Oculopharyngodistal myopathy in three unrelated Korean patients: Early distal limb weakness is characteristic finding in oculopharyngodistala myopathy
- Author:
Sun-Young Kim
;
Jeong-Keun Lim
;
Young-Eun Park
;
Chang-Hoon Lee
;
HyangSook Kim
;
Dae-Seong Kim
- Publication Type:Journal Article
- From:Neurology Asia
2011;16(4):315-320
- CountryMalaysia
- Language:English
-
Abstract:
Objectives: To determine the clinical characteristics of Korean patients with sporadic oculopharyngodistal
myopathy. Methods: Three consecutive unrelated Korean patients with sporadic oculopharyngodistal
myopathy participated in this study. Stepwise approaches were performed including careful clinical
examination, electrophysiological study, pathology evaluation and genetic study. The literature on
oculopharyngodistal myopathy was reviewed. Results: Clinically, our patients showed consistent
features of initial distal limb weakness with variable ophthalmoplegia, bulbar weakness or proximal
weakness. All the patients showed rimmed vacuoles and myopathic changes.
Conclusion: Through the literature review and this study, we believe that initial distal limb weakness
is a characteristic fi nding in patients with oculopharyngodistal myopathy in some ethnic groups.
- Full text:P020150818605948953396.pdf