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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 2005 Jan;76(1):132–134. doi: 10.1136/jnnp.2003.031609

HyperIgEaemia in patients with juvenile muscular atrophy of the distal upper extremity (Hirayama disease)

S Ito 1, S Kuwabara 1, T Fukutake 1, Y Tokumaru 1, T Hattori 1
PMCID: PMC1739290  PMID: 15608015

Abstract

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.

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