Motor neurone disease
| Incidental findings | |
| +ve signs | Fasiculations, LMN signs upper limbs, spasticity lower limbs, brisk jaw jerk |
| Differential diagnosis | MND, cervical spine compression |
| Function | ?Wheelchair/tracheostomy |
| Tests | MR spine – exclude cervical cord compression |
Notes
- 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