ISCHEMIC STROKE IN AFIBRINOGENEMIA, MECHANISMS AND THERAPEUTIC DIFFICULTIES

Abstract

Objective: Afibrinogenemia is a rare bleeding disorder. Spontaneous bleeding is the principal manifestation. The aim of our work is to describe a case of ischemic stroke in a patient with congenital afibrinogenemia by highlighting etiopathogenic and therapeutic features. Design and method: We report the case of the patient I.A aged 30 years hospitalized in our service the month of June 2018. Results: Patient, I.A, Single 30-year-old, with a history of afibrinogenemia complicated by multiple episodes of hemorrhagic stroke and left upper limb paresis. He has a grade 3 HTA since 2010 complicated by haemorrhagic stroke whose exploration had found coarctation of the aorta, treated with angioplasty, currently stable under valsartan and amlodipine in fixed combination. A phenomenon of Raynaud to the upper limbs uncomplicated. In addition the patient had a bone cyst of the right tibia, complicated of fracture operated in 2017. In June 2018, the patient consults emergency for sudden onset cerebellar syndrome dominated by a disorder of gait and balance. One week before the event, the patient had headache and dizziness for which he had benefited from several courses of fibrinogen and free-frozen plasma. the examination confirms the static and kinetic cerebellar syndrome. Brain MRI shows a focus of cerebellar ischemic stroke. The echo-doppler of the supra-aortic vessels found an occlusion of the right vertebral artery the patient had been hospitalized for 3 days. He had been treated with aspirin 100 mg / day and early rehabilitation. The evolution was favorable until the 6th day. on the 7th day, the patient reconsults for the recovery of the symptomatology. aspirin was stopped urgently, with regression of signs. Currently, after 6 months of decline, the patient is stable. Conclusions: Congenital afibrinogenemia is associated with many vascular phenomena dominated by hemorrhagic complications. In our case ischemic complications especially ischemic stroke and Reynaud phenomenon are noted. The mechanisms are multiple, embolic complications, iatrogenic, thrombotic phenomenon related to The disease or even complications of hypertension. Treatment is difficult because of increased bleeding risk.