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[Carotid dissection associated with Ehlers-Danlos syndrome. Report of one case]
Anne-Marie Chassin-Trubert Contreras1
1Servicio de Medicina Interna, Hospital San José, Santiago, Chile. annechassin@gmail.com
Insights
Vascular Ehlers-Danlos syndrome, a collagen III defect, can cause arterial dissection and stroke. A 15-year-old patient with this condition experienced a stroke due to carotid artery dissection but responded well to antiplatelet therapy.
Area of Science:
- Vascular biology
- Connective tissue disorders
- Neurology
Background:
- Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
- Vascular EDS (vEDS) involves a defect in collagen type III synthesis, crucial for blood vessel integrity.
- This defect predisposes individuals to arterial and organ rupture.
Observation:
- A 15-year-old female with a history of EDS presented with symptoms of ischemic stroke.
- Imaging revealed dissection of the right internal carotid artery as the cause of the stroke.
- This case highlights a rare neurological complication in a young patient with EDS.
Findings:
- The patient's ischemic stroke was directly linked to spontaneous carotid artery dissection.
- Conservative management with antiplatelet agents was initiated.
- The patient demonstrated a positive clinical response to the antiplatelet therapy.
Implications:
- This case underscores the risk of cerebrovascular events, such as stroke, in patients with vascular EDS.
- It emphasizes the importance of considering arterial dissection in young EDS patients presenting with neurological deficits.
- Conservative management with antiplatelet agents may be a viable option for such cases, warranting further investigation.
Abstract:
In the vascular type of Ehlers-Danlos syndrome there is a defect in the synthesis of collagen type III, important constituent of the blood vessel wall, which may cause arterial rupture and dissection. We report a 15-year-old girl with a history of Ehlers-Danlos syndrome, who suffered an ischemic stroke secondary to dissection of the right internal carotid artery. She was managed conservatively with antiplatelet agents with a good clinical response.
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