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Case Reports
. 2023 Jun 16:14:205.
doi: 10.25259/SNI_400_2023. eCollection 2023.

Epiduritis related to IgG4 disease: A very rare cause for spinal cord compression

Ghassen Gader  1 , Fatma Ben Atig  2 , Nesrine Jemel  1 , Malek Bourgou  1 , Abdelhafidh Slimane  2 , Khalil Ghedira  2 , Mohamed Badri  1 , Ihsèn Zammel  1
Affiliations
Case Reports

Epiduritis related to IgG4 disease: A very rare cause for spinal cord compression

Ghassen Gader et al. Surg Neurol Int. .

Abstract

Background: Inflammatory pseudotumors are rare, and those attributed to immunoglobulin G4 (IgG4) diseases are even less frequently encountered. Here, we reviewed 41 cases from the literature of spinal inflammatory pseudotumors due to IgG4 and have added our single new case.

Case presentation: A 25-year-old male presented with progressive back pain, bilateral paraparesis, and sphincter dysfunction. His deficit was attributed to MR-documented posterolateral lesion between the T5 and T10 levels for which he levels underwent a T1-T1010 laminectomy. The pathology revealed an immunoglobulin G4-related inflammatory pseudotumor. Postoperatively, the patient additionally required systemic and epidural administration of glucocorticoids.

Conclusion: IgG4-related disease is an emerging clinical condition that rarely involves the central nervous system. Spinal inflammatory pseudotumors, including IgG4 disease, should be more commonly considered among the potential differential diagnoses of lesions compressing the spinal cord.

Keywords: Epiduritis; Inflammation; Neurosurgery; Spinal cord; Spine surgery.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1:
Figure 1:
Sagittal (a) and axial (b) sections of a spinal cord magnetic resonance imaging on T1-weighted image with injection of Gadolinium showing a posterior epiduritis ranging from T5 to T10 responsible for compression of the spinal cord.
Figure 2:
Figure 2:
Pathological examination showing a diffuse infiltration of inflammatory cells within the fibrofatty tissue, with presence of 50 immunoglobulin G4 (IgG4)+ cells/high-powered field and 50% IgG4+/IgG cell ratio.
See this image and copyright information in PMC

References

    1. Alrashdi MN. Immunoglobulin G4-related spinal pachymeningitis. Saudi Med J. 2020;41:652–6. - PMC - PubMed
    1. Baptista B, Casian A, Gunawardena H, D’Cruz D, Rice CM. Neurological manifestations of IgG4-related disease. Curr Treat Options Neurol. 2017;19:14. - PMC - PubMed
    1. Bridges KJ, DeDeaux CH, Than KD. IgG4-related disease presenting as intradural extramedullary lesion: A case report and review of the literature. Br J Neurosurg. 2019;33:570–6. - PubMed
    1. Cação G, Calejo M, Alves J, Medeiros P, Vila-Cha N, Mendonça T. Clinical features of hypertrophic pachymeningitis in a center survey. Neurol Sci. 2019;40:543–51. - PubMed
    1. Chan SK, Cheuk W, Chan KT, Chan JK. IgG4-related sclerosing pachymeningitis: A previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 2009;33:1249–52. - PubMed

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