Neurology: Case of the Month

Test Yourself — Patient 13

Kennedy's Disease

1. All of the following would be in accord with the diagnosis of Kennedy's disease EXCEPT:
   • [ A ] Gynecomastia
   • [ B ] Proximal and distal weakness
   • [ C ] Testicular atrophy
   • [ D ] Normal to increased reflexes
2. The following statement is TRUE regarding Kennedy's disease:
   • [ A ] Onset of the disease occurs in older age groups (60 to 69 years)
   • [ B ] The incidence is reported as 20 per 100,000
   • [ C ] Has an autosomal recessive pattern of inheritance
   • [ D ] The muscle biopsy typically shows chronic neurogenic atrophy with reinnervation
3. The following is TRUE in Kennedy's disease:
   • [ A ] The degree of weakness correlates with the dysfunction of the androgen receptor
   • [ B ] The CAG repeats are translated into polyglutamine
   • [ C ] Deletion of the AR gene simulates Kennedy's disease
   • [ D ] The abnormality in CAG repeats is unique for Kennedy's disease
4. All of the following chromosomal locations are correct EXCEPT:
   • [ A ] Dentato-rubro-pallido-Luysian atrophy (12p)
   • [ B ] Huntington's disease (4p16.3)
   • [ C ] Kennedy's disease (12q)
   • [ D ] Spinocerebellar atrophy type 1 (6p22-23)
5. All of the following are correct regarding Kennedy's disease EXCEPT:
   • [ A ] Up to 35 CAG repeats can be found in normal individuals
   • [ B ] The larger the number of CAG repeats, the later the onset of disease
   • [ C ] The polyglutamine is part of the androgen receptor protein
   • [ D ] The mechanism by which polyglutamine leads to motor neuron injury is unknown

 

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