Table 1

Signs with a high positive predictive value* for motor neurone disease where there is a history of progressive motor-only weakness

FasciculationsIf generalised (easily missed over the anterior shoulders)
Bilateral wasting of the tongueLateral borders, particularly where there are also fasciculations (best observed without protrusion) and a brisk jaw jerk or orbicularis oris reflexes
The ‘split hand’73Preferential wasting of the lateral border of the hand, that is, first dorsal interosseous and abductor pollicis brevis. This is thought possibly to reflect cortical organisation
Head dropWeakness of neck extensors. Myasthenia gravis is a consideration but this sign should not be attributed to cervical spondylosis
EmotionalityExaggerated response to emotional stimuli, usually crying, typically with bulbar weakness and often with an abnormal response to glabellar tap
Cognitive or behavioural impairmentFrontotemporal dementia overlap features
  • *Not formally validated.