Table 3.

Differential diagnosis of KD depending on the presentation and predominant signs and symptoms

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