Table 2. Clinical Characteristics of CBS patients.
| CBS | |
| Number of Patients | 17 |
| Male | 7 (41.2%) |
| Age | 64.4+/−6.6 |
| Symptom Duration (months) | 54.6+/−18.0 |
| Hyperreflexia (% patients) | 12 (70.6%) |
| Weakness (% patients) | 1 (6.3%) |
| MRCSS Total (0–60) | 59.8+/−1.0 |
| Apraxia | |
| - Oro-buccal Apraxia (0–3) | 0.7+/−0.9 |
| - Limb-Meaningful (0–6) | 3.6+/−1.8 |
| - Limb-Meaningless (0–6) | 3.9+/−1.9 |
| - Overall Apraxia Score (0–15) | 7.8+/−3.9 |
| ALSFRS-R | |
| - Bulbar | 10.7+/−1.4 |
| - Fine Motor | 5.7+/−3.4 |
| - Gross Motor | 8.5+/−2.9 |
| - Respiratory | 11.8+/−0.5 |
| - Total | 36.7+/−6.2 |
| ACE-R | |
| - Attention (18 points) | 13.1+/−5.7 |
| - Memory (26 points) | 15.5+/−8.7 |
| - Fluency (14 points) | 5.4+/−4.3 |
| - Language (26 points) | 17.2+/−7.7 |
| - Visuospatial (16 points) | 9.2+/−5.1 |
| - Total (100 points) | 60.1+/−28.7 |
| MMSE | 19.7+/−9.3 |
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.