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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

There is diffuse smooth enhancement of the pachymeninges (A), left greater than right, with apparent thickening (B). Globes are normal in their size, shape, and signal intensity on all pulse sequences. No evidence of intra or extraconal soft tissue mass. Bilateral enhancement of posterior intraconal optic nerve sheath with normal signal intensity of optic nerves.
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Figure 1: There is diffuse smooth enhancement of the pachymeninges (A), left greater than right, with apparent thickening (B). Globes are normal in their size, shape, and signal intensity on all pulse sequences. No evidence of intra or extraconal soft tissue mass. Bilateral enhancement of posterior intraconal optic nerve sheath with normal signal intensity of optic nerves.

Mentions: Non-contrast CT of the head showed no intracranial abnormality. Ophthalmological evaluation found no apparent explanation for the visual loss. Dexamethasone therapy was initiated. Contrast-enhanced MRI of the brain done 1 day later showed diffuse thickening and enhancement of the dura, greater on the left side (Figure 1A). MRI of the orbits showed bilateral enhancement of the posterior aspect of the intraconal optic nerve sheath (Figure 1B). Lumbar puncture demonstrated an opening pressure of 32 mm H2O, glucose 90 mg/dL, protein content of 44 mg/dL, RBC 1, and WBC 21 (80% lymphocytes, 16% monocytes, 4% neutrophils). Gram stain, cultures, and cytology were negative. IgG index was 1.09 (reference <0.66), and IgG was 13.3 mg/dL (range 0.8–7.7). A left frontal dural biopsy was performed.


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)

There is diffuse smooth enhancement of the pachymeninges (A), left greater than right, with apparent thickening (B). Globes are normal in their size, shape, and signal intensity on all pulse sequences. No evidence of intra or extraconal soft tissue mass. Bilateral enhancement of posterior intraconal optic nerve sheath with normal signal intensity of optic nerves.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: There is diffuse smooth enhancement of the pachymeninges (A), left greater than right, with apparent thickening (B). Globes are normal in their size, shape, and signal intensity on all pulse sequences. No evidence of intra or extraconal soft tissue mass. Bilateral enhancement of posterior intraconal optic nerve sheath with normal signal intensity of optic nerves.
Mentions: Non-contrast CT of the head showed no intracranial abnormality. Ophthalmological evaluation found no apparent explanation for the visual loss. Dexamethasone therapy was initiated. Contrast-enhanced MRI of the brain done 1 day later showed diffuse thickening and enhancement of the dura, greater on the left side (Figure 1A). MRI of the orbits showed bilateral enhancement of the posterior aspect of the intraconal optic nerve sheath (Figure 1B). Lumbar puncture demonstrated an opening pressure of 32 mm H2O, glucose 90 mg/dL, protein content of 44 mg/dL, RBC 1, and WBC 21 (80% lymphocytes, 16% monocytes, 4% neutrophils). Gram stain, cultures, and cytology were negative. IgG index was 1.09 (reference <0.66), and IgG was 13.3 mg/dL (range 0.8–7.7). A left frontal dural biopsy was performed.

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