A case-control study with 46 SCA3/MJD patients and 42 healthy, non-related control individuals with similar age and sex was performed.
Clinical evaluation was done with the ataxia scales SARA and NESSCA. Serum insulin, insulin-like growth factor 1 (IGF-1) and magnetic resonance imaging normalized volumetries of cerebellum and brain stem were also assessed. BMI was lower in SCA3/MJD patients when compared to controls (p = 0.01). BMI was associated with NESSCA, expanded CAG repeat number (CAG)n, age of onset, age, disease duration, and serum insulin levels; however, in the linear regression model, (CAG)n was the only variable independently associated with BMI, in an inverse manner (R = -0.396, p = 0.015). In this report, we present evidence that Sapanisertib purchase low BMI is not only present in SCA3/MJD, but is also directly related to the length of the expanded CAG repeats, which is the causative mutation of the disease. This association points that weight loss might be a primary disturbance of SCA3/MJD, although further detailed analyses are necessary BIRB 796 for a better understanding of the nutritional deficit and its role in the pathophysiology of SCA3/MJD.”
“Background: Neurologic deterioration (ND) occurs in one third of patients with ischemic stroke and contributes to morbidity and mortality
in these patients. Etiologies of ND and clinical outcome according to ND etiology are incompletely understood. Methods: We conducted a retrospective investigation of all Z-VAD-FMK patients with ischemic stroke admitted to our center (July 2008 to December 2010), who were known to be last seen normal less than 48 hours before
arrival. First-time episodes of ND during hospitalization were collected in which a patient experienced a 2-point increase or more in National Institutes of Health Stroke Scale score within a 24-hour period. Proposed etiologies of reversible ND include infectious, metabolic, hemodynamic, focal cerebral edema, fluctuation, sedation, and seizure, whereas new stroke, progressive stroke, intracerebral hemorrhage, and cardiopulmonary arrest were nonreversible. Results: Of 366 included patients (median age 65 years, 41.4% women, 68.3% black), 128 (34.9%) experienced ND (median age 69 years, 42.2% women, 68.7% black). Probable etiologies of ND were identified in 90.6% of all first-time ND events. The most common etiology of ND, progressive stroke, was highly associated with poor outcome but not death. Etiologies most associated with mortality included edema (47.8%), new stroke (50%), and intracerebral hemorrhage (42.1%). Conclusions: In the present study, the authors identified probable etiologies of ND after ischemic stroke. Delineating the cause of ND could play an important role in the management of the patient and help set expectations for prognosis after ND has occurred.