AXONAL DYSTROPHY IN THE GRACILE NUCLEUS IN CHILDREN AND YOUNG-ADULTS - REAPPRAISAL OF THE INCIDENCE AND ASSOCIATED DISEASES

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  • 1 January 1981
    • journal article
    • research article
    • Vol. 40  (1) , 37-45
Abstract
In 656 patients aged from 1-39 yr, the incidence of axonal dystrophy in the gracile nucleus (ADG) is correlated with underlying diseases. The age-related incidence of ADG (minimal to severe) in these patients, 13, 53, 76 and 97% in the 1st, 2nd, 3rd and 4th decades, respectively, is comparable to that observed by other investigators. The incidence drops to 8, 16, 31 and 60% when > 5 spheroids in each gracile nucleus are taken into account. Diseases with which ADG is frequently associated vary according to the patient''s age. Congenital biliary atresia and cystic fibrosis account for 71% of cases of ADG (mild to severe) in the 1st decade, while cystic fibrosis and malignancies account for 38 and 35% in the 2nd decade, respectively. In the 3rd decade, malignancies, renal diseases, cystic fibrosis and heart diseases account for 30, 14, 12 and 12% of ADG cases, respectively, while malignancies, renal diseases, heart diseases and diseases of digestive organs account for 30, 24, 15 and 11% in the 4th decade, respectively. The incidence of ADG also increases progressively with age, duration of the clinical course in the individual disease or both, regardless of types of the diseases. This fact, when viewed together with the precocious development of severe ADG in patients with the malabsorption syndromes and in those with grave illnesses with protracted course leading to cachexia, seems to indicate that malnutrition represents a common factor correlated with severe ADG in young patients. ADG in man may be in some way related to deficiency or altered metabolism of vitamin E.