Transient Periictal Brain Imaging Abnormality in a Saudi Patient with Probable Celiac Disease Epilepsy and Occipital Calcification Syndrome

Celiac disease epilepsy and occipital calcification (CEC) syndrome is a rare, emerging disease first described in 1992. To date, fewer than 200 cases have been reported worldwide. CEC syndrome is generally thought to be a genetic, noninherited, and ethnically and geographically restricted disease in...

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Main Authors: Soha Khan, Asma AlNajjar, Abdullah Alquaydheb, Shahpar Nahrir
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2019/5247961
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author Soha Khan
Asma AlNajjar
Abdullah Alquaydheb
Shahpar Nahrir
author_facet Soha Khan
Asma AlNajjar
Abdullah Alquaydheb
Shahpar Nahrir
author_sort Soha Khan
collection DOAJ
description Celiac disease epilepsy and occipital calcification (CEC) syndrome is a rare, emerging disease first described in 1992. To date, fewer than 200 cases have been reported worldwide. CEC syndrome is generally thought to be a genetic, noninherited, and ethnically and geographically restricted disease in Mediterranean countries. However, we report the first ever case of probable CEC in a Saudi patient. Furthermore, the patient manifested a magnitude of brain magnetic resonance imaging (MRI) signal abnormalities during the periictal period which, to the best of our knowledge, has never been described in CEC. The brain MRI revealed diffusion-weighted imaging (DWI) restriction with a concordant area of apparent diffusion coefficient (ADC) hypointensity around bilateral occipital area of calcification. An imbalance between the heightened energy demand during ictal phase of the seizure and unadjusted blood supply may have caused an electric pump failure and cytotoxic edema, which then led to DWI/ADC signal alteration.
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2090-6676
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spelling doaj-art-d5925495f3194dc4b211404aea3e2e282025-08-20T02:19:40ZengWileyCase Reports in Neurological Medicine2090-66682090-66762019-01-01201910.1155/2019/52479615247961Transient Periictal Brain Imaging Abnormality in a Saudi Patient with Probable Celiac Disease Epilepsy and Occipital Calcification SyndromeSoha Khan0Asma AlNajjar1Abdullah Alquaydheb2Shahpar Nahrir3King Saud Medical City, Riyadh, Saudi ArabiaKing Saud Medical City, Riyadh, Saudi ArabiaKing Saud Medical City, Riyadh, Saudi ArabiaKing Saud Medical City, Riyadh, Saudi ArabiaCeliac disease epilepsy and occipital calcification (CEC) syndrome is a rare, emerging disease first described in 1992. To date, fewer than 200 cases have been reported worldwide. CEC syndrome is generally thought to be a genetic, noninherited, and ethnically and geographically restricted disease in Mediterranean countries. However, we report the first ever case of probable CEC in a Saudi patient. Furthermore, the patient manifested a magnitude of brain magnetic resonance imaging (MRI) signal abnormalities during the periictal period which, to the best of our knowledge, has never been described in CEC. The brain MRI revealed diffusion-weighted imaging (DWI) restriction with a concordant area of apparent diffusion coefficient (ADC) hypointensity around bilateral occipital area of calcification. An imbalance between the heightened energy demand during ictal phase of the seizure and unadjusted blood supply may have caused an electric pump failure and cytotoxic edema, which then led to DWI/ADC signal alteration.http://dx.doi.org/10.1155/2019/5247961
spellingShingle Soha Khan
Asma AlNajjar
Abdullah Alquaydheb
Shahpar Nahrir
Transient Periictal Brain Imaging Abnormality in a Saudi Patient with Probable Celiac Disease Epilepsy and Occipital Calcification Syndrome
Case Reports in Neurological Medicine
title Transient Periictal Brain Imaging Abnormality in a Saudi Patient with Probable Celiac Disease Epilepsy and Occipital Calcification Syndrome
title_full Transient Periictal Brain Imaging Abnormality in a Saudi Patient with Probable Celiac Disease Epilepsy and Occipital Calcification Syndrome
title_fullStr Transient Periictal Brain Imaging Abnormality in a Saudi Patient with Probable Celiac Disease Epilepsy and Occipital Calcification Syndrome
title_full_unstemmed Transient Periictal Brain Imaging Abnormality in a Saudi Patient with Probable Celiac Disease Epilepsy and Occipital Calcification Syndrome
title_short Transient Periictal Brain Imaging Abnormality in a Saudi Patient with Probable Celiac Disease Epilepsy and Occipital Calcification Syndrome
title_sort transient periictal brain imaging abnormality in a saudi patient with probable celiac disease epilepsy and occipital calcification syndrome
url http://dx.doi.org/10.1155/2019/5247961
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