Vasculopathies

Vasculopathies represent a broad category of disorders involving blood vessels. The most common are the vasculitides, moyamoya disease, fibromuscular dys-plasia, and cerebral amyloid angiopathy.

A. Vasculitides 1. General Considerations

The vasculitides are a diverse group of infrequent disorders characterized by underlying inflammation and necrosis of the blood vessel wall. They are heterogeneous with respect to presentation, distribution, size of the affected vessel, and underlying etiology. Central nervous system (CNS) vasculitis is a potentially serious disorder with symptoms arising from ischemia or hemorrhage. Rapid diagnosis and treatment are prerequisite to favorably altering the disease course.

2. Classification

The vasculitides are generally divided into primary and secondary disorders (Table X). The primary vasculi-tides are subdivided based on the size of the vessel affected (i.e., large, medium, or small). Large-vessel vasculitides include temporal arteritis, Takayasu's arteritis, and primary angiitis of the CNS. Polyarteritis nodosa and Kawasaki disease typify the medium-vessel vasculitides. The small-vessel vasculitides include Churg-Strauss syndrome and Wegener's gran-ulomatosis. The secondary vasculitides represent the most frequent cause of CNS vasculitis. They are a varied group and result from underlying autoimmune diseases, intoxications, and infectious and neoplastic processes.

3. Pathogenesis

The pathogenesis of vasculitis is complex and not well understood. There is increasing evidence that anti-neutrophil cytoplasmic antibodies (ANCAs) play a role in the immunopathogenesis of the vasculitides by activating neutrophils and thereby causing endothelial injury. Various immunological mechanisms have been proposed, but ultimately inflammation causes leukocytes to adhere to the vessel wall. The inflammation is usually segmental in nature, with skip lesions of intense inflammation interrupting otherwise normal vascula-ture. One or more layers of the vessel wall can be affected. The inflammation tends to resolve, leaving fibrosis and hypertrophy, which cause secondary occlusion.

4. Clinical Presentation

Vasculitis can present with CNS symptoms and signs alone or combined with nonspecific or multiorgan symptoms. The sequelae depend on the number, size, and site of the involved blood vessels. Focal injuries tend to cause wall rupture and hemorrhage. Segmental injuries affect the entire wall circumference and tend to cause stenosis, occlusion, and infarction. Collectively, patients can present with cerebral ischemia, encepha-lopathy, intracerebral hemorrhage, or seizures.

5. Diagnosis

The diagnosis of vasculitis can be difficult. A complete history and physical examination, augmented with appropriate laboratory tests, are necessary. A complete blood count should be performed and markers of inflammation, such as ESR and C-reactive protein, will be elevated. Other laboratory tests that can help

Table X

Classification of Vasculitides Affecting the Central Nervous System

Table X

Classification of Vasculitides Affecting the Central Nervous System

Primary vasculitides

Secondary vasculitides

Large vessel

Autoimmune diseases

Temporal arteritis

Systemic lupus erythematosis

Takayasu's arteritis

Sjogren's syndrome

Primary angiitis of the

Behcet's syndrome

central nervous system

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