Clinical features of motor neurone disease

Presentation of motor neurone disease depends on the particular pathways involved:

• combined upper and lower motor neurone features are present in 65% of cases
• bulbar features are present in 25%
• muscle weakness and atrophy are present in 10%

Features usually develop gradually. The initial complaint is often non-specific, e.g. a weakening hand grip.

Characteristic complaints:

• hand - wasting, weakness, fasciculation
• bulbar involvement - dysarthria and dysphagia
• lower limb - weakness with spasticity
• difficulty in breathing

Characteristic negative features:

• no sensory loss or cognitive loss c.f. MS
• sphincters are never affected } c.f. myasthenia
• ocular muscles are never affected } gravis

Reference

1. McDermott CJ, Shaw PJ. Diagnosis and management of motor neurone disease. BMJ. 2008 Mar 22;336(7645):658-62