Large-vessel occlusion in a patient with Emery-Dreifuss muscular dystrophy

Abstract

During the night, about 3 hours after falling asleep, the patient experienced sudden left movement disorder (there were some dystonic movements on the left side observed and described by her husband) along with marked restlessness. The husband called the emergency service and the patient was admitted to the ICU of the stroke center. Stroke due to embolization to the middle cerebral artery (MCA) was dia gnosed. Brain CT revealed, that penumbra was only aff ected to a small extent; ASPECTS (Alberta Stroke Programm Early CT Scale) was 3 points. CTA of the cerebral arteries showed the occlusion of the terminal section of the internal carotid artery (ICA) on the right side (segment C7) with a transition to the M1/MCA segment (10 mm) (Fig. 1). The patient was consulted at a comprehensive stroke center that did not recommend intravenous thrombolysis or mechanical thrombectomy due to the presence of signifi cant and extensive ischemic changes in the brain tissue. The fi nding was evaluated as an emboligenic occlusion of the distal ICA, most likely of cardiac origin. On the next day, somnolence, dysarthria, dysphagia and left-sided hemiplegia were present. A follow-up CT of the brain was performed with the fi nding of expansively behaving ischemia in the right hemisphere with a midline shift, with a subphalcinic and descending transtentorial herniation (Fig. 2). The patient was transferred to neurosurgery, where an extensive right-sided hemicraniectomy was performed. This was followed by a stay at the Anesthesiology and Resuscitation Department. After disconnection from complete mechanical ventilation (immediate postoperative), she was transferred to a neurological ICU. Here, the patient’s environmental cooperation gradually improved, and passive and active rehabilitation was started. The neurological fi nding was dominated by signifi cant psychological changes with fl uctuations in cooperation, partial neglect syndrome, plegia of the left upper limb, and severe paresis of the left lower limb