HyperIgEaemia in patients with juvenile muscular atrophy of the distal upper extremity (Hirayama disease).
BACKGROUND
Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is characterised by anterior horn cell loss in the lower cervical cord, presumably caused by anterior displacement of the dural sac during neck flexion. A recent report suggests that atopy and IgE may contribute to anterior horn damage.


OBJECTIVE
To investigate whether IgE is a contributing factor in Hirayama disease.


METHODS
Serum total IgE and allergen specific IgE were examined in 20 consecutive patients, and their correlations with clinical profiles investigated.


RESULTS
Past or present history of allergy/atopy was found in only four patients (20%), but serum IgE was raised in 14 (70%). Patients with hyperIgEaemia had more severe clinical disabilities than those without (p = 0.01). In patients whose history of Hirayama disease was less than five years, serum total IgE was higher than in those with the disease for five years or more (p = 0.05).


CONCLUSIONS
The results suggest that hyperIgEaemia is often associated with Hirayama disease and can facilitate its pathophysiology, particularly in the early phases of the disease. HyperIgEaemia does not appear to involve the anterior horn cells primarily.
Ito
S
S
Department of Neurology (D3), Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan. sito@faculty.chiba-u.jp
Kuwabara
S
S
Fukutake
T
T
Tokumaru
Y
Y
Hattori
T
T
D000293:Adolescent; D000328:Adult; D000870:Anterior Horn Cells / Q000503:physiopathology; D001132:Arm / Q000503:physiopathology; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007073:Immunoglobulin E / Q000097:blood*; D008297:Male; D014897:Spinal Muscular Atrophies of Childhood / Q000097:blood / Q000209:etiology* / Q000503:physiopathology; D014459:Ulnar Nerve / Q000503:physiopathology
D007073:Immunoglobulin E