Cerebral involvement in spinal and bulbar muscular atrophy (Kennedy's disease): a pilot study of PET.

OBJECTIVE

To investigate possible cerebral involvement in patients with spinal and bulbar muscular atrophy (SBMA) by (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET).

DESIGN

Ten patients with molecularly-confirmed SBMA and 5 age- and gender-matched healthy controls were recruited for brain FDG-PET studies. The data were analyzed and compared using the statistical parametric mapping (SPM) method.

RESULTS

Glucose hypometabolism in frontal areas of the cerebrum was found in patients with SBMA. However, no significant correlation with clinical variables, such as CAG repeat length, age at onset, or serum testosterone levels, was noted.

CONCLUSIONS

The perturbation of cerebral glucose metabolism in patients with SBMA argues against SBMA being a pure lower motor and sensory neuron syndrome. Mutations in the androgen receptor gene might have a more widespread effect in the cerebrum than previously recognized.