Online edition:ISSN 2758-089X

Spontaneous Reduction of Diverticular Outpouchings and Multiple Dissecting Aneurysms of Cervicocranial Arteries with Fibromuscular Dysplasia ― The Relation to Alpha-1-Antitrypsin Deficiency ―

The authors experienced a patient with fibromuscular dysplasia and multiple dissecting aneurysms of the cervicocranial arteries due to an alpha -1 - antitrypsin deficiency. This 48 -year-old woman presented with gait disturbance and hypalgesia of the right extremities. Magnetic resonance imaging revealed a small infarction of the left lateral part of the medulla oblongata. Angiography revealed dissecting aneurysms of the right internal carotid artery and the left vertebral artery near the infarction, and demonstrated multiple diverticular outpouchings of the cervical vertebral arteries bilaterally and the typical "string of beads" appearance in the bilateral brachial arteries. Her alpha -1 - antitrypsin plasma level was 173 mg/dl (normal range 184-243 mg/dl) on the first admission. Angiography one year later revealed diminishing of the size of the dissecting aneurysms and diverticular outpouchings, and the patient's alpha -1 - antitrypsin plasma level had normalized (223 mg/dl). The plasma level of alpha-1-antitrypsin may be useful for following this disease.

Watanabe A, et al