又名弥散性进行性脑灰质变性,婴儿进行性脑灰质营养不良,Chrinstensen-Krabbe综合征。多种致病因素(缺氧、癫痫后、创伤后遗、家族性因素等)引起大脑灰质中层的退行性改变,也见于基底节和小脑皮质。临床呈现惊厥、肌阵挛、痉挛、舞蹈-手足徐动、痴呆、小脑性共济失调。预后很差。 Also known as disseminated progressive gray matter degeneration, progressive dyschinococcal infant, Chrinstensen-Krabbe syndrome. A variety of pathogenic factors (hypoxia, epilepsy, trauma, familial factors, etc.) cause degenerative changes in the middle cerebral gray matter, also found in the basal ganglia and cerebellar cortex. Clinical seizures, myoclonus, spasms, dance - hand, foot and Xu, dementia, cerebellar ataxia. The prognosis is poor.