Cerebrotendinous Xanthomatosis: A Clinical Series Illustrating the Radiological Findings

Cerebrotendinous xanthomatosis is a rare autosomal recessive disorder that occurs due to defective bile acid biosynthesis, causing unusual cholesterol and cholestanol deposition in multiple soft tissues. It is manifested by various neurological and non-neurological symptoms. The characteristic imag...

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Main Authors: Shubham Saini, Neha Bagri
Format: Article
Language:English
Published: European Medical Journal 2023-08-01
Series:European Medical Journal Neurology
Online Access:https://www.emjreviews.com/neurology/article/cerebrotendinous-xanthomatosis-a-clinical-series-illustrating-the-radiological-findings/
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author Shubham Saini
Neha Bagri
author_facet Shubham Saini
Neha Bagri
author_sort Shubham Saini
collection DOAJ
description Cerebrotendinous xanthomatosis is a rare autosomal recessive disorder that occurs due to defective bile acid biosynthesis, causing unusual cholesterol and cholestanol deposition in multiple soft tissues. It is manifested by various neurological and non-neurological symptoms. The characteristic imaging features and clinical symptoms can help to make an early diagnosis and induce timely treatment to prevent neurological sequelae. The authors present two adults with differing clinical symptoms, whose imaging provided pivotal cues in diagnosing cerebrotendinous xanthomatosis.
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institution DOAJ
issn 2054-4529
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publishDate 2023-08-01
publisher European Medical Journal
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series European Medical Journal Neurology
spelling doaj-art-009f5d822a9243a59f95a5eaf06a10e72025-08-20T02:43:42ZengEuropean Medical JournalEuropean Medical Journal Neurology2054-45292023-08-0111111910.33590/emjneurol/10302157Cerebrotendinous Xanthomatosis: A Clinical Series Illustrating the Radiological FindingsShubham Saini0Neha Bagri1Department of Radiodiagnosis, Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi, IndiaDepartment of Radiodiagnosis, Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi, India Cerebrotendinous xanthomatosis is a rare autosomal recessive disorder that occurs due to defective bile acid biosynthesis, causing unusual cholesterol and cholestanol deposition in multiple soft tissues. It is manifested by various neurological and non-neurological symptoms. The characteristic imaging features and clinical symptoms can help to make an early diagnosis and induce timely treatment to prevent neurological sequelae. The authors present two adults with differing clinical symptoms, whose imaging provided pivotal cues in diagnosing cerebrotendinous xanthomatosis.https://www.emjreviews.com/neurology/article/cerebrotendinous-xanthomatosis-a-clinical-series-illustrating-the-radiological-findings/
spellingShingle Shubham Saini
Neha Bagri
Cerebrotendinous Xanthomatosis: A Clinical Series Illustrating the Radiological Findings
European Medical Journal Neurology
title Cerebrotendinous Xanthomatosis: A Clinical Series Illustrating the Radiological Findings
title_full Cerebrotendinous Xanthomatosis: A Clinical Series Illustrating the Radiological Findings
title_fullStr Cerebrotendinous Xanthomatosis: A Clinical Series Illustrating the Radiological Findings
title_full_unstemmed Cerebrotendinous Xanthomatosis: A Clinical Series Illustrating the Radiological Findings
title_short Cerebrotendinous Xanthomatosis: A Clinical Series Illustrating the Radiological Findings
title_sort cerebrotendinous xanthomatosis a clinical series illustrating the radiological findings
url https://www.emjreviews.com/neurology/article/cerebrotendinous-xanthomatosis-a-clinical-series-illustrating-the-radiological-findings/
work_keys_str_mv AT shubhamsaini cerebrotendinousxanthomatosisaclinicalseriesillustratingtheradiologicalfindings
AT nehabagri cerebrotendinousxanthomatosisaclinicalseriesillustratingtheradiologicalfindings