tokens: We describe a 4 year old girl with classical PDE who demonstrated significant improvements in clinical , neurological and developmental outcomes including absence of clinical seizures and cessation of antiepileptic medications since age 3 months , normalisation of EEG , significant improvement in the white matter signal throughout the cerebrum on neuroimaging and significant reduction in urine P6C and pipecolic acid levels post- combined therapy with lysine restricted diet in conjunction with pyridoxine and folinic acid .