Research reports on muscular atrophy from Mayo Clinic provide new insights.(Clinical report)

According to recent research from the United States, "Although spinal-bulbar muscular atrophy (SBMA) is generally believed to be associated with better survival and function compared to other motor neuron diseases, no systematic study of long-term functional status or survival has been reported. We report the results a retrospective review of 39 patients with genetically confirmed diagnosis and compared their survival to normal, population-based, age-and gender-matched controls."

"We assessed the functional status of 25 of the 33 survivors by completing the revised ALS Functional Rating Scale (ALSFRS-r) by telephone. The subjects with SBMA had a 10-year survival of 82% compared to 95% among the age-matched controls (p = 0.053). The mean ALSFRS-r score for the survivors was 37 (range 27 - 43). The ALSFRS-r subscores indicated mild deficits in all areas in most, with the greatest limitation being the use of stairs. While all reported bulbar symptoms, none had disabling deficits. None of the subjects required a percutaneous endoscopic gastrostomy tube and one subject used noninvasive positive pressure ventilation. The long-term survival of subjects with spinal-bulbar muscular atrophy is ...