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Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation

Spontaneous intracranial hypotension (SIH) occurs due to a leakage of the cerebrospinal fluid (CSF) lowering the pressure of subarachnoid space, mostly caused by a dural breach or discogenic microspur. As a result of less support provided by CSF pressure, intracranial structures are stretched downwa...

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Autores principales: Bin Wan Hassan, Wan Muhammad Nazief, Mistretta, Francesco, Molinaro, Stefano, Russo, Riccardo, Bosco, Giovanni, Gambino, Andrea, Bergui, Mauro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10179697/
https://www.ncbi.nlm.nih.gov/pubmed/37176727
http://dx.doi.org/10.3390/jcm12093287
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author Bin Wan Hassan, Wan Muhammad Nazief
Mistretta, Francesco
Molinaro, Stefano
Russo, Riccardo
Bosco, Giovanni
Gambino, Andrea
Bergui, Mauro
author_facet Bin Wan Hassan, Wan Muhammad Nazief
Mistretta, Francesco
Molinaro, Stefano
Russo, Riccardo
Bosco, Giovanni
Gambino, Andrea
Bergui, Mauro
author_sort Bin Wan Hassan, Wan Muhammad Nazief
collection PubMed
description Spontaneous intracranial hypotension (SIH) occurs due to a leakage of the cerebrospinal fluid (CSF) lowering the pressure of subarachnoid space, mostly caused by a dural breach or discogenic microspur. As a result of less support provided by CSF pressure, intracranial structures are stretched downward, leading to a constellation of more or less typical MRI findings, including venous congestion, subdural effusions, brainstem sagging and low-lying cerebellar tonsils. Clinic examination and an MRI are usually enough to allow for the diagnosis; however, finding the location of the dural tear is challenging. SIH shares some MRI features with Chiari malformation type I (CM1), especially low-lying cerebellar tonsils. Since SIH is likely underdiagnosed, these findings could be interpreted as signs of CM1, leading to a misdiagnosis and an incorrect treatment pathway. Medical treatment, including steroids, bed rest, hydration caffeine, and a blind epidural blood patch, have been used in this condition with variable success rates. For some years, CSF venous fistulas have been described as the cause of SIH, and a specific diagnostic and therapeutic pathway have been proposed. The current literature on SIH with a focus on diagnosis, treatment, and differential diagnosis with CM1, is reviewed and discussed.
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spelling pubmed-101796972023-05-13 Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation Bin Wan Hassan, Wan Muhammad Nazief Mistretta, Francesco Molinaro, Stefano Russo, Riccardo Bosco, Giovanni Gambino, Andrea Bergui, Mauro J Clin Med Review Spontaneous intracranial hypotension (SIH) occurs due to a leakage of the cerebrospinal fluid (CSF) lowering the pressure of subarachnoid space, mostly caused by a dural breach or discogenic microspur. As a result of less support provided by CSF pressure, intracranial structures are stretched downward, leading to a constellation of more or less typical MRI findings, including venous congestion, subdural effusions, brainstem sagging and low-lying cerebellar tonsils. Clinic examination and an MRI are usually enough to allow for the diagnosis; however, finding the location of the dural tear is challenging. SIH shares some MRI features with Chiari malformation type I (CM1), especially low-lying cerebellar tonsils. Since SIH is likely underdiagnosed, these findings could be interpreted as signs of CM1, leading to a misdiagnosis and an incorrect treatment pathway. Medical treatment, including steroids, bed rest, hydration caffeine, and a blind epidural blood patch, have been used in this condition with variable success rates. For some years, CSF venous fistulas have been described as the cause of SIH, and a specific diagnostic and therapeutic pathway have been proposed. The current literature on SIH with a focus on diagnosis, treatment, and differential diagnosis with CM1, is reviewed and discussed. MDPI 2023-05-05 /pmc/articles/PMC10179697/ /pubmed/37176727 http://dx.doi.org/10.3390/jcm12093287 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Bin Wan Hassan, Wan Muhammad Nazief
Mistretta, Francesco
Molinaro, Stefano
Russo, Riccardo
Bosco, Giovanni
Gambino, Andrea
Bergui, Mauro
Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation
title Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation
title_full Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation
title_fullStr Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation
title_full_unstemmed Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation
title_short Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation
title_sort overview of spontaneous intracranial hypotension and differential diagnosis with chiari i malformation
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10179697/
https://www.ncbi.nlm.nih.gov/pubmed/37176727
http://dx.doi.org/10.3390/jcm12093287
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