Are cognitive and behavioural deficits a part of the clinical picture in Kennedy's disease? A case study

Neurocase. 2009 Aug;15(4):332-7. doi: 10.1080/13554790902842003. Epub 2009 Apr 2.

Abstract

Two years prior to diagnosis of Kennedy's disease (KD), a 53-year-old man began experiencing neurological symptoms, including nasal speech, postural tremor, tremor in the upper extremities, and muscle weakness. Genetic analysis revealed 46 CAG repeats in the androgen receptor gene. The patient's altered social conduct and complaints of forgetfulness led to a neuropsychological assessment. A mild impairment in visuospatial and visuoconstructive abilities, visual short-term memory, and a personality disorder were detected. Although cognition and behaviour in KD are typically normal, our findings suggest that the disease may cause mild cognitive and behavioural changes as part of the disease's clinical manifestation.

Publication types

  • Case Reports

MeSH terms

  • Activities of Daily Living
  • Bulbo-Spinal Atrophy, X-Linked / complications
  • Bulbo-Spinal Atrophy, X-Linked / physiopathology*
  • Bulbo-Spinal Atrophy, X-Linked / psychology*
  • Central Nervous System / metabolism
  • Central Nervous System / pathology
  • Central Nervous System / physiopathology
  • Cognition Disorders / etiology
  • Cognition Disorders / physiopathology*
  • Cognition Disorders / psychology*
  • Disability Evaluation
  • Disease Progression
  • Humans
  • Male
  • Memory Disorders / diagnosis
  • Memory Disorders / etiology
  • Memory Disorders / psychology
  • Mental Disorders / etiology
  • Mental Disorders / physiopathology*
  • Mental Disorders / psychology*
  • Middle Aged
  • Motor Neurons / metabolism
  • Motor Neurons / pathology
  • Neuropsychological Tests
  • Perceptual Disorders / diagnosis
  • Perceptual Disorders / etiology
  • Perceptual Disorders / psychology
  • Receptors, Androgen / deficiency*
  • Receptors, Androgen / genetics*
  • Speech Disorders / etiology
  • Tremor / etiology

Substances

  • Receptors, Androgen