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Hemifacial seizures and cerebellar ganglioglioma: An epilepsy syndrome of infancy with seizures of cerebellar origin
Author(s) -
Harvey A. Simon,
Jayaka Prasanna,
Duchowny Michael,
Resnick Trevor,
Prats Antonio,
Altman Nolan,
Renfroe J. Benjamin
Publication year - 1996
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410400115
Subject(s) - ictal , epilepsy , hemifacial spasm , electroencephalography , cerebellar hemisphere , medicine , ganglioglioma , magnetic resonance imaging , psychology , ictal interictal spect analysis by spm , neuroscience , anatomy , radiology , facial nerve
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.