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A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review

Paraneoplastic neurological disorders are rare in children, with paraneoplastic cerebellar degeneration (PCD) considered highly atypical. We describe a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy presenting in super-refractory status epil...

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Autores principales: Aboseif, Albert, Palmer, Kaitlyn, Abrams, Aaron W., Lachhwani, Deepak, Knight, Elia Margarita Pestana, Valappil, Ahsan Moosa Naduvil, Zeft, Andrew
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10621035/
https://www.ncbi.nlm.nih.gov/pubmed/37928156
http://dx.doi.org/10.3389/fneur.2023.1250261
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author Aboseif, Albert
Palmer, Kaitlyn
Abrams, Aaron W.
Lachhwani, Deepak
Knight, Elia Margarita Pestana
Valappil, Ahsan Moosa Naduvil
Zeft, Andrew
author_facet Aboseif, Albert
Palmer, Kaitlyn
Abrams, Aaron W.
Lachhwani, Deepak
Knight, Elia Margarita Pestana
Valappil, Ahsan Moosa Naduvil
Zeft, Andrew
author_sort Aboseif, Albert
collection PubMed
description Paraneoplastic neurological disorders are rare in children, with paraneoplastic cerebellar degeneration (PCD) considered highly atypical. We describe a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy presenting in super-refractory status epilepticus. After an extensive evaluation, her clinical picture was suggestive of probable autoimmune encephalitis (AE). Further diagnostic testing revealed a molecularly undefined neural-restricted autoantibody in both serum and CSF, raising suspicion over an adrenal mass previously considered incidental. Surgical resection led to a robust clinical improvement, and pathology revealed a benign ganglioneuroma. This report widens the spectrum of paraneoplastic manifestations of ganglioneuroma, reviews the diagnostic approach to antibody-negative pediatric AE, and raises important clinical considerations regarding benign and incidentally found tumors in the setting of a suspected paraneoplastic neurologic syndrome.
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spelling pubmed-106210352023-11-03 A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review Aboseif, Albert Palmer, Kaitlyn Abrams, Aaron W. Lachhwani, Deepak Knight, Elia Margarita Pestana Valappil, Ahsan Moosa Naduvil Zeft, Andrew Front Neurol Neurology Paraneoplastic neurological disorders are rare in children, with paraneoplastic cerebellar degeneration (PCD) considered highly atypical. We describe a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy presenting in super-refractory status epilepticus. After an extensive evaluation, her clinical picture was suggestive of probable autoimmune encephalitis (AE). Further diagnostic testing revealed a molecularly undefined neural-restricted autoantibody in both serum and CSF, raising suspicion over an adrenal mass previously considered incidental. Surgical resection led to a robust clinical improvement, and pathology revealed a benign ganglioneuroma. This report widens the spectrum of paraneoplastic manifestations of ganglioneuroma, reviews the diagnostic approach to antibody-negative pediatric AE, and raises important clinical considerations regarding benign and incidentally found tumors in the setting of a suspected paraneoplastic neurologic syndrome. Frontiers Media S.A. 2023-10-19 /pmc/articles/PMC10621035/ /pubmed/37928156 http://dx.doi.org/10.3389/fneur.2023.1250261 Text en Copyright © 2023 Aboseif, Palmer, Abrams, Lachhwani, Knight, Valappil and Zeft. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Aboseif, Albert
Palmer, Kaitlyn
Abrams, Aaron W.
Lachhwani, Deepak
Knight, Elia Margarita Pestana
Valappil, Ahsan Moosa Naduvil
Zeft, Andrew
A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review
title A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review
title_full A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review
title_fullStr A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review
title_full_unstemmed A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review
title_short A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review
title_sort not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10621035/
https://www.ncbi.nlm.nih.gov/pubmed/37928156
http://dx.doi.org/10.3389/fneur.2023.1250261
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