American Journal of Neuroradiology最新文献

{"title":"MR Imaging Appearance of Ruptured Rathke Cleft Cyst and Associated Bone Marrow Enhancement.","authors":"Ian T Mark, Christine M Glastonbury","doi":"10.3174/ajnr.A8009","DOIUrl":"10.3174/ajnr.A8009","url":null,"abstract":"<p><p>Rathke cleft cysts are common cystic pituitary lesions seen on MR imaging. A subset of Rathke cleft cysts can rupture within the sella and are uncommon. The imaging appearance of a ruptured Rathke cleft cyst has been previously described with nonspecific imaging findings. We present 7 cases of ruptured Rathke cleft cysts and basisphenoid bone marrow enhancement below the sella that could be used to potentially distinguish a ruptured Rathke cleft cyst from other cystic lesions.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1314-1317"},"PeriodicalIF":3.1,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10631529/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41092598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Factors Predictive of Treatment Success in CT-Guided Fibrin Occlusion of CSF-Venous Fistulas: A Multicenter Retrospective Cross-Sectional Study.","authors":"Andrew L Callen, Lalani Carlton Jones, Vincent M Timpone, Jack Pattee, Daniel J Scoffings, David Butteriss, Thien Huynh, Peter Y Shen, Mark D Mamlouk","doi":"10.3174/ajnr.A8005","DOIUrl":"10.3174/ajnr.A8005","url":null,"abstract":"<p><strong>Background and purpose: </strong>CSF-to-venous fistulas contribute to spontaneous intracranial hypotension. CT-guided fibrin occlusion has been described as a minimally invasive treatment strategy; however, its reproducibility across different institutions remains unclear. This multi-institution study evaluated the clinical and radiologic outcomes of CT-guided fibrin occlusion, hypothesizing a correlation among cure rates, fibrin injectate spread, and drainage patterns.</p><p><strong>Materials and methods: </strong>A retrospective evaluation was conducted on CT-guided fibrin glue treatment in patients with CSF-to-venous fistulas from 6 US and UK institutions from 2020 to 2023. Patient information, procedural characteristics, and injectate spread and drainage patterns were examined. Clinical improvement assessed through medical records served as the primary outcome.</p><p><strong>Results: </strong>Of 119 patients at a mean follow-up of 5.0 months, fibrin occlusion resulted in complete clinical improvement in 59.7%, partial improvement in 34.5%, and no improvement in 5.9% of patients. Complications were reported in 4% of cases. Significant associations were observed between clinical improvement and concordant injectate spread with the fistula drainage pattern (<i>P</i> = .0089) and pretreatment symptom duration (<i>P</i> < .001). No associations were found between clinical improvement and cyst puncture, intravascular extension, rebound headache, body mass index, age, or number of treatment attempts.</p><p><strong>Conclusions: </strong>Fibrin occlusion performed across various institutions shows cure when associated with injectate spread matching the CVF drainage pattern and shorter pretreatment symptom duration, emphasizing the importance of accurate injectate placement and early intervention.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1332-1338"},"PeriodicalIF":3.1,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10631531/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41106822","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Partial (SAVE) versus Complete (Solumbra) Stent Retriever Retraction Technique for Mechanical Thrombectomy: A Randomized In Vitro Study.","authors":"Magda Jablonska, Jiahui Li, Riccardo Tiberi, Pere Canals, Santiago Ortega, Alejandro Tomasello, Marc Ribo","doi":"10.3174/ajnr.A7996","DOIUrl":"10.3174/ajnr.A7996","url":null,"abstract":"<p><strong>Background and purpose: </strong>Mechanical thrombectomy has become a first-line treatment for acute ischemic stroke. Several techniques combining stent retrievers and distal aspiration catheters have been described. We aimed to characterize the efficacy of 2 commonly used techniques according to clot characteristics.</p><p><strong>Materials and methods: </strong>Soft (mean stiffness = 95.77 [SD, 5.80] kPa) or stiff (mean stiffness = 205.63 [SD, 6.70] kPa) clots (3 × 10 mm and 2 × 10 mm, respectively) were embolized to the distal M1 segment of the MCA in an in vitro model. The technique was randomly allocated (1:1): stent retriever assisted vacuum-locked extraction (SAVE) versus complete retraction (Solumbra). The primary end point was the percentage of first-pass recanalization. Secondary end points were periprocedural distal embolization measures.</p><p><strong>Results: </strong>A total of 130 mechanical thrombectomies were performed (50 for soft clots and 15 for stiff clots per arm). Overall, the rate of first-pass recanalizaton was 35% with Solumbra and 15% with SAVE (<i>P </i>< .01). For stiff clots, the first-pass recanalizaton was equal for both methods (27%; <i>P</i> = 1.00). With soft clots, the first-pass recanalizaton was higher with Solumbra (38%) than with SAVE (12%; <i>P </i>< .01). When we used soft clots, the maximum embolus size (mean, 1.19 [SD, 0.9] mm versus 2.16 [SD, 1.48] mm; <i>P </i>< .01) and total area of emboli (mean, 1.82 [SD, 2.73] versus 3.34 [SD, 3.2]; <i>P </i>= .01) were also lower with Solumbra than with SAVE.</p><p><strong>Conclusions: </strong>Clot characteristics may influence the efficacy of the thrombectomy technique. In occlusions caused by soft clots, complete retrieval into the distal aspiration catheters achieved higher rates of first-pass recanalizaton and lower embolization.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1165-1170"},"PeriodicalIF":3.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10549948/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10234208","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuroimaging Findings in Axenfeld-Rieger Syndrome: A Case Series.","authors":"Samuel White, Ajay Taranath, Prasad Hanagandi, Deepa A Taranath, Minh-Son To, Emmanuelle Souzeau, Owen M Siggs, Jamie E Craig","doi":"10.3174/ajnr.A7995","DOIUrl":"10.3174/ajnr.A7995","url":null,"abstract":"<p><p>Axenfeld-Rieger syndrome is an autosomal dominant condition associated with multisystemic features including developmental anomalies of the anterior segment of the eye. Single nucleotide and copy number variants in the paired-like homeodomain transcription factor 2 (<i>PITX2</i>) and forkhead box C1 (<i>FOXC1</i>) genes are associated with Axenfeld-Rieger syndrome as well as other CNS malformations. We determined the association between Axenfeld-Rieger syndrome and specific brain MR imaging neuroradiologic anomalies in cases with or without a genetic diagnosis. This case series included 8 individuals with pathogenic variants in <i>FOXC1</i>; 2, in <i>PITX2</i>; and 2 without a genetic diagnosis. The most common observation was vertebrobasilar artery dolichoectasia, with 46% prevalence. Other prevalent abnormalities included WM hyperintensities, cerebellar hypoplasia, and ventriculomegaly. Vertebrobasilar artery dolichoectasia and absent/hypoplastic olfactory bulbs were reported in >50% of individuals with <i>FOXC1</i> variants compared with 0% of <i>PITX2</i> variants. Notwithstanding the small sample size, neuroimaging abnormalities were more prevalent in individuals with <i>FOXC1</i> variants compared those with <i>PITX2</i> variants.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1231-1235"},"PeriodicalIF":3.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10549946/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10238338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Peritumoral Signal on Postcontrast FLAIR Images: Description and Proposed Biomechanism in Vestibular Schwannomas.","authors":"John C Benson, Matthew L Carlson, John I Lane","doi":"10.3174/ajnr.A7979","DOIUrl":"10.3174/ajnr.A7979","url":null,"abstract":"<p><strong>Background and purpose: </strong>Anecdotally, postcontrast FLAIR images of vestibular schwannomas can show peritumoral hyperintense signal, hypothesized to represent gadolinium extravasation. This study assessed the incidence of this phenomenon in a cohort of patients with treatment-naïve sporadic vestibular schwannomas.</p><p><strong>Materials and methods: </strong>A retrospective review of 20 patients with presumed vestibular schwannoma based on characteristic imaging findings and with dedicated internal auditory canal imaging, including postcontrast T1W1 and postcontrast FLAIR, was performed. Tumor size and location were recorded, as was the presence or absence of a fundal fluid cleft. Images were reviewed for the presence of peritumoral hyperintense signal on FLAIR images (a \"halo\") and for both subjectively and objectively elevated signal in the ipsilateral cochlea and fundus.</p><p><strong>Results: </strong>Patients were randomly selected from an institutional vestibular schwannoma registry. Eleven (55.0%) were women. A peritumoral halo was present in 90% of patients, averaging 1.0 (SD, 0.2) mm in thickness. The maximum mean FLAIR signal in the ipsilateral fundus (205.9 [SD, 110.2]) was significantly greater than on the contralateral side (121.6 [SD, 27.8]) (<i>P </i>= .02). Maximum mean ipsilateral intracochlear signal (167.8 [SD, 104.5]) was also significantly greater than on the contralateral side (113.4 [SD, 40.1]) (<i>P </i>= .04).</p><p><strong>Conclusions: </strong>A peritumoral halo on postcontrast FLAIR images was present in 90% of our cohort with randomly selected, treatment-naïve sporadic vestibular schwannomas. Although its mechanism is unknown, this signal is hypothesized to represent gadolinium extravasation, given an ipsilateral increased signal in the adjacent internal auditory canal fundus and cochlea.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1171-1175"},"PeriodicalIF":3.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10549947/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10131522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Systematic Literature Review of Machine Learning Algorithms Using Pretherapy Radiologic Imaging for Glioma Molecular Subtype Prediction.","authors":"Jan Lost, Tej Verma, Leon Jekel, Marc von Reppert, Niklas Tillmanns, Sara Merkaj, Gabriel Cassinelli Petersen, Ryan Bahar, Ayyüce Gordem, Muhammad A Haider, Harry Subramanian, Waverly Brim, Ichiro Ikuta, Antonio Omuro, Gian Marco Conte, Bernadette V Marquez-Nostra, Arman Avesta, Khaled Bousabarah, Ali Nabavizadeh, Anahita Fathi Kazerooni, Sanjay Aneja, Spyridon Bakas, MingDe Lin, Michael Sabel, Mariam Aboian","doi":"10.3174/ajnr.A8000","DOIUrl":"10.3174/ajnr.A8000","url":null,"abstract":"<p><strong>Background: </strong>The molecular profile of gliomas is a prognostic indicator for survival, driving clinical decision-making for treatment. Pathology-based molecular diagnosis is challenging because of the invasiveness of the procedure, exclusion from neoadjuvant therapy options, and the heterogeneous nature of the tumor.</p><p><strong>Purpose: </strong>We performed a systematic review of algorithms that predict molecular subtypes of gliomas from MR Imaging.</p><p><strong>Data sources: </strong>Data sources were Ovid Embase, Ovid MEDLINE, Cochrane Central Register of Controlled Trials, Web of Science.</p><p><strong>Study selection: </strong>Per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, 12,318 abstracts were screened and 1323 underwent full-text review, with 85 articles meeting the inclusion criteria.</p><p><strong>Data analysis: </strong>We compared prediction results from different machine learning approaches for predicting molecular subtypes of gliomas. Bias analysis was conducted for each study, following the Prediction model Risk Of Bias Assessment Tool (PROBAST) guidelines.</p><p><strong>Data synthesis: </strong>Isocitrate dehydrogenase mutation status was reported with an area under the curve and accuracy of 0.88 and 85% in internal validation and 0.86 and 87% in limited external validation data sets, respectively. For the prediction of <i>O6-methylguanine-DNA methyltransferase</i> promoter methylation, the area under the curve and accuracy in internal validation data sets were 0.79 and 77%, and in limited external validation, 0.89 and 83%, respectively. PROBAST scoring demonstrated high bias in all articles.</p><p><strong>Limitations: </strong>The low number of external validation and studies with incomplete data resulted in unequal data analysis. Comparing the best prediction pipelines of each study may introduce bias.</p><p><strong>Conclusions: </strong>While the high area under the curve and accuracy for the prediction of molecular subtypes of gliomas are reported in internal and external validation data sets, limited use of external validation and the increased risk of bias in all articles may present obstacles for clinical translation of these techniques.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":"44 10","pages":"1126-1134"},"PeriodicalIF":3.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10549943/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41096975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Update from the 5th Edition of the WHO Classification of Nasal, Paranasal, and Skull Base Tumors: Imaging Overview with Histopathologic and Genetic Correlation.","authors":"A Agarwal, A A Bhatt, G Bathla, S Kanekar, N Soni, J Murray, K Vijay, P Vibhute, P H Rhyner","doi":"10.3174/ajnr.A7960","DOIUrl":"10.3174/ajnr.A7960","url":null,"abstract":"<p><p>Sinonasal and skull base tumors are a heterogeneous group of neoplasms with considerable histologic variation and overlapping imaging features. In 2022, the World Health Organization updated the head and neck tumor classification, further emphasizing the importance of molecular data and genetic alterations in sinonasal neoplasms. The changes include the addition of new entities and discussion of emerging entities, as well as changes to the taxonomy and characterization of tumors. The new classification focuses on entities that develop in these sites either exclusively (eg, olfactory neuroblastoma) or most frequently. Another change includes reduction in the number of categories by creating separate category-specific chapters for soft-tissue, hematolymphoid, and neuroectodermal lesions. In this review, we briefly discuss the various categories in the new classification with a more detailed description of the 2 new entities (SWItch/Sucrose Non-Fermentable complex-deficient sinonasal carcinomas and human papillomavirus-related multiphenotypic sinonasal carcinoma). We also highlight the emerging entities including <i>IDH</i>-mutant sinonasal malignancies and <i>DEK-AFF2</i> carcinoma, presently classified as sinonasal undifferentiated carcinoma and nonkeratinizing squamous cell carcinoma, respectively.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1116-1125"},"PeriodicalIF":3.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10549938/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10021552","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Performance of Neck Imaging Reporting and Data System (NI-RADS) for Diagnosis of Recurrence of Head and Neck Squamous Cell Carcinoma: A Systematic Review and Meta-analysis.","authors":"Akira Baba, Ryo Kurokawa, Mariko Kurokawa, Takafumi Yanagisawa, Ashok Srinivasan","doi":"10.3174/ajnr.A7992","DOIUrl":"10.3174/ajnr.A7992","url":null,"abstract":"<p><strong>Background: </strong>The Neck Imaging Reporting and Data System (NI-RADS) is a reporting template used in head and neck cancer posttreatment follow-up imaging.</p><p><strong>Purpose: </strong>Our aim was to evaluate the pooled detection rates of the recurrence of head and neck squamous cell carcinoma based on each NI-RADS category and to compare the diagnostic accuracy between NI-RADS 2 and 3 cutoffs.</p><p><strong>Data sources: </strong>The MEDLINE, Scopus, and EMBASE databases were searched.</p><p><strong>Study selection: </strong>This systematic review identified 7 studies with a total of 694 patients (1233 lesions) that were eligible for the meta-analysis.</p><p><strong>Data analysis: </strong>The meta-analysis of pooled recurrence detection rate estimates for each NI-RADS category and the diagnostic accuracy of recurrence with NI-RADS 3 or 2 as the cutoff was performed.</p><p><strong>Data synthesis: </strong>The estimated recurrence rates in each category for primary lesions were 74.4% for NI-RADS 3, 29.0% for NI-RADS 2, and 4.2% for NI-RADS 1. The estimated recurrence rates in each category for cervical lymph nodes were 73.3% for NI-RADS 3, 14.3% for NI-RADS 2, and 3.5% for NI-RADS 1. The area under the curve of the summary receiver operating characteristic for recurrence detection with NI-RADS 3 as the cutoff was 0.887 and 0.983, respectively, higher than 0.869 and 0.919 for the primary sites and cervical lymph nodes, respectively, with NI-RADS 2 as the cutoff.</p><p><strong>Limitations: </strong>Given the heterogeneity of the data of the studies, the conclusions should be interpreted with caution.</p><p><strong>Conclusions: </strong>This meta-analysis revealed estimated recurrence rates for each NI-RADS category for primary lesions and cervical lymph nodes and showed that NI-RADS 3 has a high diagnostic performance for detecting recurrence.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1184-1190"},"PeriodicalIF":3.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10549942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10234209","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dandy-Walker Phenotype with Brainstem Involvement: 2 Distinct Subgroups with Different Prognosis.","authors":"C A P F Alves, J Sidpra, A Manteghinejad, S Sudhakar, F V Massey, K A Aldinger, P Haldipur, L T Lucato, S F Ferraciolli, S R Teixeira, Ö Öztekin, D Bhattacharya, A Taranath, S P Prabhu, D M Mirsky, S Andronikou, K J Millen, A J Barkovich, E Boltshauser, W B Dobyns, M J Barkovich, M T Whitehead, K Mankad","doi":"10.3174/ajnr.A7967","DOIUrl":"10.3174/ajnr.A7967","url":null,"abstract":"<p><strong>Background and purpose: </strong>Although cardinal imaging features for the diagnostic criteria of the Dandy-Walker phenotype have been recently defined, there is a large range of unreported malformations among these patients. The brainstem, in particular, deserves careful attention because malformations in this region have potentially important implications for clinical outcomes. In this article, we offer detailed information on the association of brainstem dysgenesis in a large, multicentric cohort of patients with the Dandy-Walker phenotype, defining different subtypes of involvement and their potential clinical impact.</p><p><strong>Materials and methods: </strong>In this established multicenter cohort of 329 patients with the Dandy-Walker phenotype, we include and retrospectively review the MR imaging studies and clinical records of 73 subjects with additional brainstem malformations. Detailed evaluation of the different patterns of brainstem involvement and their potential clinical implications, along with comparisons between posterior fossa measurements for the diagnosis of the Dandy-Walker phenotype, was performed among the different subgroups of patients with brainstem involvement.</p><p><strong>Results: </strong>There were 2 major forms of brainstem involvement in patients with Dandy-Walker phenotype including the following: 1) the mild form with anteroposterior disproportions of the brainstem structures \"only\" (57/73; 78%), most frequently with pontine hypoplasia (44/57; 77%), and 2) the severe form with patients with tegmental dysplasia with folding, bumps, and/or clefts (16/73; 22%). Patients with severe forms of brainstem malformation had significantly increased rates of massive ventriculomegaly, additional malformations involving the corpus callosum and gray matter, and interhemispheric cysts. Clinically, patients with the severe form had significantly increased rates of bulbar dysfunction, seizures, and mortality.</p><p><strong>Conclusions: </strong>Additional brainstem malformations in patients with the Dandy-Walker phenotype can be divided into 2 major subgroups: mild and severe. The severe form, though less prevalent, has characteristic imaging features, including tegmental folding, bumps, and clefts, and is directly associated with a more severe clinical presentation and increased mortality.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1201-1207"},"PeriodicalIF":3.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10549954/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10021542","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Venous Sinus Stenosis with Prominent Emissary Veins: A New Common Cranial MRI Finding of Mucopolysaccharidosis I.","authors":"Shiwei Huang, Ashish Gupta, Paul Orchard, Troy Lund, David Nascene","doi":"10.3174/ajnr.A7997","DOIUrl":"10.3174/ajnr.A7997","url":null,"abstract":"<p><p>Mucopolysaccharidosis I-Hurler (MPSIH) syndrome is the most severe form of a group of hereditary lysosomal diseases. This study aims to describe previously unreported common cranial findings of sigmoid sinus stenosis with prominent emissary veins in MPSIH. A retrospective review was conducted of 66 patients with MPSIH who were treated at our institution. A total of 12 cranial MR imaging studies from 12 different patients demonstrating the venous sinus anatomy were reviewed. All 12 patients exhibited various degrees of sigmoid or transverse sinus stenosis. Eleven had various forms of emissary veins. Of those 12 patients with imaging of the venous sinuses, 9 had a lumbar puncture within the same months as the acquisition of the venogram without any correlation between elevated opening pressure and the severity of the venous sinus stenosis. Stenotic cerebral venous sinuses with associated emissary veins, common in patients with MPSIH, may be abnormal findings due to posterior fossa horns from glycosaminoglycan depositions rather than signs of elevated intracracranial pressure or requirement of CSF diversion.</p>","PeriodicalId":7875,"journal":{"name":"American Journal of Neuroradiology","volume":" ","pages":"1236-1239"},"PeriodicalIF":3.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10549949/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10238337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}