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Acute hydrocephalus in nonketotic hyperglycinemia
J L Van Hove1, P S Kishnani, P Demaerel
1Department of Pediatrics, University Hospital Gasthuisberg, Leuven, Belgium.
Neurology
|February 19, 2000
Abstract:
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.