = 0.004). CONCLUSIONS: Patients with high-flow ventral CSF leaks resulting from spiculated osteophyte or calcified disc as identified by dynamic myelography are more likely to have a BMI below the U.S. national average, matched for gender and age-range. This exploratory analysis requires confirmation as well as further characterization of potential pathophysiologic mechanisms and impact on radiographic and clinical assessments.

Diagnosis and treatment of spontaneous intracranial hypotension due to cerebrospinal fluid leakage.

INTRODUCTION: Spontaneous intracranial hypotension is one of the causes of a postural headache in young people. In this study, the diagnosis and treatment results of a case of intracranial hypotension headache due to spinal cerebrospinal fluid leakage were reported. Up to now, there is not absolutely effective treatment for intracranial hypotension headache. CASE DESCRIPTION: A 32-year-old woman complained, a headache after prolonged sitting that presented with nausea; vomiting; increased pain during walking; and decreased or absent pain after lying down. The dramatic improvement of this cephalalgia with epidural blood patch treatment confirmed the diagnosis. DISCUSSION AND EVALUATION: To the best of our knowledge, this is the first reported of radiographic contrast before and after epidural blood patch. Improved clinical diagnosis and treatment of spontaneous intracranial hypotension. The patient didn't feel any discomfort, no complications such as infection etc. were observed. A small dose of intrathecal gadolthis is the first reported case ofinium during CEMRM allows for improved detection of CSF leakage. CONCLUSIONS: Leakage of spinal CSF is a major cause of spontaneous intracranial hypotension. In order to improve clinical diagnosis and provide effective treatment, the precise etiology of spontaneous intracranial hypotension should be investigated in each patient.

Intracranial hypotension secondary to spinal pathology: Diagnosis and treatment.

Spinal pathology resulting in cerebrospinal fluid (CSF) leak and intracranial hypotension is an infrequently reported and a potentially severe cause of headaches. We present a case of cerebrospinal fluid (CSF) leak caused by a thoracic disk herniation successfully treated with two targeted epidural blood patches. Although patients typically present with orthostatic headaches, the imaging findings of intracranial hypotension should prompt investigation of the spine for site and cause of the CSF leakage. Treatment includes autologous blood patch and surgery in refractory cases.

Targeted epidural catheter injection of polyethylene glycol hydrogel for repair of a spontaneous dural tear: a novel case report.

The authors describe the novel approach of using a blunt-tipped catheter to repair a spontaneous dural tear. This case shows an effective percutaneous method of delivering dural sealant through an easily positioned catheter for the treatment of intracranial hypotension due to spontaneous CSF leakage.