Isolated bulbar phenotype of amyotrophic lateral sclerosis.

  title={Isolated bulbar phenotype of amyotrophic lateral sclerosis.},
  author={J R Burrell and Steve Vucic and Matthew C Kiernan},
  journal={Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases},
  volume={12 4},
Typical bulbar-onset ALS generally portends a poor prognosis. To determine whether a relatively isolated bulbar phenotype (IBP) may have a better prognosis, patients with bulbar onset presentations were prospectively assessed, with IBP defined by an absence of limb progression over an initial six-month period. Clinical features and neurophysiological characteristics were compared. From a cohort of 300 consecutive referrals, 32 patients with bulbar onset disease (21 females, 11 males) were… CONTINUE READING