Acute hydrocephalus in nonketotic hyperglycinemia.

Journal Article (Journal Article)

We present four patients with typical neonatal onset non-ketotic hyperglycinemia (NKH) who developed hydrocephalus requiring shunting in early infancy. Brain imaging revealed acute hydrocephalus, a megacisterna magna or posterior fossa cyst, pronounced atrophy of the white matter, and an extremely thin corpus callosum in all. The three older patients had profound developmental disabilities. This suggests that the development of hydrocephalus in NKH is an additional poor prognostic sign.

Full Text

Duke Authors

Cited Authors

  • Van Hove, JL; Kishnani, PS; Demaerel, P; Kahler, SG; Miller, C; Jaeken, J; Rutledge, SL

Published Date

  • February 8, 2000

Published In

Volume / Issue

  • 54 / 3

Start / End Page

  • 754 - 756

PubMed ID

  • 10680820

International Standard Serial Number (ISSN)

  • 0028-3878

Digital Object Identifier (DOI)

  • 10.1212/wnl.54.3.754


  • eng

Conference Location

  • United States