Supranuclear gaze palsy in familial Creutzfeldt-Jakob disease.

  title={Supranuclear gaze palsy in familial Creutzfeldt-Jakob disease.},
  author={John M. Bertoni and L S Label and J C Sackelleres and Sonja Hicks},
  journal={Archives of neurology},
  volume={40 10},
Seven affected individuals from three generations in a kindred having over 250 members were identified as having possible, probable, or definite Creutzfeldt-Jakob disease. Spongiform encephalopathy was found at postmortem examination in two cases. Detailed inpatient neurological examinations were performed on four of the subjects, three of whom were first observed with supranuclear gaze paralysis, gait ataxia, and rapidly progressive dementia. Supranuclear gaze paresis can be seen as an early… CONTINUE READING