Key feature is combination of upper and lower motor neurone signs.
Ocular movements never involved and never causes cerebellar signs.
Differentials are: cercical spine compression, old polio, spinal muscular atrophy of juvenile onset type 3
Disease patterns: (i) Bulbar: Bulbar/pseudobulbar palsy (ii)
Amyotrophic lateral sclerosis: flaccid arms, spastic legs (iii)
Progressive muscular atrophy: distal muscles (iv) Primary lateral
sclerosis: progresses from UMN to LMN type
Riluzole (glutamate antagonist) has evidence in
patients with bulbar onset with lower risk of death or tracheostomy,
however not clear if this translates to improved quality of life
