We report on a patient with transient nonketotic hyperglycinemia (NKH). Onset, clinical
features, biochemical and electroencephalographic findings were consistent with neonatal
nonketotic hyperglycinemia. The normalization of clinical, biochemical and electroencephalographic
features on day 9 without specific therapy suggested a transient form. At the last
follow-up the girl was 13 years old, had a normal mental development, no EEG abnormalities
or neurological deficits.
Nonketotic hyperglycinemia - Glycine cleavage system
