Cough, Exertional, and Sex Headaches.

In this article, the authors review the most common presentations of cough and exertional headaches and headaches associated with sexual activity. The authors elaborate on the most commonly described etiologies and identify those which are most critical to treat. The authors outline the recommendations for further evaluation and discuss effective treatment modalities for each headache type.

Reversible Cerebral Vasoconstriction Syndrome and Female Sex: A Narrative Review.

Reversible cerebral vasoconstriction syndrome (RCVS) refers to segmental, multifocal constriction of intracranial arteries along with acute headache and resolves within weeks. It occurs more commonly in women, and 1 well-known manifestation of RCVS is postpartum angiopathy. Furthermore, the female sex is included in scoring systems designed to assist with diagnosing RCVS. Nonetheless, the literature is mixed regarding the true role of female and pregnancy-related factors in the pathophysiology of RCVS, and it is similarly unclear whether management of this disorder differs by sex. Given the association of RCVS with female sex and the importance of highlighting, recognizing, and managing stroke etiologies in women, herein, the author reviews what is currently known and unknown about the topic of RCVS in women.

A case report on concomitant reversible cerebral vasoconstriction syndrome and transient global amnesia.

Reversible Cerebral Vasoconstriction Syndrome clinically presents as severe headaches with or without neurological deficits accompanied by multilocal caliber variation of the cerebral arteries on imaging studies. Transient Global Amnesia is a benign neurological condition that implies sudden temporary antero- and retrograde amnesia. The exact pathophysiological mechanisms involved in transient global amnesia and reversible cerebral vasoconstriction syndrome remain unclear but suggest similar pathways as both can be triggered by factors that activate the sympathetic nervous system. We herein discuss a potential relationship of the two conditions in a 65-year-old woman that initially presented herself to the emergency department with temporary memory impairment, indicating Transient Global Amnesia. Four days later, the patient revealed a thunderclap headache accompanied by a subarachnoid hemorrhage with transient segmental narrowing of the arteries of the anterior circulation on neuroimaging. In this case report we hypothesize that Reversible Cerebral Vasoconstriction Syndrome might be a potential cause for the clinical symptoms and imaging patterns with Transient Global Amnesia as a possible prodromal stage of Reversible Cerebral Vasoconstriction Syndrome.

Primary Cough Headache.

PURPOSE OF REVIEW: In this review article, we summarize the most recent findings and observations in patients experiencing primary cough headache. RECENT FINDINGS: Recent studies draw attention to further characterization of patients with primary cough headache in a pulmonology clinic. There have also been recent case studies describing new therapy considerations, including noninvasive vagus nerve stimulation and lumbar punctures. Primary cough headache has been a recognized entity since as early as 1932. Patients generally report a sudden onset, sharp headache lasting a few hours that is precipitated by Valsalva maneuver or coughing. It remains an uncommon diagnosis, and secondary causes are crucial to rule out with imaging studies. Additionally, there can be an overlap with other primary headache disorders such as primary exercise headache and headache associated with sexual activity, thus requiring a detailed history. Many hypotheses exist regarding the pathophysiology including relative pressure gradients and a crowded posterior fossa. Generally, patients are responsive to indomethacin, but more recent case reports have suggested benefits of other medications and procedures that may offer patients an alternative option, including vagus nerve stimulation, lumbar punctures, and beta blockers. Future case series and studies should focus on management of these patients, with a specific focus on individuals who do not tolerate indomethacin.

Bath-related headache: a case report.

BACKGROUND: Bath-related headache (BRH) is a rare primary headache disorder with only about 50 cases reported from 2000 to 2017 and none since. It is an abrupt onset excruciating headache occurring predominantly in middle-aged Asian women, most commonly following exposure to hot water. This is the first report in a Sri Lankan woman. CASE PRESENTATION: A 60-year-old Sri Lankan woman presented with an abrupt onset, severe throbbing holocephalic headache immediately following a hot-water shower. The headache was not associated with photo- or phonophobia, nausea, or vomiting, and she did not report a past history of migraine. However, she had experienced a similar headache 2 years previously precipitated by a hot-water shower. Her neurological examination, blood investigations, and magnetic resonance imaging of brain and intracranial vessels were normal. She was treated with opioid and nonsteroidal antiinflammatory drug analgesics, but the headache resolved only after treatment with nimodipine. The headache did not recur during a follow-up of 2 years since she avoided hot-water showers. CONCLUSIONS: Bath-related headache is a thunderclap primary headache disorder with a benign prognosis, but its recognition requires awareness to differentiate it from subarachnoid hemorrhage. It warrants inclusion in the International Classification of Headache Disorders.

Sudden-onset headache due to reversible cerebral vasoconstriction syndrome.

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by reversible segmental vasoconstriction of the cerebral arteries that spontaneously resolve within 3 months. Occurrence of RCVS peaks at around 40 years and the syndrome is common in women. Here, we report an adolescent boy case of RCVS.

The link between reversible cerebral vasoconstriction syndrome and transient global amnesia.

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.

Reversible cerebral vasoconstriction syndrome: review of neuroimaging findings.

Reversible cerebral vasoconstriction syndrome (RCVS) is a group of disorders characterized by segmental narrowing and dilatation of medium-to-large cerebral arteries, clinically presenting with recurrent episodes of sudden-onset thunderclap headaches, with or without focal neurological deficits. Cerebral vasoconstriction is typically reversible, with spontaneous resolution within 3 months. Although the syndrome has generally a benign course, patients with neurological deficits may experience worse outcome. The main imaging finding is segmental constriction of intracranial arteries, which can be associated with subarachnoid hemorrhage and/or ischemic foci. Other possible findings are intracranial hemorrhage, subdural bleeding and cerebral edema. The latter may have a pattern which can resemble that of posterior reversible encephalopathy syndrome, a condition that can overlap with RCVS. New imaging techniques, such as vessel wall imaging and arterial spin labeling, are proving useful in RCVS and are giving new insights into the pathophysiology of this condition. In this paper, we aim to review neuroimaging findings of RCVS.

Cryptococcal meningitis due to Cryptococcus gattii in an immunocompetent patient: uncommon presentation of a thunderclap headache.

Thunderclap headache is a medical emergency presented as the worst headache ever, is characterised by an abrupt onset and maximal intensity within seconds to minutes. However, cerebrovascular causes are among the most common causes of thunderclap headache, and other non-vascular life-threatening aetiologies should be considered in evaluating a patient. We describe a 23-year-old previously healthy Latino woman who presented to our hospital after a month of repetitive severe, abrupt-onset headaches. Her prior medical history was unremarkable. After a normal brain MRI with angio-MRI, a lumbar puncture was performed with normal opening pressure, hypoglycorrhachia, increased proteins and a leucocyte; India ink staining was positive for encapsulated yeast, cultures were positive for Cryptococcus gattii The patient received appropriate antifungal treatment with a good response. This case highlights the particular presentation of cryptococcal meningitis due to C. gattii among immunocompetent patients.

Thunderclap Headache in Children and Adolescents.

PURPOSE OF REVIEW: This work aimed to review the epidemiology, clinical criteria, and primary and secondary diagnoses of pediatric thunderclap headache and to compare to adult thunderclap headache. RECENT FINDINGS: Thunderclap headache among children aged 6-18 years are rare; this headache presented in 0.08% of the patients admitted to a pediatric emergency department in a tertiary pediatric center. In that recent single-center study, thunderclap was a headache of grade 10 on the pain scale and conferred a benign course. Contrary to adults, in children, most thunderclap headaches are due to either a primary thunderclap headache or another type of primary headache. A number of case reports have attributed pediatric thunderclap to reversible vasoconstriction syndrome and bleeding due to intracranial aneurysm. However, 3-year data from a pediatric emergency department of one center did not find these reasons to be causes of secondary thunderclap headache. This may be due to the rarity of these diagnoses in children compared to adults. Four of the 19 patients with thunderclap headache reported in that single study had secondary thunderclap; the causes were infection in three and malignant hypertension in one. All the patients had a benign course. Although urgent imaging and lumbar puncture are required in the workup of pediatric thunderclap, severe causes are very rare. More research is needed to investigate pediatric thunderclap headache.

Thunderclap headache syndrome presenting to the emergency department: an international multicentre observational cohort study.

BACKGROUND: Most headache presentations to emergency departments (ED) have benign causes; however, approximately 10% will have serious pathology. International guidelines recommend that patients describing the onset of headache as 'thunderclap' undergo neuroimaging and further investigation. The association of this feature with serious headache cause is unclear. The objective of this study was to determine if patients presenting with thunderclap headache are significantly more likely to have serious underlying pathology than patients with more gradual onset and to determine compliance with guidelines for investigation. METHODS: This was a planned secondary analysis of an international, multicentre, observational study of adult ED patients presenting with a main complaint of headache. Data regarding demographics, investigation strategies and final ED diagnoses were collected. Thunderclap headache was defined as severe headache of immediate or almost immediate onset and peak intensity. Proportion of patients with serious pathology in thunderclap and non-thunderclap groups were compared by χ² test. RESULTS: 644 of 4536 patients presented with thunderclap headache (14.2%). CT brain imaging and lumbar puncture were performed in 62.7% and 10.6% of cases, respectively. Among patients with thunderclap headache, serious pathology was identified in 10.9% (95%CI 8.7% to 13.5%) of cases-significantly higher than the proportion found in patients with a different headache onset (6.6% (95% CI 5.9% to 7.4%), p<0.001.). The incidence of subarachnoid haemorrhage (SAH) was 3.6% (95% CI 2.4% to 5.3%) in those with thunderclap headache vs 0.3% (95% CI 0.2% to 0.5%) in those without (p<0.001). All cases of SAH were diagnosed on CT imaging. Non-serious intracranial pathology was diagnosed in 87.7% of patients with thunderclap headache. CONCLUSIONS: Thunderclap headache presenting to the ED appears be associated with higher risk for serious intracranial pathology, including SAH, although most patients with this type of headache had a benign cause. Neuroimaging rates did not align with international guidelines, suggesting potential need for further work on standardisation.

Transcranial Doppler findings in a population with clinical probable reversible cerebral vasoconstriction syndrome.

OBJECTIVE: To describe transcranial Doppler (TCD) findings in a population with clinical probable RCVS. Exploratory objectives included the study of clinical characteristics of probable RCVS patients with and without spasm detected by TCD. METHODS: Cross-sectional cohort study of patients with thunderclap headache (TCH) without subarachnoid hemorrhage (SAH) of our neurology and headache center between 2010 and 2019, selecting patients with clinical diagnosis of probable RCVS (negative angiography study) by ICHD-3 criteria and with at least two TCD studies. RESULTS: From 114 TCH patients, 36/114 had probable RCVS by ICHD-3 criteria and had at least two TCD studies available. The mean age at RCVS onset was 42.9years (21-72years); 29/36 (80.6%) were female, 7/28 (25%) had cardiovascular risk factors and 20/36 (55.6%) had history of migraine. Most common triggers were stressful emotion, drugs, valsalva maneuvers and sexual activity. Five/36 (13.9%) had complications and 3/36 (8.3%) had late recurrence. Initial TCD was performed on average of 16 (6-26) days after headache onset. Twenty-nine had vasospasm on TCD, presenting mean flow velocity of MCA (VMCA) of 135.7±17.0cm/s and mean maximum VMCA of 138.3±17.2. Vasospasm was mild in 21/29 patients (72.4%) and moderate in 8/29 (27.6%). Complete VMCA normalization occurred on average 41 (30-70) days after headache onset and 24 (11-47) days after initial TCD. The group of patients with vasospasm detected by TCD had more female patients (26/29, 89.7% vs. 3/7, 42.8%, P=0.016), and more TCH attacks (mean of 3.6 vs. 2.14, P=0.049). CONCLUSION: TCD may be a useful tool in the identification of vasospasm in patients with probable RCVS, supporting the diagnosis of RCVS in patients presenting with recurrent TCH without SAH.

Reversible Cerebral Vasoconstriction Syndrome in the Setting of COVID-19 and Pleomorphic Sarcoma: A Case Report.

INTRODUCTION: Reversible cerebral vasonstriction syndrome (RCVS) is an increasingly recognized clinical and radiologic syndrome. However, it has been rarely reported in the setting of the novel coronavirus disease-2019 (COVID-19) infection or sarcomatous tumors. RCVS might be the initial manifestations of COVID-19 infection or noncatecholamine producing masses including sarcoma. CASE REPORT: A 44-year-old male who developed COVID-19-related symptoms followed by rapid onset of severe headaches in the setting of persistently elevated blood pressure (BP). Brain imaging showed multifocal arterial narrowing in the anterior and posterior circulation consistent with RCVS. Serial imaging demonstrated resolution of the arterial narrowing after BP control was achieved with improvement in the patient's headaches. Further investigation for secondary causes of the patient's elevated BP revealed a right renal mass, and the patient underwent right nephrectomy, and the biopsy results confirmed the diagnosis of pleomorphic sarcoma. CONCLUSION: Our case suggests a possible association between severe acute respiratory syndrome coronavirus 2 with development of RCVS, but further studies are needed to validate this observation, establish a causal relationship and define a pathophysiological mechanism. Considering tumors other than catecholamine-producing masses as a potential risk factor for developing RCVS might lead to earlier detection and treatment of any underlying malignancy in patients whom the main and sole presentation could be RCVS.

Type of headache at onset and risk for complications in reversible cerebral vasoconstriction syndrome.

BACKGROUND: In a recent Italian study, 30% of patients with reversible cerebral vasoconstriction syndrome (RCVS) presented without thunderclap headache (TCH), and tended to present more severe forms of RCVS than patients with TCH. We aimed to analyze the risk for complications of RCVS in patients with and without TCH at onset. METHODS: In a pooled cohort of 345 French patients with RCVS, we compared patients with and without TCH at onset regarding rates of neurological complications, and the functional outcome at 3 months. RESULTS: As compared to the 281 patients with TCH at onset, the 64 patients without TCH had a higher risk for any neurological complication (61% vs. 24%, OR 4.9, 95% CI 2.8-8.7, p < 0.001). The association was strongest for cervical artery dissections (28% vs. 5%, OR 8.1, 95% CI 3.7-17.6, p < 0.001), followed by posterior reversible encephalopathy syndrome (17% vs. 3%, OR 7.1, 95% CI 2.7-18.4, p < 0.001), seizures (9% vs. 2.5%, OR 4.1, 95% CI 1.3-12.5, p = 0.019), and subarachnoid hemorrhage (41% vs. 16%, OR 3.5, 95% CI 1.9-6.3, p < 0.001). In multivariable analysis, the risk for any neurological complication remained significantly elevated in the absence of TCH (OR 3.5, 95% CI 1.8-6.8, p < 0.001). The functional outcome was equal in both groups, with a modified Rankin scale score of 0-1 in ≥90% of patients. CONCLUSIONS: Absence of TCH at onset might predict a higher risk of complications in RCVS. Our results warrant further multicentric studies to prove this finding.

Reversible cerebral vasoconstriction syndrome: A narrative review for emergency clinicians.

INTRODUCTION: Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a rare cause of severe headache that can mimic other causes of sudden, severe headache and result in frequent emergency department (ED) visits. OBJECTIVE: This narrative review provides an evidence-based update concerning the presentation, evaluation, and management of RCVS for the emergency clinician. DISCUSSION: RCVS can present as recurrent, severe headaches that may be maximal in onset, known as a thunderclap headache. Distinguishing from other causes of thunderclap headache such as aneurysmal subarachnoid hemorrhage, cerebral venous thrombosis, and posterior reversible encephalopathy syndrome is challenging. Risk factors for RCVS include use of vasoactive substances, exertion, coughing, showering, sexual activity, and cervical artery dissection. Diagnosis relies on clinical features and imaging. Cerebral catheter digital subtraction angiography (DSA) is considered the gold standard imaging modality; however, computed tomography angiography or magnetic resonance angiography are reliable non-invasive diagnostic modalities. Treatment focuses on avoiding or removing the offending agent, administration of calcium channel blockers such as nimodipine, and reversing anticoagulation if bleeding is present. Although most cases have a benign course and resolve within 3 months, focal subarachnoid hemorrhage, intracerebral hemorrhage, permanent neurologic disability, or death can occur in a minority of cases. CONCLUSIONS: Diagnosis and appropriate management of RCVS can be aided by understanding key aspects of the history and examination. The emergency clinician can then obtain indicated imaging, confirming the diagnosis and allowing for appropriate management.