Clinical Characteristics of CBS patients.

CBS patients had marked functional impairment with reduced ALSFRS-R fine motor and gross motor sub-scores, despite normal limb power. CBS patients were at least moderately cognitively impaired, with deficits in multiple cognitive domains including visuospatial function. MRCSS = medical research council sum score, ALSFRS-R = amyotrophic lateral sclerosis functional rating score – revised, ACE-R = Addenbrooke's cognitive examination – Revised, MMSE = mini-mental status examination.