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 electrophysiological and laboratory findings …