Spontaneous non-aneurysmal subarachnoid hemorrhage in Takayasu arteritis: a case implicating hyperperfusion and cerebral dysautoregulation

Abstract

Takayasu arteritis (TA) is a systemic chronic inflammatory large-vessel vasculitis that affects the aorta, its major branches, and the pulmonary arteries. In this report, we describe a case of a young female with TA presenting with spontaneous subarachnoid hemorrhage (SAH), an unusual manifestation of the disease. Magnetic resonance angiography (MRA) of the head and neck demonstrates multifocal carotid and vertebral arterial stenoses, but no aneurysm or vascular malformation to account for SAH. A novel and unexpected finding in this case was increased cerebral perfusion in the right frontotemporal parenchyma and transient abnormally reduced augmentation of flow in response to the cerebral vasodilator acetazolamide. The etiology of SAH thus may be related to hyperperfusion and loss of cerebrovascular autoregulation leading to small vessel damage.