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Pseudotumoral neuro-behcet’s disease: case series and review of literature
Background Behcet’s disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet’s disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas...
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| Published in: | Acta neurologica Belgica 2024-04, Vol.124 (2), p.431-445 |
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| container_end_page | 445 |
| container_issue | 2 |
| container_start_page | 431 |
| container_title | Acta neurologica Belgica |
| container_volume | 124 |
| creator | Hadj Taieb, Mohamed Amine Slimane, Houssem Mhiri, Mariem Ben Dhia, Rihab Daoussi, Nizar Frih-Ayed, Mahbouba |
| description | Background
Behcet’s disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet’s disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas in only a few cases.
Methods
We report here the main characteristics, treatment, and outcome of 43 patients (4 personal cases and 39 patients from the literature) with a pseudotumoral presentation of NBD (PT NBD). We compared our findings with those of the classical form of NBD.
Results
The median age was 35.86 (12–59 years) years, with a male predominance (67.4%). PT NBD was the inaugural of the disease in 51.2% of cases. The neurological manifestations included headache (
n
= 31), pyramidal syndrome (
n
= 28), cerebellar syndrome (
n
= 5), behavioral changes (
n
= 5), and pseudobulbar signs (
n
= 2). Ophthalmologic examination revealed papilledema in 3 cases. On cerebral imaging, the most affected regions of the brain were the capsulothalamic region (
n
= 15, 37.5%) and the brainstem (
n
= 14, 35). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoral or infectious lesions. Treatment consisted of corticosteroids (
n
= 40, 93%) and immunosuppressive agents (
n
= 28, 65.11%), leading to complete clinical and imaging remission in 41.5% of patients.
Conclusion
PT NBD is a rare but life-threatening condition. |
| doi_str_mv | 10.1007/s13760-024-02477-1 |
| format | article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_3003438605</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>3003438605</sourcerecordid><originalsourceid>FETCH-LOGICAL-c298t-affe4ac95e64d0c9b4f8e1d47f09b550beddaa9da5654376acf3929d591dd41f3</originalsourceid><addsrcrecordid>eNp9kMtOwzAQRS0EolXpD7BAWbIJjB9JanYI8ZIqwQLWlmOPIVUexU5A7PgNfo8vwSWFJSPNzGLuXOkeQg4pnFCA4jRQXuSQAhObLoqU7pApYwJSJiXfJVPgAKkEkBMyD2EFsUTOaJHvkwlfcJlTCVOyvA842K4fms7rOmlx8F1a4rPB_uvjMyS2CqgDniUmziSgrzAkurWJx9cK35LOJXXVo9f94PGA7DldB5xv94w8Xl0-XNyky7vr24vzZWqYXPSpdg6FNjLDXFgwshRugdSKwoEsswxKtFZraXWWZyKm1MZxyaTNJLVWUMdn5Hj0XfvuZcDQq6YKButat9gNQcXkXPBFDlmUslFqfBeCR6fWvmq0f1cU1AakGkGqCFH9gFQ0Ph1t_YeyQfv38ostCvgoCPHUPqFXq27wbcz8n-03Ztp_-g</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>3003438605</pqid></control><display><type>article</type><title>Pseudotumoral neuro-behcet’s disease: case series and review of literature</title><source>Springer Link</source><creator>Hadj Taieb, Mohamed Amine ; Slimane, Houssem ; Mhiri, Mariem ; Ben Dhia, Rihab ; Daoussi, Nizar ; Frih-Ayed, Mahbouba</creator><creatorcontrib>Hadj Taieb, Mohamed Amine ; Slimane, Houssem ; Mhiri, Mariem ; Ben Dhia, Rihab ; Daoussi, Nizar ; Frih-Ayed, Mahbouba</creatorcontrib><description>Background
Behcet’s disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet’s disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas in only a few cases.
Methods
We report here the main characteristics, treatment, and outcome of 43 patients (4 personal cases and 39 patients from the literature) with a pseudotumoral presentation of NBD (PT NBD). We compared our findings with those of the classical form of NBD.
Results
The median age was 35.86 (12–59 years) years, with a male predominance (67.4%). PT NBD was the inaugural of the disease in 51.2% of cases. The neurological manifestations included headache (
n
= 31), pyramidal syndrome (
n
= 28), cerebellar syndrome (
n
= 5), behavioral changes (
n
= 5), and pseudobulbar signs (
n
= 2). Ophthalmologic examination revealed papilledema in 3 cases. On cerebral imaging, the most affected regions of the brain were the capsulothalamic region (
n
= 15, 37.5%) and the brainstem (
n
= 14, 35). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoral or infectious lesions. Treatment consisted of corticosteroids (
n
= 40, 93%) and immunosuppressive agents (
n
= 28, 65.11%), leading to complete clinical and imaging remission in 41.5% of patients.
Conclusion
PT NBD is a rare but life-threatening condition.</description><identifier>ISSN: 0300-9009</identifier><identifier>EISSN: 2240-2993</identifier><identifier>DOI: 10.1007/s13760-024-02477-1</identifier><identifier>PMID: 38396190</identifier><language>eng</language><publisher>Cham: Springer International Publishing</publisher><subject>Adolescent ; Adult ; Behcet Syndrome - complications ; Behcet Syndrome - diagnosis ; Biomedical and Life Sciences ; Biomedicine ; Brain - diagnostic imaging ; Brain - pathology ; Cerebellar Diseases ; Child ; Female ; Humans ; Immunosuppressive Agents - therapeutic use ; Magnetic Resonance Imaging ; Male ; Medicine/Public Health ; Middle Aged ; Neoplasm Recurrence, Local ; Neurology ; Neuroradiology ; Neurosciences ; Original Article ; Young Adult</subject><ispartof>Acta neurologica Belgica, 2024-04, Vol.124 (2), p.431-445</ispartof><rights>The Author(s) under exclusive licence to Belgian Neurological Society 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.</rights><rights>2024. The Author(s) under exclusive licence to Belgian Neurological Society.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c298t-affe4ac95e64d0c9b4f8e1d47f09b550beddaa9da5654376acf3929d591dd41f3</cites><orcidid>0000-0001-5215-9919</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,786,790,27957,27958</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38396190$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hadj Taieb, Mohamed Amine</creatorcontrib><creatorcontrib>Slimane, Houssem</creatorcontrib><creatorcontrib>Mhiri, Mariem</creatorcontrib><creatorcontrib>Ben Dhia, Rihab</creatorcontrib><creatorcontrib>Daoussi, Nizar</creatorcontrib><creatorcontrib>Frih-Ayed, Mahbouba</creatorcontrib><title>Pseudotumoral neuro-behcet’s disease: case series and review of literature</title><title>Acta neurologica Belgica</title><addtitle>Acta Neurol Belg</addtitle><addtitle>Acta Neurol Belg</addtitle><description>Background
Behcet’s disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet’s disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas in only a few cases.
Methods
We report here the main characteristics, treatment, and outcome of 43 patients (4 personal cases and 39 patients from the literature) with a pseudotumoral presentation of NBD (PT NBD). We compared our findings with those of the classical form of NBD.
Results
The median age was 35.86 (12–59 years) years, with a male predominance (67.4%). PT NBD was the inaugural of the disease in 51.2% of cases. The neurological manifestations included headache (
n
= 31), pyramidal syndrome (
n
= 28), cerebellar syndrome (
n
= 5), behavioral changes (
n
= 5), and pseudobulbar signs (
n
= 2). Ophthalmologic examination revealed papilledema in 3 cases. On cerebral imaging, the most affected regions of the brain were the capsulothalamic region (
n
= 15, 37.5%) and the brainstem (
n
= 14, 35). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoral or infectious lesions. Treatment consisted of corticosteroids (
n
= 40, 93%) and immunosuppressive agents (
n
= 28, 65.11%), leading to complete clinical and imaging remission in 41.5% of patients.
Conclusion
PT NBD is a rare but life-threatening condition.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Behcet Syndrome - complications</subject><subject>Behcet Syndrome - diagnosis</subject><subject>Biomedical and Life Sciences</subject><subject>Biomedicine</subject><subject>Brain - diagnostic imaging</subject><subject>Brain - pathology</subject><subject>Cerebellar Diseases</subject><subject>Child</subject><subject>Female</subject><subject>Humans</subject><subject>Immunosuppressive Agents - therapeutic use</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Medicine/Public Health</subject><subject>Middle Aged</subject><subject>Neoplasm Recurrence, Local</subject><subject>Neurology</subject><subject>Neuroradiology</subject><subject>Neurosciences</subject><subject>Original Article</subject><subject>Young Adult</subject><issn>0300-9009</issn><issn>2240-2993</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><recordid>eNp9kMtOwzAQRS0EolXpD7BAWbIJjB9JanYI8ZIqwQLWlmOPIVUexU5A7PgNfo8vwSWFJSPNzGLuXOkeQg4pnFCA4jRQXuSQAhObLoqU7pApYwJSJiXfJVPgAKkEkBMyD2EFsUTOaJHvkwlfcJlTCVOyvA842K4fms7rOmlx8F1a4rPB_uvjMyS2CqgDniUmziSgrzAkurWJx9cK35LOJXXVo9f94PGA7DldB5xv94w8Xl0-XNyky7vr24vzZWqYXPSpdg6FNjLDXFgwshRugdSKwoEsswxKtFZraXWWZyKm1MZxyaTNJLVWUMdn5Hj0XfvuZcDQq6YKButat9gNQcXkXPBFDlmUslFqfBeCR6fWvmq0f1cU1AakGkGqCFH9gFQ0Ph1t_YeyQfv38ostCvgoCPHUPqFXq27wbcz8n-03Ztp_-g</recordid><startdate>20240401</startdate><enddate>20240401</enddate><creator>Hadj Taieb, Mohamed Amine</creator><creator>Slimane, Houssem</creator><creator>Mhiri, Mariem</creator><creator>Ben Dhia, Rihab</creator><creator>Daoussi, Nizar</creator><creator>Frih-Ayed, Mahbouba</creator><general>Springer International Publishing</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-5215-9919</orcidid></search><sort><creationdate>20240401</creationdate><title>Pseudotumoral neuro-behcet’s disease: case series and review of literature</title><author>Hadj Taieb, Mohamed Amine ; Slimane, Houssem ; Mhiri, Mariem ; Ben Dhia, Rihab ; Daoussi, Nizar ; Frih-Ayed, Mahbouba</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c298t-affe4ac95e64d0c9b4f8e1d47f09b550beddaa9da5654376acf3929d591dd41f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Behcet Syndrome - complications</topic><topic>Behcet Syndrome - diagnosis</topic><topic>Biomedical and Life Sciences</topic><topic>Biomedicine</topic><topic>Brain - diagnostic imaging</topic><topic>Brain - pathology</topic><topic>Cerebellar Diseases</topic><topic>Child</topic><topic>Female</topic><topic>Humans</topic><topic>Immunosuppressive Agents - therapeutic use</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Medicine/Public Health</topic><topic>Middle Aged</topic><topic>Neoplasm Recurrence, Local</topic><topic>Neurology</topic><topic>Neuroradiology</topic><topic>Neurosciences</topic><topic>Original Article</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hadj Taieb, Mohamed Amine</creatorcontrib><creatorcontrib>Slimane, Houssem</creatorcontrib><creatorcontrib>Mhiri, Mariem</creatorcontrib><creatorcontrib>Ben Dhia, Rihab</creatorcontrib><creatorcontrib>Daoussi, Nizar</creatorcontrib><creatorcontrib>Frih-Ayed, Mahbouba</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Acta neurologica Belgica</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hadj Taieb, Mohamed Amine</au><au>Slimane, Houssem</au><au>Mhiri, Mariem</au><au>Ben Dhia, Rihab</au><au>Daoussi, Nizar</au><au>Frih-Ayed, Mahbouba</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pseudotumoral neuro-behcet’s disease: case series and review of literature</atitle><jtitle>Acta neurologica Belgica</jtitle><stitle>Acta Neurol Belg</stitle><addtitle>Acta Neurol Belg</addtitle><date>2024-04-01</date><risdate>2024</risdate><volume>124</volume><issue>2</issue><spage>431</spage><epage>445</epage><pages>431-445</pages><issn>0300-9009</issn><eissn>2240-2993</eissn><notes>ObjectType-Case Study-2</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Review-3</notes><notes>content type line 23</notes><notes>ObjectType-Feature-5</notes><notes>ObjectType-Article-4</notes><notes>ObjectType-Report-1</notes><abstract>Background
Behcet’s disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet’s disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas in only a few cases.
Methods
We report here the main characteristics, treatment, and outcome of 43 patients (4 personal cases and 39 patients from the literature) with a pseudotumoral presentation of NBD (PT NBD). We compared our findings with those of the classical form of NBD.
Results
The median age was 35.86 (12–59 years) years, with a male predominance (67.4%). PT NBD was the inaugural of the disease in 51.2% of cases. The neurological manifestations included headache (
n
= 31), pyramidal syndrome (
n
= 28), cerebellar syndrome (
n
= 5), behavioral changes (
n
= 5), and pseudobulbar signs (
n
= 2). Ophthalmologic examination revealed papilledema in 3 cases. On cerebral imaging, the most affected regions of the brain were the capsulothalamic region (
n
= 15, 37.5%) and the brainstem (
n
= 14, 35). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoral or infectious lesions. Treatment consisted of corticosteroids (
n
= 40, 93%) and immunosuppressive agents (
n
= 28, 65.11%), leading to complete clinical and imaging remission in 41.5% of patients.
Conclusion
PT NBD is a rare but life-threatening condition.</abstract><cop>Cham</cop><pub>Springer International Publishing</pub><pmid>38396190</pmid><doi>10.1007/s13760-024-02477-1</doi><tpages>15</tpages><orcidid>https://orcid.org/0000-0001-5215-9919</orcidid></addata></record> |
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| issn | 0300-9009 2240-2993 |
| language | eng |
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| source | Springer Link |
| subjects | Adolescent Adult Behcet Syndrome - complications Behcet Syndrome - diagnosis Biomedical and Life Sciences Biomedicine Brain - diagnostic imaging Brain - pathology Cerebellar Diseases Child Female Humans Immunosuppressive Agents - therapeutic use Magnetic Resonance Imaging Male Medicine/Public Health Middle Aged Neoplasm Recurrence, Local Neurology Neuroradiology Neurosciences Original Article Young Adult |
| title | Pseudotumoral neuro-behcet’s disease: case series and review of literature |
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