Table 4.
Site of involvement | Neurological signs |
---|---|
Peripheral motor neuron involvement limited or diffused to the four limbs |
- Fasciculations - Cramps - Amyotrophy - Predominant motor deficit in the lower and proximal limbs, - Decrease or abolition of osteo-tendinous reflexes |
Progressive peripheral bulbar involvement |
- Atrophy of the tongue and fasciculations that may extend to the lips, chin, or perioral area (often with a myokymic appearance). - Dysarthria, with potentially a discreet nasal voice - Dysphagia |
Sensory involvement | - Most often subclinical and distal |
Absence of evidence of central motor neuron involvement | - Absence of spasticity, clonus, exaggeration and / or diffusion of reflexes, or Babinski’s sign |
Postural tremor of the upper limbs |