Chronic noninfectious inflammatory CNS diseases

In clinical practice the differentiation between infl ammatory diseases of the central nervous system (CNS) as well as chronic and subacute CNS diseases may be challenging due to similarities in the symptomatology and overlapping changes on magnetic resonance imaging (MRI). However, the distinction between these diseases is important due to major differences in the treatment strategies. Since cerebrospinal fl uid (CSF) is in direct contact with the brain and spinal tissue, the analysis of CSF composition provides additional information needed in differential diagnostics. Changes in the composition of the CSF occur in primary autoimmune diseases of the CNS but also in nearly all infection-induced infl ammation and neurological manifestations of systemic autoimmune diseases such as systemic lupus erythematosus (SLE), primary Sjogren’s syndrome (PSS), and mixed connective tissue disease (MCTD). Infl ammatory lesions in the brain in primary angiitis of the CNS and in systemic vasculitides such as granulomatosis with polyangitis, Behcet’s disease, and polyarteritis nodosa may also mimic MS. This chapter reviews the CSF fi ndings in CNS diseases including multiple sclerosis and other infl ammatory demyelinating diseases as well as in major chronic and subacute CNS manifestations associated with systemic autoimmune diseases.