Clinical and immunological findings of patients with Hirayama disease
| Patient No | |||||
|---|---|---|---|---|---|
| 1 | 2 | 3 | 4 | 5 | |
| Age of onset (y) | 12 | 13 | 15 | 16 | 17 |
| Age at examination (y) | 13 | 15 | 22 | 17 | 19 |
| Sex | F | F | M | M | M |
| Onset of neurological illness | Insidious | Insidious | Insidious | Insidious | Insidious |
| Muscle atrophy of distal upper limb | R>L | R | R<L | R | R>L |
| Coexistent atopic/allergic disorders: | |||||
| Bronchial asthma | +1-150 | — | — | (+)1-151 | — |
| Allergic rhinitis | — | + | + | + | — |
| Pollinosis | — | — | + | — | — |
| Atopic dermatitis | — | — | — | — | — |
| others | + | — | — | — | — |
| Family history of atopic/allergic disorders | Brother: AD | Father: pol | Father: AR | Father: AR | Brother: AD |
| Sister: AD | Mother: pol | Brother: AR | Sister: AR | Sister: AD | |
| Uncle: AR | |||||
| Peripheral blood eosinophils (%)1-152 | 7.8 | 7.6 | 2.7 | 4.5 | 6.7 |
| Serum total IgE (U/ml) | 1874 | 1225 | 204 | 2400 | 150 |
| Allergen specific IgE (IU/ml): | |||||
| D pteronyssinus | 85.96 | >100 | 9.19 | >100 | 1.52 |
| D farinae | 90.66 | >100 | 7.75 | >100 | 2.63 |
| Cedar pollen | — | 1.53 | 54.68 | 0.52 | — |
| Soya bean | — | — | 0.38 | NE | — |
| Others | — | — | — | NE | — |
UL=Upper limb; AD=atopic dermatitis; AR=allergic rhinitis; pol=pollinosis; NE=not examined;D=Dermatophagoides.
↵1-150 Although patient 1 had asthma when she developed amyotrophy, the neurological illness occurred insidiously and showed no relation to asthma attacks.
↵1-151 History of atopic/allergic disorders, which were not present at the time of neurological illness, is shown in parentheses.
↵1-152 Eosinophil percentage higher than 4% is considered abnormal.