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 Following Exposure to Oleoresin Capsicum "Pepper Spray".

OBJECTIVES: To report a case associating the use of Oleoresin Capsicum Pepper Spray (OCPS) during law enforcement training with development of Reversible Cerebral Vasoconstriction Syndrome (RCVS). MATERIALS AND METHODS: RCVS is radiographically characterized by multifocal smooth narrowing of cerebral arteries heralded by clinical manifestations of recurrent thunderclap headaches. 70% of cases with RCVS have a clear precipitating factor and agents commonly implicated were cannabis, selective serotonin reuptake inhibitors, nasal decongestants, cocaine, postpartum state, eclampsia and strenuous physical/sexual activity.1 RESULTS: 24-year-old female police officer with no past medical history who presented with thunderclap headaches after exposure to pepper spray to her face during work training. Neurological examination was unremarkable. CT angiogram (CTA) of the head and neck and subsequent conventional angiogram revealed multifocal mild arterial narrowing of bilateral middle cerebral arteries (MCA), bilateral posterior cerebral arteries (PCA) and left anterior cerebral artery (ACA) concerning for RCVS. Eight weeks later, she had a repeat MRA head and neck demonstrating complete resolution of the previously noted narrowing of her cerebral arteries. CONCLUSIONS: OCPS is widely used in law enforcement training as well as by general population as a self- defense tool. It is generally assumed to be safe, although the consequences of its use can never be predicted with certainty.2 As our case highlights, use of OCPS may be associated with development of RCVS and awareness needs to be raised regarding this rare but serious complication.

Secondary headache attributed to exposure to or overuse of a substance.

BACKGROUND: Secondary headaches attributed to exposure to or the overuse of a substance are classified under chapter eight in the International Classification of Headache Disorders 3rd edition. Three distinct sub-chapters consider: 1. Headache attributed to exposure to a substance, 2. Medication overuse headache, and 3. Headache attributed to substance withdrawal. Headache attributed to exposure to a substance refers to a headache with onset immediately or within hours after the exposure, while medication overuse headache is a headache occurring on 15 or more days per month that has developed as a consequence of regular usage of acute headache medication(s) for more than three consecutive months in a patient with a pre-existing primary headache disorder. The withdrawal of caffeine, oestrogen, and opioids is most often associated with the development of headache. DISCUSSION: Despite the current headache classification, there is no certainty of a causal relationship between the use of any substance and the development of headache. Some substances are likely to provoke headache in patients that suffer from a primary headache disorder like migraine, tension-type headache or cluster headache, while others were described to cause headache even in people that generally do not get headaches. Toxic agents, such as carbon monoxide (CO) are difficult to investigate systematically, while other substances such as nitric oxide (NO) were specifically used to induce headache experimentally. If a patient with an underlying primary headache disorder develops a headache, in temporal relation to exposure to a substance, which is significantly worse than the usual headache it is considered secondary. This is even more the case if the headache phenotype is different from the usually experienced headache characteristics. Medication overuse headache is a well-described, distinct disease entity with only marginally understood pathophysiology and associated psychological factors. Managing medication overuse headache patients includes education, detoxification, prophylactic treatments and treating comorbidities, which is reflected in available guidelines. Viewing medication overuse headache as a separate entity helps clinicians and researchers better recognise, treat and study the disorder. CONCLUSION: Identification of substances that may cause or trigger secondary headache is important in order to educate patients and health care professionals about potential effects of these substances and prevent unnecessary suffering, as well as deterioration in quality of life. Treatment in case of medication overuse and other chronic headache should be decisive and effective.

Reversible cerebral vasoconstriction syndrome secondary to caffeine withdrawal.

We describe a 39-year-old man who developed thunderclap headaches during a hospital admission for accidental superficial burns. His magnetic resonance brain imaging was normal expect for diffuse segmental vasoconstriction. Prior to admission, he was consuming excessive amounts of caffeine which was restarted and slowly tapered and stopped over weeks. Repeat magnetic resonance angiogram showed resolution of segmental vasoconstriction. The implications of prescribed and non-prescribed drugs on cerebral vasculature have been discussed.

Isoflavones and gastrointestinal infection: Two potential triggers for reversible cerebral vasoconstriction syndrome.

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

Herbal Supplements Association with Reversible Cerebral Vasoconstriction Syndrome: A Case Report.

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

Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome.

OBJECTIVE: Factors predicting poor outcome in patients with the reversible cerebral vasoconstriction syndrome (RCVS) have not been identified. METHODS: In this single-center retrospective study, we analyzed the clinical, brain imaging, and angiography data in 162 patients with RCVS. Univariable and multivariable regression analysis were performed to identify predictors of persistent (nontransient) clinical worsening, radiologic worsening, early angiographic progression, and poor discharge outcome (modified Rankin Scale score 4-6). RESULTS: The mean age was 44 ± 13 years; 78% of patients were women. Persistent clinical worsening occurred in 14% at 6.6 ± 4.1 days after symptom onset, radiologic worsening in 27% (mainly new infarcts), and angiographic progression in 15%. Clinical worsening correlated with angiographic progression and new nonhemorrhagic lesions. Age and sex did not independently predict any type of worsening. Infarction on baseline imaging predicted poor outcome. Prior serotonergic antidepressant use predicted clinical and angiographic worsening but not poor outcome. Intra-arterial vasodilator therapy independently predicted clinical worsening and poor discharge outcome but was offered to more severe cases. Glucocorticoid treatment proved to be an independent predictor of clinical, imaging, and angiographic worsening and poor outcome. Of the 23 patients with clinical worsening, 17 received glucocorticoids (15 within the preceding 2 days). There were no significant differences in baseline brain lesions and angiographic abnormalities between glucocorticoid-treated and untreated patients. CONCLUSION: Patients with RCVS at risk for worsening can be identified on basis of baseline features. Iatrogenic factors such as glucocorticoid exposure may contribute to worsening.

Diffuse cerebrovascular dilation: Case report of amezinium metilsulfate-induced reversible cerebral vasoconstriction syndrome.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches with reversible cerebral vasoconstriction, and often precipitated by the postpartum state and vasoactive medications. We describe a case of a patient with RCVS induced by amezinium metilsulfate, a sympathomimetic drug, in whom magnetic resonance angiography (MRA) initially revealed diffusely dilated cerebral arteries. CASE DESCRIPTION: A 34-year-old woman was prescribed amezinium metilsulfate for hypotension. Twelve days later, she suffered from abrupt severe headaches and was referred to our department. She had no neurological deficits; however, MRA revealed diffusely dilated anterior, middle, and posterior cerebral arteries with vasoconstriction. She was tentatively diagnosed with RCVS and successfully treated with verapamil for headache. Nevertheless, follow-up MRAs disclosed widespread segmental vasoconstriction that resolved in two months. DISCUSSION: Diffuse cerebrovascular dilation has not been addressed but may be associated with RCVS pathophysiology. In addition, physicians should bear in mind that amezinium metilsulfate can potentially induce RCVS.

Reversible cerebral vasoconstriction syndrome possibly induced by etoricoxib.

Etoricoxib is a newer cyclooxygenase (COX)-2 inhibitor anti-inflammatory drug with a favorable safety profile. However, several randomized trials have provided evidence of an increased risk for acute myocardial infarction associated with the use of COX-2 inhibitors. Fewer data are available concerning the risk for ischemic stroke associated with COX-2 inhibitors. Although sporadic classes of drug-induced reversible cerebral vasoconstriction syndrome (RCVS) have been reported, this was not the case for etoricoxib. We report a patient who developed thunderclap headache, reversible cerebral arterial vasoconstriction, high blood pressure, and ischemic stroke (ie, RCVS) with recent exposure to etoricoxib. Although the association is hypothetical, the authors suggest consideration of RCVS in hypertensive patients presenting with headache, focal deficits, and evidence of cerebral ischemia during COX-2 inhibitors use.

Reversible cerebral vasoconstriction syndrome following indomethacin.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe thunderclap headaches and transient segmental cerebral arterial vasoconstriction. Precipitating factors, including the postpartum state and exposure to vasoactive substances are identified in approximately 50% of cases. Non-steroidal anti-inflammatory drugs have rarely been associated with RCVS. CASE DESCRIPTION: We report a case of a 51-year-old female with RCVS after administration of indomethacin given to relieve pain caused by renal colic. Cerebral imaging showed non-aneurysmal cortical subarachnoid hemorrhage, and formal angiography demonstrated widespread multifocal segmental narrowing of medium-sized cerebral arteries. These changes resolved on repeat angiography at 3 weeks. DISCUSSION: Indomethacin is a commonly used drug for treatment of certain primary headache disorders. To date, its mechanism of action remains unclear. A well described side effect of indomethacin is headache, which may be secondary to its vasoconstrictive effects. In our case, we postulate indomethacin, either alone or in combination with emotional stress from pain, triggered or exacerbated an underlying predisposition to RCVS.

Stabbing headache in an 8-year-old girl: primary or drug induced headache?

The occurrence of stabbing headaches in children requires a thorough diagnostic approach that excludes secondary headaches. The organic background should be taken into consideration when alarming symptoms occur, such as a purely 1-sided location, a change in the character of the headache, or possibly a link to physical activity. The current study describes the case of an 8-year-old girl who suffered short-lasting stabbing headache attacks. The headaches with increasing intensity and frequency started 1 month before her hospitalization and were usually preceded by physical activity (dancing, running). The pain, which was located in the right supraorbital region, lasted 1 second and occurred several times during the day. No associated symptoms were observed. In addition, the girl suffered from allergic rhinitis and was on antiallergic treatment (levocetirizine, fluticasone nasal spray). On admission she was in good general condition, and a pediatric and neurologic examination revealed no abnormalities. Her brain MRI was normal. The initial diagnosis was that the patient was suffering from primary stabbing headaches. However, during a follow-up visit 4 months later, a relationship was observed between the cessation of the headache attacks and the discontinuation of an antihistaminic drug. Six months later, the girl remained headache free. In cases involving differential diagnoses of stabbing headaches, it is important to consider the adverse reactions of the drugs used.

The reversible cerebral vasoconstriction syndrome in association with venlafaxine and methenamine.

The reversible cerebral vasoconstriction syndrome (RCVS) is characterised by thunderclap headache and multifocal vasoconstriction of cerebral arteries on angiography. It is often drug induced, but it can occur postpartum, and as a result of a number of other precipitants. To make the diagnosis, it is necessary to exclude other causes of severe headache (such as aneurysmal subarachnoid haemorrhage, carotid dissection and primary angiitis of the central nervous system). However, it is also important to show that the vasoconstriction has resolved with repeat angiography at the 3-month stage. Here we report two cases of RCVS in association with venlafaxine and the urinary antiseptic, methenamine. Serotonin-norepinephrine reuptake inhibitors have recently been reported as a possible precipitant, but this is the first report to implicate methenamine. Although RCVS is relatively uncommon, it should be considered in the differential of those presenting with thunderclap headache.

Reversible cerebral vasoconstriction syndrome induced by adrenaline.

INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by acute severe thunderclap headaches and evidence of multifocal, segmental, reversible vasoconstrictions of the cerebral arteries. Several precipitating factors have been identified and reported, including the use of recreational substances or sympathomimetic drugs and the postpartum state. CASE DESCRIPTION: Here we present the case of a woman who developed RCVS after the administration of adrenaline (epinephrine) in the setting of an anaphylactic reaction during antibiotic allergy testing. DISCUSSION: To our knowledge, this is the first reported case of RCVS following the administration of exogenous adrenaline. This case contributes to the understanding of the physiopathological mechanisms underlying reversible cerebral vasoconstriction.

[A case of reversible cerebral vasoconstriction syndrome (RCVS) triggered by human placenta extract].

We present a case of a female patient with reversible cerebral vasoconstriction syndrome (RCVS) arising after receiving subcutaneous injection of human placenta extract. A 44-year-old woman started taking human placenta extract with the aim of improving her menopausal symptoms, fatigue, and beauty. However, 18 days after taking human placenta extract, she had three episodes of thunderclap headache. Repeated cranial CT did not show subarachnoid hemorrhage; CSF examination showed neither xanthochromia nor inflammation. Brain diffusion weighted and FLAIR images were normal. However, magnetic resonance angiography showed multifocal segmental stenosis of the right middle cerebral artery and bilateral anterior cerebral arteries. Follow-up angiography, which performed 12 days after the oncet of thunderclap headache, revealed almost normalized flow in all cerebral arteries; we made a diagnosis of RCVS. She has had no symptoms and signs since the third attack of headache. The only identified etiologic factor was subcutaneous injection of human placenta extract started 18 days prior to onset. This is the first report of RCVS triggered by human placenta extract.

Reversible cerebral vasoconstriction syndrome associated with hormone therapy for intrauterine insemination.

INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) comprises a heterogeneous group of acute neurological diseases which are characterized by thunderclap headache and evidence of reversible multifocal constriction of cerebral arteries. A number of precipitating factors have been described in the literature, including recent childbirth and use of vasoactive substances. CASE DESCRIPTION: Here we present the case of a female patient with RCVS which occurred in the setting of hormonal ovarian stimulation for intrauterine insemination. DISCUSSION: This case possibly contributes to the understanding of the pathophysiological mechanisms underlying reversible cerebral vasoconstriction.

Nitroglycerin headache and nitroglycerin-induced primary headaches from 1846 and onwards: a historical overview and an update.

Nitroglycerin (NTG) (glyceryl trinitrate) was synthesized by the Italian chemist Ascanio Sobrero in Paris in 1846. A very unstable explosive, Alfred Nobel while working on explosives, combined it with Kiselguhr and patented it as dynamite in 1867. NTG was introduced in 1879 in medicine in the treatment of angina pectoris by the English doctor William Murrell. NTG-induced headache was quickly recognized as an important adverse event both in the industrial use of NTG, where it was used to produce dynamite, as well as in the use of NTG as drug. This review traces the evolution of our understanding of NTG headache.