Table 1

Clinical and immunological findings of patients with Hirayama disease

Patient No
Age of onset (y)1213151617
Age at examination (y)1315221719
Onset of neurological illnessInsidiousInsidiousInsidiousInsidiousInsidious
Muscle atrophy of distal upper limbR>LRR<LRR>L
Coexistent atopic/allergic disorders:
 Bronchial asthma+1-150 (+)1-151
 Allergic rhinitis+++
 Atopic dermatitis
Family history of atopic/allergic disordersBrother: ADFather: polFather: ARFather: ARBrother: AD
Sister: ADMother: polBrother: ARSister: ARSister: AD
Uncle: AR
Peripheral blood eosinophils (%)1-152
Serum total IgE (U/ml)187412252042400150
Allergen specific IgE (IU/ml):
D pteronyssinus 85.96>1009.19>1001.52
D farinae 90.66>1007.75>1002.63
 Cedar pollen1.5354.680.52
 Soya bean0.38NE
  • 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.