Clinical and imaging features of parenchymal and non-parenchymal neuro-Behçet’s disease: a systematic review of case reports and series
Abstract Background Neuro-Behçet’s Disease (NBD) is a serious neurological complication of Behçet’s Disease, but there’s still a lack of research on its early-stage symptoms and imaging findings. While previous research highlights MRI abnormalities and cerebrospinal fluid changes, there hasn’t been...
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2025-05-01
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| author | Omar Al-omoush Majd M. AlBarakat Deya’a Alasmar Ali Al-Khalaileh Ahmad Alzoubi Husam Tarakhan Zaid Shakhatreh Zaid Hatamleh Mohammed Abu-saleh Othman Saleh Abdalrahman Altiti Wafa’ A. Hazaymeh Sakhr Alshwayyat Hamdah Hanifa Ethar Hazaimeh |
| author_facet | Omar Al-omoush Majd M. AlBarakat Deya’a Alasmar Ali Al-Khalaileh Ahmad Alzoubi Husam Tarakhan Zaid Shakhatreh Zaid Hatamleh Mohammed Abu-saleh Othman Saleh Abdalrahman Altiti Wafa’ A. Hazaymeh Sakhr Alshwayyat Hamdah Hanifa Ethar Hazaimeh |
| author_sort | Omar Al-omoush |
| collection | DOAJ |
| description | Abstract Background Neuro-Behçet’s Disease (NBD) is a serious neurological complication of Behçet’s Disease, but there’s still a lack of research on its early-stage symptoms and imaging findings. While previous research highlights MRI abnormalities and cerebrospinal fluid changes, there hasn’t been a systematic effort to identify patterns specific to newly diagnosed patients. Our study aimed to investigate the clinical and imaging characteristics of newly diagnosed parenchymal and non-parenchymal NBD based on case reports and case series studies. Methods We synthesized case reports and case series from MEDLINE, Scopus, EMBASE, PubMed, Web of Science, and Cochrane Library until October 17th,2023. Multiple statistical tests, such as the chi-square and Mann-Whitney tests, were used, with the corresponding 95% confidence interval (CI). The study protocol was registered with PROSPERO (ID: CRD42023472436). We included case reports and case series of adult patients (≥ 18 years) with a confirmed diagnosis of parenchymal or non-parenchymal Neuro-Behçet’s disease (NBD), based on neurological symptoms attributed to Behçet’s disease. Studies were required to report MRI findings demonstrating central or peripheral nervous system involvement. Results Our analysis of 107 case reports and a case series (n = 174 patients) revealed that parenchymal NBD patients (n = 141; mean age 35.54 ± 13.33 years) frequently presented with ocular manifestations (80.9%), pyramidal signs (57.5%), cranial nerve palsies (50.4%), and headache (32.6%) while non-parenchymal NBD patients (n = 19; mean age 28.68 ± 10.67 years) commonly experienced headache (86.4%), papilledema (47.4%), cranial nerve palsies (42.1%), and nausea (31.6%). For the mixed-type group (n = 14; mean age 27.5 ± 11.57 years), commonly presented with headache (64.3%), pyramidal sign (50%), fever (50%), and diplopia (35.7%). Brainstem involvement for parenchymal NBD predominated in acute and chronic cases. Contrast enhancement was significantly more prevalent in acute NBD compared to chronic progressive NBD. Conclusion Neuro-Behçet’s disease is an uncommon but serious medical condition with varying symptoms based on its type. Parenchymal and non-parenchymal NBD have unique patterns, with parenchymal cases exhibiting primarily ocular symptoms and pyramidal signs, while non-parenchymal cases present with headache and papilledema. Patients with both types of features were more likely to experience headaches, pyramidal symptoms, fever, and diplopia. Recognizing these patterns can help diagnose and treat the disease earlier. |
| format | Article |
| id | doaj-art-7fb03c9b23ef45d8913f5da5b5ccefb7 |
| institution | OA Journals |
| issn | 1471-2377 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | BMC |
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| series | BMC Neurology |
| spelling | doaj-art-7fb03c9b23ef45d8913f5da5b5ccefb72025-08-20T02:00:12ZengBMCBMC Neurology1471-23772025-05-0125111210.1186/s12883-025-04250-0Clinical and imaging features of parenchymal and non-parenchymal neuro-Behçet’s disease: a systematic review of case reports and seriesOmar Al-omoush0Majd M. AlBarakat1Deya’a Alasmar2Ali Al-Khalaileh3Ahmad Alzoubi4Husam Tarakhan5Zaid Shakhatreh6Zaid Hatamleh7Mohammed Abu-saleh8Othman Saleh9Abdalrahman Altiti10Wafa’ A. Hazaymeh11Sakhr Alshwayyat12Hamdah Hanifa13Ethar Hazaimeh14Internal Medicine and Family Medicine Department, The Hashemite UniversityFaculty of Medicine, Jordan University of Science and TechnologyFaculty of Medicine, The Hashemite UniversityFaculty of Medicine, Jordan University of Science and TechnologyFaculty of Medicine, The Hashemite UniversityFaculty of Medicine, The Hashemite UniversityFaculty of Medicine, Jordan University of Science and TechnologyFaculty of Medicine, Jordan University of Science and TechnologyFaculty of Medicine, The Hashemite UniversityFaculty of Medicine, The Hashemite UniversityFaculty of Medicine, The Hashemite UniversityThe College of Education, Humanities and Social Sciences, Al-Ain UniversityResearch Associate, King Hussein Cancer CenterFaculty of Medicine, University of KalamoonNeurology Specialist, Neuropedia for Training and Scientific ResearchAbstract Background Neuro-Behçet’s Disease (NBD) is a serious neurological complication of Behçet’s Disease, but there’s still a lack of research on its early-stage symptoms and imaging findings. While previous research highlights MRI abnormalities and cerebrospinal fluid changes, there hasn’t been a systematic effort to identify patterns specific to newly diagnosed patients. Our study aimed to investigate the clinical and imaging characteristics of newly diagnosed parenchymal and non-parenchymal NBD based on case reports and case series studies. Methods We synthesized case reports and case series from MEDLINE, Scopus, EMBASE, PubMed, Web of Science, and Cochrane Library until October 17th,2023. Multiple statistical tests, such as the chi-square and Mann-Whitney tests, were used, with the corresponding 95% confidence interval (CI). The study protocol was registered with PROSPERO (ID: CRD42023472436). We included case reports and case series of adult patients (≥ 18 years) with a confirmed diagnosis of parenchymal or non-parenchymal Neuro-Behçet’s disease (NBD), based on neurological symptoms attributed to Behçet’s disease. Studies were required to report MRI findings demonstrating central or peripheral nervous system involvement. Results Our analysis of 107 case reports and a case series (n = 174 patients) revealed that parenchymal NBD patients (n = 141; mean age 35.54 ± 13.33 years) frequently presented with ocular manifestations (80.9%), pyramidal signs (57.5%), cranial nerve palsies (50.4%), and headache (32.6%) while non-parenchymal NBD patients (n = 19; mean age 28.68 ± 10.67 years) commonly experienced headache (86.4%), papilledema (47.4%), cranial nerve palsies (42.1%), and nausea (31.6%). For the mixed-type group (n = 14; mean age 27.5 ± 11.57 years), commonly presented with headache (64.3%), pyramidal sign (50%), fever (50%), and diplopia (35.7%). Brainstem involvement for parenchymal NBD predominated in acute and chronic cases. Contrast enhancement was significantly more prevalent in acute NBD compared to chronic progressive NBD. Conclusion Neuro-Behçet’s disease is an uncommon but serious medical condition with varying symptoms based on its type. Parenchymal and non-parenchymal NBD have unique patterns, with parenchymal cases exhibiting primarily ocular symptoms and pyramidal signs, while non-parenchymal cases present with headache and papilledema. Patients with both types of features were more likely to experience headaches, pyramidal symptoms, fever, and diplopia. Recognizing these patterns can help diagnose and treat the disease earlier.https://doi.org/10.1186/s12883-025-04250-0Neuro-Behçet’s diseaseNBDBehçet’s diseaseVasculitisSystematic review |
| spellingShingle | Omar Al-omoush Majd M. AlBarakat Deya’a Alasmar Ali Al-Khalaileh Ahmad Alzoubi Husam Tarakhan Zaid Shakhatreh Zaid Hatamleh Mohammed Abu-saleh Othman Saleh Abdalrahman Altiti Wafa’ A. Hazaymeh Sakhr Alshwayyat Hamdah Hanifa Ethar Hazaimeh Clinical and imaging features of parenchymal and non-parenchymal neuro-Behçet’s disease: a systematic review of case reports and series BMC Neurology Neuro-Behçet’s disease NBD Behçet’s disease Vasculitis Systematic review |
| title | Clinical and imaging features of parenchymal and non-parenchymal neuro-Behçet’s disease: a systematic review of case reports and series |
| title_full | Clinical and imaging features of parenchymal and non-parenchymal neuro-Behçet’s disease: a systematic review of case reports and series |
| title_fullStr | Clinical and imaging features of parenchymal and non-parenchymal neuro-Behçet’s disease: a systematic review of case reports and series |
| title_full_unstemmed | Clinical and imaging features of parenchymal and non-parenchymal neuro-Behçet’s disease: a systematic review of case reports and series |
| title_short | Clinical and imaging features of parenchymal and non-parenchymal neuro-Behçet’s disease: a systematic review of case reports and series |
| title_sort | clinical and imaging features of parenchymal and non parenchymal neuro behcet s disease a systematic review of case reports and series |
| topic | Neuro-Behçet’s disease NBD Behçet’s disease Vasculitis Systematic review |
| url | https://doi.org/10.1186/s12883-025-04250-0 |
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