Pes cavus (PC) and hammer toes (HT) were present (+) in all patients with distal hereditary motor neuronopathy with pyramidal features. Tone was increased (↑) in the lower limbs in two patients, while it was normal (N) in the remaining four patients. Muscle weakness was predominantly evident in the ankle and foot, and graded according to the Medical Research Council Score (MRC). Deep tendon reflexes (DTR) were increased (3+) in most of the patients and absent (−) at the ankles in one patient. Plantar reflexes were extensor (E) in three patients and flexor (F) in three. Cortical excitability was assessed in 52 amyotrophic lateral sclerosis (ALS) patients, all of which exhibited a combination of upper and lower motor neuron signs. The ALS patients were clinically graded using the amyotrophic lateral sclerosis functional rating scale—revised (ALSFRS-R), with a maximum score of 48 when there was no disability. Hand function was assessed using the Triggs hand function score graded as follows: 0 = normal; 1 = mild to moderate hand weakness without impairment of dexterity; 2 = weak with significant impairment of dexterity (ie, difficulty with handwriting and buttoning clothes); and 3 = marked weakness—major disability and loss of fine motor control. Muscle strength was clinically assessed using the MRC for abductor pollicis brevis, as this muscle was utilised for excitability testing. In 30 ALS patients, the site of disease onset was in the upper limb (UL) and in 12 the lower limb (LL); in 10 patients, the disease was bulbar (B) in onset.