An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature

Ecchordosis physaliphora (EP) is a benign notochordal remnant, which is often an incidental finding; however, it can rarely present with neurological symptoms. We performed a systematic review of the literature for cases of symptomatic EP published in PubMed, Web of Science and Embase from January 1...

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Main Authors: Rajesh K. Gupta, Thejasvi A. Reddy, Ashutosh Gupta, Rohan Samant, Carlos A. Perez, Anam Haque
Format: Article
Language:English
Published: MDPI AG 2023-09-01
Series:Neurology International
Subjects:
Online Access:https://www.mdpi.com/2035-8377/15/4/75
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author Rajesh K. Gupta
Thejasvi A. Reddy
Ashutosh Gupta
Rohan Samant
Carlos A. Perez
Anam Haque
author_facet Rajesh K. Gupta
Thejasvi A. Reddy
Ashutosh Gupta
Rohan Samant
Carlos A. Perez
Anam Haque
author_sort Rajesh K. Gupta
collection DOAJ
description Ecchordosis physaliphora (EP) is a benign notochordal remnant, which is often an incidental finding; however, it can rarely present with neurological symptoms. We performed a systematic review of the literature for cases of symptomatic EP published in PubMed, Web of Science and Embase from January 1982 to May 2023. This is the largest review to date and revealed 60 cases including ours. Headache (55%) and CSF rhinorrhea (32%) were the most frequent clinical manifestations. The majority of symptomatic EP lesions were located in the prepontine region (77%) and required surgical resection (75%). EP should be considered in patients with neurologic symptoms in the setting of prepontine or posterior sphenoid sinus lesions. While symptomatic patients often require surgical intervention, rare cases may respond to oral corticosteroids.
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spelling doaj.art-ef1f9cdcbe934487beea395755758f872023-12-22T14:29:30ZengMDPI AGNeurology International2035-83772023-09-011541200121110.3390/neurolint15040075An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the LiteratureRajesh K. Gupta0Thejasvi A. Reddy1Ashutosh Gupta2Rohan Samant3Carlos A. Perez4Anam Haque5Division of Multiple Sclerosis and Neuroimmunology, Department of Neurology, University of Texas Health Science Center, Houston, TX 77030, USAMcGovern Medical School, UT Health Science Center, Houston, TX 77030, USAMcGovern Medical School, UT Health Science Center, Houston, TX 77030, USADepartment of Radiology, UT Health Science Center, Houston, TX 77030, USADepartment of Neurology, Baylor College of Medicine, Houston, TX 77030, USAMcGovern Medical School, UT Health Science Center, Houston, TX 77030, USAEcchordosis physaliphora (EP) is a benign notochordal remnant, which is often an incidental finding; however, it can rarely present with neurological symptoms. We performed a systematic review of the literature for cases of symptomatic EP published in PubMed, Web of Science and Embase from January 1982 to May 2023. This is the largest review to date and revealed 60 cases including ours. Headache (55%) and CSF rhinorrhea (32%) were the most frequent clinical manifestations. The majority of symptomatic EP lesions were located in the prepontine region (77%) and required surgical resection (75%). EP should be considered in patients with neurologic symptoms in the setting of prepontine or posterior sphenoid sinus lesions. While symptomatic patients often require surgical intervention, rare cases may respond to oral corticosteroids.https://www.mdpi.com/2035-8377/15/4/75ecchordosis physaliphoranotochordchordomaCNS
spellingShingle Rajesh K. Gupta
Thejasvi A. Reddy
Ashutosh Gupta
Rohan Samant
Carlos A. Perez
Anam Haque
An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature
Neurology International
ecchordosis physaliphora
notochord
chordoma
CNS
title An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature
title_full An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature
title_fullStr An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature
title_full_unstemmed An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature
title_short An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature
title_sort ecchordosis physaliphora a rare entity involving the central nervous system a systematic review of the literature
topic ecchordosis physaliphora
notochord
chordoma
CNS
url https://www.mdpi.com/2035-8377/15/4/75
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