title: [Comparison of clinical and physiological characteristics between Kennedy disease and amyotrophic lateral sclerosis].
abstract: OBJECTIVE
  To study the clinical presentations of Kennedy disease (KD) and compare the neurophysiological features between KD and amyotrophic lateral sclerosis(ALS).
  
  
  METHODS
  Nine patients with KD, 13 patients with ALS and 26 normal control subjects were recruited. The clinical presentations of KD were analyzed, and the results of nerve conduction studies and electromyography were compared among the 3 groups.
  
  
  RESULTS
  The rates of tongue atrophy and facial fasciculation were 100% and 88.9%, respectively, in the early course and mid-course of KD, sensory damages might be perceived. 2)The sural nerve sensory nerve action potential (SNAP) was not elicited in 56.3% of the patients with KD, and sural nerve SNAP amplitudes were significantly lower in KD (7.9. ± 3.4 µV) than in ALS patients (20.0 ± 5.2 µV) and normal control subjects (26.1 ± 16.8 µV) (P<0.05).
  
  
  CONCLUSIONS
  B The onset of clinical presentations mimicking motor neuron disease, appearance of tongue atrophy and facial fasciculation in the early and mid-course, and presence of sensory impairment with a decreased sural nerve SNAP amplitude may suggest the diagnosis of KD and should prompt a genetic test for KD.
authors.affiliation: Southern Medical University, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China. E-mail: xiao_m_best@163.com.
authors.forename: Jialing
authors.initials: J
authors.lastname: Yang
authors.forename: Qun
authors.initials: Q
authors.lastname: Wang
authors.forename: Lizhen
authors.initials: L
authors.lastname: Lin
authors.forename: Dongmei
authors.initials: D
authors.lastname: Wang
authors.forename: Hui
authors.initials: H
authors.lastname: Zheng
authors.forename: Yuqing
authors.initials: Y
authors.lastname: Guan
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000503:physiopathology*; D055534:Bulbo-Spinal Atrophy, X-Linked / Q000503:physiopathology*; D004576:Electromyography; D005071:Evoked Potentials; D006801:Humans