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IgG4-Related Pachymeningitis: Evidence of Intrathecal IgG4 on Cerebrospinal Fluid Analysis

Emanuel Della Torre, MD; Enrica Paola Bozzolo, MD; Gabriella Passerini, MD; Claudio Doglioni, MD; and Maria Grazia Sabbadini, MD
[+] Article and Author Information

Acknowledgment: The authors thank Dr. Nicoletta Anzalone (Unit of Neuroradiology, San Raffaele Scientific Institute) and Dr. Paola Mapelli (Department of Nuclear Medicine, San Raffaele Scientific Institute) for radiologic studies and Dr. Roberto Furlan (Neuroimmunology Unit–DIBIT and Department of Neuroscience, San Raffaele Scientific Institute) for helpful input and review of the manuscript.

Potential Conflicts of Interest: None disclosed.


From San Raffaele Scientific Institute, 20132 Milan, Italy.


Ann Intern Med. 2012;156(5):401-403. doi:10.7326/0003-4819-156-5-201203060-00025
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Grahic Jump Location

Figure. IgG4-related pachymeningitis with evidence of intrathecal IgG4 synthesis.

A. Brain magnetic resonance with gadolinium shows diffuse thickening and enhancement of the dura mater involving the posterior cranial fossa and the foramen magnum with extension over the cervical spinal canal to the fourth vertebra (arrowheads). B. Positron emission tomography alone (left) and with computed tomography (right) fusion images show an increased 18F-fluorodeoxyglucose uptake along the meningeal thickening. C and D. After 2 months of treatment, a marked improvement is evident both on radiologic (arrowheads) (C) and nuclear imaging studies (D). Cerebrospinal fluid (CSF) and serum (S) isoelectric focusing demonstrate an intrathecal IgG synthesis with oligoclonal bands (arrowheads). F. Hematoxylin–eosin staining of the meningeal specimen shows a dural perivascular dense lymphoplasmacytic infiltrate surrounded by stromal fibrosis (original magnification, ×200); immunohistochemistry reveals numerous IgG4+ plasma cells with an IgG4+–IgG+ ratio ranging from 40% to 70% (inset) (original magnification, ×400).

Grahic Jump Location

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