ISCHEMIC STROKE / CLASSIFICATION AND ETIOPATHOGENESIS / NON-INFLAMMATORY VASCULOPATHIES
Reversible Cerebral Vasoconstriction Syndrome (RCVS)
Updated on 12/01/2024, published on 17/03/2022
- Reversible Cerebral Vasoconstriction Syndrome (RCVS) is characterized by severe headache (thunderclap headache – TCH) and the spontaneous development of reversible cerebral vasoconstriction
- it affects all age groups, but is more prevalent in women (F:M ratio is 2.4:1) between the ages of 20-50
- patients with RCVS usually have a history of migraine headaches
- patients with RCVS usually have a history of migraine headaches
- may last from several weeks to months, with varying degrees of severity; attacks may occur repeatedly
- if not promptly diagnosed and treated, RCVS can lead to serious complications, such as a stroke
- the exact cause of RCVS is unknown; it has been associated with certain conditions (secondary RCVS)
- Call-Fleming syndrome
- acute benign cerebral angiopathy
- CNS pseudovasculitis
- cerebral vasculopathy
- drug-induced cerebral arteritis
- isolated benign cerebral vasculitis
- migraine angiitis
- postpartum cerebral angiopathy
- thunderclap headache associated with vasospasms
- drugs
- SSRIs
- cannabinoids, ecstasy, cocaine, amphetamine
- nasal decongestants or triptans [Kato, 2016]
- immunosuppressive drugs (tacrolimus, cyclophosphamide, IVIG
- oral contraception
- ergotamine
- sumatriptan
- bromocriptine
- red blood cell transfusion, erythropoietin
- alcohol (especially binge drinking)
- uncontrolled hypertension
- endocrine disorders
- certain tumors
- hypercalcemia
- migraine
- pheochromocytoma
- bronchial carcinoid
- head trauma
- pregnancy and puerperium
- also known as postpartum cerebral angiopathy (postpartum angiitis) (Calado, 2006)
- it typically develops a few days to weeks after an otherwise uncomplicated delivery
Clinical presentation
- headaches
- a common symptom, fluctuating, often dramatic, referred to as a “thunderclap” headache (TCH)
- short duration (hours) and usually recurring over days or weeks
- rapid and abrupt onset may mimic SAH
- a common symptom, fluctuating, often dramatic, referred to as a “thunderclap” headache (TCH)
- other symptoms:
- photosensitivity/photophobia
- nausea, vomiting
- confusion
- changes in vision
- complications of RCVS:
- TIA/ischemic stroke (typically occurring after 3-4 days of persistent headache) with associated focal deficits
- epileptic seizures
- intracerebral hemorrhage (ICH)
- subarachnoid hemorrhage (SAH)
Diagnostic evaluation
Brain imaging (CT/MRI)
- usually, normal findings at the onset of symptoms; neuroimaging must exclude SAH
- a maximum of parenchymal changes (in T2/FLAIR) may be seen within 2-3 weeks as a complication of RCVS [Chen, 2018]
- vasogenic or cytotoxic edema (hyperintense lesions)
- nontraumatic, non-aneurysmal convexial SAH (sulcal hyperintensities on FLAIR)
- lobar hemorrhage
- border zone (watershed) infarcts
- vessel wall imaging (T1C+ black blood)
- RCVS shows no or only mild contrast enhancement of the affected arterial wall (DDx of vasculitis where a prominent enhancement is typically present)
- intracranial atherosclerotic plaques may also exhibit some degree of focal enhancement (Mandell, 2016)
- some parenchymal lesions may be reversible
Vascular imaging (CTA, MRA, DSA)
TCD/TCCD
- used for detection and bedside monitoring of vasospasms
- increased velocities in stenosed segments can be observed + increased PI and RI proximal to the stenosis
Laboratory test
- CSF is normal
- exclude hypercalcemia
- consider drugs test
- order blood samples to rule out vasculitis → more here
- brain and meningeal biopsy should be considered if an atypical course raises suspicion of PACNS
Differential diagnosis
- vasculitis (particularly PACNS)
- RCVS is probably responsible for some of the so-called benign primary CNS vasculitides (BACNS)
- thunderclap headache is absent in PACNS + neuroimaging is consistently abnormal, often involving both parenchymal and neurovascular elements
- SAH with vasospasms
- reversible posterior hypertensive encephalopathy (PRES)
- arterial dissection
- cerebral venous thrombosis
- intracranial infections
Therapy
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- in some cases with mild symptoms, the condition resolves without treatment
- calcium channel blockers (nimodipine, verapamil) induce arterial dilation – verapamil (ISOPTIN, LEKOPTIN, VERAPAMIL) 160-240 mg PO 1x daily, nimodipine (NIMOTOP 60 mg) 60mg PO 4-6x daily for 4-12 weeks
- may relieve spasms and lead to pain relief, and may also serve as a preventive measure
- may be administered orally (PO) or intravenously (IV)
- symptomatic therapy:
- analgetics for headache
- blood pressure correction
- maintenance of adequate fluid intake
- corticosteroids are not proven to be effective and are not recommended
- if a stroke is confirmed, standard acute stroke therapy and secondary prevention should be implemented