First Author: R.Hamam LEBANON
Co Author(s): R. Antonios 0 0 0 0 0 0 0 0 0
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To present the case of a fourty-one year old male patient who presented to us with right eye visual disturbance that evolved into subacute loss of vision and was subsequently diagnosed with Rosai Dorfman disease.
The patient was examined at the Ophthalmology Department at the American University of Beirut Medical Center.
Retrospective chart review of the case and a PUBMED literature review of Rosai Dorfman disease and ocular manifestations.
The patient presented with blurring of vision affecting the right eye of two weeks duration. He reported history of hearing loss a year earlier that was diagnosed as inflammatory inner ear disease and he was maintained on systemic corticosteroids (Prednisone 25 mg PO daily). Investigation of the patient revealed visual acuity of 20/32, right eye and 20/20, left eye. Color plates showed deficiency on the right side. Biomicroscopic eye exam was normal bilaterally. OCT of the ONH was normal and fluorescein angiography was normal. Visual evoked potential revealed delayed latency and decreased amplitude on the right side. MRI brain revealed multiple meningeal lesions with no mass compression of the visual pathway. Meanwhile, patient presented with acute painless loss of vision to light perception, right eye. Biopsy of the brain lesion revealed histiocytes consistent with Rosai Dorfman disease. The vision responded to pulse intravenous Solumedrol therapy with improvement to 20/30.
Rosai Dorfman disease rarely presents as isolated intracranial meningeal lesions without extra-cranial lymphadenopathy. It has been associated with autoimmune manifestations, however, optic neuritis as a presenting sign of Rosai Dorfman disease has not been described previously. Treatment with high dose corticosteroids controlled the ocular symptoms in our patient.