Cargando…

Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome

We present longitudinal imaging data of a child with Sturge–Weber syndrome (SWS). At age 8 months, 3 weeks after initial seizures and prolonged motor deficit, MRI showed extensive right hemispheric SWS involvement with severe glucose hypometabolism on PET. She was treated with levetiracetam and aspi...

Descripción completa

Detalles Bibliográficos
Autores principales: John, Flóra, Maqbool, Mohsin, Jeong, Jeong‐won, Agarwal, Rajkumar, Behen, Michael E., Juhász, Csaba
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5899918/
https://www.ncbi.nlm.nih.gov/pubmed/29687028
http://dx.doi.org/10.1002/acn3.546
_version_ 1783314334001135616
author John, Flóra
Maqbool, Mohsin
Jeong, Jeong‐won
Agarwal, Rajkumar
Behen, Michael E.
Juhász, Csaba
author_facet John, Flóra
Maqbool, Mohsin
Jeong, Jeong‐won
Agarwal, Rajkumar
Behen, Michael E.
Juhász, Csaba
author_sort John, Flóra
collection PubMed
description We present longitudinal imaging data of a child with Sturge–Weber syndrome (SWS). At age 8 months, 3 weeks after initial seizures and prolonged motor deficit, MRI showed extensive right hemispheric SWS involvement with severe glucose hypometabolism on PET. She was treated with levetiracetam and aspirin. Follow‐up imaging at age 29 months showed a robust interval expansion of enlarged deep medullary veins throughout the affected hemisphere along with a dramatic recovery of hemispheric metabolism and normalized neurocognitive functioning. These findings demonstrate a robust, multilobar hemispheric remodeling of deep venous collaterals that likely contributed to reversal of initial metabolic and neurocognitive deficits.
format Online
Article
Text
id pubmed-5899918
institution National Center for Biotechnology Information
language English
publishDate 2018
publisher John Wiley and Sons Inc.
record_format MEDLINE/PubMed
spelling pubmed-58999182018-04-23 Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome John, Flóra Maqbool, Mohsin Jeong, Jeong‐won Agarwal, Rajkumar Behen, Michael E. Juhász, Csaba Ann Clin Transl Neurol Case Study We present longitudinal imaging data of a child with Sturge–Weber syndrome (SWS). At age 8 months, 3 weeks after initial seizures and prolonged motor deficit, MRI showed extensive right hemispheric SWS involvement with severe glucose hypometabolism on PET. She was treated with levetiracetam and aspirin. Follow‐up imaging at age 29 months showed a robust interval expansion of enlarged deep medullary veins throughout the affected hemisphere along with a dramatic recovery of hemispheric metabolism and normalized neurocognitive functioning. These findings demonstrate a robust, multilobar hemispheric remodeling of deep venous collaterals that likely contributed to reversal of initial metabolic and neurocognitive deficits. John Wiley and Sons Inc. 2018-03-12 /pmc/articles/PMC5899918/ /pubmed/29687028 http://dx.doi.org/10.1002/acn3.546 Text en © 2018 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association. This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Case Study
John, Flóra
Maqbool, Mohsin
Jeong, Jeong‐won
Agarwal, Rajkumar
Behen, Michael E.
Juhász, Csaba
Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome
title Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome
title_full Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome
title_fullStr Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome
title_full_unstemmed Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome
title_short Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome
title_sort deep cerebral vein expansion with metabolic and neurocognitive recovery in sturge–weber syndrome
topic Case Study
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5899918/
https://www.ncbi.nlm.nih.gov/pubmed/29687028
http://dx.doi.org/10.1002/acn3.546
work_keys_str_mv AT johnflora deepcerebralveinexpansionwithmetabolicandneurocognitiverecoveryinsturgewebersyndrome
AT maqboolmohsin deepcerebralveinexpansionwithmetabolicandneurocognitiverecoveryinsturgewebersyndrome
AT jeongjeongwon deepcerebralveinexpansionwithmetabolicandneurocognitiverecoveryinsturgewebersyndrome
AT agarwalrajkumar deepcerebralveinexpansionwithmetabolicandneurocognitiverecoveryinsturgewebersyndrome
AT behenmichaele deepcerebralveinexpansionwithmetabolicandneurocognitiverecoveryinsturgewebersyndrome
AT juhaszcsaba deepcerebralveinexpansionwithmetabolicandneurocognitiverecoveryinsturgewebersyndrome