Clinical and electrophysiological features and pulmonary function of 8 patients with Kennedy's disease
10.3760/cma.j.issn.1671-8925.2015.01.012
- VernacularTitle:肯尼迪病患者的临床、电生理及肺功能研究
- Author:
Biying YANG
1
;
Xiaomei HOU
;
Baoxin DU
;
Ming LU
;
Yu ZHENG
Author Information
1. 广东省中医院神经四科
- Keywords:
Kennedy's disease;
Electromyography;
Pulmonary function
- From:
Chinese Journal of Neuromedicine
2015;14(1):60-63
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical and electrophysiological features and pulmonary function of 8 Chinese patients with Kennedy's disease (KD) and to enhance the understanding for the disease.Methods Eight patients with KD,admitted to out hospital from July 2010 to December 2013 and confirmed by gene examination,were chosen in our study; their clinical and electrophysiological features,and laboratory characteristics and pulmonary function were compared with those in the healthy volunteers.Results The average age of onset in the 8 patients was (45.13±17.47) years,and the average age of diagnosis was (55.63±12.11) years.The most common complaint was leg weakness.All patients presented hemifacial spasm,tongue muscle atrophy and fibrillation,amyotrophy and gynaecomastia; creatine kinase level was elevated and endocrine disorder appeared in different degrees.The electromyogram characteristics included widespread neurogenic changes accompanied with/without sensory or motor conduction abnormalities.The forced vital capacity,forced expiratory volume in first second,maximal voluntary ventilation and peak expiratory flow rate were significantly lower,and the residual volume in the KD patients was statistically higher than that in the healthy volunteers (P＜0.05).Respiratory muscle strength (maximum inspiratory pressure and maximum expiratory pressure) decreased in the KD patients.Conclusion KD is a degenerative disease with slow clinical progression which has its own characteristics of inheritance pattern and natural course; the age of onset,repeat number of CAG sequences,pulmonary function and respiratory muscle strength may be valuable for illness evaluation.
