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CC BY 4.0 · Arq Neuropsiquiatr 2024; 82(01): s00431772600
DOI: 10.1055/s-0043-1772600
Images in Neurology

IgG4-related hypertrophic pachymeningitis with cerebral venous thrombosis

Paquimeningite hipertrófica relacionada à IgG4 associada a trombose venosa cerebral
Arthur da Veiga Kalil Coelho 
1   Hospital do Servidor Público Estadual de São Paulo, Departamento de Neurologia, São Paulo SP, Brazil.
,
Alexia Carneiro de Almeida 
1   Hospital do Servidor Público Estadual de São Paulo, Departamento de Neurologia, São Paulo SP, Brazil.
,
Sonia Maria Cesar de Azevedo Silva Moura Magalhães Gomes 
1   Hospital do Servidor Público Estadual de São Paulo, Departamento de Neurologia, São Paulo SP, Brazil.
2   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Neurologia, São Paulo SP, Brazil.
,
Antonio José da Rocha 
3   Santa Casa de São Paulo, Faculdade de Ciências Médicas, Departamento de Radiologia, São Paulo SP, Brazil.
,
Herval Ribeiro Soares Neto 
1   Hospital do Servidor Público Estadual de São Paulo, Departamento de Neurologia, São Paulo SP, Brazil.
4   Hospital Israelita Albert Einstein, Departamento de Neurologia, São Paulo SP, Brazil.
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A 58-year-old female patient presented with a 4-year history of right-sided headache with tinnitus. She had been hospitalized previously due to dysfunction of multiple cranial nerves, such as diplopia, right hemiface hypoesthesia, right peripheral facial palsy, right hearing loss, dysphonia, and dysphagia, followed by venous thrombosis of the right sigmoid sinus ([Figure 1]), which was treated with warfarin. The cerebrospinal fluid yielded 90 leukocytes/mm3 (92% of lymphocytes) and 158 mg/dL of proteins. A magnetic resonance imaging (MRI) scan revealed tentorium-temporo-parietal hypertrophic pachymeningitis ([Figure 2]). A meningeal biopsy demonstrated dense lymphoplasmacytic infiltrate and storiform fibrosis ([Figure 3]), two of the three histopathological criteria.[1] [2] Therefore, the diagnosis was made, and rituximab[3] [4] was initiated, with a very important response in terms of symptoms and imaging exams ([Figure 4]).

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Figure 1 Venous magnetic resonance angiography showing venous thrombosis at the right side.
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Figure 2 T2 (upper) and postcontrast T1 (bottom) weighted magnetic resonance imaging (MRI) scans, showing dural thickening on coronal view before treatment.
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Figure 3 Meningeal biopsy in hematoxylin and eosin stain demonstrating dense lymphoplasmacytic infiltrate (A) and storiform fibrosis (B), two of the three histopathological criteria of IgG4-related disease.
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Figure 4 Postcontrast weighted MRI scan showing improvement in dural thickening at the right side on coronal view after treatment with rituximab.

Authors' Contributions

AVKC: writing o the original draft, writing – review and editing; ACA: writing of the original draft; SMCASMMG, AJR, and HRSN: supervision.




Publikationsverlauf

Eingereicht: 23. Februar 2023

Angenommen: 19. April 2023

Artikel online veröffentlicht:
25. Januar 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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Bibliographical Record
Arthur da Veiga Kalil Coelho, Alexia Carneiro de Almeida, Sonia Maria Cesar de Azevedo Silva Moura Magalhães Gomes, Antonio José da Rocha, Herval Ribeiro Soares Neto. IgG4-related hypertrophic pachymeningitis with cerebral venous thrombosis. Arq Neuropsiquiatr 2024; 82: s00431772600.
DOI: 10.1055/s-0043-1772600
 

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