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.

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.

Relationship of Bern Score, Spinal Elastance, and Opening Pressure in Patients With Spontaneous Intracranial Hypotension.

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 H2O, 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 H2O, 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.

Novel Medications for the Treatment of Migraine.

OBJECTIVE: To describe the new classes of medication for headache management and their roles in clinical practice. BACKGROUND: Calcitonin gene-related peptide (CGRP) is a key component in the underlying pathophysiology of migraine. Research focused on targeting CGRP for headache treatment has led to the development of entirely new classes of medications - the gepants and the CGRP monoclonal antibodies (mAbs) - for both acute and preventive treatment. A third class, the ditans, is being developed to target the 5-HT1F receptor to provide acute treatment without vasoconstrictive effects. METHODS: This article reviews the pathophysiology of migraine that has led to these new pharmacologic developments. Available information from randomized controlled trials, abstracts, press releases, and relevant preclinical studies is summarized for each class of medications. RESULTS: At the time of this writing, one ditan has been submitted to the U.S. Food and Drug Administration (FDA) for approval. One gepant is anticipated to be submitted within the first quarter of 2019, and others are in clinical trials. Three CGRP mAbs have been FDA approved and are now available in clinical practice, and a fourth was submitted in the first quarter of 2019. CONCLUSIONS: The development of new migraine-specific classes of medications provides more treatment options for both acute and preventive treatment of migraine.

Nummular Headache.

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.

The Neuralgias.

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.