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First description of pharmacoresistant epilepsy due to independent bilateral hypothalamic hamartomas

  • Case Report - Brain Tumors
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Abstract

Hypothalamic hamartomas (HHs) are rare developmental malformations consisting of mixed neurons and glial cells, usually unilaterally attached to the tuber cinereum or mammillary bodies. We report on two patients, both suffering from pharmacoresistant epilepsy, behavioural and cognitive disturbances. Ictal and interictal electroencephalographic (EEG) abnormalities appeared bilaterally and multiregionally with right-sided preponderance. Magnetic-resonance imaging (MRI) revealed independent bilateral hypothalamic hamartomas, more prominently on the right side. Endoscopic surgery of the right HH was performed in each patient, resulting in a significant seizure reduction in both cases. To the best of our knowledge, there are no other reports of independent bilateral HHs in the literature.

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Acknowledgments

We would like to thank Dr Maria-Paola Valenti, Dr Mustapha Benmekhbi, Prof Edouard Hirsch of the Medical and Surgical Epilepsy Units, University of Strasbourg, France, for scientific advice and assistance in technical aspects as well as Dr Thomas Bast, head of the paediatric section of the Kork Epilepsy Centre, and Dr Anne-Sophie Wendling, neuropsychologist for proofreading the manuscript.

This paper is dedicated to the neurosurgeon who performed the endoscopic resections, Prof Pierre Kehrli, head of the Department of Neurosurgery, University Hospital, Strasbourg, who passed away in May 2014.

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The patients and their next of kin have consented to the submission of the case report for submission to the journal.

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Authors and Affiliations

  1. Kork Epilepsy Centre, Landstrasse 1, 77694, Kehl-Kork, Germany

    Anke Maren Staack, Karl Strobl & Bernhard J. Steinhoff

  2. Medical and Surgical Epilepsy Units University of Strasbourg, Strasbourg, France

    Julia Scholly

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  1. Anke Maren Staack
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  2. Karl Strobl
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  3. Julia Scholly
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  4. Bernhard J. Steinhoff
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Correspondence to Anke Maren Staack.

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Staack, A.M., Strobl, K., Scholly, J. et al. First description of pharmacoresistant epilepsy due to independent bilateral hypothalamic hamartomas. Acta Neurochir 157, 57–61 (2015). https://doi.org/10.1007/s00701-014-2274-7

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  • DOI: https://doi.org/10.1007/s00701-014-2274-7

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