Table.
Summary of History, Neurological Findings and Neurophysiological Findings of the Patients in Generation III.
| Patients | Age (years) | Duration (years) | |||
|---|---|---|---|---|---|
| At examination | At onset | Onset to cane walk | Onset to wheel-chair | ||
| III-2 | 75 | 57 | 0 | 14 | |
| III-3 | 72 | 52 | 6 | 19 | |
| III-4 | 68 | 53 | NE | NE | |
| III-5 | 64 | 50 | 2 | - | |
| III-6 | 66 | 52 | 4 | 8 | |
| Clinical neurological findings | ||||
|---|---|---|---|---|
| Dysarthria | Tongue atrophy and fasciculation | Dementia | Muscular weakness | |
| + | - | + | B L/E | |
| + | - | - | B L/E | |
| + | - | + | B L/E | |
| + | + | - | B L/E | |
| + | - | - | B L/E | |
| Muscular atrophy | Hyporeflexia | Babinski sign | Fasciculation (except for tongue) |
|
|---|---|---|---|---|
| B L/E | NE | NE | - | |
| B L/E | B U/E L/E | - | - | |
| B L/E | B U/E L/E | L+ | - | |
| B L/E | B U/E L/E | B+ | - | |
| B L/E | B U/E L/E | R+ | - | |
| Neurophysiological examination | |||||
|---|---|---|---|---|---|
| Needle EMG | MEP | ||||
| NE | NE | ||||
| C | NE | ||||
| NE | NE | ||||
| NE | NE | ||||
| C | P | ||||
EMG: electromyography, MEP: motor evoked potential, B: bilateral, R: right, L: left, L/E: lower extremities, U/E: upper extremities, NE: not examined or not identified from clinical history, C: Chronic denervation pattern (High amplitude motor unit potential was observed.), P: prolonged MEP latency and CMCT