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The 7th Annual Conference of the Chinese Stroke Society and the Tiantan International Cerebrovascular Disease Conference 2021 (CSA & TISC 2021) will be held on July 9-11, 2021 at the Beijing National Convention Center
.
At the Headache and Stroke Ⅱ Session, Professor Liu Yonghui from Guangxi University of Traditional Chinese Medicine gave a sharing report on RCVS and Pili-like headache.
The article is organized as follows
.
The author of this article: Yimaitong Tiantan will report the report group Yimaitong compiled reports, please do not reprint without authorization
.
Definition of reversible cerebral vasoconstriction syndrome (RCVS): Reversible cerebral vasoconstriction syndrome (RCVS) is a group of diseases characterized by reversible multifocal narrowing of cerebral arteries.
Its clinical manifestations usually include thunderclap headache, and Rare focal neurological dysfunction related to stroke, seizure or cerebral edema
.
In the past, RCVS patients have been misdiagnosed as primary central nervous system vasculitis or aneurysmal subarachnoid hemorrhage because they have common features, such as headache, stroke, and cerebral angiography showing narrowing of blood vessels
.
Other names are described comprising: migraine vasospasm, vasospasm associated with thunder headache, drug-induced cerebral arteritis, postpartum cerebrovascular disease, benign central nervous system disease, central nervous system vasculitis pseudo
.
Risk factors: RCVS is related to a variety of factors, including pregnancy, migraine, use of vasoconstrictive drugs and other drugs, neurosurgical procedures, hypercalcemia, unruptured saccular aneurysm, carotid artery dissection, and cerebral venous thrombosis Formation and other factors
.
The clinical manifestations of RCVS The clinical manifestations of RCVS are usually very prominent.
The sudden severe headache reaches the peak of pain within a few seconds, which meets the definition of "thunderclap headache"
.
Less than 10% of RCVS patients present with subacute or less severe headaches
.
Headache attacks are usually diffuse, or located in the occiput or top; patients often have nausea and sensitivity to light
.
Most patients have pain relief within a few minutes to a few hours, followed by another sudden severe headache attack, which will continue for several days
.
Many patients report triggers such as orgasm, physical activity, acute stress or emotional state, fatigue, coughing, sneezing, bathing, and swimming
.
Headache is the only symptom in 1/2-3/4 patients
.
Published case series studies show that the incidence of focal neurological dysfunction is 9%-63%, mainly manifested as ischemic or hemorrhagic stroke, seizures, reversible cerebral edema, tremor and reflexes Hyperactivity, ataxia, and aphasia
.
The thunderclap headache resolves within a few days to a few weeks
.
Most patients’ other focal neurological symptoms or signs subside within a few days to several weeks, but some patients have less or moderate permanent neurological deficits; the relief of abnormal cerebral angiography takes longer.
It can be up to 3 months
.
The pathogenesis of RCVS is currently unknown.
Cerebrovascular tone control disorder may be a key factor in the pathogenesis of RCVS.
This change in vascular tone may be spontaneous or caused by various exogenous or endogenous factors.
The unpredictable and transient failure of sympathetic nerve overexcitement on cerebrovascular tension control seems to play a role in the occurrence of RCVS
.
Patients with RCVS have reversible lesions suggesting transient brain edema, and patients with posterior reversible posterior leukoencephalopathy syndrome (PRES) have a higher incidence of reversible cerebral angiography abnormalities.
These conditions suggest the pathophysiology of RCVS and PRES Some overlap
.
Therefore, endothelial dysfunction has also been proposed to be related to the occurrence of RCVS
.
Diagnosis of RCVS The diagnosis of RCVS is based on typical clinical features, angiographic features and brain imaging features
.
A study published in 2016 provided diagnostic criteria for RCVS and distinguished it from primary vasculitis of the central nervous system (PACNS)
.
This standard has 98%-100% specificity and almost high positive predictive value.
Even if there is no cerebral angiography or subsequent imaging does not show reversible vasoconstriction, it can also be used for bedside diagnosis upon admission: repeated thunderclap headaches, Or single thunderclap headache, neuroimaging shows normal or junctional infarction or vasogenic edema, or no thunderclap headache but abnormal angiography and neuroimaging shows no brain disease (no brain disease is almost ruled out PACNS)
.
The imaging features of RCVS The main diagnostic feature of RCVS is abnormal cerebrovascular angiography.
The abnormal cerebrovascular angiography is dynamic and progressing to the proximal end, resulting in a "sausage string-like" appearance in the Willis artery ring and its branches
.
DSA, CTA, or MRA can be used to confirm segmental narrowing of cerebral arteries and vasodilatation, but normal results cannot rule out the diagnosis
.
Because the disease begins at the distal end, the initial cerebrovascular angiography results may be normal.
If RCVS is highly suspected clinically, follow-up examinations after 3-5 days may be reasonable
.
The most common parenchymal lesions are ischemic stroke and subarachnoid hemorrhage on the cortical surface, followed by reversible angiogenic cerebral edema and parenchymal hemorrhage
.
Infarcts are usually bilateral and symmetrical, located in the "watershed" area of cerebral hemispheric arteries or at the junction of cortex and subcortex.
Larger infarcts are usually wedge-shaped
.
Differential diagnosis of RCVS Repeated thunderclap headache has diagnostic significance for RCVS
.
Isolated thunderclap headache can predict various adverse conditions, including aneurysmal subarachnoid hemorrhage, cerebral parenchymal hemorrhage, cerebral artery dissection, and cerebral venous sinus thrombosis.
These conditions require corresponding imaging examinations to identify them
.
If there is thunderclap headache, subarachnoid hemorrhage, and narrowing of the cerebral arteries, aneurysmal subarachnoid hemorrhage will be a major consideration for the diagnosis
.
If the imaging test results are negative and the patient has not been confirmed to have vasoconstriction, it is considered as primary thunderclap headache
.
Migraine is another disease that needs to be differentiated from RCVS.
If it is misdiagnosed as migraine, drugs to treat migraine will be used.
This improper treatment can aggravate vasoconstriction and stroke
.
Although there may be some overlap between RCVS and migraine, the difference between RCVS and migraine is: RCVS rarely recurs (migraine will recur), RCVS sudden onset headache is very different from migraine, RCVS brain and vascular imaging The academic abnormalities are not consistent with migraine, and the abnormal angiography of RCVS can last for several weeks
.
General treatment and prognosis of RCVS: rest, avoid physical exertion and other factors that cause headaches, and stop vasoconstrictor drugs
.
Drug therapy: Nimodipine, a calcium ion antagonist, can be used as the first choice
.
Short-term application of glucocorticoids has no definite effect on improving symptoms and is assumed to aggravate clinical symptoms.
Therefore, the use of such drugs should be avoided
.
Symptomatic treatment: such as pain, sedation, anticonvulsant, blood pressure management
.
Prognosis: The headaches and angiographic abnormalities of most RCVS patients can be completely resolved within a few days or weeks, and the patients can be discharged after the headache improves
.
Less than 15%-20% of the cases will have residual functional impairment due to stroke.
Follow up to find out whether the patient is taking drugs and whether the headache has recurred
.
Lock the medlive-neurology channel to view the latest information of CSA & TISC 2021 at the same time! Long press the QR code to follow the CSA & TISC 2021 special report ☟☟☟ You can also click "Read the original text" to view More CSA & TISC 2021 topics related content!
.
At the Headache and Stroke Ⅱ Session, Professor Liu Yonghui from Guangxi University of Traditional Chinese Medicine gave a sharing report on RCVS and Pili-like headache.
The article is organized as follows
.
The author of this article: Yimaitong Tiantan will report the report group Yimaitong compiled reports, please do not reprint without authorization
.
Definition of reversible cerebral vasoconstriction syndrome (RCVS): Reversible cerebral vasoconstriction syndrome (RCVS) is a group of diseases characterized by reversible multifocal narrowing of cerebral arteries.
Its clinical manifestations usually include thunderclap headache, and Rare focal neurological dysfunction related to stroke, seizure or cerebral edema
.
In the past, RCVS patients have been misdiagnosed as primary central nervous system vasculitis or aneurysmal subarachnoid hemorrhage because they have common features, such as headache, stroke, and cerebral angiography showing narrowing of blood vessels
.
Other names are described comprising: migraine vasospasm, vasospasm associated with thunder headache, drug-induced cerebral arteritis, postpartum cerebrovascular disease, benign central nervous system disease, central nervous system vasculitis pseudo
.
Risk factors: RCVS is related to a variety of factors, including pregnancy, migraine, use of vasoconstrictive drugs and other drugs, neurosurgical procedures, hypercalcemia, unruptured saccular aneurysm, carotid artery dissection, and cerebral venous thrombosis Formation and other factors
.
The clinical manifestations of RCVS The clinical manifestations of RCVS are usually very prominent.
The sudden severe headache reaches the peak of pain within a few seconds, which meets the definition of "thunderclap headache"
.
Less than 10% of RCVS patients present with subacute or less severe headaches
.
Headache attacks are usually diffuse, or located in the occiput or top; patients often have nausea and sensitivity to light
.
Most patients have pain relief within a few minutes to a few hours, followed by another sudden severe headache attack, which will continue for several days
.
Many patients report triggers such as orgasm, physical activity, acute stress or emotional state, fatigue, coughing, sneezing, bathing, and swimming
.
Headache is the only symptom in 1/2-3/4 patients
.
Published case series studies show that the incidence of focal neurological dysfunction is 9%-63%, mainly manifested as ischemic or hemorrhagic stroke, seizures, reversible cerebral edema, tremor and reflexes Hyperactivity, ataxia, and aphasia
.
The thunderclap headache resolves within a few days to a few weeks
.
Most patients’ other focal neurological symptoms or signs subside within a few days to several weeks, but some patients have less or moderate permanent neurological deficits; the relief of abnormal cerebral angiography takes longer.
It can be up to 3 months
.
The pathogenesis of RCVS is currently unknown.
Cerebrovascular tone control disorder may be a key factor in the pathogenesis of RCVS.
This change in vascular tone may be spontaneous or caused by various exogenous or endogenous factors.
The unpredictable and transient failure of sympathetic nerve overexcitement on cerebrovascular tension control seems to play a role in the occurrence of RCVS
.
Patients with RCVS have reversible lesions suggesting transient brain edema, and patients with posterior reversible posterior leukoencephalopathy syndrome (PRES) have a higher incidence of reversible cerebral angiography abnormalities.
These conditions suggest the pathophysiology of RCVS and PRES Some overlap
.
Therefore, endothelial dysfunction has also been proposed to be related to the occurrence of RCVS
.
Diagnosis of RCVS The diagnosis of RCVS is based on typical clinical features, angiographic features and brain imaging features
.
A study published in 2016 provided diagnostic criteria for RCVS and distinguished it from primary vasculitis of the central nervous system (PACNS)
.
This standard has 98%-100% specificity and almost high positive predictive value.
Even if there is no cerebral angiography or subsequent imaging does not show reversible vasoconstriction, it can also be used for bedside diagnosis upon admission: repeated thunderclap headaches, Or single thunderclap headache, neuroimaging shows normal or junctional infarction or vasogenic edema, or no thunderclap headache but abnormal angiography and neuroimaging shows no brain disease (no brain disease is almost ruled out PACNS)
.
The imaging features of RCVS The main diagnostic feature of RCVS is abnormal cerebrovascular angiography.
The abnormal cerebrovascular angiography is dynamic and progressing to the proximal end, resulting in a "sausage string-like" appearance in the Willis artery ring and its branches
.
DSA, CTA, or MRA can be used to confirm segmental narrowing of cerebral arteries and vasodilatation, but normal results cannot rule out the diagnosis
.
Because the disease begins at the distal end, the initial cerebrovascular angiography results may be normal.
If RCVS is highly suspected clinically, follow-up examinations after 3-5 days may be reasonable
.
The most common parenchymal lesions are ischemic stroke and subarachnoid hemorrhage on the cortical surface, followed by reversible angiogenic cerebral edema and parenchymal hemorrhage
.
Infarcts are usually bilateral and symmetrical, located in the "watershed" area of cerebral hemispheric arteries or at the junction of cortex and subcortex.
Larger infarcts are usually wedge-shaped
.
Differential diagnosis of RCVS Repeated thunderclap headache has diagnostic significance for RCVS
.
Isolated thunderclap headache can predict various adverse conditions, including aneurysmal subarachnoid hemorrhage, cerebral parenchymal hemorrhage, cerebral artery dissection, and cerebral venous sinus thrombosis.
These conditions require corresponding imaging examinations to identify them
.
If there is thunderclap headache, subarachnoid hemorrhage, and narrowing of the cerebral arteries, aneurysmal subarachnoid hemorrhage will be a major consideration for the diagnosis
.
If the imaging test results are negative and the patient has not been confirmed to have vasoconstriction, it is considered as primary thunderclap headache
.
Migraine is another disease that needs to be differentiated from RCVS.
If it is misdiagnosed as migraine, drugs to treat migraine will be used.
This improper treatment can aggravate vasoconstriction and stroke
.
Although there may be some overlap between RCVS and migraine, the difference between RCVS and migraine is: RCVS rarely recurs (migraine will recur), RCVS sudden onset headache is very different from migraine, RCVS brain and vascular imaging The academic abnormalities are not consistent with migraine, and the abnormal angiography of RCVS can last for several weeks
.
General treatment and prognosis of RCVS: rest, avoid physical exertion and other factors that cause headaches, and stop vasoconstrictor drugs
.
Drug therapy: Nimodipine, a calcium ion antagonist, can be used as the first choice
.
Short-term application of glucocorticoids has no definite effect on improving symptoms and is assumed to aggravate clinical symptoms.
Therefore, the use of such drugs should be avoided
.
Symptomatic treatment: such as pain, sedation, anticonvulsant, blood pressure management
.
Prognosis: The headaches and angiographic abnormalities of most RCVS patients can be completely resolved within a few days or weeks, and the patients can be discharged after the headache improves
.
Less than 15%-20% of the cases will have residual functional impairment due to stroke.
Follow up to find out whether the patient is taking drugs and whether the headache has recurred
.
Lock the medlive-neurology channel to view the latest information of CSA & TISC 2021 at the same time! Long press the QR code to follow the CSA & TISC 2021 special report ☟☟☟ You can also click "Read the original text" to view More CSA & TISC 2021 topics related content!
