Burst suppression and impairment of neocortical ontogenesis: Electroclinical and neuropathologic findings in two infants with early myoclonic encephalopathy

R. Spreafico, L. Angelini, S. Binelli, T. Granata, V. Rumi, D. Rosti, L. Runza, O. Bugiani

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

We report the electroclinical and neuropathologic correlations in 2 children aged 2.5 months affected by early myoclonic encephalopathy characterized by epileptic seizures, erratic myoclonus, and an EEG pattern of burst suppression. Despite different etiologies, the neuropathologic findings showed similar abnormalities in both cases, with no substantial impairment of the myelination processes. Islands of matrix tissue scattered in the periventricular region and neurons aligned marginally in the bulbar olives were detected. The presence of numerous large spiny neurons dispersed in the white matter along the axons of the cortical gyri was the most striking finding. These neurons have been interpreted as abnormally persisting interstitial cells in 2.5-month-old children. These early generated neurons, normally present during neocortical histogenesis, are programmed to die near the end of gestation or soon after birth. The interstitial cells are regarded as a waiting compartment of afferent fibers during cortical development. Their persistence in our patients represents an anatomic condition for cortical disconnection providing a pathophysiologic basis to burst- suppression phenomena.

Original languageEnglish
Pages (from-to)800-808
Number of pages9
JournalEpilepsia
Volume34
Issue number5
Publication statusPublished - 1993

Fingerprint

Neurons
Myoclonus
Olea
Islands
Axons
Electroencephalography
Epilepsy
Parturition
Pregnancy
Epileptic Encephalopathy, Early Infantile, 3
White Matter

Keywords

  • Child
  • Cortical synchronization
  • Development
  • Early myoclonic encephalopathy
  • Electroencephalography
  • Neocortex

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Burst suppression and impairment of neocortical ontogenesis : Electroclinical and neuropathologic findings in two infants with early myoclonic encephalopathy. / Spreafico, R.; Angelini, L.; Binelli, S.; Granata, T.; Rumi, V.; Rosti, D.; Runza, L.; Bugiani, O.

In: Epilepsia, Vol. 34, No. 5, 1993, p. 800-808.

Research output: Contribution to journalArticle

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AU - Rumi, V.

AU - Rosti, D.

AU - Runza, L.

AU - Bugiani, O.

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