Which age groups have the highest prevalence of Kennedy disease (KD)?

Updated: Sep 30, 2019
  • Author: Paul E Barkhaus, MD, FAAN, FAANEM; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

See the list below:

  • The typical age of onset is 40-60 years.

  • The disease may appear as early as the mid-20s.

  • Doyu et al reported an 84-year-old man without a family history who had difficulty climbing stairs and muscle cramps in his calves for 10 years. Clinical, endocrinologic, and electrophysiologic results suggested KD. Genetic polymerase chain reaction (PCR) testing for KD revealed CAG repeats but in the low range of abnormality. Expression of the disease in this man was mild. [48] This case emphasizes the importance of testing individuals, even those without a family history, for possible spontaneous mutations when the results of careful clinical evaluation and laboratory testing are compatible with KD.


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