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.

Reversible cerebral vasoconstriction syndrome after levonorgestrel-releasing intrauterine device placement-Case report.

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.

Reversible cerebral vasoconstriction syndrome: strategies to early diagnosis and the role of transcranial color-coded doppler ultrasonography (TCCD).

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.

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.

Vasoconstriction and long-term headache in reversible cerebral vasoconstriction syndrome.

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 (RCVS2) 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 RCVS2 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 RCVS2 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.

The Prevalence and Clinical Characteristics of Primary Stabbing Headache.

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 Baskent 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.

Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome as Syndromes of Cerebrovascular Dysregulation.

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.

Diagnosis and classification of headache associated with sexual activity using a composite algorithm: A cohort study.

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 syndrome2, 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 syndrome2 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.

Reversible cerebral vasoconstriction syndrome: a comprehensive systematic review.

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.

Evaluating thunderclap headache.

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.

Management of thunderclap headache in the emergency room: A retrospective cohort study.

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.

Vessel-wall MRI in thunderclap headache: A useful tool to answer the riddle?

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.

Reversible Cerebral Vasoconstriction Syndrome Following Carotid Endarterectomy: A Case Report.

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.

[Reversible cerebral vasoconstriction syndrome causing acute-onset headache].

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.

Determinants of Chiari I progression in pregnancy.

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.