The patient was a thin, ill-appearing man in no acute distress. Forced vital capacity was 60% of predicted. He was awake, alert, and anarthric and could appropriately respond to yes/no questions and follow commands. He was able to pick out the name of objects from a written list. His writing was illegible. He blinked to visual threat. Pupils were 3 to 4 mm in diameter and reactive to light and near vision. He had a prominent stare with eyelid retraction and a surprised look. He blinked infrequently. Horizontal and vertical pursuit movements were interrupted and slow. Horizontal and vertical saccades were slow and hypometric. Downgaze was more affected than upgaze. He was unable to suppress head turning with attempted lateral gaze. The vertical and horizontal oculocephalic reflex was normal. Square wave jerks on fixation were noted. His mouth was held open; there was weakness of the masseter and pterygoid muscles, with masseter atrophy. The face was masked with upper and lower facial diparesis and prominent facial reflexes. He was unable to voluntarily close his eyes on command. Mental fasciculations were present. The palate elevated poorly but was midline. The sternocleidomastoid muscles and tongue were weak, atrophic, and fasciculating. Rapid tongue movements were slowed. Power was Medical Research Council (MRC) grade 5 in the deltoid, bicep, and tricep muscles but MRC grade 4 in the right and left finger extensor and hand intrinsic muscles. There was atrophy of the hand intrinsic muscles. Leg power was MRC grade 5, he was able to rise from a chair without using his hands, and heel and toe walking was normal. Large-amplitude fasciculations were noted throughout the arms, hands, chest, thighs, calves, forearms, and abdominal wall. Tone was more rigid in the legs than the arms, without tremor or cogwheeling. Rapid movements were slowed in the hands and feet with reduced amplitude. Bradykinesia was present. Walking was slow with a narrow base, small steps, and absent arm swing. Turning was poor, with multiple steps. Retropulsion was prominent. There was no limb apraxia or alien hand. Sensory and cerebellar examinations yielded normal findings. There was jaw clonus. Reflexes were 3 of 4 in the arms and legs, greater on the left side than the right side, with bilateral Hoffman sign and crossed adduction. Cutaneous abdominal reflexes were absent. Toes were extensor (the so-called Babinski sign) bilaterally. Snout, glabellar, and palmomental reflexes were present.