PREDICTIVE VALUE OF CIRCULATING GONADAL AND ADRENAL STEROIDS FOR RESPIRATORY FUNCTION IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS (ALS)

GARGIULO MONACHELLI GM; SIVORI M; MEYER M; SICA REP; DE NICOLA AF; GONZALEZ DENISELLE M.C

San Diego

Conferencia; 65th Annual Meeting of the AAN; 2013

Objective: To analyze serum levels of adrenal and gonadal steroids in sporadic ALS patients and controls, and to search for a relationship between these steroids and functional and respiratory parameters in ALS. Background: Changes of circulating steroids exist in patients with ALS and animal models of motor neuron degeneration and influence progression. For instance, in the SOD1 mouse model of familial ALS, ovariectomy leads to a significant acceleration of the disease. Changes of steroids may also influence respiratory function. Lastly, ALS has been recently considered a hormonal condition. Design/Methods: Serum levels of gonadal and adrenal steroids were measured in 52 subjects (28 ALS and 24 controls) using radioimmunoassay procedures. We also evaluated ALS functional and strength scales, and respiratory parameters: forced vital capacity (FVC%), FVC% decline, maximal inspiratory pressure (MIP%) and maximal expiratory pressure (MEP%), at diagnosis. The need for non-invasive ventilation (NIV) and survival time were also recorded. Basal steroid levels and steroid ratios were correlated with respiratory parameters. Results: The results showed that female patients demonstrated higher testosterone levels than female controls. Furthermore, with increasing age testosterone declined in controls but not in ALS patients, suggesting a differential metabolic behavior. The ratios dehydroepiandrosterone sulfate (DHEAS)/cortisol and progesterone/cortisol positively correlated with FVC%. The DHEAS/cortisol ratio was also positively correlated with MIP% and MEP%. The analysis of total testosterone and free testosterone showed a significant negative relationship with greater FVC% decline. Multiple regression analysis showed that elevated cortisol levels and higher age significantly decreased the time to NIV in 2.2 and 1.7 months, respectively. Conclusions: Determination of the steroid profile was sexually dysmorphic in ALS. It may be of value to predict outcome in ALS patients. Regarding respiratory function, certain hormones may serve a protective function while others pertain a negative influence.