Within the past few years, a clearly defined case definition system for cen
tral nervous system systemic lupus erythematosus (CNS-SLE) has been establi
shed. This has allowed cross-study comparisons of patients fulfilling the s
pecific case definitions. New imaging techniques used on the subgroup of CN
S-SLE patients that did not have any evidence for infarctions suggest that
in these patients symptoms are associated with a diffuse process in the bra
in. Most likely this process leads to axonal damage and demyelination, ulti
mately leading to cerebral atrophy. With respect to the diagnostic work-up
of SLE patients with neu ro psychiatric symptoms, it has become clear that
cranial magnetic resonance imaging is the technique of choice. Preliminary
studies using quantitative magnetic resonance imaging techniques suggest th
at patients with neuropsychiatric symptoms caused by active CNS-SLE can be
differentiated from patients with the same symptoms caused by residual dise
ase. (C) 2001 Lippincott Williams & Wilkins, Inc.