ISCHEMIC STROKE / CLASSIFICATION AND ETIOPATHOGENESIS
Dolichoectasia
Created 16/12/2021, last revision 05/12/2022
- a progressive disease characterized by dilatation + tortuosity of the arteries; sometimes called dilated arteriopathy
- the vertebrobasilar arteries are most commonly affected (⇒ vertebrobasilar dolichoectasia or megadolichobasilar artery)
- ICA is also at high risk of being affected
- incidence 0.06-6% [Brutto, 2021]
- poses an increased risk of stroke recurrence (up to 19%/2 years) [Chen, 2019]
- may cause some “cryptogenic” strokes [Brutto, 2021]
- the characteristics listed below also apply to large fusiform aneurysms
Pathophysiology
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Etiology
- atherosclerosis (atherosclerotic dolichoectasia)
- affects both intra-/extracranial arteries
- usually age > 40 years
- typical vascular risk factors (e.g., hypertension) are present
- congenital disorders (non-atherosclerotic dolichoectasia)
- age < 40 years, more common in women, distal branches of the posterior circulation are affected
- Marfan syndrome, Ehler-Danlos, Fabry disease
- dolichoectasia secondary to dissection
Clinical presentation
- ischemic stroke/TIA (30% in 5 years)
- intracranial bleeding, subarachnoid hemorrhage
- brainstem compression (basilar dolichoectasia), hydrocephalus
- neurovascular compression syndrome (NVCS)
- compression of the optic nerve at the optic chiasma (ICA)
- impairment of one or more cranial nerves is associated with a tortuous and elongated BA (n. VII, VIII, VI)
- factors affecting the clinical outcome:
- the severity of arterial dilation and lateral shift
- signs and symptoms at the time of the diagnosis
- presence of mural thrombi
- ectasia progression on follow-up imaging
Management
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Prognosis
- from asymptomatic to malignant forms
- progression is more common in younger patients with ectasia in the anterior circulation
- a relatively high risk of stroke recurrence (up to 60% / 11 years)