Reversible cerebral vasoconstriction syndrome: case report

A 28-year-old woman had thunderclap headache (TCH), after 7 days she had left hemiparesis. She had a history of oral contraceptive and citalopram medications. Brain magnetic resonance (MR) angiography demonstrated multiple stenotic segments. Digital subtraction angiography (DSA) showed multiple segments of narrowing in vessel calibre. Two probable diagnoses performed; primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome (RCVS). Because of clinical characteristics and normal cerebrospinal fluid findings she was set on medication for probable RCVS. Follow-up MR angiography after 4 weeks and DSA after 7 weeks demonstrated improvement in vessel calibre. Thus, diagnosis RCVS was established. Diagnosis and management of TCH contain many potential difficulties. Clinicians should consider the imaging of cerebral arteries, even if computed tomography scan and lumbar puncture are normal in TCH. Potential precipitating factors and triggers should also be known and avoided. This case was presented as a poster at the European Headache and Migraine Trust International Congress (EHMTIC), 4–7th September 2008, London, UK entitled “The Call–Fleming Syndrome: case report”.     

Reversible cerebral vasoconstriction syndrome (RCVS): an interesting case report

BackgroundReversible Cerebral Vasoconstriction Syndrome is a condition of transient cerebral vascular spasms, which usually presents with recurrent thunderclap headaches and recovers within 3 months. Several probable triggers and underlying factors, such as sex hormones, vasoactive drugs, head trauma or surgery, and tumors, have been implicated.Case presentationIn this paper, we present a 53-year-old woman with thunderclap headaches and normal lab tests who was radio-clinically diagnosed with reversible cerebral vasoconstriction syndrome and treated accordingly. Then, she experienced the recurrence of RCVS after about 2 years and headaches after 1 year in association with high blood pressure, high blood sugar, hypothyroidism, hyperlipidemia, and a urine metanephrine level of 5 times higher than the normal limit, suggesting a diagnosis of pheochromocytoma. After confirmation of the diagnosis with further imaging studies, surgical removal of the tumor resolved all the signs and symptoms.ConclusionOften underdiagnosed, pheochromocytoma could be an important condition associated with RCVS. It is important for clinicians to bear this diagnosis in mind while dealing with similar cases of recurrent thunderclap headaches.     

Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome

Upshaw-Schulman syndrome (USS) is an inherited type of thrombotic thrombocytopenic purpura (TTP) that is extremely rare, but often diagnosed during pregnancy. Reversible cerebral vasoconstriction syndrome (RCVS) is the transient stenosis of several cerebral arteries that is frequently diagnosed post-partum.We describe a 28-year-old woman with USS complicated by RCVS after delivery that was treated by plasma exchange with a good outcome. She was referred to our hospital with thunderclap headache, anemia and thrombocytopenia that occurred immediately postpartum. She was diagnosed with TTP and multiple cerebral infarctions. Plasma exchange promptly improved her symptoms on hospital day 3. Moreover, multiple stenoses of cerebral arteries indicating RCVS were resolved. Since her sister also had an episode of thrombocytopenia during pregnancy, inherited TTP was suspected and genetic analyses confirmed USS. Pregnancy is a risk for not only TTP, but also RCVS. Endothelial damage might be an underlining cause and vasospasm after delivery is a trigger of RCVS. Plasma exchange was effective against both TTP and RCVS.     

An often unrecognized cause of thunderclap headache: reversible cerebral vasoconstriction syndrome

Thunderclap headache (TCH) can have several causes of which subarachnoid hemorrhage (SAH) is most common and well known. A rare cause of TCH is the reversible cerebral vasoconstriction syndrome (RCVS) which is characterized by a reversible segmental vasoconstriction of the intracranial vessels. We describe two patients with TCH due to RCVS and the probable precipitating factor, namely, cannabis and an anti-migraine drug. In RCVS, cerebrospinal fluid examination is (near) normal, in contrast to SAH and (primary) cerebral vasculitis. Brain MRI may be normal or shows infarction. MRA can demonstrate vasoconstriction of the great arteries, but a normal MRA does not rule out the diagnosis. Caliber changes on cerebral angiography cannot adequately differentiate between RCVS and vasculitis. Calcium-channel antagonists may be a good therapy and repeated transcranial Doppler ultrasonography can be a reliable non-invasive investigation to monitor the effect of treatment and demonstrate reversibility of the vasoconstriction.     

Reversible segmental cerebral vasoconstriction (Call-Fleming syndrome): are calcium channel inhibitors a potential treatment option?

The Call-Fleming syndrome is characterized by sudden onset of thunderclap-like headache and focal neurological deficits. The pathophysiological correlate is a reversible segmental cerebral vasoconstriction frequently associated with focal cerebral ischaemia. The syndrome has been described in a variety of clinical conditions, and recently an association between the syndrome and exposure to vasoactive drugs was observed. Effective treatment options are not known. A 63-year-old female developed sudden 'worst ever' headache. Initial neurological examination, laboratory blood tests, CSF examination and brain magnetic resonance imaging (MRI) were normal. Previous medical history was unremarkable and she did not take vasoactive drugs. Eleven days after the onset of headache she developed visual field impairment and a right-sided hemiparesis. Brain MRI revealed bilateral posterior and left parietal ischaemic strokes. Cerebral catheter angiography showed segmental arterial vasoconstriction. A vasodilative therapy with calcium channel inhibitors was started and serial transcranial Doppler ultrasonography demonstrated resolution of cerebral arterial vasoconstriction. The present case illustrates that calcium channel inhibitors may be an effective therapy for segmental cerebral arterial vasoconstriction. However, more clinical data are needed to prove this observation.     

The Typical Thunderclap Headache of Reversible Cerebral Vasoconstriction Syndrome and its Various Triggers

During the last 10 years, reversible cerebral vasoconstriction syndrome (RCVS) has emerged as the most frequent cause of thunderclap headache (TCH) in patients without aneurysmal subarachnoid hemorrhage, and as the most frequent cause of recurrent TCHs. The typical TCHs of RCVS are multiple, recurring over a few days to weeks, excruciating, short‐lived, and brought up by exertion, sexual activities, emotion, Valsalva maneuvers, or bathing, among other triggers. All these triggers induce sympathetic activation. In a minority of cases with RCVS, TCH heralds stroke and rarely death. Early diagnosis of RCVS in patients who present with isolated headache enables proper management and might reduce the risk of eventual stroke. This review describes the characteristics, triggers, diagnosis, and management of TCH in RCVS. One aim is to underline that the TCH pattern of RCVS is so typical that it enables, according to the 2013 revision of the International Classification of Headache Disorders, the diagnosis of “probable RCVS” in patients with such a headache pattern, normal cerebral angiography, and no other cause. Another objective is to discuss the role of physical and emotional stress in RCVS and in other related conditions involving similar triggers.     

Reversible Cerebral Vasoconstriction Syndrome Triggered by Typhoon Hagibis in 2019: Report of 2 Cases

Although reversible cerebral vasoconstriction syndrome (RCVS) is a relatively rare condition, we encountered 2 consecutive patients with RCVS during Typhoon Hagibis in 2019. The first patient developed headache when the atmospheric pressure rapidly fell, and the second patient developed headache when the atmospheric pressure rapidly rose. Extreme atmospheric pressure fluctuations might induce neuronal activity in the trigeminal nucleus caudalis and sympathetic activation. Our experience with these 2 patients indicates the importance of magnetic resonance angiography for individuals with thunderclap headache during a typhoon.     

Licorice consumption as a cause of posterior reversible encephalopathy syndrome: a case report

Introduction  

A 49-year-old woman was admitted to our hospital because of thunderclap headache and blurred vision. At the time of presentation, her blood pressure was 219/100 mmHg, her arterial pH was 7.64 and her potassium level was 2.7 mM/l.     

Reversible Cerebral Vasoconstriction Syndrome Without Typical Thunderclap Headache

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe headache and diffuse segmental intracranial arterial constriction that resolve within three months. Stroke, which is the major complication of RCVS, can result in persistent neurological disability, and rarely causes death. Diagnosis of RCVS early in the clinical course might improve outcomes. Although recurrent thunderclap headache is the clinical hallmark of RCVS, the absence of such a pattern should not lead to discard the diagnosis. Our literature review shows that RCVS can also manifest as an unspecific headache, such as a single severe headache episode, a mild or a progressive headache. Moreover, a subset of patients with severe RCVS presents without any headache, but frequently with seizures, focal neurological deficits, confusion or coma, in the setting of stroke or posterior reversible encephalopathy syndrome. These patients may be aphasic or in comatose state, explaining their inability to give their own medical history. They may have forgotten the headache they had a few days before more dramatic symptoms, or may have a variant of the classical RCVS. By consequence, an RCVS should be suspected in patients with any unusual headache, whether thunderclap or not, and in patients with cryptogenic stroke or convexity subarachnoid hemorrhage, whether the patient also has headache or not. Diagnosis in such cases relies on the demonstration of reversible multifocal intracranial arterial stenosis and the exclusion of other causes.     

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.     

Catheter-based renal sympathetic denervation: chronic preclinical evidence for renal artery safety

Background  

Renal sympathetic hyperactivity is associated with hypertension, a leading cause of mortality worldwide. Renal sympathetic denervation via the Symplicity Catheter System has been shown to decrease blood pressure by 33/11 mmHg by 6 months, with no radiofrequency (RF)-related adverse sequelae visible by CT/MR angiography or renal duplex ultrasound 6 months after the procedure. Here, we present preclinical work predating those clinical results. We performed therapeutic renal sympathetic denervation in a swine animal model to characterize the vascular safety and healing response 6 months after renal denervation therapy.     

A non-traumatic,rapid-onset headache that is as dangerous as subarachnoid hemorrhage (SAH) seen in the emergency department

A 36yo male with multiple non-traumatic, rapid-onset headaches had Emergency Department visits on days 3 and 10 after onset of symptoms. He is a social smoker and drinker. CT head imaging was negative. An MRI/MRA was obtained. The image represents multiple foci of vasoconstriction and dilation in medium and large cerebral vessels consistent with Reversible Cerebral Vasoconstriction Syndrome (RCVS). Multiple rapid-onset headaches and "string of beads" on MRA imaging are pathognomonic for RCVS, which has a 4:1 female to male ratio. Manifestations include the pure cephalic form, characterized by a headache; subarachnoid hemorrhage and cerebral infarction have also been reported. Vasoactive drugs and the post-partum period are recognized as common inciting events. Symptoms usually resolve in 3-6 months. Treatment with nimodipine, 1-2mg/kg/hr IV and/or 30-60mg PO QID orally over 4-8 weeks, has been reported to be effective.     

Catheter ablation of atrial fibrillation using the Navx-/Ensite-system and a CT-/MRI-guided approach

Background  Catheter ablation has become the first line of therapy in patients with symptomatic, recurrent, drug refractory atrial fibrillation. However, catheter ablation of atrial fibrillation is still a challenge. This is partially due to the high degree of variability with regard to the individual anatomy. Nevertheless, 3D imaging systems (CT, MRI) provide detailed information about the individual left atrial and pulmonary vein morphology. A 3D CT or MRI reconstruction of the left atrium can be displayed in the Navx-/Ensite-system in a synchronised way during the ablation procedure, thereby facilitating the intervention. This study summarizes our preliminary experience with different strategies of AF ablation using the Navx-/Ensite-system and a CT-/MRI-guided approach. Methods  In a total of 41 patients, cardiac MRI (n = 7) or multi-detector spiral computed tomography (n = 34) was performed prior to an ablation procedure. Catheter ablation was performed for paroxysmal atrial fibrillation in 31 patients and for persistent atrial fibrillation in 10 patients. A 3D MRI or high resolution spiral CT data acquisition was performed and a surface rendered model of the LA was created. This model was displayed in the Navx-/Ensite-system throughout the ablation procedure. Results  Catheter ablation was performed using the Navx-system (n = 38) or the Ensite-system (n = 3). Three strategies were used depending on the type of atrial fibrillation: segmental isolation of the pulmonary veins (facilitated by a 3D real-time visualization of the ablation catheter and a circumferential mapping catheter; group A: 20 patients), linear lesions (group C: 3 patients) and a combined approach (group B; 18 patients). The CT-/MRI-models provided an excellent overview over the pulmonary veins and the left atrial appendage. They revealed a high degree of variability with regard to the individual anatomy (e.g. dimensions of the left atrial appendage, pulmonary vein ostia). The CT scans provided a more detailed reconstruction of the left atrial anatomy than the MRI scans (especially in patients who were in atrial fibrillation at the time of the data acquisition). In some patients, the CT-/MRI-models revealed a very small diameter of some pulmonary veins or side branches close to the ostium (e.g. right inferior pulmonary vein). Therefore, no attempt was made to achieve complete pulmonary vein isolation in some patients. In group A, 16/20 (80%) patients had no arrhythmia recurrence [mean follow-up 359 days (SD ± 317 days)]. Twelve out of eighteen (67%) patients in group B [mean follow-up 452 days (SD ± 311 days)] and 2/3 (67%) patients in group C did not experience an arrhythmia recurrence [mean follow-up 1,000 days (SD ± 34 days)]. There were no major complications. Conclusions  The information derived from 3D CT- or MRI-reconstructions facilitates AF ablations performed with the Navx-/Ensite-mapping system and enhances the safety of these procedures. Furthermore, the availability of an additional impedance-based 3D real-time visualization of the ablation catheter and the circular mapping catheter placed in the pulmonary veins represents a major advantage of the Navx system.     

Neuron-specific-enolase is increased in plasma after hemorrhagic shock and after bilateral femur fracture without traumatic brain injury in the rat

Neuron-specific enolase (NSE) is an acknowledged marker of traumatic brain injury. Several markers originally considered reliable in the setting of traumatic brain injury have been challenged after having been studied more extensively. The aim of our experimental study was to determine whether NSE is a reliable marker of traumatic brain injury early after trauma. Hemorrhagic shock was achieved by bleeding anesthetized rats to a mean arterial pressure (MAP) of 30-35 mmHg through a femoral catheter until incipient decompensation. MAP was maintained at 30-35 mmHg until 40% of shed blood had been administered as Ringer's solution and was then increased and maintained at 40-45 mmHg for 40 min by further administration of Ringer's solution, mimicking the phase of inadequate preclinical resuscitation. Blood samples were drawn at the end of the 40-min period of inadequate resuscitation. Femur fracture was achieved in anesthetized rats by bilateral application of forceps. Blood samples were drawn 30 and 60 min after fracture. Hemorrhagic shock caused NSE increase versus laboratory controls at the end of inadequate resuscitation (P < 0.01). Bilateral femur fracture caused NSE increase versus laboratory controls 30 min after fracture, which was significant 60 min after fracture (P < 0.01). During femur fracture, MAP remained at a level that is not associated with shock in rats. Our findings show for the first time that NSE increases after hemorrhagic shock as well as after femur fracture without hemorrhagic shock in rats. From a clinical point of view, these findings indicate that NSE cannot be considered a reliable marker of traumatic brain injury early after trauma in cases associated with hemorrhagic shock and/or femur fracture.     

A common cause of sudden and thunderclap headaches: reversible cerebral vasoconstriction syndrome

Background

Thunderclap headache (TCH) is a sudden headache (SH) with accepted criteria of severe intensity and onset to peak within one minute. It is a well-known presentation for subarachnoid hemorrhage (SAH) but most patients with TCH or SH run a benign course without identifiable causes. Reversible cerebral vasoconstriction syndrome (RCVS), a recently recognized syndrome characterized by recurrent TCH attacks, has been proposed to account for most of these patients.

Methods

We recruited consecutive patients presenting with SH at our headache clinic. Computed tomography and/or magnetic resonance imaging with angiography were performed to exclude structural causes and to identify vasoconstriction. Catheter angiography and lumbar puncture were performed with patients consent. Reversibility of vasoconstriction was confirmed by follow-up study.

Results

From July 2010 to June 2013, 31 patients with SH were recruited. Twenty-four (72.7%) of these SH patients exhibited headache fulfilling the TCH criteria. The diagnosis of RCVS was confirmed in 14 (45.2%) of patients with SH and 11 (45.8%) of patients with TCH. Other diagnoses were as follows: primary headaches (SH: 41.9%, TCH: 45.8%) and other secondary causes (SH: 12.9%, TCH: 8.3%). Compared with non-RCVS patients, patients with RCVS were older (50.8 ± 9.3 years vs. 40.8 ± 10.0 years, P = 0.006) and less likely to experience short headache duration of < 1 hour (23.1% vs. 78.6%, P = 0.007). Patients with RCVS were more likely to cite bathing (42.9% vs. 0%, P = 0.004) and less likely to cite exertion (0% vs. 29.4%, P = 0.048) as headache triggers.

Conclusions

Reversible cerebral vasoconstriction syndrome is a common cause of SH and TCH. Considering the potential mortality and morbidity of RCVS, systemic examination of cerebral vessels should be performed in these patients.     

Systematic review of reversible cerebral vasoconstriction syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular disorder associated with multifocal arterial constriction and dilation. RCVS is associated with nonaneurysmal subarachnoid hemorrhage, pregnancy and exposure to certain drugs. The primary clinical manifestation is recurrent sudden-onset and severe (‘thunderclap’) headaches over 1–3 weeks, often accompanied by nausea, vomiting, photophobia, confusion and blurred vision. The primary diagnostic dilemma is distinguishing RCVS from primary CNS arteritis. Diagnosis requires demonstration of the characteristic ‘string of beads’ on cerebral angiography with resolution within 1–3 months, although many patients will initially have normal vascular imaging. Many treatments have been reported to ameliorate the headaches of RCVS, but it is unclear whether they prevent hemorrhagic or ischemic complications.     

CT和MRI多序列评估脑外伤患者病情

目的分析CT和MRI多序列评估脑外伤患者病况的价值。方法连续收集2018年2月~2020年4月本院收治的脑外伤患者70例的临床资料,所有入院后的患者均进行CT和磁共振T1WI、T2WI、液体衰减翻转翻转恢复序列（FLAIR）、弥散加权成像（DWI）、增强梯度回波T2*加权血管成像（ESWAN）序列扫描检查,记录病灶的位置、数目、形态等,并与手术病理学诊断结果进行对比,分析两种诊断方式对病情的评估价值;于伤后3月进行随访,记录患者有无神经症状,并进行格拉斯哥预后评分,采用Spearman相关进行比较分析。结果60例脑外伤患者中手术诊断硬膜下血肿42例,蛛网膜下腔出血21例,硬膜外血肿17例,脑挫伤23例。MRI在硬膜下血肿、脑挫伤、蛛网膜下腔出血的诊断率高于CT检查（P < 0.05）,两种检查方式在硬膜外血肿检查准确率差异无统计学意义（P>0.05）;60例患者微出血病灶MRI多序列检查中ESWAN检查数目最多,其后依次是FLAIR序列、DWI序列、T2WI序列、T1WI序列,CT与T2WI序列检出数目差异无统计学意义（P>0.05）。微出血灶主要分布在额叶、颞枕顶叶、胼胝体、基底节、丘脑、脑干等区域,ESWAN序列检出出血病灶的总体积为288 557 mm3,以颞枕顶叶白质体积最大为63 153 mm3;60例脑外伤患者ESWAN序列发现出血性病灶数目、体积与患者入院时格拉斯哥昏迷评分之间经Spearman相关检验均存在明显负相关（r=-0.753, P < 0.01;r=-0.736,P < 0.01）,伤后3月的格拉斯哥预后评分与ESWAN序列发现出血性病灶数目、体积负相关（r=-0.648, P < 0.01;r=-0.612,P < 0.01）。结论与CT检查相比,MRI多序列联合检查在脑外伤患者硬膜下血肿、脑挫伤、蛛网膜下腔出血诊断准确率更高,且ESWAN序列在出血性病灶的数目、体积检出方面更有优势,对患者病情及远期预后有重要参考价值。      

Thunderclap headache durch Kleinhirninfarkt

Thunderclap headache is an acute and severe headache and is often the first sign of a life-threatening neurovascular disorder. The case of a 44-year-old man is described who presented with a thunderclap headache as the only clinical symptom. The clinical examination did not reveal any other focal deficits or signs of motor or sensory failures. Routine blood tests, cerebral CT as well as cerebrospinal fluid analysis showed no pathological results. A cerebral MRI to exclude a symptomatic thunderclap headache revealed a right cerebellar infarction. This case expands the differential diagnosis of thunderclap headache and reinforces the need for magnetic resonance imaging in the evaluation of such patients, even when neurological examination, cerebral CT, and cerebrospinal fluid analysis are normal.     

Dyke-Davidoff-Masson综合征CT及MRI特征

目的 观察Dyke-Davidoff-Masson综合征(DDMS)CT及MRI特征。 方法 回顾性分析8例DDMS患者临床及影像学资料,观察其CT及MRI表现。结果 8例 CT和MRI均见患侧大脑半球萎缩、颅窝缩小;7例患侧侧脑室扩大,中线结构向患侧偏移,伴不同程度颅骨代偿性改变,包括颅骨代偿性增厚、鼻窦扩大、乳突过度气化等;5例丘脑萎缩;4例大脑脚萎缩;4例脑软化;1例豆状核萎缩伴钙化。结论 DDMS的CT及MRI表现具有一定特征性,包括受累侧大脑半球萎缩伴同侧颅骨代偿性改变。     

Reduced circulating endothelial progenitor cells in reversible cerebral vasoconstriction syndrome

Background

The pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS) remains elusive. Endothelial dysfunction might play a role, but direct evidence is lacking. This study aimed to explore whether patients with RCVS have a reduced level of circulating circulating endothelial progenitor cells (EPCs) to repair the dysfunctional endothelial vasomotor control.

Methods

We prospectively recruited 24 patients with RCVS within one month of disease onset and 24 healthy age- and sex-matched controls. Flow cytometry was used to quantify the numbers of circulating EPCs, defined as KDR⁺CD133⁺, CD34⁺CD133⁺, and CD34⁺KDR⁺ double-positive mononuclear cells. The Lindegaard index, an index of vasoconstriction, was calculated by measuring the mean flow velocity of middle cerebral arteries and distal extracranial internal carotid arteries via color-coded sonography on the same day as blood drawing. A Lindegaard index of 2 was chosen as the cutoff value for significant vasoconstriction of middle cerebral arteries based on our previous study.

Results

Patients with RCVS had a reduced number of CD34⁺KDR⁺ cells (0.009 ± 0.006% vs. 0.014 ± 0.010%, p = 0.031) but not KDR⁺CD133⁺ cells or CD34⁺CD133⁺ EPCs, in comparison with controls. The number of CD34⁺KDR⁺ cells was inversely correlated with the Lindegaard index (rs = -0.418, p = 0.047). Of note, compared to controls, patients with a Lindegaard index > 2 (n = 13) had a reduced number of CD34⁺KDR⁺ cells (0.007 ± 0.005% vs. 0.014 ± 0.010%, p = 0.010), but those with a Lindegaard index ≤ 2 did not.

Conclusions

Patients with RCVS had reduced circulating CD34⁺KDR⁺ EPCs, which were correlated with the severity of vasoconstriction. Endothelial dysfunction might contribute to the pathogenesis of RCVS.