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Systemic Lupus Erythematosus (SLE) is an autoimmune inflammatory disorder affecting multiple organ systems. 30-40 % of the patients manifest variable neuropsychiatric symptoms leading to significant morbidity and mortality. CNS involvement could be primary if directly related to SLE activity in the CNS or secondary to treatment, infections, metabolic abnormalities or other systemic manifestations such as hypertension (Futrell et al,1992). Primary NPSLE is divided into focal and diffuse disease. Focal NPSLE is characterized by focal neurologic deficits and is strongly associated with the occurrence of thromboembolic events. Diffuse primary NPSLE is a group of neurologic, psychiatric, and cognitive syndromes that vary from overt neurologic and psychiatric symptoms (eg. seizures, psychosis) to more subtle signs such as headache, mood disturbances, anxiety disorders or mild cognitive dysfunction. Neuropsychiatric lupus (NPSLE) manifestations can occur in the absence of either serologic activity or other systemic disease manifestations (Sibbit et al., 1999). Thus, in clinical practice the diagnosis of primary NPSLE is rather presumptive, after the exclusion of alternative causes of the neuropsychiatric symptoms. There is no single diagnostic test that is sensitive and specific for SLE-related neuropsychiatric manifestations. The assessment of individual patients is based on clinical, neurologic and rheumatologic evaluation, immunoserologic testing, brain imaging, and psychiatric and neuropsychological assessment. These examinations are used to support or refute the clinical diagnostic impression, rule out alternative explanations, and form the basis for prospective monitoring of clinical evolution and response to treatment interventions. The lack of a diagnostic gold standard makes the correct diagnosis of primary NPSLE a challenge. This realization has led us in developing under the auspices of the European League Against Rheumatology (EULAR) evidence and expert based recommendations for the management of NPSLE (Bertsias et al, 2010).

intech ebooks

Non-Conventional MRI Techniques in Neurophychiatric Systemic Lupus Erythematosus (NPSLE): Emerging Tools to Elucidate the Pathophysiology and Aid the Diagnosis and Management