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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. Plain Language Summary: This study explored the variety of symptoms and brain magnetic resonance imaging (MRI) characteristics among patients with spontaneous intracranial hypotension (SIH) due to spinal cerebrospinal fluid venous fistula (CVF). We studied the clinical symptoms and brain scans of 48 patients with CVF, and we found distinct "classic" and "atypical" symptom patterns and varied MRI findings that suggest different subtypes of the condition. These findings suggest that we should not rely only on the usual signs of orthostatic headache for diagnosis and indicate the need for a thorough diagnostic approach to better understand and treat SIH. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background and Purpose: Spontaneous intracranial hypotension (SIH) due to CSF venous fistula (CVF) is increasingly recognized as a secondary cause of headaches, with symptoms often overlapping with primary headache syndromes such as migraine. While brain MRI studies have focused on features indicative of SIH, findings that support an alternate headache etiology, such as the bifrontal white matter hyperintensities (WMH) often seen in migraines, have not been explored in this context. This study assesses 1) the quantity and distribution of WMH and 2) the presenting clinical features in patients with and without CVF found on dynamic decubitus CT myelography (dCTM)., Materials and Methods: 72 consecutive patients underwent clinical workup for SIH due to suspected CVF, including pre-procedural brain and spine MRI followed by dCTM. Brain imaging features were analyzed, including Bern score, quantitative WMH burden, and WMH distribution. Demographics and clinical symptoms present at the time of presentation were recorded. Imaging features were compared between groups with and without CVF using parametric or nonparametric comparisons according to variable normality. Multivariate logistic regression explored the relationships between imaging features, clinical symptoms, and the presence of CVF., Results: The cohort included 40 patients with (CVF+) and 32 patients without (CVF-), with no significant age or sex differences. CVF+ patients had significantly higher Bern scores and significantly fewer WMH. There were significant differences in the frequencies of WMH patterns between groups, with a migrainous pattern observed most frequently in CVF-patients. Logistic regression combining Bern score, WMH burden, and WMH pattern demonstrated a better fit for predicting CVF than using Bern score or WMH features alone. Fourteen clinical symptoms showed the greatest differences between CVF+ and CVF-groups. Logistic regression demonstrated a positive association between CVF detection and a pressure/throbbing headache quality, and negative associations for neck pain, facial pain, phonophobia, and anhedonia/depression., Conclusions: These findings suggest a negative association between CVF detection, increased burden of WMH, and a migrainous WMH pattern. Symptom analysis describes distinct clinical phenotypes, challenging orthostatic headache as a defining characteristic. These results support a comprehensive assessment of imaging and clinical presentations in the workup of suspected SIH., Abbreviations: SIH = spontaneous intracranial hypotension; CVF = CSF venous fistula; WMH = white matter hyperintensities; dCTM = dynamic CT myelography; dDSM = dynamic digital subtraction myelography; PPV = positive predictive value; NPV = negative predictive value Received month day, year; accepted after revision month day, year., Competing Interests: Disclosure of potential conflicts of interest should be included here., (© 2024 by American Journal of Neuroradiology.)

Background and Purpose: The inaugural "Spinal CSF Leak: Bridging the Gap" Conference was organized to address the complexities of diagnosing and treating spinal CSF leaks. This event aimed to converge the perspectives of clinicians, researchers, and patients with a patient-centered focus to explore the intricacies of spinal CSF leaks across 3 main domains: diagnosis, treatment, and aftercare., Materials and Methods: Physician and patient speakers were invited to discuss the varied clinical presentations and diagnostic challenges of spinal CSF leaks, which often lead to misdiagnosis or delayed treatment. Patient narratives were interwoven with discussions on advanced radiologic techniques and clinical assessments. Treatment-focused sessions highlighted patient experiences with various therapeutic options, including epidural blood patches, surgical interventions, and percutaneous and endovascular therapies. The intricacies of immediate and long-term postprocedural management were explored., Results: Key outcomes from the conference included the recognition of the need for increased access to specialized CSF leak care for patients and heightened awareness among health care providers, especially for atypical symptoms and presentations. Discussions underscored the variability in individual treatment responses and the necessity for personalized diagnostic and treatment algorithms. Postprocedural challenges such as managing incomplete symptom relief and rebound intracranial hypertension were also addressed, emphasizing the need for effective patient monitoring and follow-up care infrastructures., Conclusions: The conference highlighted the need for adaptable diagnostic protocols, collaborative multidisciplinary care, and enhanced patient support. These elements are vital for improving the recognition, diagnosis, and management of spinal CSF leaks, thereby optimizing patient outcomes and quality of life. The event established a foundation for future advancements in spinal CSF leak management, advocating for a patient-centered model that harmonizes procedural expertise with an in-depth understanding of patient experiences., (© 2024 by American Journal of Neuroradiology.)

Background and Purpose: CSF venous fistula leads to spontaneous intracranial hypotension. The exact mechanisms underlying the development of CSF venous fistula remain unclear: Some researchers have postulated that underlying chronic intracranial hypertension may lead to damage to spinal arachnoid granulations, given that many patients with CSF venous fistulas have an elevated body mass index (BMI). However, individuals with higher BMIs are also more prone to spinal degenerative disease, and individuals with CSF venous fistulas also tend to be older. CSF venous fistula tends to occur in the lower thoracic spine, the most frequent location of thoracic degenerative changes. The current study aimed to examine whether CSF venous fistulas are more likely to occur at spinal levels with degenerative changes., Materials and Methods: Forty-four consecutive patients with CSF venous fistulas localized on dynamic CT myelography were included in analyses. Whole-spine CT was scrutinized for the presence of degenerative changes at each spinal level. The proportion of levels positive for CSF venous fistula containing any degenerative findings was compared to levels without CSF venous fistula using the Fisher exact test. The Pearson correlation coefficient was calculated to explore the association between the burden of degenerative disease and BMI and age and between BMI and opening pressure., Results: Forty-four patients with 49 total CSF venous fistulas were analyzed (5 patients had 2 CSF venous fistulas). Mean patient age was 62.3 (SD, 9.5) years. Forty-seven CSF venous fistulas were located in the thoracic spine; 1, in the cervical spine; and 1, in the lumbar spine. Within the thoracic spine, 39/49 (79.6%) fistulas were located between levels T7-8 and T12-L1. Forty-four of 49 (89.8%) CSF venous fistulas had degenerative changes at the same level. The levels without CSF venous fistulas demonstrated degenerative changes at 694/1007 (68.9%) total levels. CSF venous fistulas were significantly more likely to be present at spinal levels with associated degenerative changes (OR = 4.03; 95% CI, 1.58-10.27; P  = .001). Age demonstrated a positive correlation with the overall burden of degenerative disease (correlation coefficient: 0.573, P  < .001), whereas BMI did not (correlation coefficient: 0.076, P  = .625). There was a statistically significant positive correlation between BMI and opening pressure (correlation coefficient: 0.321, P  = .03)., Conclusions: Results suggest a potential association between spinal degenerative disease and development of CSF venous fistula., (© 2024 by American Journal of Neuroradiology.)

Spontaneous intracranial hypotension (SIH) is a disorder of CSF dynamics that causes a complex clinical syndrome and severe disability. SIH is challenging to diagnose because of the variability of its presenting clinical symptoms, the potential for subtle imaging findings to be easily overlooked, and the need for specialized diagnostic testing. Once SIH is suggested by clinical history and/or supported by initial neuroim-aging, many patients may undergo initial nontargeted epidural blood patching with variable and indefinite benefit. However, data suggest that precise localization of the CSF leak or CSF-venous fistula (CVF) can lead to more effective and durable treatment strategies. Leak localization can be achieved using a variety of advanced diagnostic imaging techniques, although these may not be widely performed at nontertiary medical centers, leaving many patients with the potential for inadequate workup or treatment. This review describes imaging techniques including dynamic fluoroscopic and CT myelography as well as delayed MR myelography and treatment options including percutaneous, endovascular, and surgical approaches for SIH. These are summarized by an algorithmic framework for radiologists to approach the workup and treatment of patients with suspected SIH. The importance of a multidisciplinary approach is emphasized.

This work describes a NATO-university telemedicine collaboration established to perform a teleneurosurgery consult to assist a deployed soldier with a spinal cord injury.