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肾上腺白质营养不良(Adrenoleucodystrophy,简称ALD),又名肾上腺一弥漫性轴周性脑炎,系Blaw于1970年首次报告。本病是X连隐性遗传疾患,主要表现为肾上腺皮质功能不全、脑白质进行性髓鞘脱失及组织中饱和长链脂酸病理性堆积。临床上神经精神症状往往比肾上腺皮质功能低下的症状更为明显。既往常将此病误诊为弥漫性轴周性脑炎,经尸检证实及体内微量脂酸的检测,逐渐认识到临床诊断为弥漫性轴周性脑炎的大部分男性患者实际上患的是ALD,其它无肾上腺萎缩的弥漫性轴周性脑炎(男女均有)可能是患有各种类型的多发性硬化。病因 ALD为脂肪代谢紊乱引起的多系统疾病,但如何引起白质脱髓鞘及肾上腺
Adrenoleucodystrophy (ALD), also known as diffuse adrenal axonal encephalitis, was first reported by Blaw in 1970. The disease is X with occult genetic disorders, mainly manifested as adrenal insufficiency, progressive demyelination of white matter and tissue accumulation of long-chain fatty acids in the pathology. Clinically, neuropsychiatric symptoms are often more pronounced than symptoms of adrenal insufficiency. The disease is often misdiagnosed as diffuse axonal encephalitis confirmed by autopsy and the detection of trace amounts of fatty acids in the body gradually realized that the majority of men with clinically diagnosed as diffuse axonal encephalitis are actually suffering from ALD , Other non-adrenal atrophy diffuse axonal encephalitis (both men and women) may be suffering from various types of multiple sclerosis. Causes ALD is a multi-system disease caused by disorders in fat metabolism, but how to cause white matter demyelination and adrenal