Wang P.-J.WANG-TSO LEEWUH-LIANG HWUYoung C.Yau K.-I.T.Shen Y.-Z.2020-12-162020-12-1619980387-7604https://www.scopus.com/inward/record.uri?eid=2-s2.0-0031728575&doi=10.1016%2fS0387-7604%2898%2900042-4&partnerID=40&md5=c8cecdd2ba54d66eef1a229b997e0460https://scholars.lib.ntu.edu.tw/handle/123456789/526095To re-evaluate the diagnostic criteria for early myoclonic encephalopathy (EME), the following study was done. During the past 2 years, five patients with erratic, fragmentary myoclonus of neonatal onset, in association with other types of seizures, were analyzed with regard to etiologies, electroclinical features and their evolution, using a series of examinations including electroencephalographies (EEGs) and metabolic investigations. Of these five patients, three were diagnosed to have non-ketotic hyperglycinemia (NKH); one was pyridoxine-dependent; the other was cryptogenic. Only two cases (one NKH and one cryptogenic) had initial typical suppression-burst (S-B) EEG pattern, which subsequently evolved into multiple paroxysmal abnormalities with random asynchronous attenuation (MP-AA) pattern. The other two cases with NKH had MP-AA EEG pattern throughout both awake and sleep recordings in two consecutive EEG studies. All three cases with NKH survived with increasing microcephaly, muscle tonicity; all developed infantile spasm with hypsarrhythmia on EEGs. The patient with pyridoxine-dependency had an initial MP-AA EEG pattern, which converted into S-B pattern after the first use of pyridoxine, eventually becoming normal after a supplement with the second-dose of pyridoxine. In conclusion, either S-B or MP-AA pattern may reflect the severity of the underlying pathologies or the disease stages. These results suggest that, from both etiological and electroclinical viewpoints, EME may represent a broader spectrum than previously recognized. The still ongoing controversy regarding whether the S-B pattern should be recognized as the sole EEG criteria for the diagnosis of EME needs further experience to clarify. Copyright (C) 1998 Elsevier Science B.V.[SDGs]SDG3article; brain disease; clinical article; clinical trial; diagnostic error; electroencephalogram; female; human; hyperglycinemia; infant; male; myoclonus; Age of Onset; Electroencephalography; Epilepsies, Myoclonic; Female; Glycine; Humans; Hyperglycemia; Infant, Newborn; Male; Pyridoxinejournal article10.1016/S0387-7604(98)00042-498406742-s2.0-0031728575