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1–17% of patients experience [[seizure]]s. 8–43% of patients show [[Focal neurologic signs|neurologic problems]], especially visual disturbances, but also [[hemiplegia]], [[ataxia]], [[dysarthria]], [[aphasia]], and [[numbness]].<ref name="mehdi2014" /> These neurologic issues typically disappear within minutes or a few hours; more persistent symptoms may indicate a [[stroke]].<ref name="mehdi2014" />
1–17% of patients experience [[seizure]]s. 8–43% of patients show [[Focal neurologic signs|neurologic problems]], especially visual disturbances, but also [[hemiplegia]], [[ataxia]], [[dysarthria]], [[aphasia]], and [[numbness]].<ref name="mehdi2014" /> These neurologic issues typically disappear within minutes or a few hours; more persistent symptoms may indicate a [[stroke]].<ref name="mehdi2014" />


This condition features the unique property that the patient's cerebral [[arteries]] can spontaneously constrict and relax back and forth over a period of time without intervention and without clinical findings. Vasospasm is common post [[subarachnoid hemorrhage]] and cerebral [[aneurysm]], but in RCVS only 25% of patients have symptoms post subarachnoid hemorrhage.<ref>{{cite journal |author=Moustafa RR, Allen CM, Baron JC |title=Call-Fleming syndrome associated with subarachnoid haemorrhage: three new cases |journal=J. Neurol. Neurosurg. Psychiatr. |volume=79 |issue=5 |pages=602–5 |date=May 2008 |pmid=18077478 |doi=10.1136/jnnp.2007.134635}}</ref>
This condition features the unique property that the patient's cerebral [[arteries]] can spontaneously constrict and relax back and forth over a period of time without intervention and without clinical findings. Vasospasm is common post [[subarachnoid hemorrhage]] and cerebral [[aneurysm]], but in RCVS only 25% of patients have symptoms post subarachnoid hemorrhage.<ref>{{cite journal |author=Moustafa RR, Allen CM, Baron JC |title=Call-Fleming syndrome associated with subarachnoid haemorrhage: three new cases |journal=Journal of Neurology, Neurosurgery & Psychiatry |volume=79 |issue=5 |pages=602–5 |year=2008 |pmid=18077478 |doi=10.1136/jnnp.2007.134635}}</ref>


==Causes==
==Causes==
The direct cause of the symptoms is believed to be either [[vasoconstriction|constriction]] or [[Vasodilation|dilation]] of blood vessels in the brain.<ref name="mehdi2014" /> Although the pathology is not known definitively, it is thought [[SSRI]]s, uncontrolled [[hypertension]], endocrine abnormality, and neurosurgical trauma are indicated to potentially cause [[vasospasm]].<ref>{{cite journal |author=Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB |title=Narrative review: reversible cerebral vasoconstriction syndromes |journal=Ann. Intern. Med. |volume=146 |issue=1 |pages=34–44 |date=January 2007 |pmid=17200220 |url=http://www.annals.org/content/146/1/34.full.pdf |doi=10.7326/0003-4819-146-1-200701020-00007}}</ref>
The direct cause of the symptoms is believed to be either [[vasoconstriction|constriction]] or [[Vasodilation|dilation]] of blood vessels in the brain.<ref name="mehdi2014" /> The [[pathogenesis]] is not known definitively, and the condition is likely to result from multiple different disease processes.<ref name="miller2015" />


Up to two-thirds of RCVS cases are associated with an underlying condition or exposure, particularly [[vasoactive]] or recreational drug use, [[complications of pregnancy]] ([[eclampsia]] and [[pre-eclampsia]]), and the adjustment period following childbirth called ''puerperium''.<ref name="mehdi2014" /> Implicated drugs include [[selective serotonin reuptake inhibitors]], weight-loss pills such as [[Hydroxycut]], [[alpha-sympathomimetic decongestant]]s, acute [[migraine]] medications, [[pseudoephedrine]], [[epinephrine]], [[cocaine]], and [[cannibis]], among many others.<ref name="mehdi2014" /> It sometimes follows [[blood transfusion]]s, certain surgical procedures, swimming, bathing, [[Effects of high altitude on humans|high altitude]] experiences, sexual activity, exercise, or coughing.<ref name="mehdi2014" />
Up to two-thirds of RCVS cases are associated with an underlying condition or exposure, particularly [[vasoactive]] or recreational drug use, [[complications of pregnancy]] ([[eclampsia]] and [[pre-eclampsia]]), and the adjustment period following childbirth called ''puerperium''.<ref name="mehdi2014" /> Vasoactive drug use is found in about 50% of cases.<ref name="miller2015" /> Implicated drugs include [[selective serotonin reuptake inhibitors]], weight-loss pills such as [[Hydroxycut]], alpha-[[Sympathomimetic drug|sympathomimetic]] [[decongestant]]s, acute [[migraine]] medications, [[pseudoephedrine]], [[epinephrine]], [[cocaine]], and [[cannabis (drug)|cannabis]], among many others.<ref name="mehdi2014" /> It sometimes follows [[blood transfusion]]s, certain surgical procedures, swimming, bathing, [[Effects of high altitude on humans|high altitude]] experiences, sexual activity, exercise, or coughing.<ref name="mehdi2014" /> Symptoms can take days or a few months to manifest after a trigger.<ref name="miller2015" />


==Diagnosis==
==Diagnosis==
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==Prognosis==
==Prognosis==
All symptoms normally resolve within three weeks, and may only last days.<ref name="mehdi2014" /> Residual deficits are seen in a minority of patients, ranging from under 10% to 20% in various studies.<ref name="mehdi2014" /> Less than 5% of patients experience progressive vasoconstriction, which can lead to stroke, progressive [[cerebral edema]], or even death.<ref name="mehdi2014" />
All symptoms normally resolve within three weeks, and may only last days.<ref name="mehdi2014" /> Permanent deficits are seen in a minority of patients, ranging from under 10% to 20% in various studies.<ref name="mehdi2014" /> Less than 5% of patients experience progressive vasoconstriction, which can lead to stroke, progressive [[cerebral edema]], or even death.<ref name="mehdi2014" /> Severe complications appear to be more common in [[postpartum]] mothers.<ref name="miller2015" />


==Epidemiology==
==Epidemiology==
The [[Incidence (epidemiology)|incidence]] is unknown.<ref name="mehdi2014" /> A 2014 [[review article]] speculates that it is "not uncommon", and likely under-diagnosed.<ref name="mehdi2014" /> One small study found that the condition was eventually diagnosed in 45% of [[outpatients]] with sudden headache, and 46% of outpatients with thunderclap headache.<ref name="mehdi2014" />
The [[Incidence (epidemiology)|incidence]] of RCVS is unknown, but it is believed to be "not uncommon", and likely under-diagnosed.<ref name="mehdi2014" /><ref name="miller2015" /> One small, possibly [[sample bias|biased]] study found that the condition was eventually diagnosed in 45% of [[outpatients]] with sudden headache, and 46% of outpatients with thunderclap headache.<ref name="mehdi2014" />


The average age of onset is 42, but RCVS has been observed in patients aged from 19 months to 70 years.<ref name="mehdi2014" /> Children are rarely affected.<ref name="mehdi2014" /> It is more common in females, with a female-to-male ratio between 4:1 and 2:1.<ref name="mehdi2014" />
The average age of onset is 42, but RCVS has been observed in patients aged from 19 months to 70 years.<ref name="mehdi2014" /> Children are rarely affected.<ref name="mehdi2014" /> It is more common in females, with a female-to-male ratio of 2.4:1.<ref name="miller2015">{{cite journal | author = Miller, T. R., Shivashankar, R., Mossa-Basha, M., & Gandhi, D. | year = 2015 | title = Reversible cerebral vasoconstriction syndrome, part 1: epidemiology, pathogenesis, and clinical course | journal = American Journal of Neuroradiology | doi = 10.3174/ajnr.A4214 | url = http://www.ajnr.org/content/early/2015/01/15/ajnr.A4214.full.pdf}}</ref>


==Treatment==
==Treatment==
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==History==
==History==
[[Case studies]] of the condition first appeared in the 1960s.<ref name="mehdi2014" /> In 1983, French researchers published a [[case series]] of 11 patients, terming the condition ''acute benign cerebral angiopathy''.<ref name="mehdi2014" /> Gregory Call and Marie Fleming authored a 1988 report in which doctors from [[Massachusetts General Hospital]] (including [[C. Miller Fisher]]) described 19 patients with the characteristic symptoms and abnormal cerebral angiogram findings.<ref name="mehdi2014" /><ref name="CallFleming">{{cite journal |author=Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM |title=Reversible cerebral segmental vasoconstriction |journal=Stroke |volume=19 |issue=9 |pages=1159–70 |date=September 1988 |pmid=3046073 |url=http://stroke.ahajournals.org/cgi/reprint/19/9/1159 |doi=10.1161/01.str.19.9.1159}}</ref> The name ''Call-Fleming syndrome'' refers to these researchers.<ref name="mehdi2014" />
[[Case studies]] of the condition first appeared in the 1960s, but it was not then recognized as a distinct entity.<ref name="miller2015" /> In 1983, French researchers published a [[case series]] of 11 patients, terming the condition ''acute benign cerebral angiopathy''.<ref name="mehdi2014" /> Gregory Call and Marie Fleming authored a 1988 report in which doctors from [[Massachusetts General Hospital]] (including [[C. Miller Fisher]]) described 4 patients, alongside 12 previous case studies, with the characteristic symptoms and abnormal cerebral angiogram findings.<ref name="miller2015" /><ref name="CallFleming">{{cite journal |author=Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM |title=Reversible cerebral segmental vasoconstriction |journal=Stroke |volume=19 |issue=9 |pages=1159–70 |year= 1988 |pmid=3046073 |url=http://stroke.ahajournals.org/cgi/reprint/19/9/1159 |doi=10.1161/01.str.19.9.1159}}</ref> The name ''Call-Fleming syndrome'' refers to these researchers.<ref name="mehdi2014" />


A 2007 review proposed the name ''reversible cerebral vasoconstriction syndrome'', which has gained widespread acceptance.<ref name="mehdi2014" /> Other names may still be used for more particular forms of the condition.<ref name="mehdi2014" />
A 2007 review proposed the name ''reversible cerebral vasoconstriction syndrome'', which has gained widespread acceptance.<ref name="mehdi2014" /><ref>{{cite journal |author=Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB |title=Narrative review: reversible cerebral vasoconstriction syndromes |journal=Annals of Internal Medicine |volume=146 |issue=1 |pages=34–44 |year=2007 |pmid=17200220 |url=http://www.annals.org/content/146/1/34.full.pdf |doi=10.7326/0003-4819-146-1-200701020-00007}}</ref> This name merges various conditions that were previously treated as distinct entities, including Call-Fleming syndrome, postpartum angiopathy, and drug-induced angiopathy.<ref name="miller2015" /> Other names may still be used for particular forms of the condition.<ref name="mehdi2014" />


==References==
==References==

Revision as of 23:21, 23 April 2015

Reversible cerebral vasoconstriction syndrome
SpecialtyNeurology Edit this on Wikidata

Reversible cerebral vasoconstriction syndrome (RCVS, sometimes called Call-Fleming syndrome) is a disease characterized by a weeks-long course of thunderclap headaches, sometimes focal neurologic signs, and occasionally seizures.[1] Symptoms are thought to arise from transient abnormalities in the blood vessels of the brain.[1] In some cases, it may be associated with childbirth, vasoactive or illicit drug use, or complications of pregnancy.[1]

For the vast majority of patients, all symptoms disappear on their own within three weeks.[1] Deficits persist in a small minority of patients, with severe complications or death being very rare.[1] Because symptoms resemble a variety of life-threatening conditions, differential diagnosis is necessary.[1]

Characteristics

The key symptom of RCVS is recurrent thunderclap headaches, which over 95% of patients experience.[1] In two-thirds of cases, it is the only symptom.[1] These headaches are typically bilateral, very severe and peak in intensity within a minute.[1] They may last from minutes to days, and may be accompanied by nausea, photophobia, phonophobia or vomiting.[1] Some patients experience only one headache, but on average there are four attacks over a period of one to four weeks.[1] A milder, residual headache persists between severe attacks for half of patients.[1]

1–17% of patients experience seizures. 8–43% of patients show neurologic problems, especially visual disturbances, but also hemiplegia, ataxia, dysarthria, aphasia, and numbness.[1] These neurologic issues typically disappear within minutes or a few hours; more persistent symptoms may indicate a stroke.[1]

This condition features the unique property that the patient's cerebral arteries can spontaneously constrict and relax back and forth over a period of time without intervention and without clinical findings. Vasospasm is common post subarachnoid hemorrhage and cerebral aneurysm, but in RCVS only 25% of patients have symptoms post subarachnoid hemorrhage.[2]

Causes

The direct cause of the symptoms is believed to be either constriction or dilation of blood vessels in the brain.[1] The pathogenesis is not known definitively, and the condition is likely to result from multiple different disease processes.[3]

Up to two-thirds of RCVS cases are associated with an underlying condition or exposure, particularly vasoactive or recreational drug use, complications of pregnancy (eclampsia and pre-eclampsia), and the adjustment period following childbirth called puerperium.[1] Vasoactive drug use is found in about 50% of cases.[3] Implicated drugs include selective serotonin reuptake inhibitors, weight-loss pills such as Hydroxycut, alpha-sympathomimetic decongestants, acute migraine medications, pseudoephedrine, epinephrine, cocaine, and cannabis, among many others.[1] It sometimes follows blood transfusions, certain surgical procedures, swimming, bathing, high altitude experiences, sexual activity, exercise, or coughing.[1] Symptoms can take days or a few months to manifest after a trigger.[3]

Diagnosis

The clinician should first rule out conditions with similar symptoms, such as subarachnoid hemorrhage, ischemic stroke, pituitary apoplexy, cerebral artery dissection, meningitis, and spontaneous cerebrospinal fluid leak.[1] This may involve a CT scan, lumbar puncture, MRI, and other tests.[1] Posterior reversible encephalopathy syndrome has a similar presentation, and is found in 10–38% of RCVS patients.[1]

RCVS is diagnosed by detecting diffuse reversible cerebral vasoconstriction.[1] Catheter angiography is ideal, but computed tomography angiography and magnetic resonance angiography can identify about 70% of cases.[1] Multiple angiographies may be necessary.[1] Because other diseases (such as atherosclerosis) have similar angiographic presentations, it can only be conclusively diagnosed if vasoconstriction resolves within 12 weeks.[1]

Prognosis

All symptoms normally resolve within three weeks, and may only last days.[1] Permanent deficits are seen in a minority of patients, ranging from under 10% to 20% in various studies.[1] Less than 5% of patients experience progressive vasoconstriction, which can lead to stroke, progressive cerebral edema, or even death.[1] Severe complications appear to be more common in postpartum mothers.[3]

Epidemiology

The incidence of RCVS is unknown, but it is believed to be "not uncommon", and likely under-diagnosed.[1][3] One small, possibly biased study found that the condition was eventually diagnosed in 45% of outpatients with sudden headache, and 46% of outpatients with thunderclap headache.[1]

The average age of onset is 42, but RCVS has been observed in patients aged from 19 months to 70 years.[1] Children are rarely affected.[1] It is more common in females, with a female-to-male ratio of 2.4:1.[3]

Treatment

As of 2014, no treatment strategy has yet been investigated in a randomized clinical trial.[1] Verapamil, nimodipine, and other calcium channel blockers may help reduce the intensity and frequency of the headaches.[1] A clinician may recommend rest and the avoidance of activities or vasoactive drugs which trigger symptoms (see § Causes).[1] Analgesics and anticonvulsants can help manage pain and seizures, respectively.[1]

History

Case studies of the condition first appeared in the 1960s, but it was not then recognized as a distinct entity.[3] In 1983, French researchers published a case series of 11 patients, terming the condition acute benign cerebral angiopathy.[1] Gregory Call and Marie Fleming authored a 1988 report in which doctors from Massachusetts General Hospital (including C. Miller Fisher) described 4 patients, alongside 12 previous case studies, with the characteristic symptoms and abnormal cerebral angiogram findings.[3][4] The name Call-Fleming syndrome refers to these researchers.[1]

A 2007 review proposed the name reversible cerebral vasoconstriction syndrome, which has gained widespread acceptance.[1][5] This name merges various conditions that were previously treated as distinct entities, including Call-Fleming syndrome, postpartum angiopathy, and drug-induced angiopathy.[3] Other names may still be used for particular forms of the condition.[1]

References

  1. ^ a b c d e f g h i j k l m n o p q r s t u v w x y z aa ab ac ad ae af ag ah ai aj ak al am an Mehdi, A., & Hajj-Ali, R. A. (2014). "Reversible cerebral vasoconstriction syndrome: a comprehensive update". Current Pain and Headache Reports. 18 (9): 1–10. doi:10.1007/s11916-014-0443-2.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. ^ Moustafa RR, Allen CM, Baron JC (2008). "Call-Fleming syndrome associated with subarachnoid haemorrhage: three new cases". Journal of Neurology, Neurosurgery & Psychiatry. 79 (5): 602–5. doi:10.1136/jnnp.2007.134635. PMID 18077478.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  3. ^ a b c d e f g h i Miller, T. R., Shivashankar, R., Mossa-Basha, M., & Gandhi, D. (2015). "Reversible cerebral vasoconstriction syndrome, part 1: epidemiology, pathogenesis, and clinical course" (PDF). American Journal of Neuroradiology. doi:10.3174/ajnr.A4214.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  4. ^ Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM (1988). "Reversible cerebral segmental vasoconstriction". Stroke. 19 (9): 1159–70. doi:10.1161/01.str.19.9.1159. PMID 3046073.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  5. ^ Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB (2007). "Narrative review: reversible cerebral vasoconstriction syndromes" (PDF). Annals of Internal Medicine. 146 (1): 34–44. doi:10.7326/0003-4819-146-1-200701020-00007. PMID 17200220.{{cite journal}}: CS1 maint: multiple names: authors list (link)
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