Phenotyping non-idiopathic pseudotumor cerebri syndrome - A prospective cohort study.

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.

Transorbital sonography: A non-invasive bedside screening tool for detection of pseudotumor cerebri syndrome.

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.

Airplane flights triggering spontaneous intracranial hypotension: Observations from the Danish headache centre.

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.

Dizziness and vertigo during the prodromal phase and headache phase of migraine: A systematic review and meta-analysis.

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%, I2 = 99%) and 33.9% for vertigo (95% CI = 26.7-41.5%, I2 = 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.

Neurostimulation for the treatment of chronic migraine and cluster headache.

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.

A boy with bilateral SUNA: A case report.

Background Short-lasting unilateral neuralgiform headache attacks (SUNA) is a primary headache characterized by frequent attacks of severe headaches in association with ipsilateral cranial autonomic features. SUNA is defined as a strictly unilateral pain and bilateral cases are very unusual, so secondary causes should be searched for vigorously if there are bilateral symptoms. Despite a number of therapeutic trials, effective management for the majority of SUNA patients is not available at present. Management of SUNA is often difficult. Case We report the case of a young boy with bilateral SUNA attacks, with no detected underlying cause, who is responsive to indomethacin. Conclusion Rarely, primary SUNA can present with bilateral symptoms. According to our experience in this case, indomethacin should always be offered to patients with suspected SUNA.

Management and Outcome of Pregnancy in Patients With Idiopathic Intracranial Hypertension: A Prospective Case Series Study.

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.

Effectiveness and safety of erenumab in chronic migraine: A Croatian real-world experience.

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.

Awareness, Diagnosis and Management of Idiopathic Intracranial Hypertension.

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.

Predictive role of presenting symptoms and clinical findings in idiopathic intracranial hypertension.

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.

Bilateral Vertebral Artery Dissection - Multiple Artery Affection or Early Recurrence?

In a certain percentage of patients with craniocervical artery dissection, dissections affect multiple arteries. Some investigators consider that the dissections diagnosed as multiple might have occurred sequentially within a short time frame. We describe an oligosymptomatic patient with bilateral progressive vertebral artery dissection. Careful history taking added new data on transient left arm weakens two months earlier, as a possibility of the earlier disease onset.

Prevalence of chronic headache in Croatia.

BACKGROUND: Chronic headache describes the presence of headache for >15 days per month on average for >3 months and fulfills the rest of the IHS criteria. The prevalence of chronic headache is within the range of 0.5-7.3% worldwide. The aim of this study was to determine the 1-year prevalence of chronic headache in adult Croatian population. METHODS: The data were collected from a cross-sectional survey of an adult population (>18 years of age) sample. Randomly selected patients from the general population in four Croatian cities were asked to fulfill a self-completed questionnaire. The prevalence of chronic headache was calculated in the sample representing 3,383,769 Croatian adults. RESULTS: The total sample included 1542 responders among which 616 were with headache. The 1-year prevalence of chronic headache was 2.4%, and 0.9% of responders declared having headache 30 days per month. According to these results, 81,192 adult inhabitants in Croatia suffer from chronic headache. CONCLUSIONS: The prevalence of chronic headache in Croatia is comparable to other countries worldwide. These patients require special attention and should be offered multidisciplinary medical support.

Psychoneuroimmunologic aspects of skin diseases.

As mental and psychological issues are important in the development of many dermatologic diseases, these factors are of special interest in research. Psychoneuroimmunology is the study of interaction between psychological processes and the nervous and immune systems of the human body, and it was comprehensively described for the first time about 30 years ago. Communication between the mind and the skin involves the psycho-immuno-endocrine-cutaneous system, encompassing the activities of the brain, the immune system and the skin, with participation of different neuropeptides, interleukins, and immune system messengers. Many common dermatologic diseases have some form of psychomediated pathogenesis that partially accounts for the development of skin lesions. There is a link between emotional stressors (acute or chronic), psychiatric diseases, and dermatoses (e.g., psoriasis, atopic dermatitis, urticaria, viral warts, herpes simplex, vitiligo, acnes, alopecia, prurigo, etc.) and different cytokines and mediators produced in the skin and involved in their pathogenesis. A prominent role is played by those agents that belong to the hypothalamic-pituitary-adrenal axis.

Microembolus detection by transcranial Doppler sonography: review of the literature.

Transcranial Doppler can detect microembolic signals which are characterized by unidirectional high intensity increase, short duration, random occurrence, and a "whistling" sound. Microembolic signals have been detected in a number of clinical settings: carotid artery stenosis, aortic arch plaques, atrial fibrillation, myocardial infarction, prosthetic heart valves, patent foramen ovale, valvular stenosis, during invasive procedures (angiography, percutaneous transluminal angioplasty), surgery (carotid, cardiopulmonary bypass, orthopedic), and in certain systemic diseases. Microembolic signals are frequent in large artery disease, less commonly detected in cardioembolic stroke, and infrequent in lacunar stroke. This article provides an overview about the current state of technical and clinical aspects of microembolus detection.

Report of the Croatian Society for Neurovascular Disorders, Croatian Medical Association. Evidence based guidelines for treatment of primary headaches--2012 update.

These guidelines have been developed to assist the physician in making appropriate choices in work-up and treatment of patients with headaches. The specific aim of the Evidence Based Guidelines for Treatment of Primary Headaches--2012 Update is to provide recommendations for establishing an accurate diagnosis and choose the most appropriate therapy in the group of patients with primary headaches, based on a comprehensive review and meta-analysis of scientific evidence with regard to treatment possibilities in Croatia. These data are based on our previous Evidence Based Guidelines for Treatment of Primary Headaches published in 2005 and other recommendations and guidelines for headache treatment.

Management of patients with transient ischemic attack (TIA) at Sestre milosrdnice University Hospital Center.

Improved outcomes were observed in transient ischemic attack (TIA) patients after implementation of recommendations for stroke management and after multiple interventions such as public campaigns focused on raising awareness of stroke and reorganization of health services. The aim of this study was to describe reorganization of in-hospital services to improve the management of patients suspected of having TIA or stroke, and to validate these measures with patient outcomes. Data on 5219 patients examined between January 1 and December 31, 2008 at emergency neurology outpatient department were analyzed. Patients were referred by general practitioners, emergency physicians, or were brought by relatives without being previously seen by health services staff. The emergency services department is intended to improve care for TIA patients, providing a short standardized clinical assessment followed by initiation of a comprehensive stroke prevention program. Demographic data, risk factors, stroke type, previous TIA history, ABCD2 scores and admission rates were analyzed. A total of 1057 patients suspected of having stroke or TIA were examined. There were 447 patients with ischemic stroke (mean age 73 +/- 11 years, 196 males) and 99 patients with TIA (mean age 67 +/- 14 years, 55 males). Parenchymal hemorrhage was diagnosed in 56 and subarachnoid hemorrhage in 49 patients, while 406 patients had nonspecific symptoms or other systemic or neurologic diseases. TIA preceded stroke in 29 (6.5%) patients and 197 (44%) patients were examined for worsening of stroke symptoms (133 within 24 hours, 47 within 48 hours, and 17 within 7 days). The mean ABCD2 score was 2.95. In all examined patients, a comprehensive stroke prevention program was started; 427/447 (95%) strokes and 31/99 (31%) TIAs were hospitalized at neurology department. Four (4%) TIA patients developed stroke and were hospitalized, three of them after 2 days (ABCD2 score 3.4 and 5) and one after 7 days (ABCD2 score 5). Preventive measures resulted in a low number of strokes after TIA (< 7%), but a relatively high percentage (44%) of stroke patients ignored initial symptoms and sought medical attention after persistence or worsening of the symptoms.

Is iron deficiency anemia related to menstrual migraine? Post hoc analysis of an observational study evaluating clinical characteristics of patients with menstrual migraine.

The aim of this study was to determine the relative prevalence and clinical characteristics of patients with pure menstrual migraine (PMM) and menstrually related migraine (MRM), and to compare them with the women free of it in an outpatient clinic-based population. Adult menstruating women with ICHD-2 migraine were included. Demographic data were obtained by verbal report. Study women were asked to keep a headache diary for 6 months, in which they recorded information on headache characteristics, medication use and presence of menses. Iron deficiency anemia was considered to be present if the patient had received therapy for iron deficiency anemia or laboratory tests indicated low iron and/or hemoglobin levels (within one year). A total of 289 women were included, 52 (18.0%) with PMM and 116 (40.1%) with MRM, whereas 121 (41.9%) women had not observed any relationship between migraine and their menstrual cycle (non-MM). Duration of migraine attacks was longer in PMM/MRM patients (P < 0.0001). No significant difference was observed according to other migraine-associated symptoms. Women with PMM/ MRM took significantly more tryptans (P < 0.0001) and iron deficiency anemia was significantly more common in women with PMM/MRM (P = 0.008). In conclusion, this study supported earlier findings that PMM/MRM has similar clinical characteristics as non-MM, except for longer duration. In addition, iron deficiency anemia is more common in women with PMM/MRM, which may be an underlying mechanism aggravating migraine attacks.