progressive bulbar paralysis (Duchenne syndrome)

Pathology: 1) atrophy & gliosis of the motor nuclei of the 5th, 7th, 9th, 10th & 12th cranial nerves 2) subcortical involvement of the corticobulbar tracts 3) death with 1-3 years a) respiratory arrest b) aspiration pneumonia Epidemiology: affects patients in their 5th & 6th decades of life Clinical manifestations: 1) drooling 2) difficulty chewing 3) dysphagia 4) dysarthria 5) nasal regurgitation 6) spasticity of arms & legs 7) hyperactive deep tendon reflexes (DTR) 8) ophthalmoplegia 9) fasciculation of muscles in tongue & lips 10) emotional lability

General

brainstem disease spinal cord disease; myelopathy (disease/disorder primarily affecting spinal cord) syndrome

References

DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 871