儿童和青年成人薄束核中的轴索性营养不良。对发病率及相关疾病的重新评估。

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

作者信息

Sung J H, Mastri A R, Park S H

出版信息

J Neuropathol Exp Neurol. 1981 Jan;40(1):37-45.

PMID:7205326

Abstract

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.

摘要

在656例年龄从1岁至39岁的患者中，薄束核轴索性营养不良（ADG）的发生率与基础疾病相关。这些患者中ADG（从轻度到重度）的年龄相关发生率在第一个十年、第二个十年、第三个十年和第四个十年分别为13%、53%、76%和97%，与其他研究者观察到的情况相当。当考虑每个薄束核中有超过五个轴突球时，发生率降至8%、16%、31%和60%。与ADG经常相关的疾病因患者年龄而异。先天性胆道闭锁和囊性纤维化在第一个十年的ADG（轻度至重度）病例中占71%，而囊性纤维化和恶性肿瘤在第二个十年分别占38%和35%。在第三个十年，恶性肿瘤、肾脏疾病、囊性纤维化和心脏病在ADG病例中分别占30%、14%、12%和12%，而在第四个十年，恶性肿瘤、肾脏疾病、心脏病和消化器官疾病分别占30%、24%、15%和11%。无论疾病类型如何，ADG的发生率也随着年龄、个体疾病临床病程的持续时间或两者同时增加而逐渐升高。这一事实，再结合吸收不良综合征患者以及病程迁延导致恶病质的重症患者中严重ADG的早熟发展来看，似乎表明营养不良是年轻患者中与严重ADG相关的一个共同因素。此时忽视人类ADG在某种程度上与维生素E缺乏或代谢改变相关这一假设问题似乎还为时过早。