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Acute confusional state represents the severest clinical subsets in neuropsychiatric SLE (NPSLE), having been identified as one of the poor prognostic factors towards mortality [1]. The frequencies of ACS in NPSLE differ depending on case series, presumably because of the lack of accepted consensus in the diagnostic criteria. In addition, clinical investigations focusing on ACS are limited in number. Under such circumstances, precise understanding of this clinical entity has been hampered for many of the physicians who are involved in the treatment of NPSLE.

In order to evaluate the disease status of the patients with NPSLE in an objective and precise way, several clinical examinations have been performed such as electroencephalogram, brain magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analyses, and measurement of autoantibodies in serum and in CSF. Among them, brain MRI has been the first choice imaging technique in the diagnosis of NPSLE, especially for focal diseases represented by infarctions. Also for diffuse NPSLE, cortical atrophy, small white matter lesions, perivascular/diffuse lesions can be observed, although none of these findings are specific for NPSLE [2]. Many studies have been conducted to elucidate the clinical significance of MRI abnormalities in patients NPSLE, but this type of investigation specific for ACS has not been designed so far.

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