Document Detail


Agyria-pachygyria: clinical, neuroimaging, and neurophysiologic correlations.
MedLine Citation:
PMID:  12393126     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Jao Shwann Liang; Wang Tso Lee; Chainllie Young; Steven Shinnforng Peng; Yu-Zen Shen
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric neurology     Volume:  27     ISSN:  0887-8994     ISO Abbreviation:  Pediatr. Neurol.     Publication Date:  2002 Sep 
Date Detail:
Created Date:  2002-10-23     Completed Date:  2003-01-23     Revised Date:  2006-05-23    
Medline Journal Info:
Nlm Unique ID:  8508183     Medline TA:  Pediatr Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  171-6     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Brain / abnormalities*,  pathology,  physiopathology
Child
Child, Preschool
Electroencephalography*
Epilepsy, Tonic-Clonic / congenital,  diagnosis,  physiopathology
Female
Humans
Infant
Magnetic Resonance Imaging*
Male
Mental Retardation / diagnosis,  physiopathology
Neural Conduction / physiology
Neurologic Examination
Neurons / physiology
Prognosis
Psychomotor Disorders / congenital,  diagnosis,  physiopathology
Reaction Time / physiology
Spasms, Infantile / congenital,  diagnosis,  physiopathology

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