We report on a patient with transient nonketotic hyperglycinemia (NKH)
. Onset, clinical features, biochemical and electroencephalographic fi
ndings were consistent with neonatal nonketotic hyperglycinemia. The n
ormalization of clinical, biochemical and electroencephalographic feat
ures 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 deve
lopment, no EEG abnormalities or neurological deficits.