Hemifacial seizures and cerebellar ganglioglioma: An epilepsy syndrome of infancy with seizures of cerebellar origin

A. Simon Harvey, Prasanna Jayakar, Michael Duchowny, Trevor Resnick, Antonio Prats, Nolan Altman, J. Benjamin Renfroe

Research output: Contribution to journalArticle

96 Scopus citations

Abstract

We investigated a 6-month-old infant with daily episodes of left hemifacial contraction, head and eye deviation to the right, nystagmoid jerks to the right, autonomic dysfunction, and retained consciousness. The episodes began on day 1 of life and were unresponsive to antiepileptic medication. Interictal and ictal scalp electroencephalographic (EEG) recordings were unremarkable. Magnetic resonance imaging revealed a mass in the left cerebellar hemisphere and peduncle. Ictal single-photon emission computed tomography revealed focal hyperperfusion in the region of the cerebellar mass. Ictal EEG recordings with implanted cerebellar electrodes demonstrated focal seizure discharges in the region of the mass. Resection of the mass resulted in remission of seizures and histopathology revealed ganglioglioma. Six previously reported infants with similar semiology (described as 'hemifacial spasm'), imaging findings, and histopathology are reviewed. We believe the previously reported infants and our patient constitute a rare but important clinicopathological syndrome of infancy characterized by epileptic seizures of cerebellar origin.

Original languageEnglish (US)
Pages (from-to)91-98
Number of pages8
JournalAnnals of neurology
Volume40
Issue number1
DOIs
StatePublished - Jul 1996

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Harvey, A. S., Jayakar, P., Duchowny, M., Resnick, T., Prats, A., Altman, N., & Renfroe, J. B. (1996). Hemifacial seizures and cerebellar ganglioglioma: An epilepsy syndrome of infancy with seizures of cerebellar origin. Annals of neurology, 40(1), 91-98. https://doi.org/10.1002/ana.410400115