RESEARCH ARTICLE Neuropsychological Investigation in Chinese Patients with Progressive Muscular Atrophy

Background Progressive muscular atrophy (PMA) is a rare type of degenerative motor neuron disease (MND) of which the onset happens in adult period. Despite its well-defined clinical charac-teristics, its neuropsychological profile has remained poorly understood, considering the consensus of cognitive and behavioral impairment reached in amyotrophic lateral sclerosis (ALS). Methods We conducted a cross-sectional evaluation of Chinese PMA patients with a series of com-prehensive batteries emphasizing the executive and attention function, and covering other domains of memory, language, visuospatial function, calculation and behavior as well. Their performances were compared with those of age- and education-matched ALS and healthy controls (HC). Results 21 patients newly diagnosed with PMA were consecutively enrolled into our ALS and other MND registry platform, accounting for 14.7 % of all the incident MND cases registered during the same period. 20 patients who completed the neuropsychological batteries were includ-ed into analysis. Compared with HC, PMA performed significantly worse in maintenance function of attention, while they exhibited quantitative similarity to ALS in all behavioral in-ventories and neuropsychological tests except the time for Stroop interference effect. Conclusion PMA could display mild cognitive dysfunction in the same frontal-mediated territory of ALS but in a lesser degree, whereas they did not differ from ALS behaviorally