Weakness and progressive muscle tightness diagnosed as Kennedy disease

Kennedy disease is a rare Xlinked neurodegenerative disorder that affects patients in 3050 years of age. It is caused by CAGrepeat in androgen receptor gen. There is no known effective treatment for Kennedy disease. We report a 60yearold man who had fasciculations and proximal and distal muscle weakness. Physical examination showed involvement of the bulbar musculature accompanied by tongue atrophy and perioral muscle weakness. Furthermore, he had bilateral gynecomastia. Laboratory and imaging findings were normal, except electromyography that showed chronic proximal and distal denervation. Finally, the patient diagnosed with Kennedy disease according to clinical presentation and EMG abnormality that confirmed with genetic study.