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OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT

INTRODUCTION: Paragangliomas (PGs) are rare neuroendocrine tumours that can arise from parasympathetic glossopharyngeal and vagus nerves. Atypical presentations involving seizures and dementia can occur due to excessive catecholamine release. Physiological intracranial arteriovenous fistulae (AVF) c...

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Autores principales: Sokhi, Dilraj, Said, Jamil, Nyambane, Eunice, Hooker, Juzar, Maina, Ben, Cheserem, Beverly, Mogere, Edwin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10616582/
http://dx.doi.org/10.1093/noajnl/vdad121.032
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author Sokhi, Dilraj
Said, Jamil
Nyambane, Eunice
Hooker, Juzar
Maina, Ben
Cheserem, Beverly
Mogere, Edwin
author_facet Sokhi, Dilraj
Said, Jamil
Nyambane, Eunice
Hooker, Juzar
Maina, Ben
Cheserem, Beverly
Mogere, Edwin
author_sort Sokhi, Dilraj
collection PubMed
description INTRODUCTION: Paragangliomas (PGs) are rare neuroendocrine tumours that can arise from parasympathetic glossopharyngeal and vagus nerves. Atypical presentations involving seizures and dementia can occur due to excessive catecholamine release. Physiological intracranial arteriovenous fistulae (AVF) can occur concurrently due to increased vascularity, especially in the cavernous and sigmoid sinuses. METHODS: We present a case of a patient who developed a highly vascular AVF from an ipsilateral glomus jugulare PG, presenting with rapidly progressive but reversible dementia and epilepsy. CASE: A 64-year-old lady presented on several occasions with new-onset stereotyped episodes of confusional dysphasia suggestive of seizures, confirmed on electroencephalography. Further history revealed two years of progressive deafness and imbalance, and one year of increasing forgetfulness and executive dysfunction, which had deteriorated rapidly in the period of the patient's current presentations. Montreal cognitive assessment (MoCA) score was 21/30 at baseline (normal score >25/30), and she had no lateralising signs. Serial brain magnetic resonance imaging (MRI) revealed progressively worsening vasculopathic white matter hyper-intensities (WMHs). Eventual MR angiography showed a 14x22×23mm right glomus jugulotympanicum PG with numerous feeding arteries, and associated multiple AVFs (Cognard grade III). She was initially managed with anti-seizure medications and then discussed in our multidisciplinary neurosciences meeting. The patient underwent staged right glomus jugulare embolization over several weeks. Her cognitive function gradually improved, and at three months she had fully returned to normal functioning with a MoCA of 30/30 and no residual neurological deficit. The WMHs disappeared on follow-up MRI scans. CONCLUSION: Our case highlights a rare clinical presentation of dementia and seizures from a right glomus jugulare PG with associated AVF. Multidisciplinary involvement was key in excluding other causes, and definitive treatment of the PG led to complete reversal of the patient’s dementia.
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spelling pubmed-106165822023-11-01 OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT Sokhi, Dilraj Said, Jamil Nyambane, Eunice Hooker, Juzar Maina, Ben Cheserem, Beverly Mogere, Edwin Neurooncol Adv Final Category: Other INTRODUCTION: Paragangliomas (PGs) are rare neuroendocrine tumours that can arise from parasympathetic glossopharyngeal and vagus nerves. Atypical presentations involving seizures and dementia can occur due to excessive catecholamine release. Physiological intracranial arteriovenous fistulae (AVF) can occur concurrently due to increased vascularity, especially in the cavernous and sigmoid sinuses. METHODS: We present a case of a patient who developed a highly vascular AVF from an ipsilateral glomus jugulare PG, presenting with rapidly progressive but reversible dementia and epilepsy. CASE: A 64-year-old lady presented on several occasions with new-onset stereotyped episodes of confusional dysphasia suggestive of seizures, confirmed on electroencephalography. Further history revealed two years of progressive deafness and imbalance, and one year of increasing forgetfulness and executive dysfunction, which had deteriorated rapidly in the period of the patient's current presentations. Montreal cognitive assessment (MoCA) score was 21/30 at baseline (normal score >25/30), and she had no lateralising signs. Serial brain magnetic resonance imaging (MRI) revealed progressively worsening vasculopathic white matter hyper-intensities (WMHs). Eventual MR angiography showed a 14x22×23mm right glomus jugulotympanicum PG with numerous feeding arteries, and associated multiple AVFs (Cognard grade III). She was initially managed with anti-seizure medications and then discussed in our multidisciplinary neurosciences meeting. The patient underwent staged right glomus jugulare embolization over several weeks. Her cognitive function gradually improved, and at three months she had fully returned to normal functioning with a MoCA of 30/30 and no residual neurological deficit. The WMHs disappeared on follow-up MRI scans. CONCLUSION: Our case highlights a rare clinical presentation of dementia and seizures from a right glomus jugulare PG with associated AVF. Multidisciplinary involvement was key in excluding other causes, and definitive treatment of the PG led to complete reversal of the patient’s dementia. Oxford University Press 2023-10-31 /pmc/articles/PMC10616582/ http://dx.doi.org/10.1093/noajnl/vdad121.032 Text en © The Author(s) 2023. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Final Category: Other
Sokhi, Dilraj
Said, Jamil
Nyambane, Eunice
Hooker, Juzar
Maina, Ben
Cheserem, Beverly
Mogere, Edwin
OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT
title OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT
title_full OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT
title_fullStr OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT
title_full_unstemmed OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT
title_short OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT
title_sort other-11 dementia and epilepsy reversed successfully through staged embolisation of a symptomatic hypervascular paraganglioma : a case report
topic Final Category: Other
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10616582/
http://dx.doi.org/10.1093/noajnl/vdad121.032
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