Reappraising the Tolosa‐Hunt Syndrome Diagnostic Criteria: A Case Series

Introduction Tolosa‐Hunt syndrome (THS), a condition characterized by painful ophthalmoplegia and accompanied by cranial nerve palsies, remains as a diagnosis of exclusion. Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic crite...

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Veröffentlicht in:Headache 2020-01, Vol.60 (1), p.259-264
Hauptverfasser: Mullen, Evan, Rutland, John W., Green, Mark W., Bederson, Joshua, Shrivastava, Raj
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container_title Headache
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creator Mullen, Evan
Rutland, John W.
Green, Mark W.
Bederson, Joshua
Shrivastava, Raj
description Introduction Tolosa‐Hunt syndrome (THS), a condition characterized by painful ophthalmoplegia and accompanied by cranial nerve palsies, remains as a diagnosis of exclusion. Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic criteria to require the demonstration of granulomatous inflammation on magnetic resonance imaging or biopsy. We sought to assess the effectiveness of the new criteria in arriving at accurate diagnoses. Methods We extracted all patient charts from our institution’s electronic medical record associated with ICD‐9 code 378.55 (external ophthalmoplegia). We then completed the retrospective diagnostic workups to determine if subjects met ICHD‐3 criteria for THS and compared our findings with their final diagnoses. Results Of 62 patients associated with ICD‐9 code 378.55, 10 (16%) was identified to have presenting symptoms concerning THS. The average age at the first onset of THS‐like symptoms was 58 years. Five of the 10 (50%) met ICHD‐3 criteria for THS. Two of the 5 (40%) meeting ICHD‐3 criteria for THS were discovered to have other diagnoses. Two of the 5 (40%) patients not meeting ICHD‐3 criteria for THS nevertheless received a final diagnosis of THS. Discussion Our false‐negative rate of 40% is consistent with previous case series found in the literature. Our false‐positive rate of 40% is, to our knowledge, a new contribution to the literature as no other case series includes more than a single false‐positive. The false‐positive rate is most concerning, as a preliminary misdiagnosis of THS can delay treatment tailored to the true etiology. Furthermore, infectious etiologies can be exacerbated with steroid treatment. Conclusion Our case series suggests that ICHD‐3 criteria are suboptimal for the accurate diagnosis of THS. We recommend a close follow‐up for all patients with symptoms concerning THS until a definitive responsible etiology is discovered and we encourage further studies assessing ICHD‐3 guidelines to optimize their sensitivity and specificity in the diagnosis of THS.
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Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic criteria to require the demonstration of granulomatous inflammation on magnetic resonance imaging or biopsy. We sought to assess the effectiveness of the new criteria in arriving at accurate diagnoses. Methods We extracted all patient charts from our institution’s electronic medical record associated with ICD‐9 code 378.55 (external ophthalmoplegia). We then completed the retrospective diagnostic workups to determine if subjects met ICHD‐3 criteria for THS and compared our findings with their final diagnoses. Results Of 62 patients associated with ICD‐9 code 378.55, 10 (16%) was identified to have presenting symptoms concerning THS. The average age at the first onset of THS‐like symptoms was 58 years. Five of the 10 (50%) met ICHD‐3 criteria for THS. Two of the 5 (40%) meeting ICHD‐3 criteria for THS were discovered to have other diagnoses. Two of the 5 (40%) patients not meeting ICHD‐3 criteria for THS nevertheless received a final diagnosis of THS. Discussion Our false‐negative rate of 40% is consistent with previous case series found in the literature. Our false‐positive rate of 40% is, to our knowledge, a new contribution to the literature as no other case series includes more than a single false‐positive. The false‐positive rate is most concerning, as a preliminary misdiagnosis of THS can delay treatment tailored to the true etiology. Furthermore, infectious etiologies can be exacerbated with steroid treatment. Conclusion Our case series suggests that ICHD‐3 criteria are suboptimal for the accurate diagnosis of THS. We recommend a close follow‐up for all patients with symptoms concerning THS until a definitive responsible etiology is discovered and we encourage further studies assessing ICHD‐3 guidelines to optimize their sensitivity and specificity in the diagnosis of THS.</description><identifier>ISSN: 0017-8748</identifier><identifier>EISSN: 1526-4610</identifier><identifier>DOI: 10.1111/head.13692</identifier><identifier>PMID: 31681980</identifier><language>eng</language><publisher>HOBOKEN: Wiley</publisher><subject>Adult ; Age of Onset ; Aged ; Aged, 80 and over ; Biopsy ; cavernous sinus syndrome ; Clinical Neurology ; corticosteroids ; Cranial nerves ; Criteria ; Diagnosis ; Diagnostic systems ; diagnostic criteria ; Electronic health records ; Electronic medical records ; Etiology ; Female ; Headache ; Humans ; Life Sciences &amp; Biomedicine ; Magnetic resonance imaging ; Middle Aged ; Neurosciences &amp; Neurology ; Ophthalmoplegia ; Ophthalmoplegia - diagnosis ; Ophthalmoplegia - etiology ; painful ophthalmoplegia ; Patients ; Practice Guidelines as Topic - standards ; Retrospective Studies ; Science &amp; Technology ; Sensitivity analysis ; Sensitivity and Specificity ; Signs and symptoms ; Steroids ; Tolosa-Hunt Syndrome - diagnosis ; Tolosa-Hunt Syndrome - etiology</subject><ispartof>Headache, 2020-01, Vol.60 (1), p.259-264</ispartof><rights>2019 American Headache Society</rights><rights>2019 American Headache Society.</rights><rights>2020 American Headache Society</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>true</woscitedreferencessubscribed><woscitedreferencescount>8</woscitedreferencescount><woscitedreferencesoriginalsourcerecordid>wos000506067400026</woscitedreferencesoriginalsourcerecordid><citedby>FETCH-LOGICAL-c3572-34ad13fa45b058c4966dd72111b47617ac92041603e8eb75ef494a1605686f583</citedby><cites>FETCH-LOGICAL-c3572-34ad13fa45b058c4966dd72111b47617ac92041603e8eb75ef494a1605686f583</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fhead.13692$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fhead.13692$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>315,781,785,1418,27929,27930,28253,45579,45580</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31681980$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Mullen, Evan</creatorcontrib><creatorcontrib>Rutland, John W.</creatorcontrib><creatorcontrib>Green, Mark W.</creatorcontrib><creatorcontrib>Bederson, Joshua</creatorcontrib><creatorcontrib>Shrivastava, Raj</creatorcontrib><title>Reappraising the Tolosa‐Hunt Syndrome Diagnostic Criteria: A Case Series</title><title>Headache</title><addtitle>HEADACHE</addtitle><addtitle>Headache</addtitle><description>Introduction Tolosa‐Hunt syndrome (THS), a condition characterized by painful ophthalmoplegia and accompanied by cranial nerve palsies, remains as a diagnosis of exclusion. Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic criteria to require the demonstration of granulomatous inflammation on magnetic resonance imaging or biopsy. We sought to assess the effectiveness of the new criteria in arriving at accurate diagnoses. Methods We extracted all patient charts from our institution’s electronic medical record associated with ICD‐9 code 378.55 (external ophthalmoplegia). We then completed the retrospective diagnostic workups to determine if subjects met ICHD‐3 criteria for THS and compared our findings with their final diagnoses. Results Of 62 patients associated with ICD‐9 code 378.55, 10 (16%) was identified to have presenting symptoms concerning THS. The average age at the first onset of THS‐like symptoms was 58 years. Five of the 10 (50%) met ICHD‐3 criteria for THS. Two of the 5 (40%) meeting ICHD‐3 criteria for THS were discovered to have other diagnoses. Two of the 5 (40%) patients not meeting ICHD‐3 criteria for THS nevertheless received a final diagnosis of THS. Discussion Our false‐negative rate of 40% is consistent with previous case series found in the literature. Our false‐positive rate of 40% is, to our knowledge, a new contribution to the literature as no other case series includes more than a single false‐positive. The false‐positive rate is most concerning, as a preliminary misdiagnosis of THS can delay treatment tailored to the true etiology. Furthermore, infectious etiologies can be exacerbated with steroid treatment. Conclusion Our case series suggests that ICHD‐3 criteria are suboptimal for the accurate diagnosis of THS. We recommend a close follow‐up for all patients with symptoms concerning THS until a definitive responsible etiology is discovered and we encourage further studies assessing ICHD‐3 guidelines to optimize their sensitivity and specificity in the diagnosis of THS.</description><subject>Adult</subject><subject>Age of Onset</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Biopsy</subject><subject>cavernous sinus syndrome</subject><subject>Clinical Neurology</subject><subject>corticosteroids</subject><subject>Cranial nerves</subject><subject>Criteria</subject><subject>Diagnosis</subject><subject>Diagnostic systems</subject><subject>diagnostic criteria</subject><subject>Electronic health records</subject><subject>Electronic medical records</subject><subject>Etiology</subject><subject>Female</subject><subject>Headache</subject><subject>Humans</subject><subject>Life Sciences &amp; Biomedicine</subject><subject>Magnetic resonance imaging</subject><subject>Middle Aged</subject><subject>Neurosciences &amp; 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Neurology</topic><topic>Ophthalmoplegia</topic><topic>Ophthalmoplegia - diagnosis</topic><topic>Ophthalmoplegia - etiology</topic><topic>painful ophthalmoplegia</topic><topic>Patients</topic><topic>Practice Guidelines as Topic - standards</topic><topic>Retrospective Studies</topic><topic>Science &amp; Technology</topic><topic>Sensitivity analysis</topic><topic>Sensitivity and Specificity</topic><topic>Signs and symptoms</topic><topic>Steroids</topic><topic>Tolosa-Hunt Syndrome - diagnosis</topic><topic>Tolosa-Hunt Syndrome - etiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Mullen, Evan</creatorcontrib><creatorcontrib>Rutland, John W.</creatorcontrib><creatorcontrib>Green, Mark W.</creatorcontrib><creatorcontrib>Bederson, Joshua</creatorcontrib><creatorcontrib>Shrivastava, Raj</creatorcontrib><collection>Web of Science - Science Citation Index Expanded - 2020</collection><collection>Web of Science Core Collection</collection><collection>Science Citation Index Expanded</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>Toxicology Abstracts</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest Health &amp; Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>Headache</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mullen, Evan</au><au>Rutland, John W.</au><au>Green, Mark W.</au><au>Bederson, Joshua</au><au>Shrivastava, Raj</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Reappraising the Tolosa‐Hunt Syndrome Diagnostic Criteria: A Case Series</atitle><jtitle>Headache</jtitle><stitle>HEADACHE</stitle><addtitle>Headache</addtitle><date>2020-01</date><risdate>2020</risdate><volume>60</volume><issue>1</issue><spage>259</spage><epage>264</epage><pages>259-264</pages><issn>0017-8748</issn><eissn>1526-4610</eissn><abstract>Introduction Tolosa‐Hunt syndrome (THS), a condition characterized by painful ophthalmoplegia and accompanied by cranial nerve palsies, remains as a diagnosis of exclusion. Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic criteria to require the demonstration of granulomatous inflammation on magnetic resonance imaging or biopsy. We sought to assess the effectiveness of the new criteria in arriving at accurate diagnoses. Methods We extracted all patient charts from our institution’s electronic medical record associated with ICD‐9 code 378.55 (external ophthalmoplegia). We then completed the retrospective diagnostic workups to determine if subjects met ICHD‐3 criteria for THS and compared our findings with their final diagnoses. Results Of 62 patients associated with ICD‐9 code 378.55, 10 (16%) was identified to have presenting symptoms concerning THS. The average age at the first onset of THS‐like symptoms was 58 years. Five of the 10 (50%) met ICHD‐3 criteria for THS. Two of the 5 (40%) meeting ICHD‐3 criteria for THS were discovered to have other diagnoses. Two of the 5 (40%) patients not meeting ICHD‐3 criteria for THS nevertheless received a final diagnosis of THS. Discussion Our false‐negative rate of 40% is consistent with previous case series found in the literature. Our false‐positive rate of 40% is, to our knowledge, a new contribution to the literature as no other case series includes more than a single false‐positive. The false‐positive rate is most concerning, as a preliminary misdiagnosis of THS can delay treatment tailored to the true etiology. Furthermore, infectious etiologies can be exacerbated with steroid treatment. Conclusion Our case series suggests that ICHD‐3 criteria are suboptimal for the accurate diagnosis of THS. We recommend a close follow‐up for all patients with symptoms concerning THS until a definitive responsible etiology is discovered and we encourage further studies assessing ICHD‐3 guidelines to optimize their sensitivity and specificity in the diagnosis of THS.</abstract><cop>HOBOKEN</cop><pub>Wiley</pub><pmid>31681980</pmid><doi>10.1111/head.13692</doi><tpages>6</tpages></addata></record>
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subjects Adult
Age of Onset
Aged
Aged, 80 and over
Biopsy
cavernous sinus syndrome
Clinical Neurology
corticosteroids
Cranial nerves
Criteria
Diagnosis
Diagnostic systems
diagnostic criteria
Electronic health records
Electronic medical records
Etiology
Female
Headache
Humans
Life Sciences & Biomedicine
Magnetic resonance imaging
Middle Aged
Neurosciences & Neurology
Ophthalmoplegia
Ophthalmoplegia - diagnosis
Ophthalmoplegia - etiology
painful ophthalmoplegia
Patients
Practice Guidelines as Topic - standards
Retrospective Studies
Science & Technology
Sensitivity analysis
Sensitivity and Specificity
Signs and symptoms
Steroids
Tolosa-Hunt Syndrome - diagnosis
Tolosa-Hunt Syndrome - etiology
title Reappraising the Tolosa‐Hunt Syndrome Diagnostic Criteria: A Case Series
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