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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.)

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., (© 2024 The Author(s). Headache: The Journal of Head and Face Pain published by Wiley Periodicals LLC on behalf of American Headache Society.)

Background and Objectives: 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., 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, and elastance and whether a leak was found were analyzed., Results: Seventy-two consecutive dCTMs were performed in 53 patients. Twelve CSF-venous fistulae, 2 ruptured meningeal diverticula, 2 dural defects, and 1 dural bleb were found (17/53, 32%). Among patients with imaging-proven CSF leak/fistula, OP was normal in all but 1 patient and was not significantly different in those with a leak compared with those without (15.1 vs 13.6 cm H 2 O, p = 0.24, A = 0.40). The average Bern score in individuals with a leak was significantly higher than that 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). The average elastance in those with a leak was higher than that in those without, but this difference was not statistically significant (2.05 vs 1.20 mL/cm H 2 O, p = 0.19, A = 0.40). Increased elastance was significantly associated with an increased Bern score (95% CI -0.55 to 0.12, p < 0.01) 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., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Purpose of Review: This article reviews nummular headache (NH), including the latest literature on the epidemiological and clinical features, the most recent proposed pathophysiology, and novel management., Recent Findings: NH is characterized by continuous or intermittent head pain confined to a focal circumscribed area (1-6 cm in diameter). It is usually of mild to moderate intensity, although some patients have severe continuous pain or disabling exacerbations. NH is a primary headache, though many secondary cases have been described. Evaluation requires exclusion of systemic and structural diseases. Gabapentin can be recommended as a first-line preventive treatment. If poorly tolerated or in refractory cases, botulinum toxin is a reasonable alternative. NH is a fairly common disorder in patients presenting to a headache clinic. Increased understanding of NH will lead to improved outcomes. Clinical trials would lead to enhanced knowledge of NH.

Purpose of Review: Neuralgias are characterized by pain in the distribution of a cranial or cervical nerve. Typically, they are brief, paroxysmal, painful attacks, although continuous neuropathic pain may occur. The most commonly encountered conditions are trigeminal, postherpetic, and occipital neuralgia. Less common neuralgias include glossopharyngeal, superior laryngeal, auriculotemporal, and nervus intermedius neuralgia, among others. The approach to diagnosis and treatment of this group of disorders is reviewed., Recent Findings: Recent guidelines of medication administration, the use of botulinum toxin, and more targeted procedures have improved treatment of neuralgias. Patients who present with neuralgias should have imaging studies to investigate for structural abnormalities unless the etiology is apparent. Management of both common and rare neuralgias can be challenging and is best guided by the most recent available evidence.