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Bilateral Vision Loss Secondary to Pachymeningitis in a Patient with IgG4-Related Disease.

Ramirez L, D'Auria A, Popalzai A, Sanossian N - Front Neurol (2014)

Bottom Line: IgG4-related disease (IgG4-RD) is a recently recognized fibroinflammatory condition associated with disease in nearly every organ, including the meninges.A proportion of idiopathic hypertrophic pachymeningitis cases may involve a component of meningeal IgG4-RD.We present a patient with severe bilateral vision loss found to have thickening of the dura mater on MRI, and subsequently diagnosed with IgG4-RD after dural biopsy.

View Article: PubMed Central - PubMed

Affiliation: Roxanna Todd Hodges Comprehensive Stroke Clinic, University of Southern California , Los Angeles, CA , USA.

ABSTRACT
IgG4-related disease (IgG4-RD) is a recently recognized fibroinflammatory condition associated with disease in nearly every organ, including the meninges. A proportion of idiopathic hypertrophic pachymeningitis cases may involve a component of meningeal IgG4-RD. We present a patient with severe bilateral vision loss found to have thickening of the dura mater on MRI, and subsequently diagnosed with IgG4-RD after dural biopsy.

No MeSH data available.


Related in: MedlinePlus

Hematoxylin and eosin (H&E) stain at high power (A) and low power (B) demonstrate dural thickening with prominent lymphoplasmacytic infiltrate. On high power (A), the infiltrate is composed of mostly plasma cells. Immunohistochemical stain for IgG (C) and IgG4 (D) demonstrate that most of the plasma cells are positive for IgG and that the vast majority of the plasma cells are IgG4 positive.
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Figure 2: Hematoxylin and eosin (H&E) stain at high power (A) and low power (B) demonstrate dural thickening with prominent lymphoplasmacytic infiltrate. On high power (A), the infiltrate is composed of mostly plasma cells. Immunohistochemical stain for IgG (C) and IgG4 (D) demonstrate that most of the plasma cells are positive for IgG and that the vast majority of the plasma cells are IgG4 positive.

Mentions: The patient’s vision continued to improve with steroid therapy and on day 10, VA was noted to be 20/20 on the OD and 20/30 on OS. Pathological evaluation showed pachymeningitis with increased IgG4 plasma cells (Figure 2). Given the clinical, laboratory, and pathology results, a diagnosis of IgG4-related disease (IgG4-RD) was made. Serum IgG4 drawn on the day of hospital discharge revealed elevated levels of 182.0 (reference range 4–86).


Bilateral Vision Loss Secondary to Pachymeningitis in a Patient with IgG4-Related Disease.

Ramirez L, D'Auria A, Popalzai A, Sanossian N - Front Neurol (2014)

Hematoxylin and eosin (H&E) stain at high power (A) and low power (B) demonstrate dural thickening with prominent lymphoplasmacytic infiltrate. On high power (A), the infiltrate is composed of mostly plasma cells. Immunohistochemical stain for IgG (C) and IgG4 (D) demonstrate that most of the plasma cells are positive for IgG and that the vast majority of the plasma cells are IgG4 positive.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4195318&req=5

Figure 2: Hematoxylin and eosin (H&E) stain at high power (A) and low power (B) demonstrate dural thickening with prominent lymphoplasmacytic infiltrate. On high power (A), the infiltrate is composed of mostly plasma cells. Immunohistochemical stain for IgG (C) and IgG4 (D) demonstrate that most of the plasma cells are positive for IgG and that the vast majority of the plasma cells are IgG4 positive.
Mentions: The patient’s vision continued to improve with steroid therapy and on day 10, VA was noted to be 20/20 on the OD and 20/30 on OS. Pathological evaluation showed pachymeningitis with increased IgG4 plasma cells (Figure 2). Given the clinical, laboratory, and pathology results, a diagnosis of IgG4-related disease (IgG4-RD) was made. Serum IgG4 drawn on the day of hospital discharge revealed elevated levels of 182.0 (reference range 4–86).

Bottom Line: IgG4-related disease (IgG4-RD) is a recently recognized fibroinflammatory condition associated with disease in nearly every organ, including the meninges.A proportion of idiopathic hypertrophic pachymeningitis cases may involve a component of meningeal IgG4-RD.We present a patient with severe bilateral vision loss found to have thickening of the dura mater on MRI, and subsequently diagnosed with IgG4-RD after dural biopsy.

View Article: PubMed Central - PubMed

Affiliation: Roxanna Todd Hodges Comprehensive Stroke Clinic, University of Southern California , Los Angeles, CA , USA.

ABSTRACT
IgG4-related disease (IgG4-RD) is a recently recognized fibroinflammatory condition associated with disease in nearly every organ, including the meninges. A proportion of idiopathic hypertrophic pachymeningitis cases may involve a component of meningeal IgG4-RD. We present a patient with severe bilateral vision loss found to have thickening of the dura mater on MRI, and subsequently diagnosed with IgG4-RD after dural biopsy.

No MeSH data available.


Related in: MedlinePlus