Idiopathic Hypertrophic Cranial Pachymeningitis Associated With Intermediate Uveitis

Abstract

The authors report a case with idiopathic hypertrophic cranial pachymeningitis associated with intermediate uveitis. The patient complained of decreased vision in both eyes, especially the right. Ophthalmic examination revealed right optic disc pallor, bilateral vitritis, and cystoid macular edema. Magnetic resonance imaging revealed marked enhancement of a dural lesion. The macular edema responded well to medical treatment. Intermediate uveitis has not yet been reported in the context of idiopathic hypertrophic cranial pachymeningitis.

Keywords: cystoid macular oedema; idiopathic hypertrophic cranial pachymeningitis; intermediate uveitis.

FIGURE 1

Fluorescein angiography images show cystoid macular oedema (arrows) and peripheral vascular leakage (arrowheads) (a, b, c). One month later images show resolution of macular oedema and a decrease in the peripheral vascular leakage (d, e, f).

FIGURE 2

Humphrey visual field test shows central scotoma, enlargement of the blind spot, and peripheral scotoma in the right eye (a). The test performed 2 weeks later showed partial regression of scotomas (b).

FIGURE 3

Coronal post-gadolinium image performed in April 2008 shows the lesion extending from right cavernous sinus to the prechiasmatic part of right optic nerve (a, arrow). Axial post-gadolinium image of the brain 9 years after diagnosis shows residual thickening of the tentorium cerebelli (on prednisone and azathioprine therapy) (b, arrow).