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Background and Objectives: Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial pressure without an identifiable cause that mostly affects obese persons of childbearing age. In this prospective case series, we have evaluated the overall outcome of pregnancy and birth in participants with IIH and their newborn children. We also provide a proposal for the management of pregnant persons with IIH., Methods: In this observational study, neuro-ophthalmological findings, the course of IIH-related symptoms, disease management, and pregnancy outcomes were evaluated. The participants were divided into 3 groups according to the course of the disease during pregnancy: stable, worsened, and new diagnosed. Furthermore, the type of delivery and outcome of newborn children such as gestational age, weight at birth, and the presence of asphyxia were compared between the groups., Results: We observed 47 pregnancies in 42 participants; 2 had spontaneous abortions. There were 19 (47%) participants in the stable, 18 (45%) in the worsened, and 3 (8%) in the new diagnosed groups, respectively. A relapse of IIH occurred in 2 (5%). Worsening of IIH-related symptoms was experienced by 18/37 (49%) participants: headache by 17/18 (94%), tinnitus by 11/18 (61%), and vision by 7/18 (39%) (mostly in the first and second trimester). In 8/18 (44%), the symptoms were transient or alleviated in the second and third trimester. Body mass index before and after pregnancy did not significantly differ among the groups. A total of 8 participants were treated with acetazolamide. The frequency of cesarean section was 17/40 (43%). Preterm delivery occurred in 22%. No increased risk of asphyxia was observed, and all infants, but one, were healthy., Discussion: Worsening of headache, tinnitus, and/or vision were experienced by half of pregnant participants with IIH, mostly transient in the first and second trimester, rarely required specific treatment, and were not identified as a relapse of IIH. There was no difference in gestational age and weight at birth in children among the groups, and no perinatal asphyxia was noted. Weight gain in the participants was not identified as a risk factor for relapse of IIH in pregnancy. The rate of cesarean and preterm delivery was higher than in the non-IIH population. A proposal for the management of IIH in pregnancy is provided., Competing Interests: V. Vukovic-Cvetkovic: lectures for Novartis, Merck, and MSD and investigator in clinical trials with Lundbeck and Allergan. D. Beier: advisory board of Pfizer, Lundbeck, AbbVie, Novartis, Eli Lilly and Company, and Teva and participation in clinical trials of Teva, Lundbeck, and Novartis. L. Buchgreitz: lecture for AbbVie/Allergan. J.J. Korsbæk: funding from Lundbeck of Candy Foundation. R. Jensen: lectures for Pfizer, Eli Lilly and Company, Merck, TEVA, Novartis, Lundbeck, and Allergan; investigator in clinical trials with Eli Lilly and Company, Novartis, and Lundbeck; director of Danish Headache Center and Lifting The Global Burden of Headache; founder of Master of Headache Disorders at the University of Copenhagen; and received research funding from the University of Copenhagen, Rigshospitalet, Lundbeck Foundation, The Medical Society in Copenhagen, The Capital Region, Novo Nordisk Foundation, and Tryg Foundation. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp., (© 2024 American Academy of Neurology.)

Objective: To identify the most frequent causes of secondary pseudotumor cerebri syndrome and compare phenotype, clinical presentation, and symptoms of secondary pseudotumor cerebri syndrome to the primary form of pseudotumor cerebri syndrome, idiopathic intracranial hypertension., Methods: The study was a prospective cohort study including patients with new-onset pseudotumor cerebri syndrome. Diagnostic work up was standardized. Patients were diagnosed with secondary pseudotumor cerebri syndrome or idiopathic intracranial hypertension according to the revised Friedman criteria. Secondary pseudotumor cerebri syndrome patients were categorized into five causes: medication, systemic causes, sleep apnea, cerebrovascular causes, and several competing causes. Phenotype, clinical presentation, symptoms and neuroimaging were compared between groups., Results: Out of 278 cases, 28 secondary pseudotumor cerebri syndrome and 120 idiopathic intracranial hypertension patients were included. The most frequent causes of secondary pseudotumor cerebri syndrome were medication (n  =  8, 28.6%) and systemic causes (n = 8, 28.6%), followed by sleep apnea (n = 5, 17.9%), cerebrovascular causes (n = 4, 14.3%) and several competing causes (n = 3, 10.7%). Secondary pseudotumor cerebri syndrome and idiopathic intracranial hypertension patients were phenotypically alike and predominately female, premenopausal, and obese. Symptoms and objective findings at disease onset were similar between groups., Conclusion: Secondary pseudotumor cerebri syndrome should be considered in all patients with suspected pseudotumor cerebri syndrome as secondary pseudotumor cerebri syndrome and idiopathic intracranial hypertension patients are phenotypically and clinically alike. A thorough diagnostic workup is needed as treatment of idiopathic intracranial hypertension and secondary pseudotumor cerebri syndrome is markedly different.

Background: Our objective was to assess optic nerve sheath diameter (a marker of elevated intracranial pressure) and optic disc elevation (a marker of papilledema) in pseudotumor cerebri syndrome using transorbital sonography., Methods: The study was a prospective case-control study. We included patients with new-onset pseudotumor cerebri syndrome and matched healthy controls. All had fundoscopy, lumbar puncture with opening pressure and transorbital sonography. Sonography was assessed by a blinded observer., Results: We evaluated 45 patients and included 23 cases. We recruited 35 controls. Optic nerve sheath diameter was larger in pseudotumor cerebri syndrome compared to controls (6.3 ± 0.9 mm versus 5.0 ± 0.5 mm, p < 0.001) and so was optic disc elevation (0.9 ± 0.4 mm versus 0.4 ± 0.1 mm, p < 0.001). The optimal cut-off point for optic nerve sheath diameter was 6 mm with a sensitivity of 74% for prediction of pseudotumor cerebri syndrome and 68% for prediction of elevated opening pressure. Specificity was 94%. The optimal cut-off point for optic disc elevation was 0.6 mm. Sensitivity was 100% and specificity 83% for prediction of pseudotumor cerebri syndrome., Conclusion: Optic disc elevation and optic nerve sheath diameter are increased in new-onset pseudotumor cerebri syndrome. Optic disc elevation achieved high specificity and excellent sensitivity for diagnosis of pseudotumor cerebri syndrome. Transorbital sonography (TOS) is a potential, non-invasive screening tool for pseudotumor cerebri syndrome in headache clinics.

Objective: Spontaneous intracranial hypotension (SIH) manifests as orthostatic headache, which can be confirmed by radiological signs of low intracranial pressure on magnetic resonance imaging of the brain. The most common mechanisms of SIH are ruptured meningeal diverticula, ventral dural tears and CSF-venous fistulas. SIH is associated with connective tissue disorders, and cases of SIH onset after trivial trauma have been reported. As SIH is often underdiagnosed, the aim of this study is to identify possible new risk factors of SIH onset in a case series of SIH patients., Materials and Methods: We retrospectively reviewed the medical records of 36 patients diagnosed with SIH. We reviewed and identified potential factors that led to or presented at headache onset in SIH patients., Results: We identified 4/36 (11%) patients that had a close temporal relationship between the onset of SIH symptoms and airplane travel. In all four patients, the clinical and imaging features confirmed the diagnosis of SIH., Conclusion: This is the first report of a case series of four patients with SIH that could be related to airplane travel. Describing four cases (11%) is not proof but should alert us to a possible causal relationship, which calls for further research. We suggest that when taking medical history, thorough details about the patient's activities, such as headache onset, should be documented because of their importance in correctly diagnosing SIH, which is a debilitating, yet treatable, disease., (© 2022 The Authors. Acta Neurologica Scandinavica published by John Wiley & Sons Ltd.)

Objective: To determine the real-world effectiveness and safety of erenumab after 6 months of treatment in chronic migraine patients with therapeutic resistance to multiple classes of prophylactic medication., Methods: The patients were recruited from the Headache Outpatient Clinic of the University Hospital Centre Zagreb, Croatia between March 2019 and November 2019. All participants received erenumab 70 mg for 6 months. Interviews with participants were conducted at baseline and after the treatment period. The following parameters were analysed: reduction percentage of monthly migraine days, monthly migraine days (MMDs), monthly non-migraine headache days (MNDs), monthly headache days (MHDs), pain intensity measured by the visual-analogue scale (VAS), monthly acute migraine medication intake and reported side-effects. Additionally, we analysed the data for migraine with and without aura separately., Results: There was a significant decrease in the mean value of headache frequency and pain intensity parameters after 6 months of treatment with erenumab. Out of the 54 participants included in the analysis, 70.37% had a ≥ 50% reduction in MMDs, while 40.74% had a ≥ 75% reduction. The mean values of MMDs and MHDs were reduced from 10.37 ± 0.38 to 4.59 ± 0.43 days (P < 0.001) and from 22.24 ± 0.70 to 9.74 ± 0.91 days (P < 0.001), respectively. Furthermore, the mean migraine VAS score decreased from 10.00 ± 0 to 6.69 ± 0.24 (P < 0.001). There was no significant difference in effectiveness between participants with migraine with and without aura. Nine participants reported side-effects., Conclusion: Our study indicates that erenumab is a safe and effective therapeutic option for chronic migraine patients., (Copyright © 2022 Elsevier B.V. All rights reserved.)

The diagnosis and management of idiopathic intracranial hypertension (IIH) can be difficult and multiple medical subspecialities are often involved. Several national and international guidelines regarding the investigations and management of IIH have been published in recent years but still there is no consensus about the optimal organization of IIH-care. The objective of this review was to propose and describe a referral pathway and an organization scheme for diagnosis and management of IIH. An extensive search of existing literature was conducted and summarized. In total, 237 IIH-articles were identified and hereof 43 included. The clinical practice in our specialized IIH-clinic is characterized and described. We conclude that an educational campaign involving medical care providers and patients with chronic headaches is necessary. A detailed organizational proposal for a referral pathway and management of IIH patients based on the literature search and our clinical experience from a highly specialized IIH outpatient clinic is suggested and discussed.

Objective: To assess the proportion of individuals who report dizziness and/or vertigo during the prodromal phase or headache phase of migraine., Methods: The databases of MEDLINE and EMBASE were searched for studies on dizziness and/or vertigo during the prodromal phase or headache phase of migraine. Pooled relative frequencies were estimated using a random-effects meta-analysis., Results: We identified nine articles eligible for inclusion. Of these, one study reported results for the prodromal phase, seven studies for the headache phase and one study for both the prodromal and headache phase. In the prodromal phase, 9.0% of individuals with migraine reported dizziness, while 3.3% reported vertigo. During the headache phase, relative frequency of dizziness ranged from 6.7% to 59.6%, while vertigo ranged from 6.4% to 44.7%. The meta-analysis showed a relative frequency of 35.7% for dizziness (95% CI = 13.7-61.5%, I 2  = 99%) and 33.9% for vertigo (95% CI = 26.7-41.5%, I 2  = 87%). Study quality was rated 5/9 or below for seven studies and 6/9 or above for two studies., Conclusion: We found that there is a scarcity of literature on dizziness and vertigo as prodromal- and headache-associated symptoms in individuals with migraine. Methodological variations confound comparisons of epidemiological patterns, although it appears that dizziness and vertigo are more frequent during the headache phase of migraine, compared with the prodromal phase. Future studies should ensure use of standardized definitions and rigorous methodology to enable accurate measurements of dizziness and vertigo in migraine.

Background: The aim of the study was to evaluate the presenting symptoms and signs of idiopathic intracranial hypertension (IIH) in a large cohort of patients and to estimate their possible role in establishing the diagnosis of IIH., Methods: This prospective cohort study in two tertiary centers, the Danish Headache Center in Rigshospitalet-Glostrup and the Neurology Clinic of the Clinical Center of Serbia, included 286 patients referred by attending specialists for possible IIH evaluation. Patients were divided into two groups: one with confirmed IIH diagnosis and one with rejected IIH diagnosis., Results: The diagnosis of IIH was confirmed in 219 (76.6%) patients. It was more often confirmed if the patient was referred by an ophthalmologist than if the referral was from a neurologist (83.6% vs. 69.8%, p = .029) and in patients with higher body mass index (BMI) (p = .032). Transient visual obscurations (p = .006), double vision (p = .033), neck pain (p = .025), and tinnitus (p = .013) were presenting symptoms more frequently reported by patients with IIH diagnosis. In the same group of patients, papilledema (p < .001) and sixth nerve palsy (p = .010) were noted significantly more often. Papilledema was extracted by multivariate analysis as an independent predictor of IIH diagnosis (p < .001)., Conclusion: Although studies investigating IIH report an abundance of presenting symptoms, our results indicate that these symptoms are not diagnostic for IIH. Papilledema is the most reliable clinical sign predicting the correct IIH diagnosis in patients with suspected IIH., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Small subsets of patients who fail to respond to pharmacological treatment may benefit from alternative treatment methods. In the last decade, neurostimulation is being explored as a potential treatment option for the patients with chronic, severely disabling refractory primary headaches. To alleviate pain, specific nerves and brain areas have been stimulated, and various methods have been explored: deep brain stimulation, occipital nerve stimulation, and sphenopalatine ganglion stimulation are among the more invasive ones, whereas transcranial magnetic stimulation and supraorbital nerve stimulation are noninvasive. Vagal nerve stimulation can be invasive or noninvasive, though this review included only data for noninvasive VNS. Most of these methods have been tested in small open-label patient series; recently, more data from randomized, controlled, and blinded studies are available. Although neurostimulation treatments have demonstrated good efficacy in many studies, it still has not been established as a standard treatment in refractory patients. This review analyzes the available evidence regarding efficacy and safety of different neurostimulation modalities for the treatment of chronic migraine and cluster headache., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)