Connective tissue diseases such as systemic lupus erythematosus can have neurological manifestations. Furthermore, systemic vasculitides can result in neurological disease (Sigal, 1987; Moore, 1989b) and some vasculitides are restricted to the nervous system (Dyck et al., 1987; Moore, 1989a; Crane, Kerr & Spiera, 1991). There are three possible means by which connective tissue diseases and vasculitides could be associated with neurological disorders. Firstly, the neurological complications of these conditions could be due to ischaemia secondary to vascular occlusion. Secondly, neurological complications could be due to a specific immune response directed against antigens in the parenchyma of the nervous system. Thirdly, neurological disturbance could result from a separate autoimmune neurological disorder occurring in an individual predisposed to autoimmune disease. This chapter reviews central nervous system (CNS) and peripheral nervous system (PNS) manifestations of connective tissue diseases and vasculitides, but does not attempt a comprehensive review of these systemic disorders.

Clinical features

Systemic lupus erythematosus

The neurological manifestations of systemic lupus erythematosus (SLE) are manifold (Johnson & Richardson, 1968; Feinglass et al., 1976; Futrell, Schultz & Millikan, 1992). There are strict criteria for the diagnosis of SLE (Tan et al., 1982) and these include the presence of neurological signs. In some patients, neurological symptoms and signs are the first manifestation of SLE (Tola et al., 1992).