Review Article
Nosology of Juvenile Muscular Atrophy of Distal Upper Extremity: From Monomelic Amyotrophy to Hirayama Disease—Indian Perspective
Table 1
Clinical profile of Hirayama disease [
4,
5,
18,
24].
| | Clinical feature | Hassan et al. [5] (2012): no. (%) | Sonwalkar et al. [18] (2008): no. (%) | Raval et al. [24] (2010): no. (%) | Gourie-Devi et al. [4] (1984): no. (%) |
| | Total number of patients | 11 | 8 | 9 | 13 | | Hand wasting | 11 (100) | 6 (75) |
*
| 12 (92) | | Forearm wasting | 11 (100) | 4 (50) |
*
| 13 (100) | | Brachioradialis sparing (oblique amyotrophy) | 11 (100) | 3 (37) | 6 (67.5) | 11 (85) | | Proximal (C5,6) myotomal wasting |
- (was exclusion criteria) | 1 (12) | — | 9 (69)** | | Hand weakness | 11 (100) | 8 (100) |
*
| 12 (92) | | Forearm weakness | 8 (73) | 6 (75) |
*
| 13 (100) | | Minipolymyoclonus | 10 (91) | 5 (62) | 5 (55) | 11 (85) | | Brisk deep tendon reflexes | 2 (18) | 1 (12) | — | Nil | | Bilateral involvement | 6 (55) | 6 (75) | 1 (11) |
- (was exclusion criteria) | | Unilateral involvement | 5 (45) | 2 (25) | 8 (89) | 13 (100) | | Cold paresis | 4 (36) | — | 5 (55) | 6 (46) | | Fasciculations | 3 (27) | — | 5 (55) | 6 (46) | | Dysaesthesia on hand | 1 (9) | 1 (12) | 1 (11) | 1 (8) |
|
|
Mentioned as weakness and wasting in upper limb(s): right 4, left 4, and bilateral 1.
**Scapular 3, pectoralis 2, deltoid 5, and biceps 5.
|
