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Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms

Although transient neurological deficits in Sturge–Weber syndrome (SWS) have been acknowledged for many years, the underlying pathogenesis still constitutes a major topic of discussion. Here, we present the case of a 10-year-old boy with SWS presenting with a progressive clinically mimicking right m...

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Autor principal: Onder, Halil
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126282/
https://www.ncbi.nlm.nih.gov/pubmed/30271058
http://dx.doi.org/10.4103/jnrp.jnrp_11_18
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author Onder, Halil
author_facet Onder, Halil
author_sort Onder, Halil
collection PubMed
description Although transient neurological deficits in Sturge–Weber syndrome (SWS) have been acknowledged for many years, the underlying pathogenesis still constitutes a major topic of discussion. Here, we present the case of a 10-year-old boy with SWS presenting with a progressive clinically mimicking right middle cerebral artery syndrome. Cranial magnetic resonance imaging showed unremarkable findings except extensive right hemisphere leptomeningeal angiomas. Antiepileptic drug (AED) treatments provided recovery of the patient, and concurrently recorded electroencephalography (EEG) revealed delta slowing of the lesional hemisphere in the acute period. However, slowing of the background activity extended to the contralateral hemisphere in subacute period, while ipsilateral background activity started to recover. In the 3(rd) week of follow-up on intensive AED treatments, the patient totally recovered as well as nearly the normalization of EEG was achieved. In this report, we focus on the dramatic revolution of EEG activity in both hemispheres during the recovery period. Based on this rare illustration and limited literature data, the author suggests some hypotheses regarding the pathophysiology of this mysterious manifestation (transient neurological deficit) in SWS. Future studies of large case series, including detailed paraclinical parameters and remarkably electrophysiological data, are warranted to clarify these arguments.
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spelling pubmed-61262822018-10-01 Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms Onder, Halil J Neurosci Rural Pract Case Report Although transient neurological deficits in Sturge–Weber syndrome (SWS) have been acknowledged for many years, the underlying pathogenesis still constitutes a major topic of discussion. Here, we present the case of a 10-year-old boy with SWS presenting with a progressive clinically mimicking right middle cerebral artery syndrome. Cranial magnetic resonance imaging showed unremarkable findings except extensive right hemisphere leptomeningeal angiomas. Antiepileptic drug (AED) treatments provided recovery of the patient, and concurrently recorded electroencephalography (EEG) revealed delta slowing of the lesional hemisphere in the acute period. However, slowing of the background activity extended to the contralateral hemisphere in subacute period, while ipsilateral background activity started to recover. In the 3(rd) week of follow-up on intensive AED treatments, the patient totally recovered as well as nearly the normalization of EEG was achieved. In this report, we focus on the dramatic revolution of EEG activity in both hemispheres during the recovery period. Based on this rare illustration and limited literature data, the author suggests some hypotheses regarding the pathophysiology of this mysterious manifestation (transient neurological deficit) in SWS. Future studies of large case series, including detailed paraclinical parameters and remarkably electrophysiological data, are warranted to clarify these arguments. Medknow Publications & Media Pvt Ltd 2018 /pmc/articles/PMC6126282/ /pubmed/30271058 http://dx.doi.org/10.4103/jnrp.jnrp_11_18 Text en Copyright: © 2018 Journal of Neurosciences in Rural Practice http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Onder, Halil
Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms
title Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms
title_full Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms
title_fullStr Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms
title_full_unstemmed Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms
title_short Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms
title_sort detailed clinical and electrophysiological illustration of a patient with sturge–weber syndrome presenting with prolonged transient neurological symptoms
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126282/
https://www.ncbi.nlm.nih.gov/pubmed/30271058
http://dx.doi.org/10.4103/jnrp.jnrp_11_18
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