Infantile black dementia

Infantile black dementia is an autosomal recessive inheritance. Classical black dementia is usually normal at birth, with symptoms occurring 3 to 6 months after birth. Due to the lack of hexosaminidase or sphingolipid activator protein, GM2 gangliosides and associated sphingosyllipids are deposited, causing the brain to degenerate. Infantile black dementia is also known as Bielschowsky syndrome, Bielschowsky-Jansky syndrome, Bernheimer-Seitelberger syndrome, late-onset infant amaurotic familial infantile idiocy, late infantile waxy lipid brown GM2 gangliosidosis, GM2 gangliosidosis, juvenile GM2 gangliosidosis, etc.