Reversible cerebral vasoconstriction syndrome (RCVS)
Reversible cerebral vasoconstriction syndrome, sometimes called
Call-Fleming syndrome is defined as a condition of disease of the
arteries of which the brain develops a blood vessels spasm that leads to
multifocal arterial constriction and dilation, causing the sudden onset
of a severe headache (2)
A. Misdiagnosis
Reversible cerebral vasoconstriction syndrome (RCVS) constitutes an
under-recognised but clinically important diagnosis, because it can be
complicated by a cerebrovascular accident. The syndrome is often
misdiagnosed as it resembles. Misdiagnosis of Reversible Cerebral Vasoconstriction Syndromes as primary cerebral vasculitis and aneurysmal subarachnoid hemorrhage
is common because of overlapping clinical and
angiographic features, researchers at Cleveland Clinic Lerner College of
Medicine of Case Western Reserve University and Cleveland Clinic
Foundation, said, as reversible cerebral vasoconstriction syndromes (RCVS) comprise a group of diverse conditions, all characterized by reversible multifocal narrowing of the cerebral
arteries heralded by sudden (thunderclap), severe headaches with or
without associated neurologic deficits(11). Other study reported
demonstration of two female patients presented with severe headache with
subsequent angiographic findings of ‘bead and string’ appearances of
the cerebral arteries at Circle of Willis, which resolved spontaneously
within 3 months. Diagnosis of reversible cerebral vasoconstriction
syndrome was made. The clinical and imaging characteristics in different
modalities are discussed(10)
1. Primary Angiitis of the Central Nervous System
Primary angiitis of the central nervous system is defined as an
idiopathic disorder characterized
by vasculitis within the dural confines. Headache and encephalopathy
are the most frequent initial symptoms. Stroke or focal symptoms develop
in less than 20% of patients at the onset of disease and are uncommon
in the absence of headache or encephalopathy. Symptoms or signs of
vasculitis outside of the central nervous system are rare; serologic
markers of inflammation are typically normal(16). A Medline search was performed to identify all case reports since 1966
describing RCVS and PACNS that provide sufficient clinical detail to
permit diagnostic classification according to published criteria. RCVS
included case studies in which there was angiographic or transcranial
Doppler ultrasound evidence of near-to-complete resolution of cerebral
vasoconstriction in the absence of a well-recognized secondary cause.
PACNS included reports of histologically confirmed PACNS either through
biopsy or necropsy(17)
2. Post-partum cervicocephalic artery dissection (pp-CAD)
In a report of a 41-year-old right-handed African-American woman who developed the
syndrome of pp-CAD (headaches, trace subarachnoid hemorrhage and diffuse
cerebral arteriopathy on angiogram),
researchers at the Northwestern University, hypothesized whether
transient arterial wall abnormalities, postpartum
hormonal changes or subtle connective tissue aberrations play a similar
role in the pathogenesis of these two associated Post-partum
cervicocephalic artery dissection (pp-CAD) and Reversible cerebral segmental vasoconstriction (RCSV)(16)
3. Subarachnoid Haemorrhage
Subarachnoid hemorrhage is defined as a bleeding in the subarachnoid
space area between the brain and
the thin tissues that cover the brain, causing certain similar symptoms
to those of Reversible cerebral vasoconstriction syndrome. In the report of the case of a 51-year-old woman who presented to hospital following a
thunderclap headache, initially thought to be secondary to a subarachnoid haemorrhage (SAH). A tiny anterior choroidal artery aneurysm was demonstrated on cerebral
angiogram. At surgical clipping, no evidence of haemorrhage was
observed. Post-operatively, the patient developed delayed right-sided
hemiparesis, managed with aggressive hypertensive treatment, and later,
with onset of septicaemia, central visual loss. Computed tomography (CT)
brain scans
demonstrated oedematous changes within the parieto-occipital regions
bilaterally and later areas of infarction. The initial diagnosis of SAH
was revised to reversible cerebral vasoconstriction syndrome (RCVS), which gave rise to Posterior reversible encephalopathy syndrome (PRES)(18). Another report on a pediatric case of RCVS with cortical subarachnoid hemorrhage
(SAH). A 12-year-old boy developed acute, severe headache with
paralysis of lower extremities causing gait disturbance after
administration of eletriptan. Brain magnetic resonance angiography (MRA) revealed multifocal narrowing of the cerebral
arteries, whereas magnetic resonance imaging (MRI) demonstrated sulcal
hyperintensity on fluid-attenuated inversion recovery, consistent with
cortical SAH. The patient's clinical symptoms resolved spontaneously
after a few days and the MRI and MRA findings disappeared 3months later,
suggesting a diagnosis of RCVS. Eletriptan might cause vasoconstriction of cerebral
arteries. Although most patients with RCVS are adults and pediatric
cases are rare, RCVS should be considered in a child complaining of
severe headache.(19)
4. Orgasmic headache
Orgasmic headache (OH) is a condition of an "explosive" headache that
occurs at orgasm. In a report of 34-year-old woman who presented with
isolated and recurrent TCH at
orgasm, which fulfilled the diagnosis of OH. However, she was
post-partum and had recent exposure to ecstasy, making her symptoms
highly suggestive of RCVS. Brain magnetic resonance angiography showed segmental vasoconstriction(15)
5. Etc.
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Sources
(1) http://www.ncbi.nlm.nih.gov/pubmed/20816453
(2) http://www.ncbi.nlm.nih.gov/pubmed/19220301
(3) http://www.ncbi.nlm.nih.gov/pubmed/20936928
(4) http://www.ncbi.nlm.nih.gov/pubmed/21179608
(5) http://www.ncbi.nlm.nih.gov/pubmed/16832100
(6) http://www.ncbi.nlm.nih.gov/pubmed/18025032
(7) http://www.sciencedirect.com/science/article/pii/S038776041200006X
(8) http://www.medscape.org/viewarticle/748234_3
(9) http://www.ncbi.nlm.nih.gov/pubmed/22000400
(10) http://www.hkcr.org/publ/Journal/vol13no3/full/149-53%20Imaging.pdf
(11) http://www.ncbi.nlm.nih.gov/pubmed?term=Narrative%20Review%3A%20Reversible%20Cerebral%20Vasoconstriction%20Syndromes
(12) http://www.biomedsearch.com/nih/Cerebral-magnetic-resonance-angiography/1355864.html
(13) http://www.ncbi.nlm.nih.gov/pubmed/12105357
(14) http://www.ncbi.nlm.nih.gov/pubmed/12662190
(15) http://www.ncbi.nlm.nih.gov/pubmed/20655230
(16) http://archneur.ama-assn.org/cgi/reprint/66/6/704.pdf
(17) http://www.ncbi.nlm.nih.gov/pubmed/20534374
(18) http://www.ncbi.nlm.nih.gov/pubmed/22237929
(19) http://www.ncbi.nlm.nih.gov/pubmed/22285527
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