Neurological presentation of acute aortic syndrome: Type A intramural haematoma presenting as ischaemic hemisection of the spinal cord.

Abstract

Intramural haematoma (IMH) describes the presence of blood within the aortic wall, and is associated with a significant morbidity and mortality. Early diagnosis is essential for institution of medical, and sometimes surgical, management. Neurological complications have rarely been described during the initial presentation of IMH, or other forms of acute aortic syndrome. We describe a 56-year-old man who presented with sudden onset chest pain and left leg weakness and numbness, and the loss of right leg pain and temperature sensation. CT Angiography showed a Type A intramural haematoma extending from the ascending to the infra-renal aorta. He was managed successfully with cerebrospinal fluid drainage and thoracic endografting to cover the intimal entry lesion. His neurological symptoms improved and he remained well at 3 years with minor residual neurological deficits. Spinal cord infarction is a rare but documented complication of acute aortic syndrome; Brown-Séquard syndrome typically results from a traumatic injury. To the best of our knowledge, this is the first report of IMH presenting with Brown-Séquard syndrome. This case highlights the need to consider acute aortic syndromes in a patient presenting with chest pain and acute neurological symptoms.