Vascular Diseases of the Spinal Cord

Key points

Spinal cord anatomy

A detailed knowledge of spinal cord anatomy is indispensable in the evaluation of a patient with suspected spinal cord disease. Understanding the anatomy is necessary to interpret the patient's neurologic signs and symptoms, and to select appropriate diagnostic tests during initial evaluation. Therefore, it is important that the most relevant anatomic concepts are reviewed before discussing the various vascular pathologies of the spinal cord.1, 2, 3

Spinal cord infarction

SCI is a rare cause of acute myelopathy, frequently cited as representing only 1% of all strokes and 5 to 8% of acute myelopathies,⁹ although the exact incidence and prevalence are not well known. It has been associated with myriad pathogenic mechanisms, the most significant of which have actually changed over time. Whereas syphilitic arteritis was the most common cause of SCI until the early 20th century,¹⁰ and the cause of the first case of anterior spinal artery syndrome,¹¹ SCI is now most

Spinal AVMs

An AVM is generically defined as an abnormal communication between and artery and a vein without intervening capillary architecture. These lesions occur throughout the central nervous system (CNS), including the spinal cord, and further classification of spinal lesions is stratified by localization and morphology.⁶⁰ Spinal AVMs are a rare clinical phenomenon, although precise estimations of incidence are lacking for all but a specific subset called sdAVF, which is reported to affect 5 to 10

Spinal vasculitis

Spinal cord involvement from systemic vasculitis151, 152, 153, 154, 155, 156 or primary vasculitis of the CNS¹⁵⁷ (PCNSV) is exceedingly rare. Furthermore, several of the cases reported as likely systemic vasculitides are without true diagnoses in the absence of documented clinical or laboratory evidence of a vasculitic syndrome.152, 155, 156 Infectious vasculitis affecting the vasa nervosa, characteristic of syphilitic arteritis,¹¹ is a vasculitis per se, but has been included in the SCI

Spinal genetic and neurocutaneous vascular syndromes

Von Hippel-Lindau disease is a genetic disorder that predisposes to the development of hemangioblastomas of the CNS, retina, and abdominal viscera.¹⁵⁹ The cerebrum and cerebellum are primarily affected, but the spinal cord can be involved. In one pathologic report of five cases of Von Hippel-Lindau disease, two patients had spinal cord hemangioblastomas.¹⁶⁰ Of note, in one of those patients, there were two relatively small hemangioblastomas, one each in the cervical and lower thoracic cord,

Acknowledgments

Many thanks to our esteemed neuroradiologist colleagues, T. Kaufmann and J. Morris, for providing several of the images in this manuscript.