Kennedy's disease: A lower motor neuron model to identify upper motor neuron physiology in ALS

摘要

William Kennedy's original clinical descriptions of the disorder named after him, now more commonly referred to as spinobulbar muscular atrophy, has stood the test of time (Kennedy et al., 1968). The prominent neurological deficits are readily explained on the basis of dysfunction and loss of the anterior horn cells and dorsal root ganglion cells. To date there have been no descriptions of clinical upper motor neuron findings in Kennedy's disease. This makes Kennedy's disease a unique model to compare and help elucidate upper versus lower motor neuron failure in amyotrophic lateral sclerosis (ALS)