X-linked bulbospinal neuronopathy: Kennedy disease.

  title={X-linked bulbospinal neuronopathy: Kennedy disease.},
  author={Anne D. Sperfeld and J. Karitzky and Dagmar Brummer and Herbert Schreiber and Juergen Haeussler and Albert Christian Ludolph and Clemens Oliver Hanemann},
  journal={Archives of neurology},
  volume={59 12},
OBJECTIVE To characterize the earliest symptoms of X-linked bulbospinal neuronopathy (Kennedy disease [KD]) during the course of the disease, including a definition of the age of onset. METHODS We describe the earliest symptoms, signs on clinical investigation, electrophysiological and muscle biopsy specimen findings, and creatine kinase levels in 34 patients with KD. Correlations were made among the CAG-repeat length and clinical symptoms, age at onset, and the presence of… CONTINUE READING