Clinical features | Differential diagnosis |
---|---|
Isolated damage to the peripheral motor neuron | - Other causes of spinal amyotrophy (particularly related to SMN1 mutation), - Spinal motor forms of Charcot-Marie-Tooth neuropathy - Progressive muscular atrophy - Post-polio syndrome |
Cramps and fasciculations | -"Benign” cramps-fasciculations syndrome - Isaacs syndrome |
Progressive bulbar involvement: | - Myasthenia gravis - Congenital myopathies - Oculopharyngeal muscular dystrophy; |
Proximal motor deficit associated with a rise in CPK | - Progressive muscular dystrophies - Congenital myopathies - Muscular canalopathies - Inflammatory or metabolic myopathies; |
Amyotrophic motor deficit associated with sensory impairment | Charcot-Marie-Tooth neuropathy |