Abstract
Objective To analyze the clinical manifestations and electroencephalogram (EEG)characteristics of agyria-pachygyria for its early diagnosis, treatment and prognosis judgment in clinical practice. Methods The cli-nical manifestations and EEG features of twenty-four patients with agyria-pachygyria who were diagnosed by CT or magnetic resonance imaging(MRI) at Pediatric Neurology of Children's Hospital of Chongqing Medical University from July 2004 to July 2013 were retrospectively analyzed. Results Of twenty-four patients, eighteen cases were diagnosed as diffuse agyria-pachygyria and six cases were diagnosed as partial agyria-pachygyria.The clinical features were mainly manifested as mental retardation (twenty-four patients), and motor retardation (twenty-four patients), and epilepsy (eighteen patients). All of the twenty-four patients had abnormal EEG pattern which were mainly three tapes.Type Ⅰ had diffused high amplitude alpha and beta activity in all cortical regions, frontal-central, or parietal-occipital region (fourteen patients). Type Ⅱ showed alternating high amplitude bursts with sharp and slow waves (seven patients). Type Ⅲ was characterized by high amplitude spike or sharp wave activity generalized or multifocal distribution and δ, θ wave mixing graphics (twelve patients). Nine of twenty-four patients showed two or three EEG characteristic patterns in an awake-asleep EEG recording.During the follow-up of 1-8 years old, twelve of the thirteen patients who were diagnosed as epilepsy in diffuse agyria-pachygyria had refractory epilepsy, mainly with infantile spasms or Lennox-Gastaut syndrome.One of the five patients who was diagnosed as epilepsy in focal agyria-pachygyria had refractory epilepsy, mainly for partial epilepsy secondary generalized seizures.There was a significant diffe-rence between them (P=0.008). Eighteen of twenty patients who had moderate-severe mental retardation or dyskinesia were diagnosed as diffuse a gyria-pachygyria, while two were focal agyria-pachygyria.Both of them had a significant difference (P=0.005). Conclusions Agyria-pachygyria is a brain malformation caused by neuronal migration abnormality.Diffuse agyria-pachygyria is presented with serious clinical manifestations and poor outcome while the clinical manifestation of focal agyria-pachygyria is relatively mild and epilepsy could be controlled by antiepileptic drugs or epilepsy surgery.These characteristics of EEG patterns along with clinical findings could provide important evidence for early diagnosis, timely treatment and prognosis judgment of agyria-pachygyria. Key words: Agyria-pachygyria; Clinical manifestations; Electroencephalogram characteristics
Published Version
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