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Case Reports
. 2000 Feb 8;54(3):754-6.
doi: 10.1212/wnl.54.3.754.

Acute hydrocephalus in nonketotic hyperglycinemia

Affiliations
Case Reports

Acute hydrocephalus in nonketotic hyperglycinemia

J L Van Hove et al. Neurology. .

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.

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