International Clinical Neuroscience Journal

Vol. 2 No. 4 (2015)

Weakness and progressive muscle tightness diagnosed as Kennedy disease

International Clinical Neuroscience Journal, Vol. 2 No. 4 (2015), 5 May 2016 , Page 147-148
https://doi.org/10.22037/icnj.v2i4.11671

Abstract

Kennedy disease is a rare X-linked neurodegenerative disorder that affects patients in 30-50 years of age. It is caused by CAG-repeat in androgen receptor gen. There is no known effective treatment for Kennedy disease. We report a 60-year-old 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.

Keywords:
  • Spinobulbar muscular atrophy
  • Kennedy disease
  • x-linked
  • Neurodegenerative

How to Cite

1.
Behnam B, Arab Ahmadi M, Ashrafi F. Weakness and progressive muscle tightness diagnosed as Kennedy disease. Int Clin Neurosci J [Internet]. 2016 May 5 [cited 2026 Jul. 8];2(4):147-8. Available from: https://journals.sbmu.ac.ir/neuroscience/article/view/11671

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