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SLE is an autoimmune chronic progressive disease which affects the central and peripheral nervous system and causes different neuropsychiatric symptoms. Neuropsychiatric Syndromes of Systemic Lupus Erythematosus (NPSLE) can be observed in 10-80% of SLE patients. NPLSE syndromes have been defined by America College of Rheumatology. Vascular and neuronal damage caused by inflammatory cytokines, autoantibodies and immune complexes are involved in pathogenesis of NPLSE. Stroke is the most severe complication of SLE and the incidence rate is approximately 3-20% especially in the first five years of the disease. Many factors such as high level of aPL, hyperhomocysteinemia, lupus disease activity, cerebral vasculitis, emboli from Libman-Sacks endocarditis, accelerated atherosclerosis cause stroke in SLE. Stroke related to vasculitis is a rare manifestation of NPSLE. Immune complex deposition in vascular endothelium, intrathecal immune complexes and other inflammatory mediators are involved in the pathogenesis of vasculopathy and vasculitis. Here, we present a 41-year-old Syrian woman with diagnosis of SLE and antiphospholipid antibody syndrome (APS). Her medical history showed several SLE attacks, ischemic cerebrovascular accidents (CVA) and seizure. Neurological examination revealed confusion, poor cooperation/orientation, left-sided hemiparesia. Cranial MRI, MRA and DSA findings were suggestive of vasculitis related to lupus. For the treatment, acetylsalicylic acid, clopidogrel, warfarin, cyclophosphamid 1g IV and uromitexan were applied. On the basis of this case, diagnostic and therapeutic approaches of NPSLE were discussed
Data on the relationship between behavioral disturbances in Alzheimer's disease (AD) and global clinical deterioration is still controversial. The purpose of this study was to explore potential correlations of neuropsychiatric syndromes with global clinical deterioration in patients with AD, with particular consideration on severity levels of dementia.
Methods:
AD patients (n = 156) aged 76.7 years from Brazilian clinical centers were assessed to diagnose the five neuropsychiatric syndromes measured by the Neuropsychiatric Inventory-Clinician rating scale (NPI-C): psychosis, agitation, affective, apathy, and sleep. These syndromes were then analyzed for their correlation with the Global Deterioration Scale (GDS). To analyze the association of neuropsychiatric syndromes with the GDS, considering the total sample and patients grouped by dementia severity levels, we applied the coefficient of multiple correlation (Ryy), adjusted multiple linear regression, and the coefficient of determination (R2yx). We tested the significance of correlation coefficients using the Student t-test for simple correlations (a single independent variable) and analysis of variance (ANOVA) for multiple correlations. ANOVA was also used to compare means of demographic and some clinical variables at different levels of dementia.
Results:
For the total sample, apathy and agitation syndromes were most strongly correlated (0.74; 0.72, respectively) with clinical deterioration according to the GDS, followed by psychosis (0.59), affective (0.45), and sleep syndromes (0.34). Agitation significantly correlated with mild and moderate dementia (CDR 1: 0.45; and CDR 2: 0.69, respectively). At CDR 2, agitation and affective syndromes were most strongly correlated (0.69; 0.59, respectively) with clinical deterioration while at CDR 3, the apathy syndrome was most strongly correlated with clinical deterioration (0.52).
Conclusions:
Agitation, apathy, and affective disorders were the syndromes most strongly correlated with global deterioration in AD patients, becoming more evident at severe stages of dementia.
White matter was first depicted as a neuroanatomic structure by the great anatomist Andreas Vesalius in 1543. The anatomy of white matter is fundamental to understand its role in brain-behavior relationships. The major function of white matter can be conceived as the transfer of information within the nervous system. The development of magnetic resonance imaging (MRI) in the early 1980s has proven to be a pivotal event in the understanding of white matter and its impact on higher brain function. Ten categories of white matter disorder are: genetic, demyelinative, infectious, inflammatory, toxic, metabolic, vascular, traumatic, neoplastic, and hydrocephalic. Three major groups of syndromes emerge that capture the variety of cognitive and emotional impairments related to cerebral white matter disorders: focal neurobehavioral syndromes, white matter dementia, and neuropsychiatric syndromes. The treatment of cerebral white matter disorders depends on the specific problem disclosed by the diagnostic search.
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