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Ann Clin Neurophysiol > Volume 5(1); 2003 > Article
Ann Clin Neurophysiol. 2003; 5(1): 11-15.
Clinical and Electrodiagnostic Features of Kennedy Disease
Byeong Cheol Oh, Young-Min Lim, and Kwang Kuk Kim
Copyright © 2003 The Korean Society of Clinical Neurophysiology
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background : Kennedy disease is a X-linked recessive disease characterized by bulbar symptoms, proximal muscle weakness, and gynecomastia. Methods : We analyzed clinical symptoms and performed electrodiagnostic studies on 6 patients. Results : We found following features: 1) proximal muscle weakness 2) bulbar symptoms, as dysarthria, facial and tongue atrophy 3) hyporeflexia or areflexia 4) fasciculations, predominantly on face, and proximal upper extremities 5) decreased sensory nerve action potentials(SNAPs) 6) chronic neurogenic changes in needle EMG. Conclusions : Kennedy disease is characterized by degenerative process of anterior horn cell and dorsal root ganglion without upper motor neuron dysfunction. Increased triple uncleotide CAG repeat(>38) in androgen receptor gene of Xp21 will confirm early stage of this disease.
Key words: Kennedy disease, Triple nucleotide repeats
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