Abstracts 2023

AUTHORS: Cahal M, Roth J, Ungar OJ, Brinjikji W.

CITATION: Interventional Neuroradiology. 2023 Dec 25:15910199231221863. doi: 10.1177/15910199231221863. Epub ahead of print. PMID: 38146166.

OBJECTIVE: Fluctuating sensorineural hearing loss (SNHL) has multiple etiologies, most commonly Ménière’s disease (MD), recurrent sudden SNHL, and autoimmune inner ear disorders. Fluctuating SNHL has rarely been described as a symptom of spontaneous intracranial hypotension (SIH).

PATIENT: A 39-year-old previously healthy female presented with “Ménière’s like” symptoms responsive to steroid treatment, which worsened during the day and improved in the supine position. Conservative treatment for MD consisting of low salt and caffeine diet and betahistine medication yielded no improvement. Secondary revision of brain imaging scans showed signs indicative of SIH, and a spinal cerebrospinal fluid leak was ultimately found and treated by a novel technique of transvenous fistula embolization by means of Onyx® glue, leading to gradual clinical improvement and near-complete resolution of symptoms.

CONCLUSIONS: SIH should be considered as part of the differential diagnosis of fluctuating SNHL. Clinical and radiological features should be known and sought. We suspect that early diagnosis and treatment can lead to cure and prevent permanent auditory damage.

PMID: 38146166
DOI: 10.1177/15910199231221863

AUTHORS: Volz, Florian MD; Doria-Medina, Roberto MD; Fung, Christian MD; Wolf, Katharina MD; El Rahal, Amir MD; Lützen, Niklas MD; Urbach, Horst MD; Loidl, Theresa Bettina MD; Hubbe, Ulrich MD; Klingler, Jan-Helge MD; Beck, Jürgen MD

CITATION: Operative Neurosurgery, December 22, 2023. DOI: 10.1227/ons.0000000000001042

BACKGROUND AND OBJECTIVE: A safe working trajectory is mandatory for spinal pathologies, especially in the midline, anterior to the spinal cord. For thoracic cerebrospinal fluid (CSF) leaks, we developed a minimally invasive keyhole fenestration. This study investigates the necessary bone removal for sufficient exposure of different leak types particularly regarding weight-bearing structures.

METHODS: In this retrospective case series between January 2022 and June 2023, the volume of bone resection and the axial and sagittal diameter of hemilamina defects after closure through keyhole fenestration were quantified. The involvement of facet joints and pedicles was qualitatively rated. Demographic (age, sex, body mass index, leak type) and surgical data (blood loss, surgery time, discharge after surgery) and complications were analyzed.

RESULTS: Thirty-three patients with 34 approaches were included. The volume of resected bone was 1.5 cm3, and the diameter of the hemilamina defect was 17.8 mm in the sagittal and 15.1 mm in the axial plane. Facet joints were uninvolved in 24% and partly resected in 74%, and one facet joint was resected completely. Pedicles remained intact in 71% and were minimally involved in 29%. The median surgery time was 93 minutes, blood loss was 45 mL, and discharge was 4 days after surgery. Three patients (9%) needed revision surgery. No relevant and persisting morbidity occurred. Within the median follow-up period of 10 months, no stabilizing surgery was necessary. No permanent neurological deficit occurred.

CONCLUSIONS: The keyhole fenestration leaves weight-bearing structures like facet joints and pedicles intact in most cases. The limited, penny-sized bone resection is sufficient to reach and close thoracic CSF leaks Type 1, 2, and 3 from the anterior midline to the ganglion. For experienced centers, it is a universal minimally invasive approach for treating all CSF leaks.

DOI: 10.1227/ons.0000000000001042

AUTHORS: Christopher Marvin Jesse, Ralph T. Schär, Johannes Goldberg, Christian Fung, Christian Thomas Ulrich, Tomas Dobrocky, Eike Immo Piechowiak, Christoph J. Schankin, Jürgen Beck, Andreas Raabe, Levin Häni

CITATION: Clinical Neurology and Neurosurgery 2023 Dec 19;236:108087. doi: 10.1016/j.clineuro.2023.108087. Epub ahead of print. 

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).

CONCLUSIONS: 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.

PMID: 38134757
DOI: 10.1016/j.clineuro.2023.108087

AUTHORS: Andrew L. Callen, Mo Fakhri, Vincent M. Timpone, Ashesh A. Thaker, William P. Dillon, Vinil N. Shah

CITATION: American Journal of Neuroradiology Dec 2023, DOI: 10.3174/ajnr.A8078

BACKGROUND AND PURPOSE: CSF-venous fistula can be diagnosed with dynamic decubitus CT myelography. This study aimed to analyze the temporal characteristics of CSF-venous fistula visualization on multiphase decubitus CT myelography.

MATERIALS AND METHODS: A retrospective, multisite study was conducted on patients diagnosed with CSF-venous fistula at 2 institutions between June 2017 and February 2023. Both institutions perform decubitus CT myelography with imaging immediately following injection and usually with at least 1 delayed scan. The conspicuity of CSF-venous fistula was assessed on each phase of imaging.

RESULTS: Forty-eight patients with CSF-venous fistula were analyzed. CSF-venous fistulas were better visualized on the early pass in 25/48 cases (52.1%), the delayed pass in 6/48 cases (12.5%) and were seen equally on both passes in 15/48 cases (31.3%). Of 25 cases in which the CSF-venous fistula was better visualized on the early pass, 21/25 (84%) fistulas were still at least partially visible on a delayed pass. Of 6 cases in which the CSF-venous fistula was better visualized on a delayed pass, 4/6 (67%) were partially visible on the earlier pass. Six of 48 (12.5%) CSF-venous fistulas were visible only on a single pass. Of these, 4/6 (66.7%) were seen only on the first pass, and 2/6 (33.3%) were seen only on a delayed pass. One fistula was found with one pass only, and one fistula was discovered upon contralateral decubitus imaging without a dedicated second injection.

CONCLUSIONS: A dynamic decubitus CT myelography imaging protocol that includes an early and delayed phase, likely increases the sensitivity for CSF-venous fistula detection. Further studies are needed to ascertain the optimal timing and technique for CSF-venous fistula visualization on dynamic decubitus CT myelography and its impact on patient outcomes.

PMID: 36894299
DOI: 10.3174/ajnr.A8078

AUTHORS: Victor Liaw, Morgan McCreary, Deborah I Friedman

CITATION: Neurology October 2023, DOI: 10.1212/WNL.0000000000207763

BACKGROUND AND OBJECTIVES: Spontaneous intracranial hypotension (SIH) is a debilitating condition typically producing orthostatic headache limiting upright time. SIH is often difficult to diagnose and treat, negatively affecting quality-of-life (QoL) in patients with the disorder. We studied QOL in confirmed and suspected SIH patients using standardized instruments, including suicidality.

METHODS: We performed a cross-sectional survey of adult patients with confirmed and clinically suspected SIH evaluated in our Headache and Facial Pain Program from 2016 to 2022. Using an online data collection tool (REDCap V 11.2.2), participants completed validated questionnaires assessing general well-being (SF-36), depression (PHQ-9), general anxiety disorder-7 (GAD-7), spiritual well-being during chronic therapy (FACIT-Sp-12) and headache impact (HIT-6). Subsequently, we interviewed willing participants to administer the Columbia-Suicide Severity Rating Scale (C-SSRS) assessing suicidal behavior and ideation.

RESULTS: 234 patients met inclusion criteria and were invited to participate in the study and 95 (59 confirmed and 36 clinically suspected) completed the questionnaires. The cohort’s average age was 51.1 years (SD: 15.5), predominantly female (69.5%), White (91.6%), and married (69.5%). Three-quarters (74.5%) scored within the most severe headache category (HIT-6). SF-36 scores were significantly inferior (p < .0001) to the general population, and lower than reported values for patients with multiple sclerosis and idiopathic intracranial hypertension. Almost half (49.1%) of respondents scored in the moderate depression range or worse (>10) and 25.4% scored with moderate anxiety or worse (>10). FACIT-Sp-12 scores were significantly worse (p < 0.0001) in symptomatic participants than in the validation cohorts of AIDS and cancer patients. Of the 67 respondents who completed the C-SSRS, over half (64.2%) endorsed a wish to be dead, and 22.4% had demonstrated suicidal behavior. Symptom-free SIH patients (n = 22) scored significantly better than symptomatic patients, comparable to the general population.

CONCLUSION: Based on our single-center cohort, SIH is associated with severe headache pain and high rates of depression, anxiety, and disability, affecting basic activities of daily living. Individuals with confirmed and suspected spinal CSF leaks scored similarly on these measures including suicidality. Outcomes were comparable to the general population following successful treatment or spontaneous remission. Improved identification and treatment of SIH are imperative to improve patients’ QoL.

PMID: 37816637
DOI: 10.1212/WNL.0000000000207763

AUTHORS: Andrew L. Callen, Rich Wojcik and Michael Bojanowski

CITATION: American Journal of Neuroradiology October 2023, DOI: https://doi.org/10.3174/ajnr.A8023

ABSTRACT SUMMARY: We describe a novel patient-positioning device for dynamic CT myelography. Dynamic CT myelography requires angling the patient’s spine to distribute dense contrast along the dependent thecal sac. The proposed device is constructed of a low-density reinforced polymer frame and can be raised or lowered to various heights with a hand-operated mechanism, allowing precise adjustment of the spinal angle and control of the contrast bolus, increasing the safety, reproducibility, and sensitivity of dynamic CT myelography.

CONCLUSIONS: We describe a dCTM patient-positioning device that offers several practical advantages over current commonly used methods for angling the spine on the CT table. By providing accurate and safe manipulation of the spinal angle, the device has the potential to increase diagnostic accuracy and procedural reproducibility and can accommodate patients of varying sizes. Patients can be positioned decubitus or prone and can start the examination in a horizontal or angled position. Because elevation is smooth and controlled, height can be adjusted while leaving the needle in place if needed. We believe that the incorporation of this device in clinical practice will improve patient outcomes and streamline the dCTM procedure for radiologists. Further studies are warranted to validate its effectiveness, assess patient and provider satisfaction as well as explore potential applications for manipulating the spinal angle in other CT-guided procedures. To date, we have performed 32 dCTM examinations using the device, all of which have been executed without compromised image quality. A patent is pending for the device, and it is our hope that it will be commercially available soon.

PMID 37827715
DOI: 10.3174/ajnr.A8023

AUTHORS: Timothy J. Amrhein, Molly McFatrich, Kate Ehle, Michael D. Malinzak, Linda Gray, Peter G. Kranz, E. Hope Weant & Christina K. Zigler

CITATION: Journal of Patient-Reported Outcomes volume 7, Article number: 82 (2023) doi: 10.1186/s41687-023-00625-4

BACKGROUND & OBJECTIVES: Spontaneous intracranial hypotension (SIH) is an underdiagnosed and debilitating condition caused by a spinal cerebrospinal fluid (CSF) leak. Although SIH can lead to substantial morbidity and disability, little data exists about patients’ perspectives. Without hearing directly from patients, our understanding of the full experience of having SIH is limited, as is our ability to identify and use appropriate patient-reported outcome measures (PROMs) within clinical care and research. The purpose of this study was to conduct qualitative interviews with confirmed SIH patients to fully describe their experiences and identify relevant concepts to measure.

METHODS: Patients were recruited from an SIH specialty clinic at a large, U.S.-based healthcare center. Patients undergoing an initial consultation who were ≥ 18 years old, English-speaking, met the International Classification of Headache Disorders-3 criteria for SIH, and had a brain MRI with contrast that was positive for SIH were eligible to participate. During semi-structured qualitative interviews with a trained facilitator, participants were asked to describe their current SIH symptoms, how their experiences with SIH had changed over time, and the aspects of SIH that they found most bothersome. Analysts reviewed the data, created text summaries, and wrote analytic reports.

RESULTS: Fifteen participants completed interviews. Common symptoms reported by patients included headache, tinnitus, ear fullness/pressure/pain, and neck or interscapular pain. Patients reported that their symptoms worsened over the course of their day and with activity. The most bothersome aspect of SIH was disruption to daily activities and limits to physical activities/exercise, which were severe. With regard to symptoms, the most bothersome and impactful included physical pain and discomfort (including headache), as well as fatigue.

CONCLUSIONS: Patients reported a diverse set of symptoms that were attributed to SIH, with devastating impacts on functioning and high levels of disability. Researchers considering use of PROMs for SIH should consider inclusion of both symptom scales and aspects of functioning, and future work should focus on evaluating the validity of existing measures for this patient population using rigorous qualitative and quantitative methods in diverse samples. Additionally, these data can be used to assist clinicians in understanding the impacts of SIH on patients.

PMID: 37581717
DOI: 10.1186/s41687-023-00625-4

AUTHORS:Sanjay Cheema, Jane Anderson, Heather Angus-Leppan, Paul Armstrong, David Butteriss, Lalani Carlton Jones, David Choi, Amar Chotai, Linda D’Antona, Indran Davagnanam, Brendan Davies, Paul J Dorman, Callum Duncan, Simon Ellis, Valeria Iodice, Clare Joy, Susie Lagrata, Sarah Mead, Danny Morland, Justin Nissen, Jenny Pople, Nancy Redfern, Parag P Sayal, Daniel Scoffings, Russell Secker, Ahmed K Toma, Tamsin Trevarthen, James Walkden, Jürgen Beck, Peter George Kranz, Wouter Schievink, Shuu-Jiun Wang, Manjit Singh Matharu.

CITATION:J Neurol Neurosurg Psychiatry. 2023 Oct;94(10):835-843. doi:10.1136/jnnp-2023-331166. Epub 2023 May 5.

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BACKGROUND AND PURPOSE:We 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 current evidence and consensus from a multidisciplinary specialist interest group (SIG).

MATERIALS AND METHODS: A 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH.

RESULTS:SIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided.

CONCLUSIONS:This multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH.

PMID: 37147116
PMCID: PMC10511987
DOI: 10.1136/jnnp-2023-331166

AUTHORS: Andrew L. Callen, Lalani Carlton Jones, Vincent M. Timpone, Jack Pattee, Daniel J. Scoffings, David Butteriss, Thien Huynh, Peter Y. Shen and Mark D. Mamlouk

CITATION: American Journal of Neuroradiology October 2023, DOI: https://doi.org/10.3174/ajnr.A8005

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BACKGROUND AND PURPOSE: CSF-to-venous fistulas contribute to spontaneous intracranial hypotension. CT-guided fibrin occlusion has been described as a minimally invasive treatment strategy; however, its reproducibility across different institutions remains unclear. This multi-institution study evaluated the clinical and radiologic outcomes of CT-guided fibrin occlusion, hypothesizing a correlation among cure rates, fibrin injectate spread, and drainage patterns.

MATERIALS AND METHODS: A retrospective evaluation was conducted on CT-guided fibrin glue treatment in patients with CSF-to-venous fistulas from 6 US and UK institutions from 2020 to 2023. Patient information, procedural characteristics, and injectate spread and drainage patterns were examined. Clinical improvement assessed through medical records served as the primary outcome.

RESULTS: Of 119 patients at a mean follow-up of 5.0 months, fibrin occlusion resulted in complete clinical improvement in 59.7%, partial improvement in 34.5%, and no improvement in 5.9% of patients. Complications were reported in 4% of cases. Significant associations were observed between clinical improvement and concordant injectate spread with the fistula drainage pattern (P = .0089) and pretreatment symptom duration (P < .001). No associations were found between clinical improvement and cyst puncture, intravascular extension, rebound headache, body mass index, age, or number of treatment attempts.

CONCLUSIONS: Fibrin occlusion performed across various institutions shows a CSF-to-venous fistula drainage pattern and shorter pretreatment symptom duration, emphasizing the importance of accurate injectate placement and early intervention.

PMID: 37798111
DOI: https://doi.org/10.3174/ajnr.A8005

AUTHORS: Vishal Uppal, MBBS, MSc; Robin Russell, MBBS; Rakesh Sondekoppam, MD; Jessica Ansari, MD; Zafeer Baber, MD; Yian Chen, MD; Kathryn DelPizzo, MD; Dan Sebastian Dîrzu, MD; Hari Kalagara, MD; Narayan R. Kissoon, MD; Peter G. Kranz, MD; Lisa Leffert, MD; Grace Lim, MD; Clara A. Lobo, MD; Dominique Nuala Lucas, MBBS; Eleni Moka, MD; Stephen E. Rodriguez, MD; Herman Sehmbi, MD; Manuel C. Vallejo, MD; Thomas Volk, MD; Samer Narouze, MD, PhD

CITATION: JAMA Netw Open. 2023;6(8):e2325387. doi:10.1001/jamanetworkopen.2023.25387

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IMPORTANCE: Postdural puncture headache (PDPH) can follow unintentional dural puncture during epidural techniques or intentional dural puncture during neuraxial procedures, such as a lumbar puncture or spinal anesthesia. Evidence-based guidance on the prevention, diagnosis, and management of this condition is, however, currently lacking.

OBJECTIVE: To fill the practice guidelines void and provide comprehensive information and patient-centric recommendations for preventing, diagnosing, and managing PDPH.

EVIDENCE REVIEW: With input from committee members and stakeholders of 6 participating professional societies, 10 review questions that were deemed important for the prevention, diagnosis, and management of PDPH were developed. A literature search for each question was performed in MEDLINE on March 2, 2022. Additional relevant clinical trials, systematic reviews, and research studies published through March 2022 were also considered for practice guideline development and shared with collaborator groups. Each group submitted a structured narrative review along with recommendations that were rated according to the US Preventive Services Task Force grading of evidence. Collaborators were asked to vote anonymously on each recommendation using 2 rounds of a modified Delphi approach.

FINDINGS: After 2 rounds of electronic voting by a 21-member multidisciplinary collaborator team, 47 recommendations were generated to provide guidance on the risk factors for and the prevention, diagnosis, and management of PDPH, along with ratings for the strength and certainty of evidence. A 90% to 100% consensus was obtained for almost all recommendations. Several recommendations were rated as having moderate to low certainty. Opportunities for future research were identified.

CONCLUSIONS AND RELEVANCE: Results of this consensus statement suggest that current approaches to the treatment and management of PDPH are not uniform due to the paucity of evidence. The practice guidelines, however, provide a framework for individual clinicians to assess PDPH risk, confirm the diagnosis, and adopt a systematic approach to its management.

PMID: 37581893
DOI: 10.1001/jamanetworkopen.2023.25387

AUTHORS: Florian Volz, Christian Fung, Katharina Wolf, Niklas Lützen, Horst Urbach, Luisa Mona Kraus, Mazin Omer, Jürgen Beck, and Amir El Rahal

CITATION: Cephalalgia. 2023;43(8). doi:10.1177/03331024231195830

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INTRODUCTION: Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid leak causes orthostatic headaches and impacts quality of life. Successful closure rates are often reported, whereas data on long-term outcome are still scarce.

METHODS: Between April 2020 and December 2022 surgically treated patients completed the Headache Impact Test-6 prior to surgery and at 14 days, three months, six months, and 12 months postoperatively. In addition to the Headache Impact Test-6 score, we extracted data related to orthostatic symptoms.

RESULTS: Eighty patients were included. Median Headache Impact Test-6 score preoperatively was 65 (IQR 61–69), indicating severe and disabling impact of headaches. At three months headache impact significantly improved to 49 (IQR 44–58) (p < 0.001) and remained stable up to 12 months (48, IQR 40–56), indicating little to no impact of headaches on quality of life. The need to lie down “always” or “very often” was reduced from 79% to 23% three months postoperatively (p < 0.001).

CONCLUSIONS: Surgical closure of spinal CSF leaks significantly improves the impact of headaches in the long term. At least three months should be expected for recovery. Despite permanent closure of the CSF-leak, a quarter of patients still have relevant long-term impairment, indicating the need for further research on its cause and possible treatment.

PMID: 37652456
DOI: 10.1177/03331024231195830

AUTHORS: J.L. Houk, S. Morrison, S. Peskoe, T.J. Amrhein and P.G. Kranz

CITATION: American Journal of Neuroradiology August 2023, DOI: https://doi.org/10.3174/ajnr.A7962

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BACKGROUND AND PURPOSE: The Bern score is a quantitative scale characterizing brain MR imaging changes in spontaneous intracranial hypotension. Higher scores are associated with more abnormalities on brain MR imaging, raising the question of whether the score can serve as a measure of disease severity. However, the relationship between clinical symptom severity and the Bern score has not been evaluated. Our purpose was to assess correlations between Bern scores and clinical headache severity in spontaneous intracranial hypotension.

MATERIALS AND METHODS: This study was a single-center, retrospective cohort of patients satisfying the International Classification of Headache Disorders-3 criteria for spontaneous intracranial hypotension. Fifty-seven patients who completed a pretreatment headache severity questionnaire (Headache Impact Test-6) and had pretreatment brain MR imaging evidence of spontaneous intracranial hypotension were included. Pearson correlation coefficients (ρ) for the Headache Impact Test-6 and Bern scores were calculated. Receiver operating characteristic curves were used to assess the ability of Bern scores to discriminate among categories of headache severity.

RESULTS: We found low correlations between clinical headache severity and Bern scores (ρ = 0.139; 95% CI, −0.127−0.385). Subgroup analyses examining the timing of brain MR imaging, symptom duration, and prior epidural blood patch showed negligible-to-weak correlations in all subgroups. Receiver operating characteristic analysis found that the Bern score poorly discriminated subjects with greater headache severity from those with lower severity.

CONCLUSIONS: Pretreatment Bern scores show a low correlation with headache severity in patients with spontaneous intracranial hypotension. This finding suggests that brain imaging findings as reflected by Bern scores may not reliably reflect clinical severity and should not replace clinical metrics for outcome assessment.

PMID: 37562827
DOI: 10.3174/ajnr.A7962

AUTHORS: P.G. Kranz, M.D. Malinzak, L. Gray, J. Willhite and T.J. Amrhein

CITATION: American Journal of Neuroradiology July 2023, DOI: https://doi.org/10.3174/ajnr.A7927

BACKGROUND AND PURPOSE: CSF-venous fistulas are an important cause of spontaneous intracranial hypotension but are challenging to detect. A newly described technique known as resisted inspiration has been found to augment the CSF-venous pressure gradient and was hypothesized to be of potential use in CSF-venous fistula detection but has not yet been investigated in patients with spontaneous intracranial hypotension. The purpose of this investigation was to determine whether resisted inspiration results in improved visibility of CSF-venous fistulas on CT myelography in patients with spontaneous intracranial hypotension.

MATERIALS AND METHODS: A retrospective cohort of patients underwent CT myelography from November 2022 to January 2023. Patients with an observed or suspected CSF-venous fistula identified during CT myelography using standard maximum suspended inspiration were immediately rescanned using resisted inspiration and the Valsalva maneuver. The visibility of the CSF-venous fistula among these 3 respiratory phases was compared, and changes in venous drainage patterns between phases were assessed.

RESULTS: Eight patients with confirmed CSF-venous fistulas who underwent CT myelography using the 3-phase respiratory protocol were included. Visibility of the CSF-venous fistula was greatest during resisted inspiration in 5/8 (63%) of cases. Visibility was optimal with the Valsalva maneuver and maximum suspended inspiration in 1 case each, and it was equivalent in all respiratory phases in 1 case. In 2/8 (25%) cases, the pattern of venous drainage shifted between respiratory phases.

CONCLUSIONS: In patients with spontaneous intracranial hypotension, resisted inspiration improved visualization of CSF-venous fistulas in most, but not all, cases. Further investigation is needed to determine the impact of this technique on the overall diagnostic yield of myelography in this condition.

PMID: 37414450
DOI: 10.3174/ajnr.A7927

AUTHORS: Andrew L. Callen MD,  Peter Lennarson MD,  Ian R. Carroll MD, MS

CITATION: Headache. 2023; 00: 1- 3. doi:10.1111/head.14584

ABSTRACT: A 24-year-old woman experienced a postdural puncture headache following a labor epidural, recovered following bedrest, and was then without headache for 12 years. She then experienced sudden onset of daily, holocephalic headache persisting for 6 years prior to presentation. Pain reduced with prolonged recumbency. MRI brain, MRI myelography, and later bilateral decubitus digital subtraction myelography showed no cerebrospinal fluid (CSF) leak or CSF venous fistula, and normal opening pressure. Review of an initial noncontrast MRI myelogram revealed a subcentimeter dural outpouching at L3–L4, suspicious for a posttraumatic arachnoid bleb. Targeted epidural fibrin patch at the bleb resulted in profound but temporary symptom relief, and the patient was offered surgical repair. Intraoperatively, an arachnoid bleb was discovered and repaired followed by remission of headache. We report that a distant dural puncture can play a causative role in the long delayed onset of new daily persistent headache.

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PMID: 37358488
DOI: 10.1111/head.14584

AUTHORS: T J Amrhein, J W Williams Jr, L Gray, M D Malinzak, S Cantrell, C R Deline, C M Carr, D K Kim, K M Goldstein, P G Kranz

CITATION: American Journal of Neuroradiology May 2023, DOI: https://doi.org/10.3174/ajnr.A7880

STUDY BACKGROUND: Spontaneous intracranial hypotension is an important cause of treatable secondary headaches. Evidence on the efficacy of epidural blood patching and surgery for spontaneous intracranial hypotension has not been synthesized.

PURPOSE OF THE STUDY: Our aim was to identify evidence clusters and knowledge gaps in the efficacy of treatments for spontaneous intracranial hypotension to prioritize future research.

DATA SOURCES: We searched published English language articles on MEDLINE (Ovid), the Web of Science (Clarivate), and EMBASE (Elsevier) from inception until October 29, 2021.

STUDY SELECTION: We reviewed experimental, observational, and systematic review studies assessing the efficacy of epidural blood patching or surgery in spontaneous intracranial hypotension.

DATA ANALYSIS: One author performed data extraction, and a second verified it. Disagreements were resolved by consensus or adjudicated by a third author.

DATA SYNTHESIS: One hundred thirty-nine studies were included (median, 14 participants; range, 3-298 participants). Most articles were published in the past decade. Most assessed epidural blood patching outcomes. No studies met level 1 evidence. Most were retrospective cohort or case series (92.1%, n = 128). A few compared the efficacy of different treatments (10.8%, n = 15). Most used objective methods for the diagnosis of spontaneous intracranial hypotension (62.3%, n = 86); however, 37.7% (n = 52) did not clearly meet the International Classification of Headache Disorders-3 criteria. CSF leak type was unclear in 77.7% (n = 108). Nearly all reported patient symptoms using unvalidated measures (84.9%, n = 118). Outcomes were rarely collected at uniform prespecified time points.

LIMITATIONS: The investigation did not include transvenous embolization of CSF-to-venous fistulas.

CONCLUSION: Evidence gaps demonstrate a need for prospective study designs, clinical trials, and comparative studies. We recommend using the International Classification of Headache Disorders-3 diagnostic criteria, explicit reporting of CSF leak subtype, inclusion of key procedural details, and using objective validated outcome measures collected at uniform time points.

PMID: 37202114
DOI: 10.3174/ajnr.A7880

AUTHORS: Richard I Farb, Sean T O’Reilly, Everardus J Hendriks, Philip W Peng, Eric M Massicotte, Yasmine Hoydonckx, Patrick J Nicholson

CITATION: Interventional Neuroradiology. 2023;0(0). doi:10.1177/15910199231172627

PURPOSE OF THE STUDY: Presented here is a strategy of sequential lateral decubitus digital subtraction myelography (LDDSM) followed closely by lateral decubitus CT (LDCT) to facilitate cerebrospinal fluid (CSF)–venous fistula (CVF) localization.

STUDY MATERIALS AND METHODS: This is a retrospective analysis of patients referred to our institution for evaluation of CSF leak. Patients with Type 1 and Type 2 leaks, and those not displaying MR brain stigmata of intracranial hypotension were excluded. All patients underwent consecutive LDDSM and LDCT. If the CVF was not localized on the first LDDSM–LDCT pair the patient returned for contralateral examinations. Images were reviewed for CVF and for accumulation of contrast within the renal pelvises expressed as a renal pelvis contrast score (RPCS) in Hounsfield units (HU).

RESULTS: Twenty-two patients were included in this study. In 21 of 22 patients (95%) a CVF was identified yielding an RPCS for the LDDSM–LDCT pair ipsilateral to the CVF ranging from 71 to 423 with an average of 146 HU. An RPCS of the negative side LDDSM–LDCT pair contralateral to a CVF was available in 8 patients and averaged 51 HU. In 4 patients the initial bilateral LDDSM–LDCT pairs did not reveal the location of the CVF however in 3 of these 4 cases the CVF was revealed on a third LDDSM repeated ipsilateral to the higher RPCS.

CONCLUSION: The strategy of sequential LDDSM–LDCT coupled with evaluation of renal accumulation of contrast agent appears to improve the rate of CVF localization and warrants further evaluation.

PMID: 37211661
DOI: 10.1177/15910199231172627

AUTHORS: Andrew Callen, Jack Pattee, Ashesh A. Thaker, Vincent M. Timpone, David Zander, Ryan Turner, Marius Birlea, Danielle Wilhour, Chantal O’Brien, Jennifer Evan, Fabio Grassia, Ian Carroll

CITATION: Neurology Apr 2023,  DOI: 10.1212/WNL.0000000000207267. Full PDF here.

BACKGROUND AND OBJECTIVES OF THE STUDY: Existing tools to diagnose spontaneous intracranial hypotension (SIH), namely spinal opening pressure (OP) and brain MRI, have limited sensitivity. We investigated whether evaluation of brain MRI using the Bern Score, combined with calculated craniospinal elastance, would aid in diagnosing SIH and provide insight into its pathophysiology.

STUDY METHODS: A retrospective chart review was performed of patients who underwent brain MRI and pressure-augmented dynamic CT myelography (dCTM) for suspicion of SIH. Two blinded Neuroradiologists assigned Bern Scores for each brain MRI. OP and incremental pressure changes after intrathecal saline infusion were recorded to calculate craniospinal elastance. The relationship between Bern Score, OP, elastance, and whether a leak was found were analyzed.

RESULTS: 72 consecutive dCTMs were performed in 53 patients. 12 CSF-venous fistulae, two ruptured meningeal diverticula, two dural defects, and one dural bleb were found (17/53=32%). Among patients with imaging proven CSF leak/fistula, OP was normal in all but one patient, and was not significantly different in those with a leak compared to those without (15.1 vs 13.6 cm H2O, p = 0.24, A=0.40). Average Bern Score in individuals with a leak was significantly higher than in those without (5.35 vs 1.85, p < 0.001, A=0.85), even when excluding pachymeningeal enhancement from the score (3.77 vs 1.57, p = 0.001, A=0.78). Average elastance in those with a leak was higher than in those without, but this difference was not statistically significant (2.05 vs 1.20 mL/cm H2O, p = 0.19, A=0.40). Increased elastance was significantly associated with an increased Bern Score (p < 0.01, 95% CI -0.55, 0.12), and was significantly associated with venous distention, pachymeningeal enhancement, prepontine narrowing, and subdural collections, but not a narrowed mamillopontine or suprasellar distance.

DISCUSSION: OP is not an effective predictor for diagnosing CSF leak, and if used in isolation would result in misdiagnosis of 94% of patients in our cohort. The Bern Score was associated with a higher diagnostic yield of dCTM. Elastance was significantly associated with certain components of the Bern Score.

PMID: 37015821
DOI: 10.1212/WNL.0000000000207267

Full PDF: https://n.neurology.org/content/neurology/early/2023/04/04/WNL.0000000000207267.full.pdf

AUTHORS: M.D. Mamlouk, P.Y. Shen, B.C. Dahlin

CITATION: American Journal of Neuroradiology Mar 2023, 44 (3) 341-346; DOI: 10.3174/ajnr.A7784

BACKGROUND AND PURPOSE OF THE STUDY: Dynamic CT myelography can identify spinal CSF leaks secondary to dural tears (type 1) and ruptured meningeal diverticula (type 2), but the radiation can be high secondary to multiple successive acquisitions. The purpose of this article is to discuss the procedural approach of a modified dynamic CT myelography technique with single scan acquisitions, reduced contrast volume, and condensed scan coverage and compare its radiation dose with that in traditional dynamic CT myelography.

STUDY MATERIALS AND METHODS: Retrospective review was performed for patients with spontaneous CSF leaks showing extradural collections on spine MR imaging who underwent traditional and modified dynamic CT myelography. The radiation doses between the 2 cohorts were compared.

RESULTS: Thirty-seven patients (25 women, 12 men) had a type 1 or 2 CSF leak on dynamic CT myelography. Thirty-one patients had a type 1 CSF leak, and 6 patients had type 2 leaks. The traditional dynamic CT myelography was performed in 25 patients, and the average number of acquisitions per dynamic CT myelography was 3.6. The mean total effective dose per dynamic CT myelography was 31.3 mSv (range, 11.3–68.4 mSv). The modified dynamic CT myelography was performed in 12 patients, and the average number of acquisitions was 2.8. The mean total effective dose per dynamic CT myelography was 15.1 mSv (range, 4.8–24.6 mSv). The effective dose and dose-length product between the cohorts were statistically significant (P  < .0001 and .01, respectively).

CONCLUSIONS: Modified dynamic CT myelography performed with single scan acquisitions, smaller volume of contrast, and reduced scan coverage can reduce the radiation dose for type 1 and 2 CSF leak detection.

PMID: 36732032
DOI: 10.3174/ajnr.A7784

AUTHORS: Najdat Zohbi, Alexander Castilho, Sera Kim, Amit M. Saindane, Jason W. Allen, Michael J. Hoch, Brent D. Weinberg

CITATION: J Neuroimaging. 2023 Mar 27. doi: https://doi.org/10.1111/jon.13102

BACKGROUND AND PURPOSE OF THE STUDY: Spontaneous intracranial hypotension (SIH) is a known cause of headaches and neurologic symptoms, but the frequency of cranial nerve symptoms and abnormalities on magnetic resonance imaging (MRI) has not been well described. The purpose of this study was to document cranial nerve findings in patients with SIH and determine the relationship between imaging findings and clinical symptoms.

STUDY METHODS: Patients diagnosed with SIH with pre-treatment brain MRI at a single institution from September 2014 to July 2017 were retrospectively reviewed to determine the frequency of clinically significant visual changes/diplopia (cranial nerves 3 and 6) and hearing changes/vertigo (cranial nerve 8). A blinded review of brain MRIs before and after treatment was conducted to assess for abnormal contrast enhancement of cranial nerves 3, 6, and 8. Imaging results were correlated with clinical symptoms.

RESULTS: Thirty SIH patients with pre-treatment brain MRI were identified. Sixty-six percent of patients had vision changes, diplopia, hearing changes, and/or vertigo. Cranial nerve 3 and/or 6 enhancement was present in nine patients on MRI, with 7/9 patients experiencing visual changes and/or diplopia (odds ratio [OR] 14.9, 95% confidence interval [CI] 2.2-100.8, p = .006). Cranial nerve 8 enhancement was present in 20 patients on MRI, with 13/20 patients experiencing hearing changes and/or vertigo (OR 16.7, 95% CI 1.7-160.6, p = .015).

CONCLUSIONS: SIH patients with cranial nerve findings on MRI were more likely to have associated neurologic symptoms than those without imaging findings. Cranial nerve abnormalities on brain MRI should be reported in suspected SIH patients as they may support the diagnosis and explain patient symptoms.

PMID: 36972143
DOI: 10.1111/jon.13102

AUTHORS: Andrew L. Callen, William P. Dillon & Vinil N. Shah

CITATION: Neuroradiology (2023). https://doi.org/10.1007/s00234-023-03136-7

PURPOSE OF THE STUDY: The most common neuroimaging manifestations of patients suffering from spontaneous intracranial hypotension (SIH) include subdural fluid collections, enhancement of the pachymeninges, engorgement of venous structures, pituitary hyperemia, sagging of the brainstem, and cerebellar hemosiderosis. However, infrequently patients may present with separate neuroradiological findings which could be easily mistaken for other pathology.

STUDY METHODS: We describe patients who presented with unique neuroimaging findings who were eventually found to have a spinal CSF leak or venous fistula. Relevant clinical history and neuroradiology findings are presented, and a relevant review of the literature is provided.

RESULTS: We present six patients with a proven CSF leak or fistula who presented with dural venous sinus thrombosis, compressive ischemic injury, spinal hemosiderosis, subarachnoid hemorrhage, pial vascular engorgement, calvarial hyperostosis, and spinal dural calcifications.

CONCLUSIONS: Radiologists should be familiar with atypical neuroimaging manifestations of SIH in order to avoid misdiagnosis and guide the clinical trajectory of the patient towards accurate diagnosis and eventual cure.

PMID: 36879063 
DOI: 10.1007/s00234-023-03136-7

AUTHORS: Wouter I. Schievink, Marcel M. Maya

CITATION: JAMA Neurol. Published online February 20, 2023. doi:10.1001/jamaneurol.2022.5388

ABSTRACT: A 61-year-old woman presented to the emergency department with neck pain after a fall in the nursing home, where she had lost her balance while trying to move clothing in her closet with her feet. She had a 7-year history of progressive neck, shoulder, and upper extremity weakness and atrophy, for which several diagnoses had been entertained, including amyotrophic lateral sclerosis, person-in-the-barrel syndrome, and upper motor neuron disease of unknown etiology. Electromyography had shown chronic neurogenic changes in the cervical myotomes. There was no history of headache or prior trauma. At presentation, she had severe atrophy of her neck, shoulder, and upper extremity musculature. Occasional fasciculations were noted. Motor examination showed flaccid paralysis (Medical Research Council score of 0 of 5) of the upper extremities except for right thumb abduction (score of 4 of 5). Lower extremity strength was normal. Deep tendon reflexes were absent in the upper extremities and normal in the lower extremities. Magnetic resonance imaging (MRI) showed an extensive ventral cervicothoracic extradural cerebrospinal fluid (CSF) collection with posterior displacement of the spinal cord. Brain MRI findings were normal. Digital subtraction myelography localized the ventral CSF leak to the thoracic 11-12 level. At surgery, through a posterior intradural approach, a ventral dural tear was found, and this was repaired. The patient recovered well from surgery, and postoperative MRI showed complete resolution of her CSF leak. At last follow-up 40 months postoperatively, stable motor examination findings were noted.

DOI: 10.1001/jamaneurol.2022.5388

AUTHORS: M.D. Mamlouk, P.Y. Shen and B.C. Dahlin

CITATION: American Journal of Neuroradiology  February 2023, DOI: https://doi.org/10.3174/ajnr.A7784

BACKGROUND AND PURPOSE OF THE STUDY: Dynamic CT myelography can identify spinal CSF leaks secondary to dural tears (type 1) and ruptured meningeal diverticula (type 2), but the radiation can be high secondary to multiple successive acquisitions. The purpose of this article is to discuss the procedural approach of a modified dynamic CT myelography technique with single scan acquisitions, reduced contrast volume, and condensed scan coverage and compare its radiation dose with that in traditional dynamic CT myelography.

MATERIALS AND METHODS: Retrospective review was performed for patients with spontaneous CSF leaks showing extradural collections on spine MR imaging who underwent traditional and modified dynamic CT myelography. The radiation doses between the 2 cohorts were compared.

RESULTS: Thirty-seven patients (25 women, 12 men) had a type 1 or 2 CSF leak on dynamic CT myelography. Thirty-one patients had a type 1 CSF leak, and 6 patients had type 2 leaks. The traditional dynamic CT myelography was performed in 25 patients, and the average number of acquisitions per dynamic CT myelography was 3.6. The mean total effective dose per dynamic CT myelography was 31.3 mSv (range, 11.3–68.4 mSv). The modified dynamic CT myelography was performed in 12 patients, and the average number of acquisitions was 2.8. The mean total effective dose per dynamic CT myelography was 15.1 mSv (range, 4.8–24.6 mSv). The effective dose and dose-length product between the cohorts were statistically significant (P < .0001 and .01, respectively).

CONCLUSIONS: Modified dynamic CT myelography performed with single scan acquisitions, smaller volume of contrast, and reduced scan coverage can reduce the radiation dose for type 1 and 2 CSF leak detection.

PMID: 36732032
DOI: 10.3174/ajnr.A7784

AUTHORS: Jürgen Beck, Ulrich Hubbe, Jan-Helge Klingler, Roland Roelz, Luisa Mona Kraus, Florian Volz, Niklas Lützen, Horst Urbach, Kristin Kieselbach, and Christian Fung

CITATION: Journal of Neurosurgery: Spine. 2023;38(1):147-152. doi: 10.3171/2022.7.SPINE2252

OBJECTIVE OF THE STUDY: Spinal CSF leaks cause spontaneous intracranial hypotension (SIH). Surgical closure of spinal CSF leaks is the treatment of choice for persisting leaks. Surgical approaches vary, and there are no studies in which minimally invasive techniques were used. In this study, the authors aimed to detail the safety and feasibility of minimally invasive microsurgical sealing of spinal CSF leaks using nonexpandable tubular retractors.

STUDY METHODS: Consecutive patients with SIH and a confirmed spinal CSF leak treated at a single institution between April 2019 and December 2020 were included in the study. Surgery was performed via a dorsal 2.5-cm skin incision using nonexpandable tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach. The primary outcome was successful sealing of the dura, and the secondary outcome was the occurrence of complications.

RESULTS: Fifty-eight patients, 65.5% of whom were female (median age 46 years [IQR 36–55 years]), with 38 ventral leaks, 17 lateral leaks, and 2 CSF-venous fistulas were included. In 56 (96.6%) patients, the leak could be closed, and in 2 (3.4%) patients the leak was missed because of misinterpretation of the imaging studies. One of these patients underwent successful reoperation, and the other patient decided to undergo surgery at another institution. Two other patients had to undergo reoperation because of insufficient closure and a persisting leak. The rate of permanent neurological deficit was 1.7%, the revision rate for a persisting or recurring leak was 3.4%, and the overall revision rate was 10.3%. The rate of successful sealing during the primary closure attempt was 96.6% and 3.4% patients needed a secondary attempt. Clinical short-term outcome at discharge was unchanged in 14 patients and improved in 25 patients, and 19 patients had signs of rebound intracranial hypertension.

CONCLUSIONS: Minimally invasive surgery with tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach is safe and effective for the treatment of spinal CSF leaks. The authors suggest performing a minimally invasive closure of spinal CSF leaks in specialized centers.

PMID: 36087332
DOI: 10.3171/2022.7.SPINE2252