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Dysplastic Cerebellar Epilepsy: Complete Seizure Control Following Resection of a Ganglioglioma

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Abstract

Subcortical epilepsy has been a controversial issue, partially settled by evidence showing seizure generation in hypothalamic hamartomas and also by reports of seizures caused by cerebellar lesions. We report 4-year-old girl with right hemifacial seizures and autonomic phenomena, in whom MRI showed an irregular mass in the right cerebellar peduncle. Despite several unremarkable video-EEG recordings, seizure origin in the lesion was hypothesized. Complete resection was feasible, histopathology showed a ganglioglioma, and she has been seizure free for 3 years. A fine line separates these developmental tumors from focal cortical dysplasia, and the homogeneous presentation of this entity led us to propose the terminology dysplastic cerebellar epilepsy.

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Compliance with Ethics Guidelines

Conflict of Interest

Dr. Palmini has received honoraria for talks and participation in advisory boards from Novartis, Abbott, Eli Lilly, and Janssen-Cilag. Drs. Martins, Paglioli, and Hemb have no disclosures relevant to this publication. This article is in accord to all coauthors and its manuscript has not been submitted in any other medical journal.

Human and Animal Rights and Informed Consent

Written informed consent was obtained from the patient’s mother for publication of this case report, accompanying images, and video records. A copy of the written consent is available for review by the editor of this journal.

Authors’ Contributions

WAM and MH drafted the manuscript for content; EP, WAM, MH, and AP revised the manuscript for content; EP and AP made the study concept; WAM and MH performed the acquisition of data; and AP and EP did the study supervision.

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Authors and Affiliations

  1. Severe Epilepsies Outpatient Clinic, Neurology Service, Hospital São Lucas, Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), Avenida Ipiranga 6690, 90610-000, Porto Alegre, RS, Brazil

    William Alves Martins, Marta Hemb & Andre Palmini

  2. Porto Alegre Epilepsy Surgery Program, Neurology and Neurosurgery Services, Hospital São Lucas, Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), Porto Alegre, Brazil

    William Alves Martins, Eliseu Paglioli, Marta Hemb & Andre Palmini

  3. Department of Internal Medicine/Neurology, Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), Porto Alegre, Brazil

    Andre Palmini

  4. Department of Surgery, Faculty of Medicine, Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), Porto Alegre, Brazil

    Eliseu Paglioli

Authors
  1. William Alves Martins
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  2. Eliseu Paglioli
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Correspondence to William Alves Martins.

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Recording of a typical seizure. Mild clonic twitching of her right cheek, associated to blinking and tonic deviation of her right eye to the right and upwards. These episodes were almost continuous and disrupted her sleep, feeding and growth. Video keywords: cerebellar seizure; hemifacial spasm; ganglioglioma. (WMV 13987 kb)

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Martins, W.A., Paglioli, E., Hemb, M. et al. Dysplastic Cerebellar Epilepsy: Complete Seizure Control Following Resection of a Ganglioglioma. Cerebellum 15, 535–541 (2016). https://doi.org/10.1007/s12311-015-0705-5

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  • DOI: https://doi.org/10.1007/s12311-015-0705-5

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