Table 2. Overview on patient population.
Patient number | Onset | CI | PPD (months) | Clinical features (weakness and atrophy) | Absent TR | Tremors | ||||
---|---|---|---|---|---|---|---|---|---|---|
Age (years) | Side | SS | Hands intrinsics MM | Fingers extensors MM | Biceps/triceps brachii MM | |||||
1 | 19 | Bilateral | Tremor and hand weakness | Yes (L > R) | 12 | No | Yes (L>R) | No | Distal reflexes at upper limbs | Yes (bilateral) |
2 | 15 | Bilateral | Tremor and hand weakness | Yes (R > L) | 36 | Yes (R > L) | Yes (R > L) | No | Distal reflexes at upper limbs | Yes (R hand) |
3 | 17 | R | Progressive hand weakness | Yes | 36 | Yes (R > L) | Yes (R > L) | Yes (tricipital) | Distal reflexes at upper limbs and R tricipital | No |
4 | 18 | R | Hyperhidrosis and hand weakness | Yes | 12 | No | Yes (R > L) | No | Distal reflexes at upper limbs | No |
5 | 17 | R | Progressive hand weakness | Yes | 12 | Yes | Yes | No | Distal reflexes at upper limbs | No |
6 | 23 | L | Tremor and hand weakness | Yes | 36 | Yes | Yes | No | Distal reflexes at upper limbs | Yes (L > R) |
7 | 16 | L | Hyperhidrosis and hand weakness | Yes | 24 | Yes | Yes | No | Distal reflexes at upper limbs | No |
8 | 18 | L | Progressive weakness | Yes | 8 | Yes | Yes | No | Distal reflexes at upper limbs | No |
SS, signs and symptoms; CI, contralateral involvement; PPD, progression phase duration; MM, muscles; TR, tendon reflexes, L, left; R, right.