Management Of Spontaneous Intracranial Hypotension Research Articles

Glucagon-Like Peptide-1 Analogue In The Management Of Rebound Intracranial Hypertension: A Case Report

Background: Rebound intracranial hypertension (RIH) is a possible complication of epidural blood patching used in the management of spontaneous intracranial hypotension (SIH). RIH has been reported to occur in up to 27% of patients after SIH treatment and is characterized by elevated intracranial pressure, resulting in severe headache, nausea, and vomiting. Although it typically resolves spontaneously, treatment may be warranted for symptom control. While acetazolamide is the most commonly used agent for RIH, there are side-effects, and its efficacy remains inconclusive. Recently, novel glucagon-like peptide-1 (GLP-1) analogues have been found to modulate CSF secretion and consequently reduce intracranial pressure. However, there are no studies that have evaluated the use of these agents in the treatment of RIH. We report of a case of a 46 year old female patient with persistent RIH after CSF leak repair that was refractory to pharmacologic and interventional treatments. The patient was treated with Semaglutide, an oral GLP-1 analogue and experienced immediate and sustained benefit of her symptoms. Conclusion: RIH is a common complication after successful patching of a CSF leak, a proportion of patients suffer from persistent and debilitating headaches. Our case report showed that GLP-1 agonist Semaglutide appeared to reduce symptoms in this one patient with refractory RIH symptoms. The use of GLP-1 agonists in the treatment of RIH should be evaluated in controlled studies to establish its safety and efficacy in this population.

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

BackgroundWe 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...

Abstract No. 183 Embolization of Spinal CSF Dural-Venous Fistulae for Management of Spontaneous Intracranial Hypotension

Abstract No. 183 Embolization of Spinal CSF Dural-Venous Fistulae for Management of Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension: from cerebral venous thrombosis to neurosurgical treatment: a case series experience from a Brazilian tertiary health care center.

Spontaneous intracranial hypotension (SIH) is a secondary cause of headache and an underdiagnosed disease. The clinical presentation can be highly variable. It typically presents with isolated classic orthostatic headache complaints, but patients can develop significant complications such as cerebral venous thrombosis (CVT). To report 3 cases of SIH diagnosis admitted and treated in a tertiary-level neurology ward. Review of the medical files of three patients and description of clinical and surgical outcomes. Three female patients with SIH with a mean age of 25.6 ± 10.0 years old. The patients had orthostatic headache, and one of them presented with somnolence and diplopia because of a CVT. Brain magnetic resonance imaging (MRI) ranges from normal findings to classic findings of SIH as pachymeningeal enhancement and downward displacement of the cerebellar tonsils. Spine MRI showed abnormal epidural fluid collections in all cases, and computed tomography (CT) myelography could determine an identifiable cerebrospinal fluid (CSF) leak in only one patient. One patient received a conservative approach, and the other two were submitted to open surgery with laminoplasty. Both of them had uneventful recovery and remission in surgery follow-up. The diagnosis and management of SIH are still a challenge in neurology practice. We highlight in the present study severe cases of incapacitating SIH, complication with CVT, and good outcomes with neurosurgical treatment.

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

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. 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. 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. 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.

Survey of healthcare professionals’ knowledge, attitudes and practices regarding spontaneous intracranial hypotension

ObjectiveTo assess the knowledge, attitudes and practices of healthcare professionals regarding the diagnosis and management of spontaneous intracranial hypotension (SIH).MethodsWe performed a cross-sectional, web-based survey of multiple healthcare professional groups...

P.195 Management of spontaneous intracranial hypotension in pregnancy: a case series

P.195 Management of spontaneous intracranial hypotension in pregnancy: a case series

Epidural Blood Patch Using a Racz Catheter for Spontaneous Intracranial Hypotension With Unclear Leak Points

Using a Racz catheter (Brevi-XL™, Epimed Inc., NY, USA) to insert an epidural blood patch (EBP) may be an effective method of reaching the target epidural space in the cervical region. We would like to present a case, wherein a targeted EBP via Racz catheter was used in the management of spontaneous intracranial hypotension. When the leak point is clear via imaging, EBP should be performed exactly at that point. However, if the leak point is unclear, with only a contrast agent pool detected via imaging, EBP should be performed to mask the entire region of the pool. In both cases, EBP via Racz catheter is a convenient and effective method for the management of spontaneous intracranial hypotension. Further cases may be needed to verify our results.

Patient experience of diagnosis and management of spontaneous intracranial hypotension: a cross-sectional online survey

ObjectivesTo present the results of a survey of patients with spontaneous intracranial hypotension (SIH) secondary to spinal cerebrospinal fluid (CSF) leak, documenting the patient experience of its diagnosis and management...

Interdisciplinary Diagnosis and Management of Cerebrospinal Fluid Leakage Due to a Meningeal Diverticulum: a Case Report

Cerebrospinal fluid leakage through meningeal diverticula represents a diagnostic and therapeutic challenge comparatively rarely encountered in the interdisciplinary management of spontaneous intracranial hypotension (SIH). Several false-positive CSF leakage signs may be observed during the imaging work-up of SIH. A 27-year-old female with orthostatic headache showing marked spinal epidural CSF collections and MRI signs of intracranial hypotension underwent a blind and CT-guided epidural blood patch (EBP) of a pathological T9/10 meningeal diverticulum (MD), detected by dynamic CT myelography (dCTM). After initial good imaging and symptomatic improvement, recurrent symptoms and a large left-sided subdural hematoma required neurosurgical MD ligation, with persisting clinical success. The following aspects of this brief report are remarkable: added value of dCTM to synchronously detect true CSF leakage and false-positive CSF leakage signs, near-complete resolution of spinal epidural CSF collections after CT fluoroscopy–guided EBP, interdisciplinary diagnosis, and definite management of CSF leakage through an anomalous MD.

Management of spontaneous intracranial hypotension: a series of 31 cases over 15-years with a challenging outlier

Spontaneous intracranial hypotension (SIH) has been classified as a triad of postural headache, low CSF opening pressure (below 60mmH20) and diffuse pachymeningeal gadolinium enhancement on MRI. SIH is due to a non-iatrogenic defect in the dura somewhere along the neuraxis (usually in the spine). The resultant leak depressurizes the system and undermines the buoyancy-providing quality of CSF. In many cases the site of leak is not identified. Epidural blood patch (EBP) is a well-established treatment of SIH but is not always effective. We retrospectively analysed thirty-one cases of SIH who required at least one EBP. They were managed over a fifteen-year period at a single institution. EBP resulted in a significant improvement in headache for 77% of patients. We report in more detail on a particularly challenging outlier where severe SIH resulted in coma. Serial epidural blood patches, burr hole evacuation of chronic subdural haematomata, and infusion of 0.9% NaCl via an intradural spinal catheter were all required to enable long-term recovery.

075 A case of spontaneous CSF rhinorrhea in a patient with marfan syndrome

IntroductionSpontaneous cerebrospinal fluid leak is uncommon condition and frequently associated with hereditary disorders of connective tissues. Nasal CSF leakage is extremely rare.1Methods and resultsWe present the case of a 40-year-old woman presented to hospital for few days history of postural headache associated with clear intermittent discharge from right nostril without any signs of meningism. There was no history of trauma. She has a background history of Marfan syndrome with associated complications of ASD repair at age 2, mechanical Aortic and Mitral valve replacement, aortic root repair, previous ST elevation MI with LV dysfunction, automated implantable cardioverter-defibrillator in situ, atrial fibrillation, and Hashimoto’s thyroiditis. Her regular medications are warfarin, bisoprolol and thyroxine. The clear nasal discharge was positive for β-2 transferrin confirming cerebrospinal fluid. Her CT Brain did not reveal any clear site of CSF leak. She had a flexible nasendoscopy which showed normal nasal passageway, no defect in nasal mucosa and no active CSF leakage. She was managed conservatively with strict bed rest and advised to minimise strenuous activity and heavy lifting.ConclusionSpontaneous cerebrospinal fluid leak is uncommon condition and frequently associated with hereditary disorders of connective tissues. Nasal CSF leakage is extremely rare.1 Testing β-2 transferrin has high sensitivity and specificity.2Initial treatment may include bed rest, oral or intravenous hydration, oral caffeine or corticosteroids.3 4 If conservative therapy fails, surgical repair with nasal endoscopic approach is recommended.2 5ReferencesOmmaya A, Di Chiro G, Baldwin M, Pennybacker J. Non-traumatic cerebrospinal fluid rhinorrhoea. Journal of Neurology, Neurosurgery & Psychiatry 1968;31(3):214-–225.Wang E, Vandergrift W, Schlosser R. Spontaneous CSF Leaks. Otolaryngologic Clinics of North America 2011;44(4):845–856.Milledge J, Ades L, Cooper M, Jaumees A, Onikul E. Severe spontaneous intracranial hypotension and Marfan syndrome in an adolescent. Journal of Paediatrics and Child Health 2005;41(1–2):68–71.Placantonakis D, Bassani L, Graffeo C, Behrooz N, Tyagi V, Wilson T, et al. Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature. Surgical Neurology International 2014;5(1):8.Spontaneous cerebrospinal fluid rhinorrhea. Medicine 2018;97(7):e9954.

A Review of Spontaneous Intracranial Hypotension.

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. 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.

Management of spontaneous intracranial hypotension – Transorbital ultrasound as discriminator

ObjectiveSpontaneous intracranial hypotension (SIH) is most commonly caused by cerebrospinal fluid (CSF) leakage. Therefore, we hypothesised that patients with orthostatic headache (OH) would show decreased optic nerve sheath diameter (ONSD)...

Localization of Cerebrospinal Fluid Leak in a Case of Spontaneous Intracranial Hypotension Using Dynamic Computed Tomography Myelography

EPIDURAL blood patch is the mainstay of treatment for persistent symptoms secondary to spontaneous intracranial hypotension.1 Determining the level of cerebrospinal fluid leak is thought to be an important consideration for optimal siting of the epidural blood patch injection.2 However, it is often difficult to determine the level of cerebrospinal fluid leak in spontaneous intracranial hypotension. “Dynamic” (as opposed to conventional) computed tomography myelography has recently emerged as a preferred diagnostic test for high-flow (“fast”) cerebrospinal fluid leaks, suspected by extra-arachnoid fluid on spinal magnetic resonance imaging.3 With dynamic computed tomography myelography, myelographic contrast is injected with the patient in the computed tomography scanner (tilted Trendelenburg using a wedge), followed by immediate and multiple computed tomography acquisitions.A 33-yr-old male patient presented to the emergency department with severe occipital headache. Computed tomography of the head demonstrated a chronic subdural hematoma. Head contrast magnetic resonance imaging showed diffuse dural gadolinium enhancement and a “sagging” brain. Spine magnetic resonance imaging revealed circumferential extradural fluid (“scattered” in cervical and “diffuse” in thoracic and lumbar regions). Surgical drainage of the subdural hematoma was performed. On postoperative day 1, headache symptoms were improved with no residual hematoma on repeated computed tomography of the head.However, persistent postural headaches prompted consultation of the acute pain service for evaluation and management of spontaneous intracranial hypotension. Despite lumbar epidural blood patch followed by 2 days of bed rest, headache symptoms returned on upright positioning. Dynamic computed tomography myelography performed on postoperative day 6 showed a high-flow cerebrospinal fluid leak between T4 and T5 (arrow in fig.), secondary to a midline spiculated osteophyte (arrow). Using fluoroscopic guidance, an epidural blood patch was performed between T3 and T4 (14 ml). Headache symptoms resolved and had not recurred at 12-month follow-up.The author declares no competing interests.

Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature

Background:Spontaneous intracranial hypotension is an uncommon clinical entity. Heritable connective tissue disorders (HCTD), such as Marfan syndrome, are frequently implicated as an underlying cause, due to dural structural weaknesses that predispose patients to spontaneous cerebrospinal fluid (CSF) leak. Due to the high prevalence of multi-system disease in HCTD, diagnosis and treatment are often complicated.Case Description:We present a 58-year-old female with Marfan syndrome on anticoagulation for a mechanical aortic valve replacement who came to medical attention with severe, acute-onset headache following a straining episode. Noninvasive magnetic resonance (MR) myelography confirmed thoracic CSF extravasations and multiple lumbar diverticula. The patient was treated conservatively and her symptoms resolved.Conclusion:We discuss the common presentation, diagnostic tools, and treatment options for spontaneous CSF leaks in patients with Marfan syndrome or related HCTD with an emphasis on noninvasive modalities and a review of the major radiographic criteria used to diagnose dural abnormalities, such as dural ectasia.

Spontaneous Intracranial Hypotension: Recommendations for Management

A literature search found no clinical trials or guidelines addressing the management of spontaneous intracranial hypotension (SIH). Based on the available literature and expert opinion, we have developed recommendations for the diagnosis and management of SIH. For typical cases, we recommend brain magnetic resonance (MR) imaging with gadolinium to confirm the diagnosis, and conservative measures for up to two weeks. If the patient remains symptomatic, up to three non-directed lumbar epidural blood patches (EBPs) should be considered. If these are unsuccessful, non-invasive MR myelography, radionuclide cisternography, MR myelography with intrathecal gadolinium, or computed tomography with myelography should be used to localize the leak. If the leak is localized, directed EPBs should be considered, followed by fibrin sealant or neurosurgery if necessary. Clinically atypical cases with normal brain MR imaging should be investigated to localize the leak. Directed EBPs can be used if the leak is localized; non-directed EBPs should be used only if there are indirect signs of SIH.

Management of Spontaneous Intracranial Hypotension With Subdural Hematoma

Management of Spontaneous Intracranial Hypotension With Subdural Hematoma

CT-Guided Epidural Blood Patching of Directly Observed or Potential Leak Sites for the Targeted Treatment of Spontaneous Intracranial Hypotension

Optimal diagnosis and management of spontaneous intracranial hypotension remains uncertain. CT-guided blood patching has been described but has not been evaluated in larger case series. We sought to evaluate the efficacy of CT-guided blood patching of observed or potential CSF leaks in spontaneous intracranial hypotension. Patients referred for evaluation of spontaneous intracranial hypotension were retrospectively reviewed. Inclusion criteria were findings of intracranial hypotension on pretreatment brain MR imaging, evaluation and treatment with CT-guided myelography and blood patching, and availability of posttreatment brain MR images. Eight patients met inclusion criteria. Imaging findings, treatment details, and clinical outcomes were assessed. Pretreatment imaging findings included dural enhancement, tonsillar ectopia, subdural collections, and syrinx. All findings resolved or significantly improved on posttreatment imaging. Presenting clinical symptoms included positional headache, neck/interscapular/shoulder pain, and tinnitus. Headaches and neck/interscapular/shoulder pain improved in all patients; tinnitus improved in 1 of 2 patients. CSF leak sites were directly visualized in 37% of patients and were targets for patching when seen. When no direct visualization of leaks was seen, irregular spinal nerve root diverticula were targeted as potential leak sites. The average number of blood patching sessions was 3 (range, 1-6) and the average number of individual sites patched per session was 5 (range, 1-10). Our results suggest that CT-guided blood patching targeting observed or potential leak sites can be effective in the treatment of intracranial hypotension. Prospective controlled studies are needed to confirm efficacy and compare outcomes with other treatment options.

Spontaneous Intracranial Hypotension Treated by Epidural Blood Patches

We present a case of spontaneous intracranial hypotension (SIH) diagnosed from the clinical symptoms and magnetic resonance imaging brain scans. After failure of conservative treatment, and lack of identification of the cerebrospinal fluid leak site, the headache was managed successfully and simply with two applications of an epidural blood patch (EBP). The strategy of our management for the patient was as follows: (1) application of an EBP to the lumbar epidural space initially and manipulating it into the proximity of the possible leak site; (2) a greater volume of autologous blood was injected at the second attempt of EBP; and (3) the patient was required to lie flat for at least 2 hours after the procedure. Better management of SIH is still developing. Traditionally, SIH is initially managed by conservative treatment. However, recent studies have shown that the success rate in arresting SIH after weeks or months of conservative treatment is not quite satisfactory. Hence, the EBP has proven to be more effective in treating SIH patients. Early EBP application may offer immediate relief of clinical symptoms. The role of the EBP in treating SIH patients should have greater emphasis and its application is worth recommendation.