Differential diagnosis of hypertrophic pachymeningitis: literature review

Hypertrophic pachymeningitis (HP) is a focal or diffuse thickening of cranial and/or spinal dura mater caused by chronic inflammatory process, which induces fibrosis and thickening of the pachymeninges. Fibrotic, thickened dura causes compression of adjacent structures that results in HP symptoms....

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Main Authors: G. Makarevičius, J. Grigaitė, M. Jokubaitis, A. Klimašauskienė
Format: Article
Language:English
Published: Vilnius University Press 2022-03-01
Series:Neurologijos seminarai
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Online Access:https://www.journals.vu.lt/neurologijos_seminarai/article/view/29288
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author G. Makarevičius
J. Grigaitė
M. Jokubaitis
A. Klimašauskienė
author_facet G. Makarevičius
J. Grigaitė
M. Jokubaitis
A. Klimašauskienė
author_sort G. Makarevičius
collection DOAJ
description Hypertrophic pachymeningitis (HP) is a focal or diffuse thickening of cranial and/or spinal dura mater caused by chronic inflammatory process, which induces fibrosis and thickening of the pachymeninges. Fibrotic, thickened dura causes compression of adjacent structures that results in HP symptoms. Usually HP presents with headache, diplopia, visual loss, and otologic symptoms. As the disease progresses, intracranial hypertension, consciousness disturbance, and seizures may also develop. There are two distinct groups of HP – idiopathic and secondary HP. There are 3 major groups of secondary HP: infectious, autoimmune, and oncological. There are no highly specific clinical and radiological features to distinguish secondary HP from idiopathic HP. For this reason, a thorough examination and dural biopsy are of great importance in the diagnosis of idiopathic HP. Idiopathic HP can be only diagnosed when secondary causes of HP are ruled out and dural biopsy confirms that there are only unspecific findings: lymphocytic-plasmacytic infiltration and fibrosis. In this article, we review the literature regarding HP and discuss clinical and radiological features of both, idiopathic and secondary HP, and their differential diagnosis.
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publisher Vilnius University Press
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series Neurologijos seminarai
spelling doaj-art-fa537bb1787946188590a75c0f31915a2025-01-20T18:22:16ZengVilnius University PressNeurologijos seminarai1392-30642424-59172022-03-01261 (91)10.29014/NS.2022.26.2Differential diagnosis of hypertrophic pachymeningitis: literature reviewG. Makarevičius0J. Grigaitė1M. Jokubaitis2A. Klimašauskienė3Vilnius University, LithuaniaVilnius University, LithuaniaVilnius University, LithuaniaVilnius University, Lithuania Hypertrophic pachymeningitis (HP) is a focal or diffuse thickening of cranial and/or spinal dura mater caused by chronic inflammatory process, which induces fibrosis and thickening of the pachymeninges. Fibrotic, thickened dura causes compression of adjacent structures that results in HP symptoms. Usually HP presents with headache, diplopia, visual loss, and otologic symptoms. As the disease progresses, intracranial hypertension, consciousness disturbance, and seizures may also develop. There are two distinct groups of HP – idiopathic and secondary HP. There are 3 major groups of secondary HP: infectious, autoimmune, and oncological. There are no highly specific clinical and radiological features to distinguish secondary HP from idiopathic HP. For this reason, a thorough examination and dural biopsy are of great importance in the diagnosis of idiopathic HP. Idiopathic HP can be only diagnosed when secondary causes of HP are ruled out and dural biopsy confirms that there are only unspecific findings: lymphocytic-plasmacytic infiltration and fibrosis. In this article, we review the literature regarding HP and discuss clinical and radiological features of both, idiopathic and secondary HP, and their differential diagnosis. https://www.journals.vu.lt/neurologijos_seminarai/article/view/29288idiopathic hypertrophic pachymeningitissecondary hypertrophic pachymeningitisdifferential diagnosis
spellingShingle G. Makarevičius
J. Grigaitė
M. Jokubaitis
A. Klimašauskienė
Differential diagnosis of hypertrophic pachymeningitis: literature review
Neurologijos seminarai
idiopathic hypertrophic pachymeningitis
secondary hypertrophic pachymeningitis
differential diagnosis
title Differential diagnosis of hypertrophic pachymeningitis: literature review
title_full Differential diagnosis of hypertrophic pachymeningitis: literature review
title_fullStr Differential diagnosis of hypertrophic pachymeningitis: literature review
title_full_unstemmed Differential diagnosis of hypertrophic pachymeningitis: literature review
title_short Differential diagnosis of hypertrophic pachymeningitis: literature review
title_sort differential diagnosis of hypertrophic pachymeningitis literature review
topic idiopathic hypertrophic pachymeningitis
secondary hypertrophic pachymeningitis
differential diagnosis
url https://www.journals.vu.lt/neurologijos_seminarai/article/view/29288
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AT mjokubaitis differentialdiagnosisofhypertrophicpachymeningitisliteraturereview
AT aklimasauskiene differentialdiagnosisofhypertrophicpachymeningitisliteraturereview