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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]

Objectives: Identify how the American Headache Society (AHS) membership manages status migrainosus (SM) among outpatients. Background: SM is defined as a debilitating migraine attack lasting more than 72 h. There is no standard of care for SM, including whether a 72‐h duration is required before the attack can be treated as SM. Methods: The Refractory Headache Special Interest Group from AHS developed a four‐question survey distributed to AHS members enquiring (1) whether they treat severe refractory migraine attacks the same as SM regardless of duration, (2) what their first step in SM management is, (3) what the top three medications they use for SM are, and (4) whether they are United Council for Neurologic Subspecialties (UCNS) certified. The survey was conducted in January 2022. Descriptive statistical analyses were performed. Results: Responses were received from 196 of 1859 (10.5%) AHS members; 64.3% were UCNS certified in headache management. Respondents treated 69.4% (136/196) of patients with a severe refractory migraine attack as SM before the 72‐h period had elapsed. Most (76.0%, 149/196) chose "treat remotely using outpatient medications at home" as the first step, 11.2% (22/196) preferred procedures, 6.1% (12/196) favored an infusion center, 6.1% (12/196) sent patients to the emergency department (ED) or urgent care, and 0.5% (1/196) preferred direct hospital admission. The top five preferred medications were as follows: (1) corticosteroids (71.4%, 140/196), (2) nonsteroidal anti‐inflammatory drugs (NSAIDs) (50.1%, 99/196), (3) neuroleptics (46.9%, 92/196), (4) triptans (30.6%, 60/196), and (5) dihydroergotamine (DHE) (21.4%, 42/196). Conclusions: Healthcare professionals with expertise in headache medicine typically treated severe migraine attacks early and did not wait 72 h to fulfill the diagnostic criteria for SM. Outpatient management with one or more medications for home use was preferred by most respondents; few opted for ED referrals. Finally, corticosteroids, NSAIDs, neuroleptics, triptans, and DHE were the top five preferred treatments for home SM management. Plain Language Summary: In this research study, we asked members of the American Headache Society how they treat status migrainosus, a very severe migraine attack lasting more than 3 days. Out of the 196 people who answered the survey, most used stronger treatments before the headache lasted 72 h, and the majority preferred to use home‐based treatments to avoid the hospital. The five most common treatments they use are corticosteroids (like dexamethasone), nonsteroidal anti‐inflammatory drugs (like ketorolac), neuroleptics (like prochlorperazine), triptans (like naratriptan), and dihydroergotamine. [ABSTRACT FROM AUTHOR]

Objective: To describe headache characteristics, medication use, disability, and quality of life in a large patient cohort from the United States who have chronic migraine (CM) and medication overuse headache (MOH). Methods: In all, 610 adult patients were enrolled into the Medication Overuse Treatment Strategy trial from 34 healthcare clinics, including headache specialty, general neurology, and primary care clinics. Descriptive statistics characterize baseline demographics, headache characteristics, medication use, disability (Headache Impact Test 6 [HIT‐6] and Migraine Functional Impact Questionnaire [MFIQ]), pain interference (PROMIS Pain Interference), and quality of life (EQ‐5D‐5L). Relationships with headache frequency were assessed. Results: Mean age was 45 years (SD 13) and 531/608 (87.3%) were females. Mean headache days per 30 was 24.3 (SD 5.5), including 13.6 (SD 7.1) with moderate to severe headache. Daily headaches were reported by 36.1% (219/607) of patients. Acute headache medications were used on 21.5 (SD 7.5) per 30 days. The most commonly overused medications were simple analgesics (378/607, 62% of patients), combination analgesics (246/607, 41%), and triptans (128/607, 21%). HIT‐6, MFIQ, PROMIS Pain Interference, and EQ‐5D‐5L scores demonstrated substantial negative impact from CM with MOH on patient functioning and quality of life. Higher headache frequency was associated with more moderate–severe headache days, more frequent acute headache medication use, greater headache‐related disability, and lower quality of life. Only 272/606 (44.9%) were taking migraine preventive medication. Conclusions: CM with MOH is associated with a large burden on patients in the United States. Higher headache frequency is associated with greater impact on functioning, pain interference, and quality of life. [ABSTRACT FROM AUTHOR]

Background: The American Registry for Migraine Research (ARMR) is a multicenter, prospective, longitudinal patient registry, biorepository, and neuroimaging repository that collects clinical data, electronic health record (EHR) data, blood samples, and brain imaging data from individuals with migraine or other headache types. In this manuscript, we outline ARMR research methods and report baseline data describing an initial cohort of ARMR participants. Methods: Adults with any International Classification of Headache Disorders (ICHD) diagnosis were prospectively enrolled from one of the 8 participating headache specialty centers. At baseline, ARMR participants complete web‐based questionnaires, clinicians enter the participant's ICHD diagnoses, an optional blood specimen is collected, and neuroimaging data are uploaded to the ARMR neuroimaging repository. Participants maintain the ARMR daily headache diary longitudinally and follow‐up questionnaires are completed by participants every 3 months. EHR data are integrated into the ARMR database from a subset of ARMR sites. Herein, we describe the ARMR methodology and report the summary data from ARMR participants who had, from February 2016 to May 2019, completed at least 1 baseline questionnaire from which data are reported in this manuscript. Descriptive statistics are used to provide an overview of patient's sociodemographics, headache diagnoses, headache characteristics, most bothersome symptoms other than headache, headache‐related disability, comorbidities, and treatments. Results: Data were available from 996 ARMR participants, enrolled from Mayo Clinic Arizona, Dartmouth‐Hitchcock Medical Center, University of Utah, University of Colorado, Thomas Jefferson University, University of Texas Health Science Center at Houston, Georgetown University Medical Center, and DENT Neurological Institute. Among ARMR participants, 86.7% (n = 864) were female and the mean age at the time of enrollment was 48.6 years (±13.9; range 18‐84). The most common provider‐reported diagnosis was chronic migraine (n = 622), followed by migraine without aura (n = 327), migraine with aura (n = 196), and medication overuse headache (n = 65). Average headache frequency was 19.1 ± 9.2 days per month (n = 751), with 68% reporting at least 15 headache days per month. Sensitivity to light was the most frequent (n = 222) most bothersome symptom overall, other than headache, but when present, cognitive dysfunction was most frequently (n = 157) the most bothersome symptom other than headache. Average migraine disability assessment (MIDAS) score was 52 ± 49 (n = 760), (very severe headache‐related disability); however, 17% of the ARMR population had MIDAS scores suggesting "no" or "mild" disability. The most common non‐headache health issues were allergies (n = 364), back pain (n = 296), neck pain (n = 296), depression (n = 292), and anxiety (n = 278). Nearly 85% (n = 695) of patients were using preventive medications and 24.7% were using non‐medication preventive therapy (eg, vitamins and neuromodulation). The most common preventive medication classes were neurotoxins, anticonvulsants, antidepressants, vitamins/supplements, and anticalcitonin gene‐related peptide ligand or receptor‐targeted monoclonal antibodies. Nearly 90% (n = 734) of ARMR participants was taking medications to treat migraine attacks, with the most common classes being triptans, non‐steroidal anti‐inflammatory drugs, antiemetics, acetaminophen, and combination analgesics. Conclusions: ARMR is a source of real‐world patient data, biospecimens, and brain neuroimaging data that provides comprehensive insight into patients with migraine and other headache types being seen in headache specialty clinics in the United States. ARMR data will allow for longitudinal and advanced analytics that are expected to lead to a better characterization of patient heterogeneity, healthcare resource utilization, identification of endophenotypes, factors that predict treatment outcomes and clinical course, and ultimately advance the field toward precision headache medicine. [ABSTRACT FROM AUTHOR]

Vitiligo has been associated with the host's genetic profile, metabolic abnormality and immunostatus. The purpose of this study was to investigate the association of vitiligo with autoimmune diseases for 31 out of 39 subjects with vitiligo and their first-degree relatives living in a small Caucasian inbred rural community. They were compared with healthy individuals. A 2.28% prevalence of vitiligo was calculated and the presence of consanguine marriages (72.3%) was noted for this community. Our results indicate an increased prevalence of thyroidopathies, diabetes mellitus and rheumatoid arthritis in families with vitiligo. We also show that the Apa-I polymorphism of the vitamin D receptor gene is associated with vitiligo. This is the first study of its kind performed in Romania suggesting that the vitamin D receptor gene might play a role in the aetiopathogenesis of skin depigmentation. [ABSTRACT FROM AUTHOR]

Objective: "Pain interference" and "headache impact" refer to negative consequences that pain and headache have on one's life. This study investigated determinants of these negative impacts in a large patient cohort who have chronic migraine with medication overuse. Methods: Six hundred and eleven adults were enrolled from 34 headache, neurology, and primary care clinics. Negative consequences of chronic migraine with medication overuse were determined using the Patient-Reported Outcomes Measurement Information System (PROMIS) Pain Interference 6b questionnaire and the Headache Impact Test 6. Relationships between PROMIS-6b and Headache Impact Test 6 scores with demographics, headache characteristics, medication use, anxiety symptoms, and depression symptoms were assessed with linear regression. Elastic Net regression was used to develop a multiple regression model. Results: PROMIS-6b T-Scores averaged 65.2 (SD 5.4) and Headache Impact Test 6 scores averaged 65.0 (SD 5.3), indicating severe negative consequences of chronic migraine with medication overuse. Chronic migraine with medication overuse interfered with enjoyment of life, concentration, daily activities, doing tasks away from home, and socializing. Depression symptom severity had the strongest relationship with pain interference and headache impact. Moderate-to-severe headache frequency, headache intensity, and anxiety symptoms were also associated with pain interference and headache impact. Conclusions: Chronic migraine with medication overuse is associated with substantial negative consequences, the extent of which is most strongly related to depression symptoms. [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.)

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.