title: Multimodal evoked potentials of Kennedy's disease.
abstract: BACKGROUND
  Kennedy's disease (KD) is an X-linked recessive polyglutamine disease. Traditionally, it is a lower motor neuron syndrome with additional features such as gynecomastia and tremor. Sensory symptoms are minimal if ever present. We used multimodal evoked potential (EPs) tests to study the distribution of the involvement of the disease.
  
  
  METHODS
  Visual, brainstem auditory, somatosensory and motor EPs were studied in six KD patients. All of them had typical presentations and had been proved genetically.
  
  
  RESULTS
  Abnormal findings were noted as follows: prolonged peak latencies of visual EPs, increased hearing threshold level, inconsistent brainstem auditory EPs, decreased amplitudes of cortical potentials of somatosensory EPs, and increased motor threshold to transcranial magnetic stimulation.
  
  
  CONCLUSIONS
  Our multimodal EP studies showed that KD involved multiple levels of the nervous system. It implies the widespread effects of the mutant androgen receptors.
authors.affiliation: Department of Neurology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Tsu-Hsien
authors.initials: TH
authors.lastname: Lai
authors.forename: Bing-Wen
authors.initials: BW
authors.lastname: Soong
authors.forename: Jen-Tse
authors.initials: JT
authors.lastname: Chen
authors.forename: Yen-Yu
authors.initials: YY
authors.lastname: Chen
authors.forename: Kuan-Lin
authors.initials: KL
authors.lastname: Lai
authors.forename: Zin-An
authors.initials: ZA
authors.lastname: Wu
authors.forename: Kwong-Kum
authors.initials: KK
authors.lastname: Liao
mesh_terms: D000161:Acoustic Stimulation; D000328:Adult; D000368:Aged; D001933:Brain Stem / Q000503:physiopathology; D004558:Electric Stimulation; D005071:Evoked Potentials / Q000502:physiology*; D005072:Evoked Potentials, Auditory / Q000502:physiology; D019054:Evoked Potentials, Motor / Q000502:physiology; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005074:Evoked Potentials, Visual / Q000502:physiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009134:Muscular Atrophy, Spinal / Q000175:diagnosis* / Q000503:physiopathology*; D009154:Mutation / Q000235:genetics; D009420:Nervous System / Q000503:physiopathology*; D009431:Neural Conduction / Q000502:physiology; D009434:Neural Pathways / Q000503:physiopathology*; D009460:Neurologic Examination; D010775:Photic Stimulation; D011237:Predictive Value of Tests; D011944:Receptors, Androgen / Q000235:genetics; D013116:Spinal Cord / Q000503:physiopathology; D050781:Transcranial Magnetic Stimulation