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Idiopathic Hypertrophic Cranial Pachymeningitis: Case Report with 7 Years of Imaging Follow-up

Yu Chang Lee, Yao Chung Chueng, Shin Wei Hsu and Chun Chung Lui
American Journal of Neuroradiology January 2003, 24 (1) 119-123;
Yu Chang Lee
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Yao Chung Chueng
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Shin Wei Hsu
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Chun Chung Lui
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  • Fig 1.
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    Fig 1.

    Contrast-enhanced CT scans show increased enhancement at the right cavernous sinus.

    A, Contrast-enhanced CT scan obtained in 1994 shows that the right tentorium is thickened with homogeneous enhancement due to an inflammatory reaction of the pachymeninges.

    B, Contrast-enhanced CT scan obtained at a more caudal level shows that involvement of the right cavernous sinus correlates with the patient’s symptom of optic neuropathy.

  • Fig 2.
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    Fig 2.

    MR images obtained in 1995 show the thickened right tentorium. No significant progression of tentorium thickening or cavernous sinus enhancement is noted.

    A, Thickened right tentorium is hypertense on axial T1-weighted image.

    B, Thickened right tentorium is hypertense on coronal T1-weighted image.

    C, Thickened right tentorium is hypointense on coronal T2-weighted image.

  • Fig 3.
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    Fig 3.

    MR images show mild increasing thickness and enhancement of the left tentorium.

    A, Axial T1-weighted contrast-enhanced MR image obtained in May of 1998, at the onset of blindness in the right eye and decreased left visual acuity, shows increased right cavernous sinus enhancement and more diffuse involvement of the tentorium bilaterally and of the posterior falx. Prominent dura enhancement can also be seen at the prepontine area, with mild compression of the brain stem.

    B, Coronal T1-weighted contrast-enhanced MR image shows a focus of nodular dural enhancement at left temporal area, with minimal perifocal edema.

  • Fig 4.
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    Fig 4.

    Coronal T1-weighted contrast-enhanced MR image obtained in June of 1998 shows regression of dural enhancement and the left temporal nodular mass with minimal dural thickening and edema.

  • Fig 5.
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    Fig 5.

    Coronal T1-weighted contrast-enhanced MR image obtained in January of 1999 at the onset of right hearing impairment shows increased enhancement of the right mastoid area, although decrease in the tentorium and falx enhancement can be seen.

  • Fig 6.
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    Fig 6.

    Coronal T1-weighted contrast-enhanced MR image obtained in December of 1999 shows increased intensity of the left mastoid at the onset of left hearing impairment.

  • Fig 7.
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    Fig 7.

    In July of 2001, the patient suffered from delirium and confusion. CT scan and MR image show enhanced dural mass at the left temporal area, with perifocal edema.

    A, Contrast-enhanced CT scan shows an irregularly enhanced left temporal mass, with compression of the left temporal horn.

    B, Coronal T1-weighted contrast-enhanced MR image shows an enhanced dural-based mass of the left tentorium, with an edematous change of the left temporal lobe. The mass is located in the same area as that revealed in Figure 3B.

  • Fig 8.
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    Fig 8.

    Cerebral angiogram of the left internal carotid artery shows nonopacification of the straight sinus.

  • Fig 9.
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    Fig 9.

    Narrowed straight sinus, which correlates with dural sinus thrombosis, was noted on MR venograms.

    A, Sagittal MR venogram shows nonopacification of the straight sinus and indicates thrombosis.

    B, Coronal view.

  • Fig 10.
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    Fig 10.

    Pathology slide of the meningeal tissue from a left temporal craniotomy shows inflammatory cells with lymphoplasmacytic infiltration, foamy histiocytes, and vascular proliferation (hematoxylin and eosin; original magnification, ×150).

Tables

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    TABLE:

    Chronologic imaging findings with clinical correlations

    TimeImaging ModalityImaging FindingsClinical Symptoms
    1994.3.24CTThickened right tentorium: hypodense on unenhanced and with strong enhancementDecreased right vision
    1995.5.6MR imagingRight tentorium thickening: hypointense on T1- and T2-weighted images and homogeneous contrast enhancement
    1998.5.8MR imagingAdditional involvement of left tentorium, posterior falx, and right cavernous sinusRight blindness, decreased left vision
    1998.6.30MR imagingRegressive change of tentorium, falx, and cavernous sinus
    1999.1.11MR imagingRegressive involvement of tentorium but increased enhancement at right mastoidRight hearing loss
    1999.2.8Temporal bone CTRight mastoid cholesteatoma with tegmen and dural plate erosion, left mastoiditis
    1999.12.18MR imagingProgressive enhancement of left mastoidBilateral hearing loss
    2001.4.21MR imagingNo significant interval change of tentorium or mastoid involvement
    2001.7.31CTIll defined enhanced left temporal lobe mass with perifocal edemaDelirium, disorientation
    2001.8.6Cerebral angiographyFaint stain at left temporal, no extra-axial tumor
    2001.8.7MR imagingEnhanced left temporal dural mass with mass effect of temporal lobe, poor opacified straight sinus
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American Journal of Neuroradiology: 24 (1)
American Journal of Neuroradiology
Vol. 24, Issue 1
1 Jan 2003
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Cite this article
Yu Chang Lee, Yao Chung Chueng, Shin Wei Hsu, Chun Chung Lui
Idiopathic Hypertrophic Cranial Pachymeningitis: Case Report with 7 Years of Imaging Follow-up
American Journal of Neuroradiology Jan 2003, 24 (1) 119-123;

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Idiopathic Hypertrophic Cranial Pachymeningitis: Case Report with 7 Years of Imaging Follow-up
Yu Chang Lee, Yao Chung Chueng, Shin Wei Hsu, Chun Chung Lui
American Journal of Neuroradiology Jan 2003, 24 (1) 119-123;
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