MD is a 75-year-old right-handed male who presented to a neurology clinic in a large tertiary hospital with a ten-year history of imbalance. His speech had become slurred over the last five years and swallow initiation had deteriorated. He developed severe bilateral sensorineural hearing loss. His background was significant for lumbar spinal surgery in the UK in the 1980s.
An examination was demonstrative of diffuse limb fasciculations in the upper and lower extremities as well as the paravertebral muscles. He had an intention tremor in the upper limbs and walked with a broad based gait. He displayed a moderate cerebellar dysarthria and had hearing aids bilaterally. Power was intact in the upper and lower limbs with brisk reflexes.