Clinical features of Kennedy's disease without sensory nerve involvement: a report of 5 cases
10.3969/j.issn.1002-0152.2017.08.008
- VernacularTitle:不伴感觉神经受累的肯尼迪病5例临床特征分析
- Author:
Xiaomeng ZHOU
1
;
Yaling LIU
;
Jina RAN
;
Ying WANG
Author Information
1. 河北医科大学第二医院 石家庄050000
- Keywords:
Kennedy' Disease;
AR mutation protein;
Multisystem lesion
- From:
Chinese Journal of Nervous and Mental Diseases
2017;43(8):485-489
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical feature,serum examination,EMG of Kennedy'Disease to reduce misdiagnosis of Kennedy's Disease.Methods Five cases of Kennedy's disease were confirmed by genetic test.The clinical data was analyzed including clinical features,laboratory findings,EMG characteristics and determination of AR gene exon 1 CAG repeat sequence.Results These cases were male without an obvious positive family history.The average age of onset was 39.8 ±7.2 years old and the average duration from onset to diagnosis was 9 ±5.2 years.Onset symptoms included Lower limbs weakness in 3 cases,facial fasciculationin 1 cases and gynecomastia in 1 case.The most prominent clinical manifestations were tongue muscle atrophy,tongue muscle fibrillation and proximal limb muscle weakness.In addition,these 5 cases did not have clinical manifestation of sensation loss nor EMG evidence of abnormal sensation.Conclusion Kennedy's disease is a neurodegenerative disease characterized by lower motor neuron damage.The clinical features of these 5 cases are approximately the same as those reported in previous literatures.Although the patients have been reported to have abnormal sensation,the present study indicates that some patients with Kennedy's disease may not present with abnormal sensation and that the diagnosis of Kennedy's disease depends on the genetic test.
