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Objective: To investigate the prevalence of neuroimaging in patients with primary headaches and the clinician-based rationale for requesting neuroimaging in China., Data Sources and Study Setting: This study included patients with primary headaches admitted to hospitals and clinicians in China. We identified whether neuroimaging was requested and the types of neuroimaging conducted., Study Design: This was a cross-sectional study, and convenience sampling was used to recruit patients with primary headaches. Clinicians were interviewed using a combination of personal in-depth and topic-selection group interviews to explore why doctors requested neuroimaging., Data Collection: We searched for the diagnosis of primary headache in the outpatient and inpatient systems according to the International Classification of Diseases-10 code of patients admitted to six hospitals in three provincial capitals by 2022.We selected three public and three private hospitals with neurology specialties that treated a corresponding number of patients., Principle Findings: Among the 2263 patients recruited for this study, 1942 (89.75%) underwent neuroimaging. Of the patients, 1157 (51.13%) underwent magnetic resonance imaging (MRI), 246 (10.87%) underwent both head computed tomography (CT) and MRI, and 628 (27.75%) underwent CT. Fifteen of the 16 interviewed clinicians did not issue a neuroimaging request for patients with primary headaches. Furthermore, we found that doctors issued a neuroimaging request for patients with primary headaches mostly, to exclude the risk of misdiagnosis, reduce uncertainty, avoid medical disputes, meet patients' medical needs, and complete hospital assessment indicators., Conclusions: For primary headaches, the probability of clinicians requesting neuroimaging was higher in China than in other countries. There is considerable room for improvement in determining appropriate strategies to reduce the use of low-value care for doctors and patients., (© 2024 The Author(s). Brain and Behavior published by Wiley Periodicals LLC.)

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is a syndrome of recurrent thunderclap headaches and reversible vasoconstriction of the cerebral arteries on neuroimaging within 3 months of onset. Initial non-contrast computed tomography (CT) can reveal abnormalities such as ischemic stroke, intracerebral hemorrhage, and subarachnoid hemorrhage (SAH) can be present in patients with RCVS and may delay diagnosis., Aims: We conducted a systematic review and meta-analysis in accordance with the PRISMA guidelines. We aimed to estimate the prevalence of imaging abnormalities on initial non-contrast CT head in adult patients with RCVS., Data Sources & Eligibility Criteria: We searched electronic databases including MEDLINE, EMBASE, and the Cochrane Register of Clinical Trials from inception to August 2, 2022. Eligible studies included articles reporting the prevalence of non-contrast CT abnormalities on initial neuroimaging in patients with RCVS, aged 18 and older. Case series, observational studies and clinical trials were included. Data was extracted directly from included papers using a standardized data charting form., Results: The search yielded 722 titles with duplicates removed. Twenty studies that included 379 patients with RCVS met inclusion criteria. We classified non-contrast CT abnormalities as either ischemic stroke, ICH, or SAH. We pooled prevalence data using a random effects model with the inverse-variance weighted method. The most common imaging finding was SAH with a pooled prevalence of 24% (95% CI:17%-33%), followed by ICH at 14% (95% CI:8%-22%), and ischemic stroke at 10% (95% CI:7%-14%). The pooled prevalence of any of these imaging abnormalities on initial non-contrast CT was 31% (95% CI:23%-40%). Risk of bias was moderate to very-high-risk for case-series and low-risk for observational studies., Conclusion: Our review demonstrates that one-third of patients with RCVS will have an abnormality on initial non-contrast CT head, including either an ischemic stroke, ICH, or SAH. These findings highlight the diagnostic challenges of RCVS imaging and contribute to our understanding of this disease., Competing Interests: The authors declare that there are no conflicts of interest., (Copyright: © 2024 Gotesman et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular transitory condition characterized by severe headache, possible concomitant acute neurological symptoms, evidence of diffuse multifocal segmental constriction of cerebral arteries, and usually spontaneously resolving within 3 months. Putative causes and/or precipitating factors are vasoactive drugs-e.g., antidepressants, α-sympathomimetics, triptans-post-partum, and immunosuppressants., Case Presentation: We report the case of a middle-aged woman referred to the emergency room (ER) with a 7-day long intense headache and vomit. Cerebral non-contrast computed tomography (CT) was negative for acute ischemic lesions or intracranial bleedings. She was again referred to ER 7 days later with additional fluctuating episodes of weakness in left arm and both lower limbs. A new brain CT was negative. Due to worsening headache, a transcranial color-coded Doppler (TCCD) was performed, which showed diffuse multifocal blood flow acceleration in all principal intracranial vessels, and particularly on the right hemisphere. These findings were subsequently confirmed at MR angiogram and digital subtraction angiography., Conclusion: TCCD imaging is a non-invasive and relatively inexpensive tool which provides real-time information on cerebrovascular function, blood flow velocities, and hemodynamic changes. TCCD may be a powerful tool in the early detection of acute infrequent cerebrovascular conditions, as well as in monitoring their course and the therapeutic response., (© 2023. The Author(s).)

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is a disease characterized by reversible multifocal narrowing of the cerebral arteries with clinical manifestations that typically include thunderclap headache and occasionally brain edema, stroke, or seizure. The exact pathophysiology of RCVS is not well known., Case: A 46-year-old female with history of episodic migraine presented with 1-month duration of worsening headaches that had become more severe over the past 2 weeks. The headaches were episodic and thunderclap in onset and aggravated by physical exertion or emotional situations. A neurological examination was unremarkable including initial head computed tomography (CT). A CT angiogram of the head showed multifocal stenosis in the right anterior cerebral artery, bilateral middle cerebral arteries, and right posterior cerebral artery. Cerebral angiogram confirmed the CT angiogram findings. A repeated CT angiogram a few days later showed improvement in the multifocal cerebral arterial stenosis. Lumbar puncture and autoimmune workup were not suggestive of neuroinflammatory etiology. She had one generalized tonic-clonic seizure during her second day of hospitalization. The patient's thunderclap onset headaches resolved in 1 week after she was managed with blood pressure control and pain medication. She denied any illicit drug use or any new medications other than the placement of a levonorgestrel-releasing intrauterine device (IUD) about 6 weeks prior to her presentation., Conclusions: Our case suggests a possible link between RCVS and levonorgestrel-releasing IUDs., (© 2023 American Headache Society.)

Background: Angiographic vasoconstriction in reversible cerebral vasoconstriction syndrome (RCVS) is often undetectable at symptom onset and the diagnosis relies on clinical presentation. Although thunderclap headache is a hallmark feature of RCVS, the incidence and predictors of long-term headaches (LTH) are incompletely understood. Our study aims were twofold: to examine the sensitivity and specificity of a recently developed score (RCVS 2 ) for vasoconstriction detection in a real-world clinical context and describe the incidence and predictors of LTH beyond the acute phase of RCVS., Methods: Retrospective analysis of consecutive patients with clinical diagnosis of RCVS in a tertiary hospital between 2017 and 2021. We examined associations between demographic factors, comorbidities, medications, imaging characteristics, and LTH (defined as at least one episode present at greater than 6-months follow-up necessitating medication). We separately examined the association between RCVS 2 score and angiographic vasoconstriction and computed its sensitivity, specificity, and negative and positive predictive value based on established cutoffs (certain ≥ 5, negative ≤ 2)., Results: We included 55 patients, 50.5 (± 13.7) years; 41 (75%) female. 25 (49%) patients had LTH; only prior history of headache was significantly associated with LTH [OR 4.3, 95% CI (1.1-16.2), p = 0.03]. We found a significant association between RCVS 2 score and angiographic vasoconstriction [OR 1.49, 95% CI (1.18-1.88), p = 0.001]; sensitivity, specificity, and positive and negative predictive value were 64%, 94%, 95% and 58% respectively., Conclusions: Approximately 50% of RCVS patients experienced LTH; only prior headache history was associated with its incidence. The RCVS2 score had a significant association with high specificity and positive predictive value for angiographic vasoconstriction in our cohort, validating its utility in improving the accuracy of diagnosis in the clinical setting., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Reversible cerebral vasoconstriction syndrome (RCVS) and transient global amnesia (TGA) are acute and self-limiting intra-cerebral conditions. Although previously studied as independent phenomena, there are increasing reports of co-occurrence of these two pathologies. We report a 55-year-old male who presented to the hospital with recurrent thunderclap headaches over the course of 1 week with sudden onset of anterograde memory loss. His medications included a selective serotonin reuptake inhibitor and intermittent use of pseudoephedrine. On examination he was amnestic to recent events and notably perseverating. Magnetic resonance imaging of the brain without contrast showed a small, punctate focus of restricted diffusion in the left hippocampus. He was diagnosed with TGA based on his clinical presentation. His headaches and amnesia resolved over the next 12 h throughout the course of his stay with acetaminophen and oral verapamil and he was discharged. Repeat computed tomography angiogram at 2 weeks revealed diffuse and segmental narrowing of the anterior and posterior intracranial circulation, which resolved on follow-up imaging at 3 months, confirming RCVS. The acute and reversible nature of these conditions and increasing reports of co-occurrence suggests a common pathophysiologic link. We review the literature highlighting similar cases and the presumed pathophysiology., (© 2022 American Headache Society.)

Objective: This study aimed to determine the prevalence and characteristics of primary stabbing headache in children and adolescents that presented because of headache. Materials and Methods: The medical files of 772 children and adolescents who presented with headache to the Pediatric Neurology Outpatient Clinic at Başkent University between 2012 and 2020 were retrospectively reviewed. In total, 77 patients (9.97%) with primary stabbing headache and those thought to have primary stabbing headache were included in the study. Patient data, including demographic features, headache characteristics, family history of primary headache, electroencephalographic (EEG) findings, and cranial magnetic resonane imaging (MRI) findings, were noted. Results: Age at presentation was <6 years in 16.9% of the patients and onset time of headache was below 3 months in 55.8%. Daily headache attacks occurred in 46.8% of the patients. Headache localization was frontal in 54.5% of patients and bilateral in 68.8%, whereas the quality of headache was undefined in 40.3%. Headache attack duration was seconds long in 37.7% of the patients, attacks occurred at any time of the day in 83.1%, and 80.5% did not have accompanying symptoms. In all, 54.5% of the patients had a negative family history of primary headache. In 95.8% of the patients, EEG findings were normal and cranial MRI findings were normal in 100% of the patients. Conclusion: The prevalence of primary stabbing headache is not rare in children and adolescents. Clinician awareness of the diagnosis and underlying causes of primary stabbing headache should be increased.

Background: To differentiate primary headache associated with sexual activity from other devastating secondary causes., Methods: In this prospective cohort, we recruited consecutive patients with at least 2 attacks of headache associated with sexual activity from the headache clinics or emergency department of a national medical center from 2005 to 2020. Detailed interview, neurological examination, and serial thorough neuroimaging including brain magnetic resonance imaging and magnetic resonance angiography scans were performed on registration and during follow-ups. Patients were categorized into four groups, i.e. primary headache associated with sexual activity, reversible cerebral vasoconstriction syndrome, probable reversible cerebral vasoconstriction syndrome, and other secondary headache associated with sexual activity through a composite clinic-radiological diagnostic algorithm. We compared the clinical profiles among these groups, including sex, age of onset, duration, quality, and clinical course ("chronic" indicates disease course ≥ 1 year). In addition, we also calculated the score of the reversible cerebral vasoconstriction syndrome 2 , a scale developed to differentiate reversible cerebral vasoconstriction syndrome from other intracranial vascular disorders., Results: Overall, 245 patients with headache associated with sexual activity were enrolled. Our clinic-radiologic composite algorithm diagnosed and classified all patients into four groups, including 38 (15.5%) with primary headache associated with sexual activity, 174 (71.0%) with reversible cerebral vasoconstriction syndrome, 26 (10.6%) with probable reversible cerebral vasoconstriction syndrome, and 7 (2.9%) with other secondary causes (aneurysmal subarachnoid hemorrhage (n = 4), right internal carotid artery dissection (n = 1), Moyamoya disease (n = 1), and meningioma with hemorrhage (n = 1)). These four groups shared similar clinical profiles, except 26% of the patients with primary headache associated with sexual activity had a 3 times greater chance of running a chronic course (≥ 1 year) than patients with reversible cerebral vasoconstriction syndrome. Of note, the reversible cerebral vasoconstriction syndrome 2 score could not differentiate reversible cerebral vasoconstriction syndrome from other groups., Conclusion: Our composite clinic-radiological diagnostic algorithm successfully classified repeated headaches associated with sexual activity, which were predominantly secondary and related to vascular disorders, and predicted the prognosis. Primary headache associated with sexual activity and reversible cerebral vasoconstriction syndrome presented with repeated attacks of headache associated with sexual activity may be of the same disease spectrum.

Purpose of Review: This article describes the causes, clinical and imaging features, management, and prognosis of posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS), in which the underlying pathophysiology is related to reversible dysregulation of the cerebral vasculature., Recent Findings: PRES and RCVS are descriptive terms, each bringing together conditions with similar clinical-imaging manifestations. Headache, visual symptoms, seizures, and confusion occur in both syndromes. RCVS is usually heralded by recurrent thunderclap headaches, whereas encephalopathy and seizures are typical in PRES. In PRES, brain imaging shows reversible vasogenic edema that is typically symmetric and located in subcortical regions (mostly posterior predominant). In RCVS, brain imaging is often normal; cerebral angiography shows segmental vasoconstriction-vasodilatation affecting the circle of Willis arteries and their branches. Aside from shared clinical features, significant imaging overlap exists. Both PRES and RCVS can be complicated by ischemic and hemorrhagic brain lesions; angiographic abnormalities frequently occur in PRES and vasogenic edematous lesions in RCVS. Common triggers (eg, eclampsia, vasoconstrictive and chemotherapeutic agents) have been identified. Abnormal cerebrovascular tone and endothelial dysfunction may explain both syndromes. Management of these syndromes includes the removal of identified triggers, symptomatic treatment of headache or seizures, and moderate blood pressure control. Both syndromes are self-limited, with clinical recovery occurring within days to weeks. Long-term deficits and mortality are uncommon., Summary: PRES and RCVS have been well characterized and acknowledged to have significant overlap. Advances in our understanding of pathophysiology and risk factors for poor outcome are expected to optimize the management of these not uncommon syndromes., (Copyright © 2021 American Academy of Neurology.)

Purpose of Review: Thunderclap headache (TCH) is an abrupt-onset of severe headache that needs to be thoroughly investigated because the most common secondary cause is subarachnoid hemorrhage (SAH). There has been no consensus guideline regarding the diagnostic workup. This review aims to provide an update on the evaluation of TCH., Recent Findings: The most important update in the 2019 American College of Emergency Physicians guideline for evaluation of acute headache in the emergency department is that negative noncontrast brain computed tomography (CT) findings within 6 h from ictus essentially excludes SAH. Additionally, the updated guideline recommends that after a negative brain CT, CT angiogram is a reasonable alternative to lumbar puncture if clinical suspicion of an intracranial source of SAH is high. An important update of reversible vasoconstriction syndrome (RCVS), the second most common etiology of TCH, is the RCVS2 score development based on clinical and radiological features, providing high specificity and sensitivity for distinguishing RCVS from other intracranial arteriopathies., Summary: Although the evaluation of TCH is exhaustive, the potentially catastrophic consequence of a missed diagnosis of sentinel headache justifies the efforts. Awareness of the clinical features and application of diagnostic tools specific for different pathological conditions can facilitate the diagnostic workup., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: We aimed to analyze clinical characteristics, treatment patterns, and prognosis of patients with reversible cerebral vasoconstriction syndrome (RCVS)., Materials and Methods: Two investigators independently searched PubMed and EMBASE, and 191 cases were included in this study. Information regarding demographics, triggering factors, brain imaging findings, treatment modalities, recurrence, and clinical outcome was collected., Results: The mean age of the patients was 39.9 years, and 155 (81.2%) were female. The most common triggering factor for RCVS was an exposure to vasoactive substances (41.4%), followed by pregnancy/postpartum (20.9%), and sexual intercourse (10.5%). Multifocal stenosis (84.0%) and beading shape (82.4%) were the leading abnormal findings on angiography, while cerebral ischemic lesions (47.6%) and cerebral hemorrhage (mainly subarachnoid hemorrhage) (35.1%) were the main findings on brain computed tomography (CT)/magnetic resonance imaging (MRI). Calcium channel blockers (nimodipine/verapamil) were the most commonly used medications (44.5%) in the treatment of RCVS. Multivariate analysis identified that RCVS was precipitated by trauma/surgery/procedure (hazard ratio (HR): 3.29, 95% confidence interval (CI) (1.21-8.88), p=0.019), and presence of aphasia/neglect/apraxia during the acute phase of the disease (HR: 3.83, 95% CI (1.33-11.05), p=0.013) were found to be the two independent risk factors for residual neurological deficit after RCVS., Conclusions: In our systematic review, vasoactive substances were the most frequent triggers for RCVS, which was most commonly accompanied by angiographic findings of multifocal stenotic lesions. Patients with RCVS precipitated by trauma or surgical procedures and those with focal cortical deficits had a higher risk of residual neurological deficits, and these patients should closely be monitored.

Introduction: The evaluation of red flags is crucial for the accurate the diagnosis of headache disorders, especially for thunderclap headache. We analysed if secondary headache disorders were adequately ruled out in patients that presented to the emergency room with thunderclap headache., Methods: In this retrospective cohort study, we screened all patients that visited the emergency room for headache, including those that described thunderclap headache. We measured the frequency with which secondary causes were not adequately ruled out. We analysed the order of the exams, the final diagnosis, and the time elapsed between arrival, initial request for imaging, and the completion of the imaging., Results: We screened 2132 patients, and 42 (1.9%) fulfilled eligibility criteria. Mean age was 43.1 ± 17.1 years, and 57% of patients were female. For 22 (52.4%) patients, the work-up was incomplete. Vascular study was missing in 16 (38.1%) patients, cerebrospinal fluid evaluation in nine (21.4%), and magnetic resonance imaging in seven (16.7%), with multiple assessments missing in six (14.3%). There were ten different combinations in which the exams were performed, with the most frequent being the second exam's cerebral spinal fluid evaluation in 18 (52.9%) and the computed tomography angiogram in 10 (29.4%). A secondary cause of thunderclap headache was found in 16 (38.1%) patients, and four (9.5%) had a primary headache diagnosis after an adequate and complete study., Conclusions: Thunderclap onset was described in one of every 50 patients that visited the emergency room for headache. More than half of these patients were not adequately managed. More than a third of thunderclap headache patients had a secondary cause.

Background: Finding an intracranial aneurysm (IA) during a thunderclap headache (TCH) attack, represents a problem because it is necessary to distinguish whether the aneurysm is responsible for the headache as a warning leak or as an incidental finding. High-Resolution Vessel-Wall (HRVW) MRI sequences have been proposed to assess the stability of the wall, as it permits to detect the presence of aneurysmal wall enhancement (AWE). In fact, AWE has been confirmed due to inflammation, recognizable preceding rupture.Case 1: A 37-year-old woman with a migraine more intense than her usual. A CTA revealed a 10 mm AComA aneurysm without subarachnoid hemorrhage (SAH) and HRVW-MRI excluded AWE. The patient's headache improved, and therefore, the aneurysm was considered an incidental finding, and the headache diagnosed as TCH attack. Subsequently, the aneurysm was surgically clipped, and typical migraine relapsed was reported at follow-up (FU).Case 2: A 67-year-old woman with no history of headaches underwent CTA for an abrupt onset of headache. A 7 mm right carotid-ophthalmic aneurysm with no sign of SAH was discovered. HRVW-MRI demonstrated AWE and thus, a TCH attack for a warning leak of an unstable wall was suspected. Endovascular coiling was immediately performed and at FU any further headache attack was reported., Conclusions: HRVW-MRI is useful in case of finding aneurysm as the cause of headaches, particularly the TCH attack. In fact, HRVW-MRI could assess the stability of the aneurysms wall, allowing different patient management and eventually the aneurysmal treatment.

Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular disorder associated with multifocal intracranial arterial constriction and dilation that occurs spontaneously or as a result of a stimulant. The authors present a case of RCVS in a patient who presented with a new-onset thunderclap headache a day after carotid endarterectomy (CEA). RCVS has been rarely reported after CEA., Case Report: A 65-year-old woman was evaluated for a new-onset thunderclap headache a day after left-sided CEA. Computed tomography (CT) of the head revealed left frontal and parietal subarachnoid hemorrhage (SAH). CT angiography did not show any saccular aneurysms or vessel stenosis. The initial impression was SAH related to reperfusion injury after carotid revascularization. Seven days postoperatively, the patient returned to the hospital with a persistent headache. CT revealed SAH in the vertex of the frontal region bilaterally. Magnetic resonance angiogram (MRA) of the head revealed multifocal stenosis of the intracranial circulation bilaterally. A follow-up MRA 9 weeks postoperatively showed interval improvement of the caliber of the circle of Willis branches and significant improvement of the multifocal stenosis. The patient was diagnosed with RCVS as a result of CEA., Conclusion: The authors advise clinicians to consider RCVS as a cause of thunderclap headache or recurrence of a severe headache shortly after CEA-particularly with the presence of a nonaneurysmal convexity SAH.

Chiari Malformation type 1 (CM-I) is congenital or an acquired anomaly of the hind brain; develops when the cerebellar tonsils recede downwards below the foramen magnum. Recurrent post tussive suboccipital headache is the common presentation in a pregnant woman and the diagnosis is usually missed or delayed due to lack of formal understanding of this neurological pathology. Much has been written regarding presentation, morphology and the treatment of CM-I; however, little is known when the etiology is acquired or an iatrogenic in its evolution. Similarly, unknown is the progression of CM-I (diagnosed or undiagnosed) in pregnancy. The objective of this study is to elucidate the causes of progression of CM-I in pregnancy, and how this can be avoided. A detailed literature review has been conducted to find the case reports or case studies on association of CM-I in pregnancy; therefore, the risk factors regarding the progression have been sought. There is a lack of literature on timing, mode of anesthesia, and the management of CM-I. Moreover, authors have sought a questionnaire to screen these patients at pre-conception, intrapartum visits if, the initial diagnosis is delayed. Crucial points of concern including but not limited to the diagnosis, pre-conception counseling, timing of intervention during pregnancy, and mode of anesthesia, have been discussed in detail. In summary, a formal management algorithm has been proposed to avoid the rapid progression of this complex neurological pathology especially, in women of child bearing age and/or during pregnancy., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Reversible cerebral vasoconstriction syndrome is caused by narrowing of cerebral arteries. The cardinal symptom is relapsing acute-onset headache also known as thunderclap headache, which may be accompanied by neurologic deficits. This is a case report of a 61-year-old woman with CT angiography-verified reversible cerebral vasoconstriction syndrome. We discuss the diagnostic work-up, i.e. lumbar puncture including test for xanthochromia and important brain imaging, differential diagnoses and treatment of the most important causes of thunderclap headache.

Aim: To examine the characteristics of primary stabbing headache (PSH) in typically developing children and adolescents., Method: In this prospective non-interventional hospital-based study, we examined 42 eligible individuals (24 females, 18 males; 26 white; mean age 12y 1mo, range 6y-16y 1mo) with spontaneous transient stabs. A headache focused history and/or a headache diary was obtained from each patient. All patients had normal neurological examination. Diagnosis of primary headache was based on the International Classification of Headache Disorders, Third Edition (ICHD-3) beta version., Results: Duration of stabs ranged from a few seconds to up to 10 minutes. Stabs were located in a variety of sites on the cranium but mostly in the extra-trigeminal regions (n=28). Intensity of stabs varied from moderate (n=4) to severe (n=38). The frequency of the stabs ranged from daily to monthly. There were up to 50 stabs per headache attack. Stabs among our patients occurred independently from those caused by other primary headache types. Accompanying symptoms during stabs were reported by eight patients. Family history of primary headaches was identified (n=17). Familial transmission of PSH was recognized among two patients. All patients had normal brain magnetic resonance imaging., Interpretation: The presentation and nature of PSH in children and adolescents varies widely. PSH in children may be a different entity to that in adults, and there is a need for further research to support changes in the ICHD-3 criteria for PSH in children and adolescents., What This Paper Adds: Presentation of childhood primary stabbing headache (PSH) varies widely. Duration of PSH could last from a few seconds up to 10 minutes., (© 2019 Mac Keith Press.)

Rationale: Reversible cerebral vasoconstriction syndrome (RCVS) is often accompanied by thunderclap headaches. Although symptoms usually resolve spontaneously within 2 months, it can cause fatal complications, such as cerebral hemorrhage, and is difficult to differentiate from a migraine and other headaches on the basis of symptoms and Imaging study. In this case report, we explore clinical findings and appropriate treatment methods for RCVS through the case study of a female patient who experienced severe headache upon defecation PATIENT CONCERNS:: A 42-year-old female patient complained of a severe throbbing headache with a Numeric Rating Scale (NRS) score of 10 after defecation. The pain subsided temporarily after treatment with diclofenac 75 mg and Tridol 50 mg propacetamol 1 g, but the headache returned upon defecation; soon after, the patient complained again of regular headaches at 4 to 6-hour intervals irrespective of defecation., Diagnosis: Brain computed tomography (CT) and head and neck magnetic resonance angiography, performed during a headache episode, revealed no specific neurological findings. Blood analysis was also normal. Head and neck CT angiography, performed one month after the start of the headaches, revealed RCVS., Interventions: Treatment commenced with pregabalin (150 mg), oxycodone HCl/naloxone (10/5 mg), Alpram (0.5 mg), milnacipran (25 mg), and frovatriptan 25 mg, but there was no improvement in the headaches. The patient received bilateral trigger point injections (TPI) in the temporal muscles on four occasions at the pain clinic., Outcomes: Medication showed no effect, but after the patient received four sessions of bilateral TPI in the temporal muscles her NRS score eventually decreased from 10 to 2. The patient is currently continuing medication while still experiencing headaches at reduced intensities., Lessons: RCVS is difficult to diagnose; moreover, it is difficult differentiate RCVS from other headaches. However, as it can cause fatal complications, it should not be overlooked. It is essential to consider diagnostic treatment for all types of headaches because RCVS can be accompanied by headaches originating from other causes.

"Other Primary Headaches" in the ICHD-3 encompasses activity-related headaches, headaches due to direct physical stimuli, epicranial headaches and a miscellanea, including hypnic headache and new daily-persistent headache. They can be primary or secondary and their etiologies differ depending on headache type. For instance, activity-related headaches can be induced by Valsalva maneuvers ("cough headache") or prolonged exercise ("exercise and sexual headaches"). Almost half of cough headaches are secondary to posterior fossa abnormality, whereas only 20% of exertional/sexual headaches are secondary, with subarachnoid hemorrhage the most frequent etiology. This article reviews the clinical diagnosis and management of these heterogeneous headaches., (Copyright © 2019 Elsevier Inc. All rights reserved.)

BACKGROUND: Pneumocephalus is intracranial air normally occurring as a consequence of trauma or cranial surgery, but rarely has been reported following the habitual use of the Valsalva maneuver. We were unable to locate any similar cases of pneumocephalus associated with high performance aircraft flight, and this case report highlights an important consequence of the inappropriate use of Valsalva on ascent to altitude. CASE REPORT: A healthy 29-yr-old F-22 instructor pilot presented with recurrent pneumocephalus following the use of the Valsalva maneuver. These episodes started after an initial use of a forceful Valsalva during an aggressive unrestricted climb to altitude. His symptoms included facial pressure and a bubbling sensation in his head followed by a severe headache for several days. After upright CT evaluation, three small sphenoid sinus defects were discovered and repaired. There was no recurrence of pneumocephalus following provocative testing after surgical repair of the sphenoid sinus defects, and the aviator returned to flying high performance aircraft. DISCUSSION: Spontaneous or Valsalva-associated pneumocephalus is an uncommon occurrence in healthy individuals. Awareness of the pathognomonic succussion splash associated with this diagnosis as well as a presentation of facial pressure and headache following flight will lead to appropriate diagnostic investigation and management. This case highlights the importance of recognizing rare diagnoses like pneumocephalus in the flying population, and future education of aerospace physiology technicians and aviators should emphasize appropriate Valsalva technique to promote flying safety and prevent similar presentations. Woodside SS, Metzler MM. Pneumocephalus associated with inappropriate Valsalva technique . Aerosp Med Hum Perform. 2019; 90(6):566-569.

Objective: To describe 3 new cases of new daily persistent headache (NDPH) starting as a single thunderclap headache., Background: NDPH is a form of chronic daily headache that is unique in its temporal profile of onset. Distinct subtypes of NDPH are being described with specific efficacious treatments. A single case of NDPH starting as a single thunderclap headache has been previously reported but no further cases have been documented in the literature. New cases need to be published to better define the syndrome., Methods: To report a case series of patients diagnosed with this unique NDPH subtype at an academic headache center from 2016 to 2018., Results: Three new cases of NDPH starting as a single thunderclap headache at onset were diagnosed. All patients were female with an average age of onset of 46 years. All presented with neurologic symptoms/stroke-like spells at headache onset. No defined triggering events were noted in any of the patients. There is a possible seasonal predilection to develop the syndrome around the fall clock change to the winter solstice. Neuroimaging was normal in the case patients. All patients rapidly responded to nimodipine. The duration of headache prior to nimodipine therapy ranged from 3 months to 4 years., Conclusion: This NDPH subtype appears to be rare. The key to diagnosis is asking about the temporal profile of the onset of the first ever NDPH headache. None of the case patients had been asked about the initial headache and its pattern of onset. Nimodipine seems to be very effective in treating this NDPH subtype. This syndrome is most likely precipitated by persistent or intermittent cerebral artery vasospasm. It is possibly a subform of the reversible cerebral vasoconstriction syndrome., (© 2019 American Headache Society.)

Objectives: To provide a unique perspective on geriatric headache and a number of novel treatment options that are not well known outside of the headache literature., Design: Review of the most current and relevant headache literature for practitioners specializing in geriatric care., Results: Evaluation and management of headache disorders in older adults requires an understanding of the underlying pathophysiology and how it relates to age-related physiological changes. To treat headache disorders in general, the appropriate diagnosis must first be established, and treatment of headaches in elderly adults poses unique challenges, including potential polypharmacy, medical comorbidities, and physiological changes associated with aging., Conclusion: The purpose of this review is to provide a guide to and perspective on the challenges inherent in treating headaches in older adults. J Am Geriatr Soc 66:2408-2416, 2018., (© 2018, Copyright the Authors Journal compilation © 2018, The American Geriatrics Society.)

Introduction: Thunderclap headache (TCH) is an excruciating headache that reaches maximal intensity within a minute. It has numerous potential etiologies, the most concerning of which is subarachnoid hemorrhage (SAH) due to high morbidity and mortality. Thus, patients with TCH must be evaluated urgently to identify the underlying cause and initiate prompt therapy. Areas covered: This paper reviews PubMed-listed research articles and presents an update of the clinical features, diagnostic evaluation, and possible causes of TCH. Expert commentary: In addition to SAH, TCH has been associated with reversible cerebral vasoconstriction syndrome (RCVS), cervical artery dissection, cerebral venous sinus thrombosis, cerebral infarction, intracerebral hemorrhage, spontaneous intracranial hypotension, intracranial infection, and pituitary apoplexy. Of note, with advances in knowledge in the past decade, RCVS has become an important cause of TCH, being diagnosed more frequently. Brain computed tomography (CT) should be performed in all patients with TCH, and lumbar puncture is indicated if the brain CT is nondiagnostic. Generally, a negative brain CT and lumbar puncture can eliminate SAH diagnosis, in which case brain magnetic resonance imaging and vascular imaging should be performed to evaluate other possible underlying causes.

Introduction We previously reported centripetal propagation of vasoconstriction at the time of thunderclap headache remission in patients with reversible cerebral vasoconstriction syndrome. Here we examine the clinical significance of centripetal propagation of vasoconstriction. Methods Participants comprised 48 patients who underwent magnetic resonance angiography within 72 h of reversible cerebral vasoconstriction syndrome onset and within 48 h of thunderclap headache remission. Results In 24 of the 48 patients (50%), centripetal propagation of vasoconstriction occurred on magnetic resonance angiography at the time of thunderclap headache remission. The interval from first to last thunderclap headache in patients with centripetal propagation of vasoconstriction (14 ± 10 days) was significantly longer than that of patients without centripetal propagation of vasoconstriction (4 ± 2 days). In the patients with centripetal propagation of vasoconstriction at the time of thunderclap headache remission, the incidence of another cerebral lesion (38%, 9 of 24 cases) was significantly higher than in patients without centripetal propagation of vasoconstriction (0%). From findings of sequential magnetic resonance angiography before and after thunderclap headache remission, we observed tendencies in which centripetal propagation of vasoconstriction gradually progressed after the onset of reversible cerebral vasoconstriction syndrome and peaked at the time of thunderclap headache remission. The progress of centripetal propagation of vasoconstriction concluded with thunderclap headache remission. Conclusions Centripetal propagation of vasoconstriction has clinical significance as an indicator of the severity of reversible cerebral vasoconstriction syndrome. The presence of centripetal propagation of vasoconstriction is associated with an increased risk of brain lesions and a longer interval from first to last thunderclap headache. Moreover, repeat magnetic resonance angiography to assess centripetal propagation of vasoconstriction during the time from onset to thunderclap headache remission can help diagnose reversible cerebral vasoconstriction syndrome.

Background: Limited histopathology studies have suggested that reversible cerebral vasoconstriction syndromes (RCVS) does not present with vascular wall inflammation. Previous vascular imaging studies have had inconsistent vascular wall enhancement findings in RCVS patients. The aim of this study was to determine whether absence of arterial wall pathology on imaging is a universal finding in patients with RCVS., Methods: We recruited patients with RCVS from Taipei Veterans General Hospital prospectively from 2010 to 2012, with follow-up until 2017 (n = 48). We analyzed the characteristics of vascular wall enhancement in these patients without comparisons to a control group. All participants received vascular wall imaging by contrasted T1 fluid-attenuated inversion recovery with a 3-T magnetic resonance machine. The vascular wall enhancement was rated as marked, mild or absent., Results: Of 48 patients with RCVS, 22 (45.8%) had vascular wall enhancement (5 marked and 17 mild). Demographics, clinical profiles, and cerebral artery flow velocities were similar across patients with versus without vascular wall enhancement, except that patients with vascular wall enhancement had fewer headache attacks than those without (p = 0.04). Follow-up imaging completed in 14 patients (median interval, 7 months) showed reduced enhancement in 9 patients, but persistent enhancement in 5., Conclusion: Almost half of our RCVS patients exhibited imaging enhancement of diseased vessels, and it was persistent for approximately a third of those patients with follow-up imaging. Both acute and persistent vascular wall enhancement may be unhelpful for differentiating RCVS from central nervous system vasculitis or subclinical atherosclerosis.

Introduction: Headaches are a common occurrence in childhood and adolescence. Most children presenting with a chief complaint of headache have a self-limited infectious disorder or primary headache syndrome that should not require extensive workup., Purpose of Review: Differentiating these conditions from other more serious causes of headache in children can sometimes be difficult. This article aims to provide information regarding "red flags" that should indicate a need for concern for disorders that require more urgent evaluation., Recent Findings: Long-held beliefs about specific "red flags" that have been analyzed in recent years as to their validity and new criteria for the diagnosis of idiopathic intracranial hypertension have been elaborated based on study. These publications are reviewed in this article. Knowledge of past and current literature on secondary headache in children, combined with thorough history taking and examination, should help determine when there is concern for a serious secondary cause for headache in children and adolescents and direct workup.

Reversible cerebral vasoconstriction syndrome is an intracranial vascular manifestation of a wide variety of diseases. It is the second most common cause of thunderclap headache, the most common cause of recurrent severe secondary headaches, and, in patients <60 years of age, has been reported as the commonest cause of isolated convexity subarachnoid haemorrhage. Radiologically, its key feature is vasoconstriction of the intracranial vessels, a dynamic process that is typically maximal at 2 weeks, varies in its distribution over the course of the disease, and typically resolves after 3 months. It can have haemorrhagic and ischaemic complications and sometimes occurs in concert with posterior reversible encephalopathy syndrome. It also has important associations with dissection and migraine. Rarer atypical cases can present with mild headache, no headache at all, or even a comatose state. This paper provides a detailed review of this syndrome, its pathophysiology, differential diagnosis, imaging findings, and work-up. It also describes the role that high-resolution magnetic resonance imaging (MRI) techniques can have in diagnosing the disease and emphasises the central role that all radiologists have in detecting this important and underdiagnosed condition., (Copyright © 2017 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Background Reversible cerebral vasoconstriction syndrome (RCVS) is an important differential diagnosis of singular or recurrent thunderclap headache. Prognosis is generally good, however complications of the transient segmental vasospasms of cerebral arteries such as stroke, subarachnoidal hemorrhage and brain edema may worsen the clinical outcome. Although the exact pathomechanism is still unclear, various vasoactive substances and conditions (e.g. post partum) have been identified as triggering RCVS. Cases We report on the clinical course and management of two cases of typical RCVS that were associated with two different precipitants previously not described: A gastrointestinal infection and isoflavones, which are phytoestrogens used for menopausal vasomotor symptoms. Discussion In the case of gastrointestinal infection, either systemic inflammatory processes might lead to disturbances of vascular tone, or the repetitive vomiting that resembles Valsalva manoeuvers known to trigger RCVS. In the case of isoflavone intake, it may be their estrogenic potential that induces dysregulation of cerebral arteries, a mechanism known from other states of hormonal change such as post-partum angiopathy. However, the association of both precipitating factors with RCVS in our two cases is not a proof for a causal relationship, and there may have been additional potential triggers for RCVS. Conclusion In patients with (gastrointestinal) infection and concomitant thunderclap headache, RCVS should be considered as an important differential diagnosis due to its major complications. Since RCVS may be triggered by various vasoactive substances, taking the medical history should always include over-the-counter drugs and dietary supplements (such as the isoflavones) beside the regular medication.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

When MRI scans of the brain are obtained for evaluation of primary headaches in children, incidental findings and anatomical variants are commonly present. After a review of the prevalence, 11 types are presented., (© 2017 American Headache Society.)

Background: Headache is a common patient complaint. The threshold for brain imaging in headache is debated, especially when the headache is transient, although even if the headache has resolved at presentation, a sentinel bleed heralding a subarachnoid haemorrhage (SAH) often remains a concern., Aim: To assess the yield of computed tomography (CT) head scan referrals for patients with transient headache symptoms., Methods: This study looked at 6 months of CT results in order to assess for brain pathology, with a particular focus on subarachnoid haemorrhage. Where any pathology was identified, detailed chart review looked for potential high-risk indicators., Results: Between January and July 2015, 531 undifferentiated headache patients were referred for head CT. Of these, 177 (33.3%) presented with a transient or episodic pattern. None of these cases had SAH on imaging nor re-presented with SAH within 3 months of initial CT. However, 7.3% (13/177) had other significant intracranial pathologies. The only significant risk factor for CT abnormality in this setting was focal neurology on presentation (odds ratio 3.1 (95% confidence interval (CI) 1.2-11.0); P = 0.044). All identified cases of SAH over the 6-month study period occurred in patients with persistent headache, and their clinical presentations showed a similar cluster of symptoms to previous literature (including thunderclap headache, vomiting and loss of consciousness)., Conclusion: This study suggests that patients with transient headache are at a low risk of SAH; however, further study is needed to quantify this, and other serious pathologies remains a concern, especially in the setting of focal neurology at presentation., (© 2017 Royal Australasian College of Physicians.)

Aims: To prospectively assess the incidence and etiology (ie, primary vs symptomatic) of headache in women during the first month postdelivery, with particular emphasis on the type of presentation as a clue for identifying potentially harmful etiologies. A secondary aim was to evaluate the relative frequency of migraine- vs tension-type headache in cases of primary headache., Methods: A total of 900 consecutive women were enrolled in the study and examined within 3 days of delivery, both clinically and with transcranial color-coded sonography (TCCS). During the course of follow-up, all subjects presenting with headache suspected of being secondary to intracranial pathology underwent a complete clinical and instrumental assessment with TCCS and magnetic resonance imaging (MRI) and angiography. A telephone interview was administered to all subjects 1 month after delivery. Two-tailed t test, Mann-Whitney test, Pearson chi-square test, and multiple logistic regression were used to analyze the data., Results: At the end of the follow-up period, 241 women (26.8% of the sample) reported at least one headache attack. In 88 of these 241 cases (9.8%), the headache attack occurred soon after delivery and was already recorded at the first visit. Thunderclap headache occurred in 34 (3.8%) of the subjects. In all but one of these subjects, the course was spontaneously benign. None of the recorded variables allowed discrimination of the subjects with thunderclap headache from those without headache. Three subjects had thunderclap headache following dural anesthesia, and one subject was found to have reversible cerebral vasoconstriction syndrome. Headache with gradual onset was recorded in 207 subjects (23%). Three of these subjects fulfilled the criteria for pre-eclampsia, and 13 had postural headache after dural anesthesia. Migraine history and urinary protein were independent predictors of gradual onset headache, and migraine history and parity were significant independent predictors of pulsating pain with gradual onset headache., Conclusion: Headache appeared early in the first days postdelivery, and its incidence increased in the first month thereafter. Predictors were different according to whether the headache had a gradual onset or a thunderclap presentation. Primary headache accounted for the overwhelming majority of the recorded cases.

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by reversible multifocal narrowing of cerebral arteries heralded by sudden (thunderclap) headaches with or without neurological deficits, resolving within 3 months. It often occurs in the peripartum period. To date, the ideal treatment remains unclear. Here, we report the case of a 31-year-old primigravida who presented with intracranial hemorrhage and went on to develop RCVS and acute respiratory distress syndrome over the course of her illness. Her condition was further complicated by uterine atony and septic shock.We describe for the first time the use of short-acting volatile sedation for prone positioning in acute respiratory distress syndrome during RCVS.

Background: Reversible cerebral vasoconstriction syndrome occurs predominantly in middle-aged women. Only nine pediatric patients with this syndrome have been reported., Patient Description: We present a ten-year-old boy with reversible cerebral vasoconstriction syndrome with radiographic findings similar to those of posterior reversible encephalopathy syndrome (PRES). He presented with a thunderclap headache without a neurological deficit. Brain magnetic resonance angiography (MRA) revealed multifocal narrowing of the cerebral arteries, whereas magnetic resonance imaging (MRI) with diffusion-weighted imaging and fluid-attenuated inversion recovery demonstrated hyperintense lesions in the occipital lobes and the left cerebellum. The patient's symptoms resolved spontaneously after a few hours with no recurrence. MRA on the second day showed a complete normalization of the affected arteries, and MRI after one month demonstrated improvement in the abnormal findings, leading to a diagnosis of RCVS with radiographic findings similar to those of PRES., Conclusions: This child's findings suggests that, RCVS, with or without PRES, may occur in children who present with a thunderclap headache., (Copyright © 2017 Elsevier Inc. All rights reserved.)

When MRI scans of the brain are obtained for evaluation of primary headaches in adults, incidental findings are commonly present. After a review of the prevalence of incidental findings and normal anatomical variants, 21 types are presented., (© 2017 American Headache Society.)

Background and Aim of the Study: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe "thunderclap" headache, with or without associated neurological symptoms and neuroimaging findings of reversible vasoconstriction of cerebral arteries. Puerperium is a recognized precipitant, but the incidence of puerperal RCVS is unknown. We conducted a prospective study to assess incidence, risk factors and clinical features of RCVS., Material and Method: Nine-hundred consecutive puerperae were prospectively enrolled within three days of delivery. Past medical history, basal demographic, anthropometric and biological variables were recorded. Transcranial Colour Coded Sonography (TCCS) was performed to assess early signs of vasospasm in brain vessels. A structured telephone interview was planned in all subjects one month postdelivery., Results: Thunderclap headache was recorded in 8 subjects (0.9%) on the first visit. At the one month follow-up interview 27 more patients reported having had at least one episode of thunderclap headache. In these 33 (3.8%) patients the course was spontaneously benign. One patient presented to the Emergency ward with throbbing thunderclap headache three weeks after delivery. Diagnostic work-up ended up in the diagnosis of RCVS, the outcome was favourable CONCLUSION: In normally coursing pregnancies and after uncomplicated delivery the risk of puerperal RCVS is negligible (0.1%). On the other way thunderclap headache may occur in a measurable proportion of (3.4%), although in the vast majority of cases (33/34=97%) it is of benign course. Transcranial Doppler sonography may be helpful to pick up those cases in whom further neuroradiological investigation is warranted., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is a clinico-radiologic syndrome characterized by thunderclap headache and reversible multifocal arterial constrictions that resolves within 3 months. RCVS can be either spontaneous or related to a trigger; vasoactive drugs including over-the-counter medicine are common culprits. Nevertheless, there are sparse data on the association of herbal supplements in the genesis of unexplained RCVS., Methods: We describe a case of RCVS with a temporal association with the consumption of a diet pill composed of green tea, L-carnitine, and conjugated linoleic acid. We reviewed the literature describing RCVS cases associated with consumption of herbal supplements or plants., Results: A 50-year-old black woman presented at the emergency room with a thunderclap headache less than 1 week after beginning a new herbal supplement with weight loss purpose. Angiographic study revealed multiple arterial constriction of virtually all intracranial territories that were reversed 28 days later. The patient was discharged with minimal symptoms. From our review, we identified 5 previous reports of herbal product-related triggers., Conclusions: Different factors can trigger RCVS. Besides our case, at least 5 other nutraceutical products were described to be associated with the disorders, 3 of them in patients without any other clear cause. Clinicians should be aware of the possible role of herbal supplements in RCVS, and their use should be systematically assessed in large RCVS cohorts to clarify this association., (Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.)