Chapter 14: Neurovascular and Neurocutaneous Diseases

CHAPTER OUTLINE

• CEREBROVASCULAR DISEASE

  • Carotid disease

  • Vertebrobasilar disease

  • Arteriovenous malformation

  • Arterial-cavernous sinus fistula

  • Aneurysms

  • Dolichoectatic arteries

• NEUROCUTANEOUS SYNDROMES

  • Neurofibromatosis

  • Encephalotrigeminal angiomatosis (Sturge-Weber syndrome)

  • Retinal angiomatosis (von Hippel disease)

  • Ataxia telangiectasia (Louis-Bar syndrome)

  • Wyburn-Mason syndrome

  • Tuberous sclerosis (Bourneville syndrome)

• KEY POINTS

INTRODUCTION

The topic of neurovascular diseases is a broad one, and a number of the disorders discussed in earlier chapters belong in this category. The goal of this chapter is not to be all-inclusive, but rather to discuss the mechanisms of vascular disease that commonly affect the visual system. Inclusion of the phakomatoses in this chapter is appropriate because many of these diseases have neurovascular manifestations.

CEREBROVASCULAR DISEASE

The vascular supply of the brain consists of the carotid system anteriorly and the vertebrobasilar system posteriorly. The circle of Willis is an anastomotic complex (of variable completeness) formed by the junction of the two systems (Figure 14–1). Common ischemic manifestations of cerebrovascular disease include transient neurological deficits and stroke. Seizures can also occur as a result of ischemia.

A transient ischemic attack (TIA) is an episode of transient neurological dysfunction that lasts less than 24 hours. Most TIAs last less than 20 minutes. The 24-hour time limit in the definition of a TIA is an arbitrary boundary; neurological episodes may last longer than 24 hours and still be reversible. Stroke occurs when ischemic events are of sufficient magnitude or duration to cause infarction. Vascular disease can cause a TIA or stroke in the carotid or the vertebrobasilar distribution (Table 14–1), producing signs and symptoms that help to localize the ischemic area of the brain and implicate specific arteries that supply the affected territory.

CAROTID DISEASE

TIAs or completed strokes in the distribution of the carotid system frequently involve the eye (Table 14–2).