Agyria-pachygyria: clinical, neuroimaging, and neurophysiologic correlations

Pediatr Neurol. 2002 Sep;27(3):171-6. doi: 10.1016/s0887-8994(02)00401-0.

Abstract

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.

MeSH terms

  • Adolescent
  • Brain / abnormalities*
  • Brain / pathology
  • Brain / physiopathology
  • Child
  • Child, Preschool
  • Electroencephalography*
  • Epilepsy, Tonic-Clonic / congenital
  • Epilepsy, Tonic-Clonic / diagnosis
  • Epilepsy, Tonic-Clonic / physiopathology
  • Female
  • Humans
  • Infant
  • Intellectual Disability / diagnosis
  • Intellectual Disability / physiopathology
  • Magnetic Resonance Imaging*
  • Male
  • Neural Conduction / physiology
  • Neurologic Examination
  • Neurons / physiology
  • Prognosis
  • Psychomotor Disorders / congenital
  • Psychomotor Disorders / diagnosis
  • Psychomotor Disorders / physiopathology
  • Reaction Time / physiology
  • Spasms, Infantile / congenital
  • Spasms, Infantile / diagnosis
  • Spasms, Infantile / physiopathology