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Marc-Antoine M, Simon H. Role of CT myelography in the diagnosis and management of spontaneous intracranial hypotension. Clin Neurol Neurosurg 2025; 249:108707. [PMID: 39740334 DOI: 10.1016/j.clineuro.2024.108707] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2024] [Revised: 12/22/2024] [Accepted: 12/22/2024] [Indexed: 01/02/2025]
Abstract
INTRODUCTION Spontaneous intracranial hypotension (SIH) is a secondary cause of headache. Its pathophysiology is complex, and relies mainly on the notion of a localized leak, and thus a loss of cerebrospinal fluid (CSF) in the spinal region. SIH is little known to the medical profession, for which CT myelography is a technique on the rise, allowing to identify and treat the leak using a blood-patch or a fibrin sealant. The aim of the study was to investigate the contribution of CT myelography in the diagnostic and therapeutic management of spontaneous intracranial hypotension, its modalities and population characteristics. MATERIAL AND METHODS A cohort of patients with spontaneous intracranial hypotension who underwent a CT myelography in our department was retrospectively studied. Nineteen patients were included between March 2020 and October 2022. RESULTS CT myelography allowed identification of an etiology in 16 patients (84 %). The etiology of the leak was mainly a dural tear, identified in 11 patients. One diverticulum and 4 CSF-venous fistulas were identified. First targeted injection of fibrin sealant allowed stopped the leak in 12 out of 17 patients. CONCLUSION CT myelography allows to identify the etiology of the CSF leak and pinpoint its topography. CT guidance can then be used to treat the leak in the same therapeutic timeframe, with greater efficacy during the first targeted injection of fibrin sealant. The current study data showed a high incidence of dural tears, and a significant proportion of venous-dural fistulas, and a low incidence of diverticulum.
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Affiliation(s)
| | - Henry Simon
- CHU de Lille, 2 Avenue Oscar Lambret, Hauts-de-France, France.
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Callen AL, Han L, Pisani Petrucci SL, Andonov N, Lennarson P, Birlea M, O'Brien C, Wilhour D, Anderson A, Bennett JL, Carroll IR. Patterns of clinical and imaging presentations in patients with spontaneous intracranial hypotension due to spinal cerebrospinal fluid venous fistula: A single-center retrospective cross-sectional study. Headache 2024; 64:939-949. [PMID: 39129307 DOI: 10.1111/head.14805] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2024] [Revised: 06/29/2024] [Accepted: 07/01/2024] [Indexed: 08/13/2024]
Abstract
OBJECTIVE To identify distinct clinical or imaging subtypes of spontaneous intracranial hypotension (SIH) due to spinal cerebrospinal fluid (CSF) venous fistula (CVF). BACKGROUND Spontaneous intracranial hypotension is classically understood to present clinically with an orthostatic headache and stereotyped brain magnetic resonance imaging (MRI) findings; however, most prior literature examining clinical and brain MRI features of SIH has focused on all types of spinal CSF leaks concurrently. This study aimed to evaluate whether data support the possibility of internally consistent subtypes based on brain imaging features and clinical symptoms analogous to those seen in primary headache syndromes. METHODS This retrospective cross-sectional single-institution study included 48 consecutive patients meeting the International Classification of Headache Disorders, 3rd edition criteria for SIH due to CVF. Clinical symptoms, pre-treatment brain MRI, and symptom duration were analyzed. Clinical and MRI data were analyzed to identify patterns and associations between symptoms and imaging findings. RESULTS A total of 20 males and 28 females were evaluated, with a mean (standard deviation) age of 61 (10) years. In all, 44/48 (92%) patients experienced headaches, though 18/48 (40%) did not endorse relief when flat, including six of the 48 (13%) with worsening symptoms when flat. In all, 19/48 (40%) patients reported at least one migraine symptom, and six of the 48 (13%) presented with at least one migraine symptom and had no relief when flat. Clinical symptoms clustered primarily into a "classic" presentation consisting of relief when flat, occipital head pain, comorbid neck pain, a pressure/throbbing headache quality, and an "atypical" presentation that was characterized by having several differences: less relief when flat (nine of 22 (41%) vs. 20/23 (87.0%), p = 0.002; odds ratio [OR] 0.110, 95% confidence interval [CI] 0.016-0.53), more frontal head pain (14/22 (64%) vs. one of 23 (4%), p < 0.001; OR 35.0, 95% CI 4.2-1681.0), less neck pain (two of 21 (4.5%) vs. nine of 13 (69.6%), p < 0.001; OR 0.023, 95% CI 0.0005-0.196), and more stabbing/sharp headache quality (nine of 22 (41%) vs. two of 23 (9%), p = 0.017; OR 7.0, 95% CI 1.18-75.9). Brain MRI findings clustered into three groups: those presenting with most imaging findings of SIH concurrently, those with brain sag but less pachymeningeal/venous engorgement, and those with pachymeningeal/venous engorgement but less brain sag. CONCLUSION This study highlights the clinical and imaging diversity among patients with SIH due to CVF, challenging the reliance on classic orthostatic headache alone for diagnosis. The findings suggest the existence of distinct SIH subtypes based on clinical and imaging presentations, underscoring the need for comprehensive evaluation in patients with suspected CVF. Future research should further elucidate the relationship between clinical symptoms and imaging findings, aiming to refine diagnostic criteria and enhance understanding of SIH's pathophysiology.
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Affiliation(s)
- Andrew L Callen
- Department of Radiology, Neuroradiology Section, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Lichy Han
- Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University, Stanford, California, USA
| | - Samantha L Pisani Petrucci
- Department of Radiology, Neuroradiology Section, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Nadya Andonov
- Department of Radiology, Neuroradiology Section, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Peter Lennarson
- Department of Neurosurgery, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Marius Birlea
- Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Chantal O'Brien
- Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Danielle Wilhour
- Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Abigail Anderson
- Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Jeffrey L Bennett
- Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
- Programs in Neuroscience and Immunology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
- Department of Ophthalmology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Ian R Carroll
- Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University, Stanford, California, USA
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El Rahal A, Haupt B, Fung C, Cipriani D, Häni L, Lützen N, Dobrocky T, Piechowiak E, Schnell O, Raabe A, Wolf K, Urbach H, Kraus LM, Volz F, Beck J. Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis. Eur J Neurol 2024; 31:e16122. [PMID: 38015455 PMCID: PMC11235863 DOI: 10.1111/ene.16122] [Citation(s) in RCA: 7] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2023] [Revised: 10/13/2023] [Accepted: 10/16/2023] [Indexed: 11/29/2023]
Abstract
BACKGROUND AND PURPOSE Spinal cerebrospinal fluid (CSF) leaks may cause a myriad of symptoms, most common being orthostatic headache. In addition, ventral spinal CSF leaks are a possible etiology of superficial siderosis (SS), a rare condition characterized by hemosiderin deposits in the central nervous system (CNS). The classical presentation of SS involves ataxia, bilateral hearing loss, and myelopathy. Unfortunately, treatment options are scarce. This study was undertaken to evaluate whether microsurgical closure of CSF leaks can prevent further clinical deterioration or improve symptoms of SS. METHODS This cohort study was conducted using data from a prospectively maintained database in two large spontaneous intracranial hypotension (SIH) referral centers in Germany and Switzerland of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. Patients with spinal CSF leaks were screened for the presence of idiopathic infratentorial symmetric SS of the CNS. RESULTS Twelve patients were included. The median latency between the onset of orthostatic headaches and symptoms attributed to SS was 9.5 years. After surgical closure of the underlying spinal CSF leak, symptoms attributed to SS improved in seven patients and remained stable in three. Patients who presented within 1 year after the onset of SS symptoms improved, but those who presented in 8-12 years did not improve. We could show a significant association between patients with spinal longitudinal extrathecal collections and SS. CONCLUSIONS Long-standing untreated ventral spinal CSF leaks can lead to SS of the CNS, and microsurgical sealing of spinal CSF leaks might stop progression and improve symptoms in patients with SS in a time-dependent manner.
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Affiliation(s)
- Amir El Rahal
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
- Department of Neurosurgery, Faculty of Medicine of GenevaGeneva University HospitalGenevaSwitzerland
| | - Benedikt Haupt
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
| | - Christian Fung
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
| | - Debora Cipriani
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
| | - Levin Häni
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
- Department of Neurosurgery, Inselspital, Bern University HospitalUniversity of BernBernSwitzerland
| | - Niklas Lützen
- Department of Diagnostic and Interventional NeuroradiologyUniversity Medical Center FreiburgFreiburgGermany
| | - Tomas Dobrocky
- Institute of Diagnostic and Interventional Neuroradiology, Inselspital, Bern University HospitalUniversity of BernBernSwitzerland
| | - Eike Piechowiak
- Institute of Diagnostic and Interventional Neuroradiology, Inselspital, Bern University HospitalUniversity of BernBernSwitzerland
| | - Oliver Schnell
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
| | - Andreas Raabe
- Department of Neurosurgery, Inselspital, Bern University HospitalUniversity of BernBernSwitzerland
| | - Katharina Wolf
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
| | - Horst Urbach
- Department of Diagnostic and Interventional NeuroradiologyUniversity Medical Center FreiburgFreiburgGermany
| | - Luisa Mona Kraus
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
| | - Florian Volz
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
| | - Jürgen Beck
- Department of NeurosurgeryUniversity Medical Center FreiburgFreiburgGermany
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Jesse CM, Schär RT, Goldberg J, Fung C, Ulrich CT, Dobrocky T, Piechowiak EI, Schankin CJ, Beck J, Raabe A, Häni L. Patient-reported symptomatology and its course in spontaneous intracranial hypotension - Beware of a chameleon. Clin Neurol Neurosurg 2024; 236:108087. [PMID: 38134757 DOI: 10.1016/j.clineuro.2023.108087] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2023] [Revised: 12/15/2023] [Accepted: 12/15/2023] [Indexed: 12/24/2023]
Abstract
OBJECTIVE Although orthostatic headache is the hallmark symptom of spontaneous intracranial hypotension (SIH), patients can present with a wide range of different complaints and thereby pose a diagnostic challenge for clinicians. Our aim was to describe and group the different symptoms associated with SIH and their course over time. METHODS We retrospectively surveyed consecutive patients diagnosed and treated for SIH at our institution from January 2013 to May 2020 with a specifically designed questionnaire to find out about their symptomatology and its course. RESULTS Of 112 eligible patients, 79 (70.5%) returned the questionnaire and were included in the analysis. Of those, 67 (84.8%) reported initial orthostatic headaches, whereas 12 (15.2%) denied having this initial symptom. All except one (98.7%) patients reported additional symptoms: most frequently cephalic pressure (69.6%), neck pain (68.4%), auditory disturbances (59.5%), nausea (57%), visual disturbances (40.5%), gait disturbance (20.3%), confusion (10.1%) or sensorimotor deficits (21.5%). Fifty-seven (72.2%) patients reported a development of the initial symptoms predominantly in the first three months after symptom onset. Age and sex were not associated with the symptomatology or its course (p > 0.1). CONCLUSION Although characteristic of SIH, a relevant amount of patients present without orthostatic headaches. In addition, SIH can manifest with non-orthostatic headaches at disease onset or during the course of the disease. Most patients report a wide range of associated complaints. A high degree of suspicion is crucial for an early diagnosis and targeted treatment.
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Affiliation(s)
- Christopher Marvin Jesse
- Department of Neurosurgery, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland.
| | - Ralph T Schär
- Department of Neurosurgery, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland
| | - Johannes Goldberg
- Department of Neurosurgery, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland
| | - Christian Fung
- Department of Neurosurgery, Medical Center - University of Freiburg, Freiburg, Germany; Department of Neurosurgery, Lindenhofspital, Bern, Switzerland
| | | | - Tomas Dobrocky
- Institute of Diagnostic and Interventional Neuroradiology, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland
| | - Eike Immo Piechowiak
- Institute of Diagnostic and Interventional Neuroradiology, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland
| | - Christoph J Schankin
- Department of Neurology, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland
| | - Jürgen Beck
- Department of Neurosurgery, Medical Center - University of Freiburg, Freiburg, Germany
| | - Andreas Raabe
- Department of Neurosurgery, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland
| | - Levin Häni
- Department of Neurosurgery, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland
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Choi SH, Lee YY, Kim WJ. Epidural blood patch for spontaneous intracranial hypotension with subdural hematoma: A case report and review of literature. World J Clin Cases 2022; 10:388-396. [PMID: 35071543 PMCID: PMC8727273 DOI: 10.12998/wjcc.v10.i1.388] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/07/2021] [Revised: 10/23/2021] [Accepted: 11/21/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Cerebrospinal fluid (CSF) leakage at C1/2 in spontaneous intracranial hypotension (SIH) is rare. Subdural hematoma (SDH), a serious complication of SIH, may lead to neurological deficits. This report presents a case of SDH after spontaneous C1/2 CSF leakage, which was treated with a targeted epidural blood patch (EBP).
CASE SUMMARY A 60-year-old man with no history of trauma was admitted to our hospital with orthostatic headache, nausea, and vomiting. Brain computed tomography imaging revealed bilateral, subacute to chronic SDH. Brain magnetic resonance imaging (MRI) findings were SDH with dural enhancement in the bilateral cerebral convexity and posterior fossa and mild sagging, suggesting SIH. Although the patient underwent burr hole trephination, the patient’s orthostatic headache was aggravated. MR myelography led to a suspicion of CSF leakage at C1/2. Therefore, we performed a targeted cervical EBP using an epidural catheter under fluoroscopic guidance. At 5 d after EBP, a follow-up MR myelography revealed a decrease in the interval size of the CSF collected. Although his symptoms improved, the patient still complained of headaches; therefore, we repeated the targeted cervical EBP 6 d after the initial EBP. Subsequently, his headache had almost disappeared on the 8th day after the repeated EBP.
CONCLUSION Targeted EBP is an effective treatment for SDH in patients with SIH due to CSF leakage at C1/2.
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Affiliation(s)
- Se Hee Choi
- Anesthesiology and Pain Medicine, Ewha Womans University Mokdong Hospital, Seoul 07985, South Korea
| | - Youn Young Lee
- Anesthesiology and Pain Medicine, School of Medicine, Ewha Womans University, Seoul 07985, South Korea
| | - Won-Joong Kim
- Anesthesiology and Pain Medicine, School of Medicine, Ewha Womans University, Seoul 07985, South Korea
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Chen ST, Wu JW, Wang YF, Lirng JF, Hseu SS, Wang SJ. The time sequence of brain MRI findings in spontaneous intracranial hypotension. Cephalalgia 2021; 42:12-19. [PMID: 34579563 DOI: 10.1177/03331024211044424] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
OBJECTIVES To investigate the time sequence of brain magnetic resonance imaging findings of spontaneous intracranial hypotension. METHODS We retrospectively reviewed the medical records and brain magnetic resonance imaging findings of consecutive patients with spontaneous intracranial hypotension hospitalized between January 2007 and December 2017. Patients were divided into quartiles based on intervals between initial spontaneous intracranial hypotension symptom onset and brain magnetic resonance imaging scan. Six categorical and five continuous brain magnetic resonance imaging findings were assessed, including venous distension sign, enlarged pituitary gland, diffuse pachymeningeal enhancement, mid-brain pons deformity, subdural fluid collection, flattening of pons, midbrain-pons angle, descent of cerebral aqueduct, mamillopontine distance, distance of suprasellar cistern, and distance of prepontine cistern. In addition, we also calculated the neuroimaging scores with a score ≥5 classified as 'high probability of spontaneous intracranial hypotension' and a score ≥3 as 'intermediate-to-high probability.' Then, we analyzed the linkage between the onset-neuroimaging interval and brain magnetic resonance imaging findings, as well as different neuroimaging scores. RESULTS A total of 173 patients (57 males and 116 females) were included in the analysis, and the range of onset-neuroimaging interval was 1 to 89 days (median [interquartile range] = 17 [7 to 30 days]). We divided the patients into quartiles based on their onset-neuroimaging interval (the first quartile: 0-6 days; the second quartile: 7-16 days; the third quartile: 17-29 days; the fourth quartile: ≥30 days). Among brain magnetic resonance imaging findings, the incidence of venous distension sign was high (>75%), with no difference among quartiles (p = 0.876). The incidence of diffuse pachymeningeal enhancement (p = 0.001), severe midbrain-pons deformity (p = 0.001), and subdural fluid collection (<0.001) followed a significant stepwise increase from the first quartile to fourth quartile. Patients with shorter onset-neuroimaging intervals were less likely to have neuroimaging scores ≥5 (<17 vs. ≥17 days: 72.9% vs. 86.4%; odds ratio = 2.3 [95% CI 1.1-5.1], p = 0.028), but not neuroimaging scores ≥3 (<17 vs. ≥17 days: 92.9% vs. 92.0%, p = 0.824). CONCLUSIONS The emergence of brain magnetic resonance imaging findings of spontaneous intracranial hypotension depended on disease duration and appeared sequentially. When using brain magnetic resonance imaging findings or neuroimaging scores for diagnostic purposes, the onset-neuroimaging interval should be considered.
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Affiliation(s)
- Shu-Ting Chen
- Department of Radiology, 46615Taipei Veterans General Hospital, Taipei Veterans General Hospital, Taipei, Taiwan.,College of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan
| | - Jr-Wei Wu
- College of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Neurology, Neurological Institute, 46615Taipei Veterans General Hospital, Taipei Veterans General Hospital, Taipei, Taiwan.,Department of Medical Education, Taipei Veterans General Hospital, Taipei, Taiwan
| | - Yen-Feng Wang
- College of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Neurology, Neurological Institute, 46615Taipei Veterans General Hospital, Taipei Veterans General Hospital, Taipei, Taiwan
| | - Jiing-Feng Lirng
- Department of Radiology, 46615Taipei Veterans General Hospital, Taipei Veterans General Hospital, Taipei, Taiwan.,College of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan
| | - Shu-Shya Hseu
- College of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Anaesthesiology, Taipei Veterans General Hospital, Taipei, Taiwan
| | - Shuu-Jiun Wang
- College of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Neurology, Neurological Institute, 46615Taipei Veterans General Hospital, Taipei Veterans General Hospital, Taipei, Taiwan.,Brain Research Center, National Yang Ming Chiao Tung University, Taipei, Taiwan
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Luetzen N, Dovi-Akue P, Fung C, Beck J, Urbach H. Spontaneous intracranial hypotension: diagnostic and therapeutic workup. Neuroradiology 2021; 63:1765-1772. [PMID: 34297176 PMCID: PMC8528761 DOI: 10.1007/s00234-021-02766-z] [Citation(s) in RCA: 54] [Impact Index Per Article: 13.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2021] [Accepted: 07/04/2021] [Indexed: 11/17/2022]
Abstract
Spontaneous intracranial hypotension (SIH) is an orthostatic headache syndrome with typical MRI findings among which engorgement of the venous sinuses, pachymeningeal enhancement, and effacement of the suprasellar cistern have the highest diagnostic sensitivity. SIH is in almost all cases caused by spinal CSF leaks. Spinal MRI scans showing so-called spinal longitudinal extradural fluid (SLEC) are suggestive of ventral dural tears (type 1 leak) which are located with prone dynamic (digital subtraction) myelography. As around half of the ventral dural tears are located in the upper thoracic spine, additional prone dynamic CT myelography is often needed. Leaking nerve root sleeves typically associated with meningeal diverticulae (type 2 leaks) and CSF-venous fistulas (type 3 leaks) are proven via lateral decubitus dynamic digital subtraction or CT myelography: type 2 leaks are SLEC-positive if the tear is proximal and SLEC-negative if it is distal, and type 3 leaks are always SLEC-negative. Although 30–70% of SIH patients show marked improvement following epidural blood patches applied via various techniques definite cure mostly requires surgical closure of ventral dural tears and surgical ligations of leaking nerve root sleeves associated with meningeal diverticulae or CSF-venous fistulas. For the latter, transvenous embolization with liquid embolic agents via the azygos vein system is a novel and valuable therapeutic alternative.
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Affiliation(s)
- Niklas Luetzen
- Department of Neuroradiology, Medical Center, University of Freiburg, Breisacher Str. 64 , 79106, Freiburg, Germany
| | - Philippe Dovi-Akue
- Department of Neuroradiology, Medical Center, University of Freiburg, Breisacher Str. 64 , 79106, Freiburg, Germany
| | - Christian Fung
- Department of Neurosurgery, Medical Center, University of Freiburg, Freiburg, Germany
| | - Juergen Beck
- Department of Neurosurgery, Medical Center, University of Freiburg, Freiburg, Germany
| | - Horst Urbach
- Department of Neuroradiology, Medical Center, University of Freiburg, Breisacher Str. 64 , 79106, Freiburg, Germany.
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Abstract
This article introduces the basic concepts of intracranial physiology and pressure dynamics. It also includes discussion of signs and symptoms and examination and radiographic findings of patients with acute cerebral herniation as a result of increased as well as decreased intracranial pressure. Current best practices regarding medical and surgical treatments and approaches to management of intracranial hypertension as well as future directions are reviewed. Lastly, there is discussion of some of the implications of critical medical illness (sepsis, liver failure, and renal failure) and treatments thereof on causation or worsening of cerebral edema, intracranial hypertension, and cerebral herniation.
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Affiliation(s)
- Aleksey Tadevosyan
- Department of Neurology, Tufts University School of Medicine, Beth Israel Lahey Hospital and Medical Center, 41 Mall Road, Burlington, MA 01805, USA.
| | - Joshua Kornbluth
- Department of Neurology, Tufts University School of Medicine, Tufts Medical Center, 800 Washington Street, Box#314, Boston, MA 02111, USA
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9
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Urbach H, Fung C, Dovi-Akue P, Lützen N, Beck J. Spontaneous Intracranial Hypotension. DEUTSCHES ARZTEBLATT INTERNATIONAL 2020; 117:480-487. [PMID: 33050997 DOI: 10.3238/arztebl.2020.0480] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
BACKGROUND Spontaneous intracranial hypotension (SIH) is an underdiagnosed disease. Its incidence is estimated at 5 per 100 000 persons per year. METHODS This review is based on a selective literature search in PubMed covering the years 2000-2019, as well as on the authors' personal experience. RESULTS The diagnostic and therapeutic methods discussed here are supported by level 4 evidence. SIH is caused by spinal leakage of cerebrospinal fluid (CSF) out of ventral dural tears or nerve root diverticula, or, in 2-5% of cases, through a fistula leading directly into the periradicular veins (CSF-venous fistula). In half of all patients, no CSF leak is demonstrable. A low CSF opening pressure on lumbar puncture is present in only one-third of patients; imaging studies are thus needed to confirm and localize a spinal CSF leak. Half of all patients in whom myelographic computed tomography (CT) reveals contrast medium reaching the epidural space have ventral dural tears, which tend to be located at upper thoracic spinal levels. Epidural blood patches applied under fluoroscopic or CT guidance can seal the CSF leak in 30-70% of patients, but 90% of patients with ventral dural tears will need operative closure. Some patients who have no visible epidural contrast medium on CT presumably do not have SIH, while others do, in fact, have a CSF leak from a diverticulum or a CSF-venous fistula and will need to have the site of the leak demonstrated with the aid of further studies, such as dynamic (subtraction) myelography in the lateral decubitus position. CONCLUSION The management of patients with SIH calls for complementary imaging studies to demonstrate the causative spinal CSF leak. Often, successful treatment requires surgical closure of the leak. In view of the sparse evidence available to date, controlled studies should be performed.
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Affiliation(s)
- Horst Urbach
- Department of Neuroradiology, Universitätsklinikum Freiburg
| | - Christian Fung
- Department of Neurosurgery, Universitätsklinikum Freiburg
| | | | - Niklas Lützen
- Department of Neuroradiology, Universitätsklinikum Freiburg
| | - Jürgen Beck
- Department of Neurosurgery, Universitätsklinikum Freiburg
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10
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Kranz PG, Gray L, Malinzak MD, Amrhein TJ. Spontaneous Intracranial Hypotension. Neuroimaging Clin N Am 2019; 29:581-594. [DOI: 10.1016/j.nic.2019.07.006] [Citation(s) in RCA: 26] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
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Kranz PG, Gray L, Amrhein TJ. Spontaneous Intracranial Hypotension: 10 Myths and Misperceptions. Headache 2018; 58:948-959. [PMID: 29797515 DOI: 10.1111/head.13328] [Citation(s) in RCA: 85] [Impact Index Per Article: 12.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/04/2018] [Indexed: 01/29/2023]
Abstract
OBJECTIVE To discuss common myths and misperceptions about spontaneous intracranial hypotension (SIH), focusing on common issues related to diagnosis and treatment, and to review the evidence that contradicts and clarifies these myths. BACKGROUND Recognition of SIH has increased in recent years. With increasing recognition, however, has come an increased demand for management by neurologists and headache specialists, some of whom have little prior experience with the condition. This dearth of practical experience, and lack of awareness of recent investigations into SIH, produces heterogeneity in diagnostic and treatment pathways, driven in part by outdated, confusing, or unsubstantiated conceptions of the condition. We sought to address this heterogeneity by identifying 10 myths and misperceptions that we frequently encounter when receiving referrals for suspected or confirmed SIH, and to review the literature addressing these topics. METHODS Ten topics relevant to diagnosis and treatment SIH were generated by the authors. A search for studies addressing SIH was conducted using PubMed and EMBASE, limited to English language only, peer reviewed publications from inception to 2018. Individual case reports were excluded. The resulting studies were reviewed for relevance to the topics in question. RESULTS The search generated 557 studies addressing SIH; 75 case reports were excluded. Fifty-four studies were considered to be of high relevance to the topics addressed, and were included in the data synthesis. The topics are presented in the form of a narrative review. CONCLUSIONS The understanding of SIH has evolved over the recent decades, leading to improvements in knowledge about the pathophysiology of the condition, diagnostic strategies, and expanded treatments. Awareness of these changes, and dispelling outdated misconceptions about SIH, is critical to providing appropriate care for patients and guiding future investigations going forward.
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Affiliation(s)
- Peter G Kranz
- Division of Neuroradiology, Department of Radiology, Duke University Medical Center, Durham, NC
| | - Linda Gray
- Division of Neuroradiology, Department of Radiology, Duke University Medical Center, Durham, NC
| | - Timothy J Amrhein
- Division of Neuroradiology, Department of Radiology, Duke University Medical Center, Durham, NC
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Petramfar P, Mohammadi SS, Hosseinzadeh F. Treatment of Idiopathic Intracranial Hypotension With Tea: A Case Report. IRANIAN RED CRESCENT MEDICAL JOURNAL 2016; 18:e24620. [PMID: 27621920 PMCID: PMC5002997 DOI: 10.5812/ircmj.24620] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 10/18/2014] [Revised: 03/30/2015] [Accepted: 04/18/2015] [Indexed: 11/16/2022]
Abstract
INTRODUCTION The syndrome of spontaneous intracranial hypotension has been increasingly diagnosed since its discovery through magnetic resonance imaging (MRI). It is a rare syndrome that is due to the leakage of cerebrospinal fluid (CSF) from a tear in the dura and can occur at any age, even among adolescents, but is most frequently seen among females in late middle age. CASE PRESENTATION Here, we describe a 32-year-old woman with a two-month history of headaches and occasional nausea and vomiting (N/V). MRI without gadolinium was normal, but meningeal enhancement was seen in MRI with gadolinium. The lumbar puncture revealed a low opening pressure. Computed tomography myelography (CT myelography) showed no leakage; Therefore, idiopathic intracranial hypotension was diagnosed. Treatment was started using tea, and the patient's headache got significantly better in about a day. CONCLUSIONS Conservative therapy, such as bed rest and caffeine treatment with eight cups of tea daily, yielded a significant improvement in our patient. Effectively, the patient constitutes a case of idiopathic intracranial hypotension due to undetectable CSF leakage or hyper-absorption, with good response to conservative management through tea-drinking. Further investigations with an appropriate sample size are needed in order to confirm this intervention in the treatment of idiopathic intracranial hypotension.
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Affiliation(s)
- Peyman Petramfar
- Department of Neurology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, IR Iran
| | - S. Saeed Mohammadi
- Student Research Committee, Shiraz University of Medical Sciences, Shiraz, IR Iran
- Corresponding Authors: S. Saeed Mohammadi, Student Research Committee, Shiraz University of Medical Sciences, Shiraz, IR Iran. E-mail: ; Farideh Hosseinzadeh, Shiraz University of Medical Sciences, Shiraz, IR Iran. E-mail:
| | - Farideh Hosseinzadeh
- Student Research Committee, Shiraz University of Medical Sciences, Shiraz, IR Iran
- Corresponding Authors: S. Saeed Mohammadi, Student Research Committee, Shiraz University of Medical Sciences, Shiraz, IR Iran. E-mail: ; Farideh Hosseinzadeh, Shiraz University of Medical Sciences, Shiraz, IR Iran. E-mail:
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Abstract
BACKGROUND Intracranial hypotension (IH) is caused by a leakage of cerebrospinal fluid (often from a tear in the dura) which commonly produces an orthostatic headache. It has been reported to occur after trivial cervical spine trauma including spinal manipulation. Some authors have recommended specifically questioning patients regarding any chiropractic spinal manipulation therapy (CSMT). Therefore, it is important to review the literature regarding chiropractic and IH. OBJECTIVE To identify key factors that may increase the possibility of IH after CSMT. METHOD A systematic search of the Medline, Embase, Mantis and PubMed databases (from 1991 to 2011) was conducted for studies using the keywords chiropractic and IH. Each paper was reviewed to examine any description of the key factors for IH, the relationship or characteristics of treatment, and the significance of CSMT to IH. In addition, other items that were assessed included the presence of any risk factors, neck pain and headache. RESULTS The search of the databases identified 39 papers that fulfilled initial search criteria, from which only eight case reports were relevant for review (after removal of duplicate papers or papers excluded after the abstract was reviewed). The key factors for IH (identified from the existing literature) were recent trauma, connective tissue disorders, or otherwise cases were reported as spontaneous. A detailed critique of these cases demonstrated that five of eight cases (63%) had non-chiropractic SMT (i.e. SMT technique typically used by medical practitioners). In addition, most cases (88%) had minimal or no discussion of the onset of the presenting symptoms prior to SMT and whether the onset may have indicated any contraindications to SMT. No case reports included information on recent trauma, changes in headache patterns or connective tissue disorders. DISCUSSION Even though type of SMT often indicates that a chiropractor was not the practitioner that delivered the treatment, chiropractic is specifically cited as either the cause of IH or an important factor. There are so much missing data in the case reports that one cannot determine whether the practitioner was negligent (in clinical history taking) or whether the SMT procedure itself was poorly administered. CONCLUSIONS This systematic review revealed that case reports on IH and SMT have very limited clinical details and therefore cannot exclude other theories or plausible alternatives to explain the IH. To date, the evidence that CSMT is not a cause of IH is inconclusive. Further research is required before making any conclusions that CSMT is a cause of IH. Chiropractors and other health practitioners should be vigilant in recording established risk factors for IH in all cases. It is possible that the published cases of CSMT and IH may have missed important confounding risk factors (e.g. a new headache, or minor neck trauma in young or middle-aged adults).
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Affiliation(s)
- P Tuchin
- Department of Chiropractic, Macquarie University, NorthRyde, NSW, Australia
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Ferraro F, Marano E, Petruzzi J, Tedeschi E, Santulli L, Elefante A. Spontaneous intracranial hypotension and epidural blood patch: a report involving seven cases. Anaesth Intensive Care 2013; 41:393-6. [PMID: 23659405 DOI: 10.1177/0310057x1304100320] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Spontaneous intracranial hypotension is a rare condition caused by spontaneous cerebrospinal fluid leak. It is characterised by orthostatic headache, diffuse pachymeningeal enhancement on brain imaging and low cerebrospinal fluid pressure. Seven patients with spontaneous intracranial hypotension were treated conservatively: of these, four responded to drug treatment and three underwent a lumbar autologous epidural blood patch (EBP). A complete response was obtained in two patients after a single EBP; one patient underwent a second EBP and then became asymptomatic. Clinical improvement coincided with a dramatic reduction of pachymeningeal enhancement. The aetiology and brain imaging findings, and the technique and effectiveness of EBP are discussed.
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Affiliation(s)
- F Ferraro
- Department of Neurological Sciences, University of Naples Federico II, Naples, Italy
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Ansari H. Occipital neuralgia secondary to CerebroSpinal fluid leak. J Headache Pain 2013. [PMCID: PMC3620309 DOI: 10.1186/1129-2377-14-s1-p151] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
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Wybrecht D, Faivre A, Aletti M, Bounolleau P, Alla P. Hypotension intracrânienne spontanée du liquide céphalo-rachidien. Presse Med 2011; 40:558-60. [DOI: 10.1016/j.lpm.2010.10.029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2010] [Revised: 10/18/2010] [Accepted: 10/22/2010] [Indexed: 11/26/2022] Open
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Abstract
Since the introduction of magnetic resonance imaging (MRI), spontaneous intracranial hypotension has been diagnosed much more frequently. The aim of this review is to discuss the symptoms and signs of the condition, in particular the characteristics of the associated headache, with sudden onset after sitting or standing, so that it can be included under the rubric of 'thunderclap headache'. This type of headache, like post lumbar puncture headaches, may be caused by cerebral vasodilatation and exacerbated by lowered pressure of the cerebrospinal fluid (CSF). Other symptoms include neck stiffness, nausea, vomiting, vertigo, tinnitus, deafness, and cognitive abnormalities. The clinical picture can sometimes mimic frontotemporal dementia, and the behaviour of some patients can sometimes be described as hypoactive-hypoalert, with somnolence, impaired attention, and stereotyped motor activity. Sagging of the brain, caused by leakeage of the CSF, can cause lesions in the brainstem with stupor, gaze palsies, and cranial nerve palsies. The condition can be a risk factor for cerebral venous thrombosis because of slowing of the blood flow and distortion of the blood vessels. The clinical picture may well suggest the diagnosis, but the headache may possibly indicate a subarachnoid haemorrhage. However, MRI will help to confirm the diagnosis and to localize the site of the CSF leak. MRI myelograms are of particular value, but if they are equivocal a computed tomography myelogram should be performed. The leakage of CSF is due to a tear in the dura, most frequently where the spinal roots leave the subarachnoid space. If this does not heal with bedrest, an epidural blood patch or a percutaneous injection of fibrin glue may be needed. More information is required on long-term follow-up.
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