Alper's disease
Alper's disease is a progressive degenerative disease of the central nervous system seen in children. The other system affected is the liver.
Features develop after the first year of life; sometimes as late as the fifth year, and include:
- seizures, which may be myoclonic or Salaam type
- progressive dementia
- spastic quadraplegia
- deafness
Pathologically there is status spongiosus of the cerebral grey matter.
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