progressive bulbar paralysis (Duchenne syndrome)

Epidemiology

affects patients in their 5th & 6th decades of life

Pathology

• atrophy & gliosis of the motor nuclei of the 5th, 7th, 9th, 10th & 12th cranial nerves
• subcortical involvement of the corticobulbar tracts
• death with 1-3 years
  • respiratory arrest
  • aspiration pneumonia

Clinical manifestations

• drooling
• difficulty chewing
• dysphagia
• dysarthria
• nasal regurgitation
• spasticity of arms & legs
• hyperactive deep tendon reflexes (DTR)
• ophthalmoplegia
• fasciculation of muscles in tongue & lips
• emotional lability

More general terms

• brainstem disease
• spinal cord disease
• syndrome