[Reversible cerebral vasoconstriction syndrome : A rare cause of stroke]. / Reversibles zerebrales Vasokonstriktionssyndrom : Eine seltene Schlaganfallursache.

Reversible cerebral vasoconstriction syndrome (RCVS) is a complex and etiologically diverse neurovascular disorder that typically presents with severe thunderclap headaches (TCH) as the primary symptom, accompanied by reversible vasoconstriction of the cerebral arteries. The clinical course may include focal neurological deficits or epileptic seizures. There are two types: idiopathic RCVS and secondary RCVS, the latter triggered by various substances, medical interventions, or diseases. In clinical practice, various medical specialists may initially encounter this condition, underscoring the importance of accurate recognition and diagnosis of RCVS. The clinical course often appears monophasic and self-limiting, with recurrences reported in only 1.7% of cases annually. Complications such as cerebral hemorrhages and cerebral ischemia can lead to death in 5-10% of cases. This article utilizes a case study to explore RCVS, its complications, and the diagnostic procedures involved.

Update in the understanding of new daily persistent headache.

Background Accumulating evidence suggests various specific triggers may lead to new daily persistent headache (NDPH)-like presentations, suggesting that new daily persistent headache is a heterogenous syndrome, and challenging the concept that new daily persistent headache is a primary headache disorder.Method We searched the PubMed database up to August 2022 for keywords including persistent daily headache with both primary and secondary etiologies. We summarized the literature and provided a narrative review of the clinical presentation, diagnostic work-ups, possible pathophysiology, treatment response, and clinical outcomes.Results and conclusion New daily persistent headache is a controversial but clinically important topic. New daily persistent headache is likely not a single entity but a syndrome with different etiologies. The issue with past studies of new daily persistent headache is that patients with different etiologies/subtypes were pooled together. Different studies may investigate distinct subsets of patients, which renders the inter-study comparison, both positive and negative results, difficult. The identification (and removal) of a specific trigger might provide the opportunity for clinical improvement in certain patients, even when the disease has lasted for months or years. Nonetheless, if there is a specific trigger, it remains unknown or unidentified for a great proportion of the patients. We need to continue to study this unique headache population to better understand underlying pathogenesis and, most importantly, to establish effective treatment strategies that hopefully resolve the continuous cycle of pain.

Update on primary headache associated with sexual activity and primary thunderclap headache.

BACKGROUND: This narrative review aims to provide an update on primary headache associated with sexual activity and primary thunderclap headache. METHODS: We conducted a literature search on PubMed with the keywords "headache associated with sexual activity", "sexual headache", "orgasmic cephalalgia", and "coital cephalalgia" in addition to "thunderclap headache" to assess the appropriateness of all published articles in this review. RESULTS: Primary headache associated with sexual activity is a "primary" headache precipitated by sexual activity, which occurs as sexual excitement increases (progressive at onset), or manifests as an abrupt and intense headache upon orgasm (thunderclap at onset) or combines these above two features. Primary headache associated with sexual activity is diagnosed after a thorough investigation, including appropriate neuroimaging studies, to exclude life-threatening secondary causes such as subarachnoid hemorrhage. According to the criteria of the third edition of the International Classification of Headache Disorders, primary thunderclap headache is also a diagnosis by exclusion. The pathophysiology of primary headache associated with sexual activity and primary thunderclap headache remains incompletely understood. Treatment may not be necessary for all patients since some patients with primary headache associated with sexual activity and primary thunderclap headache have a self-limiting course. CONCLUSION: A comprehensive neuroimaging study is needed for distinguishing primary headache associated with sexual activity or primary thunderclap headache from secondary causes. Primary headache associated with sexual activity and primary thunderclap headache are self-limited diseases and the prognoses are good, but some patients with primary headache associated with sexual activity may have a prolonged course.

A case report of reversible cerebral vasoconstriction syndrome with thunderclap headache significantly exacerbated in the supine position and alleviated in the standing position.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden onset thunderclap headache and multiple segmental reversible cerebral vasoconstrictions that improve within 3 months. The postpartum period is a well-known precipitating factor for the onset of RCVS. Cerebral venous thrombosis (CVT) causes thunderclap headaches in the postpartum period. While headache in CVT is sometimes exacerbated in the supine position, the severity of the headache in RCVS is usually independent of body position. In this study, we report a case of RCVS with thunderclap headache exacerbated in the supine position, and headache attacks that resolved quickly in the standing position during the postpartum period. CASE PRESENTATION: A 33-year-old woman presented with a sudden increase in blood pressure and thunderclap headache on the fifth postpartum day (day 1: the first sick day). The headache was severe and pulsatile, with onset in the supine position in bed, and peaked at approximately 10 s. It was accompanied by nausea and chills but there were no scintillating scotomas or ophthalmic symptoms. The headache resolved in the standing or sitting position but was exacerbated and became unbearable within a few seconds when the patient was in the supine position. Therefore, she was unable to lie supine at night. Computed tomography angiography (CTA) of the head on day 2 and magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) on day 3 showed no abnormalities. However, considering the possibility of RCVS, verapamil was initiated on day 3. The headache resolved the following day. MRA of the head on day 10 revealed diffuse and segmental stenoses in the bilateral middle and posterior cerebral arteries and basilar artery. Therefore, the patient was diagnosed with RCVS. The headache gradually resolved and disappeared completely on day 42. Cerebral vasoconstriction was also improved on MRA on day 43. CONCLUSIONS: This postpartum RCVS case was notable for the exacerbation of headaches in the supine position. For the diagnosis of thunderclap headache in the postpartum period, RCVS should be considered in addition to CVT when the patient presents with a headache that is exacerbated in the supine position.

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.

Association between impaired dynamic cerebral autoregulation and BBB disruption in reversible cerebral vasoconstriction syndrome.

BACKGROUND: Half of the sufferers of reversible cerebral vasoconstriction syndrome (RCVS) exhibit imaging-proven blood-brain barrier disruption. The pathogenesis of blood-brain barrier disruption in RCVS remains unclear and mechanism-specific intervention is lacking. We speculated that cerebrovascular dysregulation might be associated with blood-brain barrier disruption in RCVS. Hence, we aimed to evaluate whether the dynamic cerebral autoregulation is altered in patients with RCVS and could be associated with blood-brain barrier disruption. METHODS: A cross-sectional study was conducted from 2019 to 2021 at headache clinics of a national tertiary medical center. Dynamic cerebral autoregulation was evaluated in all participants. The capacity of the dynamic cerebral autoregulation to damp the systemic hemodynamic changes, i.e., phase shift and gain between the cerebral blood flow and blood pressure waveforms in the very-low- and low-frequency bands were calculated by transfer function analysis. The mean flow correlation index was also calculated. Patients with RCVS received 3-dimensional isotropic contrast-enhanced T2 fluid-attenuated inversion recovery imaging to visualize blood-brain barrier disruption. RESULTS: Forty-five patients with RCVS (41.9 ± 9.8 years old, 29 females) and 45 matched healthy controls (41.4 ± 12.5 years old, 29 females) completed the study. Nineteen of the patients had blood-brain barrier disruption. Compared to healthy controls, patients with RCVS had poorer dynamic cerebral autoregulation, indicated by higher gain in very-low-frequency band (left: 1.6 ± 0.7, p = 0.001; right: 1.5 ± 0.7, p = 0.003; healthy controls: 1.1 ± 0.4) and higher mean flow correlation index (left: 0.39 ± 0.20, p = 0.040; right: 0.40 ± 0.18, p = 0.017; healthy controls: 0.31 ± 0.17). Moreover, patients with RCVS with blood-brain barrier disruption had worse dynamic cerebral autoregulation, as compared to those without blood-brain barrier disruption, by having less phase shift in very-low- and low-frequency bands, and higher mean flow correlation index. CONCLUSIONS: Dysfunctional dynamic cerebral autoregulation was observed in patients with RCVS, particularly in those with blood-brain barrier disruption. These findings suggest that impaired cerebral autoregulation plays a pivotal role in RCVS pathophysiology and may be relevant to complications associated with blood-brain barrier disruption by impaired capacity of maintaining stable cerebral blood flow under fluctuating blood pressure.

Pathophysiology of reversible cerebral vasoconstriction syndrome.

Reversible cerebral vasoconstriction syndrome (RCVS) is a complex neurovascular disorder being recognized during the past two decades. It is characterized by multiple abrupt severe headaches and widespread cerebral vasoconstrictions, with potential complications such as ischemic stroke, convexity subarachnoid hemorrhage, intracerebral hemorrhage and posterior reversible encephalopathy syndrome. The clinical features, imaging findings, and dynamic disease course have been delineated. However, the pathophysiology of RCVS remains elusive. Recent studies have had substantial progress in elucidating its pathogenesis. It is now believed that dysfunction of cerebral vascular tone and impairment of blood-brain barrier may play key roles in the pathophysiology of RCVS, which explains some of the clinical and radiological manifestations of RCVS. Some other potentially important elements include genetic predisposition, sympathetic overactivity, endothelial dysfunction, and oxidative stress, although the detailed molecular mechanisms are yet to be identified. In this review, we will summarize what have been revealed in the literature and elaborate how these factors could contribute to the pathophysiology of RCVS.

Reversible cerebral vasoconstriction syndrome developing after an erenumab injection for migraine prevention.

BACKGROUND: Reversible cerebral vasoconstriction syndrome is normally triggered by vasoactive compounds or illicit drugs. A new type of migraine preventive medication blocks calcitonin gene-related peptide utilizing monoclonal antibodies. Calcitonin gene-related peptide is a potent vasodilator for the cerebrovascular system. Could blocking calcitonin gene-related peptide be a trigger for cerebral artery vasospasm in patients susceptible to developing reversible cerebral vasoconstriction syndrome (migraine patients) or in individuals using vasoactive compounds? We present a case of reversible cerebral vasoconstriction syndrome occurring after calcitonin gene-related peptide monoclonal antibody treatment. CASE REPORT: A 43-year -old woman with a history of episodic migraine developed an acute headache with orgasm two days after taking her second injection of erenumab. Ten days after erenumab injection she developed a thunderclap headache while completing a high intensity workout. These new headaches were only left sided. Computed tomography angiography demonstrated mild to moderate areas of narrowing involving the left middle and anterior cerebral arteries, concerning for reversible cerebral vasoconstriction syndrome. She denied exposure to any known reversible cerebral vasoconstriction syndrome precipitant medication or illicit drugs. She did endorse recent exposure to high altitude prior to erenumab therapy. She was started on verapamil 40 mg three times per day and her headache ceased within 24 h of initiating treatment. A repeat CT angiogram completed 4 weeks after the initial study noted resolution of the areas of vessel stenosis. CONCLUSION: A case of reversible cerebral vasoconstriction syndrome developing after treatment with a calcitonin gene-related peptide monoclonal antibody is presented. The timing of the new type of headache occurring 2 days post erenumab injection suggests a possible cause and effect relationship. Reversible cerebral vasoconstriction syndrome as a possible treatment-related complication to the usage of calcitonin gene-related peptide monoclonal antibodies needs to be studied further.

Time to retire 'New daily persistent headache': Mode of onset of chronic migraine and tension-type headache.

New daily persistent headache is described as an enigmatic condition with daily headache from onset. It has posed challenges diagnostically and therapeutically.Methods: We conducted a study of patients referred to headache services based in Central and North-East London, United Kingdom, meeting the International Classificaiton of Headache Disorders - 3 criteria for New daily persistent headache. Information on demographics, phenotype and treatment responses were collected. The syndrome of the daily headache was also classified according any other ICHD-3-defined syndrome.Results: Of 162 patients, females comprised 68.5% with median age of onset 35 years. The daily headache experienced was chronic migraine in 89.7% and tension-type headache in 8.8%. Thunderclap-onset New daily persistent headache occurred in 14.8%. More than one headache syndrome was experienced in 15.4%, including cough, hypnic, sexual and stabbing headache. All aura types were experienced, most commonly brainstem aura in 39%. Prior headache was reported 53.7%. A persisting sub-form was present in 51.2%, relapsing remitting in 12.3% and 14.0% reported improvement; 19.8% were lost to follow-up. Only 11.1% reported an antecedent trigger. The most common premorbid disorders were psychiatric in 35.7%. A fifth improved on preventative medication, most commonly amitriptyline, propranolol and topiramate.Conclusion: Our cohort of New daily persistent headache is consistent with a mode of onset of migraine and tension-type headache which occurs in predisposed individuals.

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.

Behavioural responses of Konik Polski horses to natural, familiar sound of thunderstorm, and unfamiliar similar-sounding sounds of volcanic eruption and sea storms.

BACKGROUND: It is not clear, if modern Konik Polski horses have retained the ability to identify sounds in terms of danger. The aim of the study was to identify differences in their behaviour in response to the reproduction of volcanic eruption and sea storm sounds, assumed to be unfamiliar to these horses, as compared to their response to a thunderclap sound, considered by the horses as potentially dangerous. The study included 13 adult mares of the Konik Polski breed, kept under a free-range system. Their behavioural responses to the reproduction of the three natural sounds with an intensity of over 50 dB, were registered. They were analysed distance of each horse to the central point of the pasture and to the exit from the enclosure, and time and/or frequencies of elements of behaviour categorised as: increased anxiety (walking, trotting and cantering), vigilance (snoring, vocalisation, high head position, high tail position, sticking together), foraging (time of grazing), comfort (playing, examining the surroundings, sniffing), maintenance of hygiene (rubbing against objects, auto- or allogrooming, rolling) and resting. The obtained data were analysed by the Dwass, Steel and Critchlow-Fligner method using the SAS program. RESULTS: Most of analysed elements increased in response to reproduced sounds and decreased after sounds were stop playing (p < 0.05), however, they were no significant differences in general response to each studied sound. CONCLUSIONS: The responses of horses to similar sounds of both known and unknown origins, i.e. the sound of a thunderstorm, sea storm and volcanic eruption, are similar. The sound stimuli applied were not too stressful for the horses.

SARS-CoV-2 vaccine-related neurological complications.

OBJECTIVE: To describe three cases with neurological symptoms after SARS-CoV-2 vaccination. METHODS: A case series followed by a review of the literature, describing hypotheses on how neurological symptoms might develop after vaccination. RESULTS: The different temporal relationship between the onset or worsening of different neurological symptoms suggests different pathophysiological mechanisms. Progression of post-infectious myoclonus, caused by a previous SARS-CoV-2-infection, shortly after vaccination suggests a renewed auto-immune mediated crossreaction of antibodies to both viral epitopes and central nervous system components. Thunderclap headache after vaccination suggests a similar pathophysiological mechanism to the headache and other flu-like symptoms described after vaccination against other viruses. This might be ascribed to the activation of immunoinflammatory mediators or accompanying fever. Although headache accompanied by encephalopathy and focal neurological deficit might occur as part of a cytokine release syndrome, this is clinically less likely. CONCLUSIONS: A variety of symptoms, including thunderclap headache, focal deficits and movement disorders, can occur after SARS-CoV-2 vaccination, and an activation or reactivation of the immune system is suggested as most likely cause. However, one should be careful about claiming a direct correlation. It remains important to exclude other causes, such as structural lesions, infections or subarachnoid hemorrhage, and future research is required to understand possible pathophysiological mechanisms and associations with the SARS-CoV-2 vaccine.

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.

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.

Severe abrupt (thunderclap) non-traumatic headache at the pediatric emergency department - a retrospective study.

BACKGROUND: Adult abrupt severe non-traumatic headache (thunderclap) is often related to serious underlying etiologies such as subarachnoid hemorrhage. However, data are sparse regarding thunderclap headache in the pediatric population. OBJECTIVE: The aim of the study was to evaluate the prevalence, characteristics and causes of thunderclap headache in the pediatric and adolescent population, aged 6-18 years, presenting to a pediatric emergency department. METHODS: The electronic database of a tertiary care pediatric emergency department was searched for children presenting with acute headache during 2016-2018. Headache severity was defined by pain scales, either a visual analogue scale or by the Faces Pain Scale-Revised. RESULTS: Thunderclap headache was diagnosed in 19/2290 (0.8%) of the included patients, all of them with a pain score of 10/10. All the patients had a benign course. Primary headache was diagnosed in 15/19 (78.9%), six patients had migraine and eight were diagnosed with primary thunderclap headache. Four of the 19 patients were diagnosed with secondary headache: three with infectious causes and one with malignant hypertension. CONCLUSIONS: Thunderclap headache is rare among children and adolescents presenting to the emergency department. This headache is generally of a primary origin. Extensive evaluation is still needed to rule out severe diagnosis problems.

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.

Reversible cerebral vasoconstriction syndrome with cardiac involvement during treatment for iron deficiency anemia: caser report.

BACKGROUND: The diagnosis and therapy of reversible cerebral vasoconstriction syndrome (RCVS) tends to focus on neurological symptoms, but less attention has been paid the occurrence of extracerebral lesion such as the myocardium. CASE PRESENTATION: A 40-year-old woman taking iron supplements for iron deficiency anemia due to menorrhagia had suffered from a thunderclap headache and seizure. Brain magnetic resonance imaging revealed high-intensity lesions bilaterally in the cerebellar and cerebral hemispheres. Her symptoms once subsided with steroids and anticonvulsant therapy; however, she experienced a severe headache again while bathing and was transferred to our hospital. Based on the clinical course and imaging data, she was diagnosed as having RCVS triggered by a rapid improvement of anemia. At the same time, she had cardiac involvement revealed by electro and echocardiographs despite without chest symptoms. After the administration of a calcium channel blocker and nitrite, her cerebral and cardiac involvements were rapidly improved. CONCLUSIONS: The case presented RCVS with transient myocardial damage. With RCVS, we should always pay attention to the complication of extracerebral lesions.

Characterization of thunderclap headache in a reversible cerebral vasoconstriction syndrome case series.

OBJECTIVES: To empirically address how thunderclap headache (TCH) is described in a relevant real-world setting. BACKGROUND: TCH refers to a highly recognizable description of a severe headache that reaches maximum severity within 1 minute and endures for at least 5 minutes. The use of a numerical rating scale (NRS) to appraise TCH severity, as well as assessment of TCH progression in patients with pre-existing headache at the time of TCH onset has not been previously evaluated. METHODS: This was a retrospective case series of adults with a diagnosis of reversible cerebral vasoconstriction syndrome (RCVS), identified through a search of the electronic health record. Individuals meeting International Classification of Headache Disorders, 3rd Edition criteria for acute headache attributed to RCVS were included. Attacks described using a verbal descriptor scale (VDS), NRS, or both were recorded to evaluate acute headache characteristics. RESULTS: In all, 56 individuals with available descriptions of 120 acute headaches were included in the study analysis. Patients were female (35, 62.5%) with a median age of 46 (range: 19-67). The majority of patients reported a RCVS trigger (39, 69.6%). Acute headaches were characterized using a VDS (52, 43.3%), NRS (51, 42.5%), or both (17, 14.1%). Acute headaches were always described as severe when a VDS was utilized, and with a median NRS of 10 (range: 4-10). Four patients (7%) did not have a single headache characterized as either severe or with a NRS 8 or greater. In the 10 cases for which there was a pre-TCH baseline headache, it was either rated as mild or with a median NRS of 3 (range: 2-6). CONCLUSIONS: TCH in RCVS can be recognized using either VDS or NRS, with a broader range of peak intensities than previously recognized. TCH remains recognizable despite pre-existing baseline headache.

Clinical features of new daily persistent headache: A retrospective chart review of 328 cases.

OBJECTIVE: To describe the clinical features of new daily persistent headache (NDPH) at an outpatient neurology clinic with a subspecialty interest in headache in Houston, Texas. METHODS: A retrospective chart review was performed by a neurologist subspecialty certified in headache medicine of all patients seen from September 1, 2011 through February 28, 2020 (8.5 years) with a provisional diagnosis of NDPH and abstracted charts meeting criteria for primary NDPH. RESULTS: A total of 328 patients met the International Classification of Headache Disorders, 3rd edition criteria including 215 (65.5%) female patients. The mean age of onset was 40.3 years (range 12-87 years) with a mean age at first consultation in the study clinic of 42.6 years (range 12-87 years). The median [25th, 75th] duration of NDPH at initial consultation was 0.7 [0.3, 2.0] years, and at last visit, it was 1.9 [0.7, 4.8] years. The headaches were side-locked unilateral in 28/328 (8.5%). 12/328 cases (3.6%) with a thunderclap onset are reported. There was no obvious seasonal or other cyclical variation. The pain was usually moderate to severe and more often severe for the migraine phenotype (MP; 260/328 [79.3%] of cases) than for the tension-type phenotype (TTP). Precipitating factors were the following: stressful life events, 67/328 (20.4%); upper respiratory infection or flu-like illness, 33/328 (10.1%); and extracranial surgery, 5/328 (1.5%). Exacerbating or aggravating factors similar to migraine triggers were more often reported with the MP than with the TTP in this sample. For the MP, vertigo or dizziness was reported by 19/260 (7.3%) and a visual aura by 21/260 (8.1%) with descriptions provided. The prognostic types were the following for all patients: persisting (refractory), 305/328 (93.0%); relapsing-remitting, 9/328 (2.7%); and remitting (self-limited), 14/328 (4.3%). CONCLUSION: NDPH is typically of moderate to severe intensity often with migraine features without obvious seasonal or other cyclical variation. Most cases are refractory. 3.6% have a thunderclap onset often with a good response to treatment.

Pediatric Case of Life-Threatening Stroke Caused by Reversible Cerebral Vasoconstriction Syndrome with Spontaneous Cervical Internal Carotid Artery Vasospasm: A Case Report.

Pediatric reversible cerebral vasoconstriction syndrome (RCVS) and spontaneous cervical internal carotid artery (ICA) vasospasm are rare conditions; the former is commonly associated with a favorable prognosis. A healthy 13-year-old girl presented with thunderclap headache, followed by left hemiparesis, during a curling match. Six days after onset, left hemiparesis worsened to hemiplegia. Magnetic resonance imaging showed progressive cerebral infarction caused by severe right middle cerebral artery and cervical ICA stenosis. She became comatose because of impending uncal herniation. Emergent surgical decompression was performed. Then, 59 days after onset, her multiple stenoses improved, which was consistent with RCVS concomitant with spontaneous cervical ICA vasospasm. This is the first case of RCVS that concurrently developed spontaneous cervical ICA vasospasm. The patient developed life-threatening stroke due to the hemodynamic impairment of the affected intracranial and cervical arteries. Spontaneous extracranial supra-aortic artery vasospasm can be a poor prognostic predictor of RCVS.