The Research Of Clinical Feature And Expression Of IL-4 MRNA And IgE In The Patients Of Hirayama Disease

Objective:The clinical data and the expression of IL-4 mRNA in peripheral blood mononuclear cells (PBMC) and plasma IgE in the patients of Hirayama disease were analyzed in order to investigate the correlation between immunologic reaction and Hirayama disease.Methods:The detailed history-taking, neurological examination, concentric needle EMG, nerve conduction study, cervical MRI and/or muscle biopsy were performed in all of the patients of Hirayama disease. The expression of IL-4 mRNA in PBMC from the patients of Hirayama disease was measured by reverse transcriptase polymerase chain reaction (RT - PCR) and the plasma IgE level was measured by enzyme-linked immunosorbent assay (ELISA).Results:We examined 32 consecutive patients of Hirayama disease. The age of onset was 14 to 22 years old (mean (SD) 18.27 (2.60)) and the ratio of male and female was 31 vs 1. Muscular weakness and atrophy were noted. The neurogenic EMG abnormalities showed an anterior horn cell damage. Cervical MRI in a neutral neck position showed lower cervical cord trophy and abnormal cervical curvature. In a fully flexed position of the neck, all of the 3 patients showed forward displacement, flattening of the lower cervical cord and a crescent-shaped high signal area behind the cord. The biopsy of flexor carpi ulnaris showed neurogenic muscular atrophy but the triangular muscle was normal. The expression of IL-4 mRNA and IgE was measured in 23 consecutive patients of Hirayama disease and 20 normal controls. The age of the patients of Hirayama disease was 17 to 29 years old (mean (SD) 19.91 (2.68) ) and the age of controls was 17 to 29 years old (mean (SD) 21.05 (2.01) ). The positive expressional rates of IL-4 mRNA in Hirayama disease was higher (86.96%) than those (60%) in normal controls (P=0.043). The expressional intensity of IL-4 mRNA in Hirayama disease was higher than that in normal controls (P=0.033). The plasma IgE level showed increasing trend but there was no statistic difference between the patients of Hirayama disease and normal controls.Conclusion:1. All the patients were in accord with the diagnosis standards of Hirayama disease.2. The expression of IL-4 mRNA in PBMC from patients of Hirayama disease was up-regulated. Plasma total IgE showed increasing trend. The immunologic abnormality maybe correlate with Hirayama disease.