Szobor A, Máttyus A, Molnár J
Jahn Ferenc University Teaching Hospital, Dept. of Neurology, Budapest.
Acta Paediatr Hung. 1988;29(3-4):299-312.
Authors review the different theoretical and practical problems of childhood and adolescent myastenia gravis, including the heterogeneous group of congenital myasthenia and the big casuistics of the literature. There are reports on 113 cases with childhood myasthenia gravis and 96 cases of adolescent myasthenia. Ratio of these forms ranged 10.76% in childhood myasthenia and 9.14% in adolescent age, resp., 19.9% of the whole patient material. A classification is given concerning juvenile myasthenia: 1. Neonatal (transitory) myasthenia. 2. Congenital (local, non-progressive) form. 3. Congenital form with late generalized symptoms. 4. Myasthenia simulating brain-stem process. 5. Generalized childhood myasthenia. 6. Adolescent type myasthenia (juvenile form). 7. Associated myasthenia, myasthenic syndrome or reaction. A new form of the disease is described in which the congenital myasthenia changes into generalized form in the later course of the disease. Report is given on thymectomies in childhood and adolescence which produce in general excellent results. Thymic pathology and activity are dealt with.
作者回顾了儿童和青少年重症肌无力的不同理论和实际问题,包括先天性肌无力这一异质性群体以及大量的文献病例。文中报道了113例儿童重症肌无力病例和96例青少年重症肌无力病例。这些类型在儿童重症肌无力中的比例为10.76%,在青少年中的比例为9.14%,分别占全部患者资料的19.9%。文中给出了青少年重症肌无力的分类:1. 新生儿(暂时性)重症肌无力。2. 先天性(局部、非进行性)形式。3. 伴有晚期全身性症状的先天性形式。4. 模拟脑干病变的重症肌无力。5. 全身性儿童重症肌无力。6. 青少年型重症肌无力(青少年形式)。7. 相关的重症肌无力、肌无力综合征或反应。文中描述了一种新的疾病形式,即先天性肌无力在疾病后期转变为全身性形式。报告了儿童和青少年胸腺切除术的情况,总体效果良好。文中还探讨了胸腺病理学及活性。