reversible cerebral vasoconstriction syndrome (Call-Fleming syndrome)
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Etiology
- 9% of patients postpartum, pregnancy
- 42% exposed to vasoconstrictor agent
- sympathomimetics (ampethamine, others)
- triptans, ergot alkaloids
- SSRI, SNRI, MAO inhibitors
- nicotine
- cocaine, methamphetamine, ecstasy
- cannabis
- head & neck trauma & neurosurgery
- acute cerebrovascular disorders
- cervical artery dissection
- ceebral endovascular procedures, cerebral angiography
- cerebral venous sinus thrombosis
- blood products (packed RBC, immune globulin)
- meningitis
- catecholamine-releasing tumors (pheochromocytoma)[3]
Epidemiology
- mean age, 42 years
- 81% women
- 2nd most common cause of thunderclap headache after subarachnoid hemorrhage [3[
Pathology
- transient segmental cerebral vasoconstriction
Clinical manifestations
- generally presents with severe recurrent thunderclap headache
- occurs over a few days or weeks
- focal neurological deficits (50%)
- case presentation of abrupt-onset severe headache lasting 6-8 hours[3]
- case presentation with throbbing, nausea, photophobia involving the entire cranium[3]
- seizures (17%)
Diagnostic procedures
- lumbar puncture (after head CT)
- CSF analysis
- no evidence of subarachnoid hemorrhage
- CSF analysis
Radiology
- non-contrast CT of the head (emergency)
- cerebrovascular imaging
- modalities
- magnetic resonance angiography (MRA)*
- CT angiography*
- cerebral angiography (invasive)
- findings
- narrowing of multiple cerebral arteries bilaterally
- abnormalities reversed over time
- infarction (39%)
- convexity subarachnoid hemorrhage (34%)
- hemorrhage (20%)
- white matter hyperintensities with dynamic temporal evolution that parallels disease severity[5]
- cerebral edema[3]
- narrowing of multiple cerebral arteries bilaterally
- modalities
* screening imaging modalities of choice
Differential diagnosis
Management
- normalization of blood pressure[3]
- eliminate offending agents[3]
- agents used of uncertain value
- glucocorticoids may worsen outcomes[3]
- calcium channel blockers
- prognosis:
- 78% of patients have no substantial residual disability
- 9% with severe disability
- mortality 2%
More general terms
References
- ↑ Singhal AB et al. Reversible cerebral vasoconstriction syndromes: Analysis of 139 cases. Arch Neurol 2011 Aug; 68:1005 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21482916
- ↑ Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol. 2009 Oct;9(5):256-67 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19762885
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2021.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ Yancy H, Lee-Iannotti JK, Schwedt TJ, Dodick DW. Reversible cerebral vasoconstriction syndrome. Headache. 2013 Mar;53(3):570-6 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23489219
- ↑ 5.0 5.1 George J. White Matter Lesions May Be Partially Reversible in RCVS. Dynamic pattern distinct from aging or other neurological disorders. MedPage Today. June 04, 2018 https://www.medpagetoday.com/neurology/generalneurology/73272?
Chen SP, Chou KH, Fuh JL et al Dynamic changes in white matter hyperintensities in reversible cerebral vasoconstriction syndrome. JAMA Neurol. Published online June 4, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29868878 https://jamanetwork.com/journals/jamaneurology/article-abstract/2682655 - ↑ Ducros A, Wolff V. The Typical Thunderclap Headache of Reversible Cerebral Vasoconstriction Syndrome and its Various Triggers. Headache. 2016 Apr;56(4):657-73. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27015869