Axonal dystrophy in the gracile nucleus in children and young adults. Reappraisal of the incidence and associated diseases.

In 656 patients aged from one to 39 years, 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 first, second, third, and fourth decades, respectively, is comparable to that observed by other investigators. The incidence drops to 8, 16, 31, and 60% when more than five 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 first decade, while cystic fibrosis and malignancies account for 38 and 35% in the second decade, respectively. In the third 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 fourth 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 the young patients. It seems to be premature at this time to disregard the hypothetical question that ADG in man is in some way related to deficiency or altered metabolism of vitamin E.