Paleococcus nigra red nuclear pigment degeneration

Paleococcus nigra red nuclear pigment degeneration, also known as Hallervorden-Spatz disease, is a genetic metabolic disease in late childhood and adolescence. It mainly involves the extrapyramidal system and is also a rare neurodegenerative disease related to iron metabolism disorders. There have been few autopsy reports in China. The disease was first reported by Hallervorden and Spatz (1922), and the disease was later named in two people. The disease is an autosomal recessive. Iron salts are deposited on bilateral pale globules, nigro reticulum, or even red nuclei, leading to neurodegeneration with loss of neurons and glialization. The main clinical manifestations are slowly progressing tonicity, less movement, dystonia, pyramidal tract signs, dementia, and pigmented retinitis in children and adolescents, and optic disc atrophy.

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