Table I.
Patient characteristics.
| V30M | Non-V30M | |
|---|---|---|
| Sex male/female, n | 34/14 | 2/5 |
| Type of amyloid fibril | ||
| Type A fibrils, n | 25 | 6 |
| Type B fibrils, n | 21 | 0 |
| Not typed, n | 2 | 1 |
| Age at onset median (range), years | 63 (26–82) | 60 (38–69) |
| Age at examination median (range), years | 68 (30–83) | 65 (41–72) |
| Previous liver transplant, n | 12 | 1 |
| PND score, na | ||
| 0 | 10 | 3 |
| I | 20 | 0 |
| II | 10 | 0 |
| IIIA | 2 | 0 |
| IIIB | 6 | 3 |
| IV | 0 | 0 |
| Not applicableb | 0 | 1 |
| Primary symptoms, n | ||
| Cardiac | 4 | 4 |
| Neuropathy | 40 | 1 |
| Other | 4 | 2 |
| Total | 48 | 7 |
Type A amyloid fibrils are composed of full-length and truncated transthyretin, and type B of full-length transthyretin only.
a
Polyneuropathy disability (PND) score: 0 = no sensory disturbances; I = sensory disturbances in the feet, but no impaired walking capability; II = walking impairment, but walking without aid; IIIA = walking with one stick or crutch; IIIB = walking with two sticks or crutches; IV = in wheelchair or immobilized.
b
Patient with post-polio syndrome that causes neurological impairment.