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Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base

OBJECTIVE: To estimate the prevalence and significance of cranial nerve (CN) imaging abnormalities in patients with hereditary neuropathy and discuss clinical implications. METHODS: We retrospectively analyzed data from patients at four tertiary academic medical centers with hereditary neuropathy di...

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Autores principales: Bartholomew, Ryan A., Zamani, Amir A., Kim, Grace S., Alyono, Jennifer C., Steinert, Haley, Fridman, Vera, Sadjadi, Reza, Jackler, Robert K., Corrales, C. Eduardo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7042653/
https://www.ncbi.nlm.nih.gov/pubmed/32128425
http://dx.doi.org/10.1002/lio2.343
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author Bartholomew, Ryan A.
Zamani, Amir A.
Kim, Grace S.
Alyono, Jennifer C.
Steinert, Haley
Fridman, Vera
Sadjadi, Reza
Jackler, Robert K.
Corrales, C. Eduardo
author_facet Bartholomew, Ryan A.
Zamani, Amir A.
Kim, Grace S.
Alyono, Jennifer C.
Steinert, Haley
Fridman, Vera
Sadjadi, Reza
Jackler, Robert K.
Corrales, C. Eduardo
author_sort Bartholomew, Ryan A.
collection PubMed
description OBJECTIVE: To estimate the prevalence and significance of cranial nerve (CN) imaging abnormalities in patients with hereditary neuropathy and discuss clinical implications. METHODS: We retrospectively analyzed data from patients at four tertiary academic medical centers with hereditary neuropathy diagnoses who had undergone gadolinium‐enhanced magnetic resonance imaging (MRI) of the brain or skull base between 2004 and 2018. MRI scans, as well as computed tomography imaging when available, were reviewed and bivariable analysis was performed to identify predictors of CN abnormalities on imaging. RESULTS: Among 39 patients meeting study criteria, 11 had clinical CN deficits (28%) and 8 had CN abnormalities on imaging (21%). Of the patients with CN abnormalities on imaging, half had CN deficits (4/8) and only a quarter had imaging abnormalities of the CNs with the deficits (2/8). Imaging abnormalities were found in varied CNs, including CNs III, V, VII, and the VII/VIII complex in the internal auditory canal. MRI obtained for the purpose of evaluating CN deficits had a statistically significant increased likelihood of containing CN imaging abnormalities. However, CN deficits themselves were not predictive of imaging abnormalities. CONCLUSION: Thickening and enhancement of CNs on MRI may be found in approximately 1/5 of patients with hereditary neuropathies and are inconsistently associated with clinical deficits. These imaging findings should not be mistaken for neoplastic and infectious processes as they may be manifestations of the patients' underlying genetic neuropathy. LEVEL OF EVIDENCE: 4.
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spelling pubmed-70426532020-03-03 Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base Bartholomew, Ryan A. Zamani, Amir A. Kim, Grace S. Alyono, Jennifer C. Steinert, Haley Fridman, Vera Sadjadi, Reza Jackler, Robert K. Corrales, C. Eduardo Laryngoscope Investig Otolaryngol COMPREHENSIVE (GENERAL) OTOLARYNGOLOGY OBJECTIVE: To estimate the prevalence and significance of cranial nerve (CN) imaging abnormalities in patients with hereditary neuropathy and discuss clinical implications. METHODS: We retrospectively analyzed data from patients at four tertiary academic medical centers with hereditary neuropathy diagnoses who had undergone gadolinium‐enhanced magnetic resonance imaging (MRI) of the brain or skull base between 2004 and 2018. MRI scans, as well as computed tomography imaging when available, were reviewed and bivariable analysis was performed to identify predictors of CN abnormalities on imaging. RESULTS: Among 39 patients meeting study criteria, 11 had clinical CN deficits (28%) and 8 had CN abnormalities on imaging (21%). Of the patients with CN abnormalities on imaging, half had CN deficits (4/8) and only a quarter had imaging abnormalities of the CNs with the deficits (2/8). Imaging abnormalities were found in varied CNs, including CNs III, V, VII, and the VII/VIII complex in the internal auditory canal. MRI obtained for the purpose of evaluating CN deficits had a statistically significant increased likelihood of containing CN imaging abnormalities. However, CN deficits themselves were not predictive of imaging abnormalities. CONCLUSION: Thickening and enhancement of CNs on MRI may be found in approximately 1/5 of patients with hereditary neuropathies and are inconsistently associated with clinical deficits. These imaging findings should not be mistaken for neoplastic and infectious processes as they may be manifestations of the patients' underlying genetic neuropathy. LEVEL OF EVIDENCE: 4. John Wiley & Sons, Inc. 2020-01-10 /pmc/articles/PMC7042653/ /pubmed/32128425 http://dx.doi.org/10.1002/lio2.343 Text en © 2020 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals, Inc. on behalf of The Triological Society. This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle COMPREHENSIVE (GENERAL) OTOLARYNGOLOGY
Bartholomew, Ryan A.
Zamani, Amir A.
Kim, Grace S.
Alyono, Jennifer C.
Steinert, Haley
Fridman, Vera
Sadjadi, Reza
Jackler, Robert K.
Corrales, C. Eduardo
Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base
title Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base
title_full Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base
title_fullStr Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base
title_full_unstemmed Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base
title_short Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base
title_sort prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: clinical implications at the skull base
topic COMPREHENSIVE (GENERAL) OTOLARYNGOLOGY
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7042653/
https://www.ncbi.nlm.nih.gov/pubmed/32128425
http://dx.doi.org/10.1002/lio2.343
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