Probable Kennedy Disease Mimicking Hirayama Disease: A Case Report
10.46518/kjnmd.2023.15.1.24
- Author:
Soo-Im JANG
1
;
Soo-Hyun PARK
;
Seul-Gi CHOI
;
Sae-Nal LEE
;
Ji-Yoon AN
;
Nam-Hee KIM
Author Information
1. Department of Neurology, Dongguk University Ilsan Hospital, Goyang, Korea
- Publication Type:Case Report
- From:
Korean Journal of Neuromuscular Disorders
2023;15(1):24-27
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Spinal and bulbar muscular atrophy (Kennedy disease) is an X-linked, adult-onset motor neuron disease characterized by slow, progressive weakness of the bulbar and extremity muscles with CAG triplet repeat expansion in the androgen receptor gene. Hirayama disease (HD) is characterized by the juvenile onset of asymmetric weakness and amyotrophy of the hand and is most common in males in Asia. We report a patient with atypical Kennedy disease presenting with asymmetric hand weakness and atrophy typical of HD.
