Agyria-pachygyria: clinical, neuroimaging, and neurophysiologic correlations.
Agyria-pachygyria complex is a disorder of neuronal migration and organization. Patients suffer either motor or intellectual retardation. We report our experiences of 10 patients with agyria-pachygyria complex and evaluate their clinical features, electroencephalography, and evoked potentials. Of nine electroencephalography examinations, five patients demonstrated characteristically high-amplitude fast activity. One of nine patients had an abnormal brainstem auditory-evoked potential. Three of seven patients had abnormal goggled visual-evoked potential. Six patients received somatosensory-evoked potential examinations, and five of these were abnormal, including four with prolonged central conduction times. Of the 10 patients, eight survived with variable intellectual and motor retardation; two died of sepsis. Patients with grades 1-4 agyria-pachygyria had high incidences of somatosensory-evoked potential abnormalities and also suffered worse neurologic outcomes. Normal brainstem auditory-evoked potential but abnormal cortical somatosensory-evoked potential components and prolonged central conduction time in these patients indicate that agyria-pachygyria is a supratentorial disease. We conclude that somatosensory-evoked potential examination is supplemental to neuroimaging in predicting the neurologic prognosis of patients with agyria-pachygyria.
Liang
Jao Shwann
JS
Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
Lee
Wang Tso
WT
Young
Chainllie
C
Peng
Steven Shinnforng
SS
Shen
Yu-Zen
YZ
D000293:Adolescent; D001921:Brain / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004830:Epilepsy, Tonic-Clonic / Q000151:congenital / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000175:diagnosis / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D009431:Neural Conduction / Q000502:physiology; D009460:Neurologic Examination; D009474:Neurons / Q000502:physiology; D011379:Prognosis; D011596:Psychomotor Disorders / Q000151:congenital / Q000175:diagnosis / Q000503:physiopathology; D011930:Reaction Time / Q000502:physiology; D013036:Spasms, Infantile / Q000151:congenital / Q000175:diagnosis / Q000503:physiopathology