Acta Rheumatologica

Abstract

Neuropsychiatric Systemic lupus erythematous (NPSLE) refers to the various psychiatric and neurologic manifestations that develop secondary to involvement of the nervous system in patients with SLE [1, 2]. These clinical features occur either due to a diffuse neurological insult (e.g. encephalopathy, coma, depression, and psychosis) or a localized neurologic insult (e.g. stroke or seizure etc) [3]. Because of the varied diagnostic criteria associated with these manifestations, the American College of Rheumatology (ACR) has formulated case definitions, reporting standards, and diagnostic testing recommendations for the 19 neuropsychiatric SLE syndromes [4]. The prevalence of neurologic and psychiatric manifestations using the ACR case definitions follows the following order from most to least: cognitive dysfunction, headache, mood disorder, cerebrovascular disease, seizures, polyneuropathy, anxiety, and psychosis [5]. A psychiatric disturbance due to CNS lupus is a diagnosis of exclusion; all other possible causes of the observed symptoms must therefore be considered, including infection, electrolyte abnormalities, renal failure, drug effects, mass lesions, arterial emboli, and primary psychiatric disorders (such as bipolar disorder or severe stress disorder resulting from a chronic and life-threatening disease) [6]. One clue to the diagnosis is that majority of acute neuropsychiatric episodes occur during the first two years after the onset of SLE [7].