Reversible cerebral vasoconstriction syndrome

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Reversible cerebral vasoconstriction syndrome
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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] If it occurs after delivery it may be referred to as postpartum cerebral angiopathy.

Contents

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]

Signs and symptoms

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] Posterior reversible encephalopathy syndrome is present in a small minority of patients. [2] [3] [4]

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. [5]

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. [6]

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. [6] 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. [6]

Following a study and publication in 2007, it is also thought SSRIs, uncontrolled hypertension, endocrine abnormality, and neurosurgical trauma are indicated to potentially cause vasospasm. [7]

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]

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]

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. [6]

Epidemiology

The incidence of RCVS is unknown, but it is believed to be "not uncommon", and likely under-diagnosed. [1] [6] 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. [6]

History

Case studies of the condition first appeared in the 1960s, but it was not then recognized as a distinct entity. [6] In 1983, French researchers published a case series of 11 patients, terming the condition acute benign cerebral angiopathy. [1] Gregory Call and Marie Fleming were the first two authors of a report in which doctors from Massachusetts General Hospital, led by C. Miller Fisher, described 4 patients, alongside 12 previous case studies, with the characteristic symptoms and abnormal cerebral angiogram findings. [6] [8] The name Call-Fleming syndrome refers to these researchers. [1]

A 2007 review by Leonard Calabrese and colleagues proposed the name reversible cerebral vasoconstriction syndrome, which has since gained widespread acceptance. [1] [9] This name merges various conditions that were previously treated as distinct entities, including Call-Fleming syndrome, postpartum angiopathy, and drug-induced angiopathy. [6] Other names may still be used for particular forms of the condition. [1]

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<span class="mw-page-title-main">Intracranial aneurysm</span> Cerebrovascular disorder

An intracranial aneurysm, also known as a cerebral aneurysm, is a cerebrovascular disorder characterized by a localized dilation or ballooning of a blood vessel in the brain due to a weakness in the vessel wall. These aneurysms can occur in any part of the brain but are most commonly found in the arteries of the circle of Willis. The risk of rupture varies with the size and location of the aneurysm, with those in the posterior circulation being more prone to rupture.

<span class="mw-page-title-main">Cerebral edema</span> Excess accumulation of fluid (edema) in the intracellular or extracellular spaces of the brain

Cerebral edema is excess accumulation of fluid (edema) in the intracellular or extracellular spaces of the brain. This typically causes impaired nerve function, increased pressure within the skull, and can eventually lead to direct compression of brain tissue and blood vessels. Symptoms vary based on the location and extent of edema and generally include headaches, nausea, vomiting, seizures, drowsiness, visual disturbances, dizziness, and in severe cases, death.

<span class="mw-page-title-main">Subarachnoid hemorrhage</span> Bleeding into the brains subarachnoid space

Subarachnoid hemorrhage (SAH) is bleeding into the subarachnoid space—the area between the arachnoid membrane and the pia mater surrounding the brain. Symptoms may include a severe headache of rapid onset, vomiting, decreased level of consciousness, fever, weakness, numbness, and sometimes seizures. Neck stiffness or neck pain are also relatively common. In about a quarter of people a small bleed with resolving symptoms occurs within a month of a larger bleed.

<span class="mw-page-title-main">Visual snow syndrome</span> Visual impairment

Visual snow syndrome (VSS) is an uncommon neurological condition in which the primary symptom is that affected individuals see persistent flickering white, black, transparent, or colored dots across the whole visual field.

<span class="mw-page-title-main">Intracranial hemorrhage</span> Hemorrhage, or bleeding, within the skull

Intracranial hemorrhage (ICH), also known as intracranial bleed, is bleeding within the skull. Subtypes are intracerebral bleeds, subarachnoid bleeds, epidural bleeds, and subdural bleeds.

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Vasospasm refers to a condition in which an arterial spasm leads to vasoconstriction. This can lead to tissue ischemia and tissue death (necrosis). Cerebral vasospasm may arise in the context of subarachnoid hemorrhage. Symptomatic vasospasm or delayed cerebral ischemia is a major contributor to post-operative stroke and death especially after aneurysmal subarachnoid hemorrhage. Vasospasm typically appears 4 to 10 days after subarachnoid hemorrhage.

A thunderclap headache is a headache that is severe and has a sudden onset. It is defined as a severe headache that takes seconds to minutes to reach maximum intensity. Although approximately 75% are attributed to "primary" headaches—headache disorder, non-specific headache, idiopathic thunderclap headache, or uncertain headache disorder—the remainder are secondary to other causes, which can include some extremely dangerous acute conditions, as well as infections and other conditions. Usually, further investigations are performed to identify the underlying cause.

Sexual headache is a type of headache that occurs in the skull and neck during sexual activity, including masturbation or orgasm. These headaches are usually benign, but occasionally are caused by intracranial hemorrhage and cerebral infarction, especially if the pain is sudden and severe. They may be caused by general exertion, sexual excitement, or contraction of the neck and facial muscles. Most cases can be successfully treated with medication.

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Posterior reversible encephalopathy syndrome (PRES), also known as reversible posterior leukoencephalopathy syndrome (RPLS), is a rare condition in which parts of the brain are affected by swelling, usually as a result of an underlying cause. Someone with PRES may experience headaches, changes in vision, and seizures, with some developing other neurological symptoms such as confusion or weakness of one or more limbs. The name of the condition includes the word "posterior" because it predominantly though not exclusively affects the back of the brain. Common underlying causes are severely elevated blood pressure, kidney failure, severe infections, certain medications, some autoimmune diseases, and pre-eclampsia. The diagnosis is usually made by a brain scan (MRI) on which areas of swelling can be identified.

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<span class="mw-page-title-main">Bonnet–Dechaume–Blanc syndrome</span> Medical condition

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Behçet's disease is recognized as a disease that cause inflammatory perivasculitis, inflammation of the tissue around a blood or lymph vessel, in practically any tissue in the body. Usually, prevalent symptoms include canker sores or ulcers in the mouth and on the genitals, and inflammation in parts of the eye. In addition, patients experience severe headache and papulopustular skin lesions as well. The disease was first described in 1937 by a Turkish dermatologist, Dr. Hulusi Behçet. Behçet's disease is most prevalent in the Middle East and the Far East regions; however, it is rare in America regions.

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References

  1. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 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. PMID   25138149. S2CID   7457809.
  2. ↑ Chen, Shih-Pin; Fuh, Jong-Ling; Wang, Shuu-Jiun (2010). "Reversible cerebral vasoconstriction syndrome: an under-recognized clinical emergency". Ther Adv Neurol Disord. 3 (3): 161–171. doi:10.1177/1756285610361795. PMC   3002654 . PMID   21179608.
  3. ↑ Miller TR, Shivashankar R, Mossa-Basha M, and Gandhi D (2015). "Reversible Cerebral Vasoconstriction Syndrome, Part 1 Epidemiology, Pathogenesis, and Clinical Course" (PDF). American Journal of Neuroradiology. 36 (8): 1392–9. doi: 10.3174/ajnr.A4214 . PMC   7964694 . PMID   25593203. S2CID   12431927.
  4. ↑ Ducros A (2012). "Reversible cerebral vasoconstriction syndrome" (PDF). The Lancet Neurology. 11 (10): 906–17. doi:10.1016/s1474-4422(12)70135-7. PMID   22995694. S2CID   13616987.
  5. ↑ 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. PMC   3029638 . PMID   18077478. S2CID   36857208.
  6. 1 2 3 4 5 6 7 8 9 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. 36 (8): 1392–1399. doi: 10.3174/ajnr.A4214 . PMC   7964694 . PMID   25593203. S2CID   12431927.
  7. ↑ Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB (January 2007). "Narrative review: reversible cerebral vasoconstriction syndromes". Ann. Intern. Med. 146 (1): 34–44. doi:10.7326/0003-4819-146-1-200701020-00007. PMID   17200220. S2CID   8628852.
  8. ↑ 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.
  9. ↑ Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB (2007). "Narrative review: reversible cerebral vasoconstriction syndromes". Annals of Internal Medicine. 146 (1): 34–44. doi:10.7326/0003-4819-146-1-200701020-00007. PMID   17200220. S2CID   8628852.
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