UNIVERSITY OPHTHALMOLOGY CONSULTANTS

 

CASE OF THE MONTH

CASE #1

NEURO-OPHTHALMOLOGY

 

Outcome: When we obtained confirmation that the spinal fluid was noninfectious, we began Solu-Medrol (methylprednisolone) therapy, 1 g daily—since the patient had lost vision in her only eye—and continued the medical evaluation. Within 12 hours, the pain subsided—for the first time in 10 months—vision improved to 20/25, and the patient correctly identified 10 of 10 Ishihara color plates. Further evaluation showed a negative PPD; the anergy panel showed no cutaneous response; the pulmonary function tests showed diminished diffusing capacity; 24-hour urine calcium levels were elevated, and the gallium scan showed increased bilateral parenchymal uptake. All of these findings are indicative of sarcoidosis.

This patient has refused to undergo bronchoscopy to obtain histologic confirmation of sarcoidosis.

 

Summary: The presence of periphlebitis at the 11-o’clock position during the initial fundus examination led us to suspect sarcoidosis as the most probable disorder in our differential diagnosis. The MRI scans also revealed clinical findings consistent with sarcoid. The optic nerve showed “stem to stern” enlargement and was enhanced from the globe to the optic chiasm; a lumpy, nodular-like enlargement of the contralateral posterior optic nerve and intrinsic chiasmal expansion were noted. The patient showed no signs of clear-cut lacrimal, meningeal, or hypothalamic enhancement or pituitary stalk involvement, which are among the other classic MRI findings for sarcoidosis involving the anterior visual pathway. Lymphocytic pleocytosis was typical.

Therapy: The mainstay of therapy for optic nerve/chiasmal sarcoidosis is treatment with systemic corticosteroids. In the unusual event that corticosteroid therapy fails, agents such as cyclosporine, methotrexate, azathioprine, and cyclophosphamide have been administered.
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Additional Reading:

Lama P, Frohman L. Systemic diseases-annual review, 1997. J Neuroophthalmol 1998;18:127-42.

Please send comments to: Dr. Larry Frohman at frohman@UMDNJ.EDU