The Monocular Duke of Urbino.

Federico da Montefeltro (1422-1482), the Duke of Urbino, was a well-known historical figure during the Italian Renaissance. He is the subject of a famous painting by Piero della Francesca (1416-1492), which displays the Duke from the left and highlights his oddly shaped nose. The Duke is known to have lost his right eye due to an injury sustained during a jousting tournament, which is why the painting portrays him from the left. Some historians teach that the Duke subsequently underwent nasal surgery to remove tissue from the bridge of his nose in order to expand his visual field in an attempt to compensate for the lost eye. In theory, removal of a piece of the nose may have expanded the nasal visual field, especially the "eye motion visual field" that encompasses eye movements. In addition, removing part of the nose may have reduced some of the effects of ocular parallax. Finally, shifting of the visual egocenter may have occurred, although this seems likely unrelated to the proposed nasal surgery. Whether or not the Duke actually underwent the surgery cannot be proven, but it seems unlikely that this would have substantially improved his visual function.

Tug of war.

A 74-year-old man had reproducible superior and inferior arcuate visual field defects in the left eye only that were initially believed to be caused by primary open-angle glaucoma. Diagnostic evaluation with the aid of optical coherence tomography revealed extrafoveal vitreomacular traction (VMT) with secondary retinal thickening and schisis. We discuss the evaluation of non-glaucomatous visual field defects and review the literature on the pathogenesis, clinical manifestations, and treatment of VMT syndrome.

HIV and cannot see.

A 55-year-old HIV-positive man presented with acute vision loss in the right eye and altered mental status. Ophthalmic evaluation revealed light perception vision OD with a right relative afferent pupillary defect, conjunctival chemosis, large mutton-fat keratitic precipitates, and diffuse cream-colored vitreous cells. Magnetic resonance imaging of the brain and orbit with and without contrast with fat saturation showed choroidal thickening OD, multifocal deep periventricular and deep ganglionic enhancing lesions, and a suprasellar mass. Brain biopsy showed diffuse large B-cell lymphoma. Intrathecal chemotherapy with methotrexate and cytarabine and whole brain radiation therapy failed. His mental status deteriorated. He developed pancytopenia, neutropenic fever, and septic shock and subsequently expired under palliative care.

A weed by any other name.

A 39-year-old white man presented with intractable headaches and papilledema. The initial workup, with normal MRI and MRV but elevated cerebrospinal fluid protein raised concerns about the putative diagnosis of idiopathic intracranial hypertension, and his condition remained refractory to maximum medical treatment. Angiography revealed cerebral venous sinus stenosis, thought to represent chronic thrombosis. The diagnosis and treatment of cerebral venous sinus stenosis and thrombosis are discussed.

Bilateral optic disk swelling plus.

A 64-year-old woman presented with bilateral optic neuropathy leading to a diagnosis of Sjögren syndrome. She improved with high-dose corticosteroids and oral azathioprine and was subsequently found to have asymptomatic bilateral iridocyclitis. Although central nervous system manifestations of Sjögren syndrome are documented in the literature, they are not widely recognized in clinical practice. Associated optic neuritis often mimics demyelinating disease such as multiple sclerosis. Treatment of CNS disease related to Sjögren syndrome is highly controversial. Uveitis is an uncommon finding associated with Sjögren syndrome.

A case of orbital Rosai-Dorfman disease responding to radiotherapy.

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder most often characterized by painless cervical lymphadenopathy, but it may also present with orbital disease. The clinical course of RDD is variable; it can be either relapsing-remitting or progressive, and the outcome relates to clinical location and treatment response. Orbital RDD can have an insidious onset and similar presentation to other ophthalmic conditions; this can result in a delayed diagnosis. Nearly all cases of orbital RDD cause visual disturbances and require treatment. Because orbital RDD is an uncommon presentation, a variety of interventions have been employed, including surgery, immunotherapy, chemotherapy, and radiotherapy. We present a case of salvage radiotherapy for progressive orbital RDD refractory to surgery and chemotherapy in a pediatric patient.

Optic neuritis in evolution.

A case of progressive optic neuropathy in a woman with a history of breast cancer is presented. Differential diagnoses including optic neuritis, infiltrative optic neuropathy, carcinomatous meningitis, and toxic optic neuropathies are discussed. Risk factors for metastatic brain lesions are also discussed.

Acute disseminated encephalomyelitis following infectious mononucleosis.

Two months following an Epstein-Barr virus infection, a 17-year-old white female presented with seizures, intermittent visual changes, and altered mental status. Magnetic resonance imaging showed white matter changes of acute disseminated encephalomyelitis with a predilection for posterior cerebral artery distributions but without radiological evidence of arteritis. Epstein-Barr virus titers and polymerase chain reaction analysis results for the virus were consistent with postinfectious acute disseminated encephalomyelitis. The symptoms and signs improved following treatment with high-dose corticosteroids and intravenous immunoglobulin. Although Epstein-Barr virus can cause acute viral encephalomyelitis, the authors report a case of acute disseminated encephalomyelitis months after acute Epstein-Barr virus infection.

Treatment of neuro-ophthalmologic manifestations of tuberculosis.

In the absence of obvious pulmonary or disseminated tuberculosis, ocular and central nervous system (CNS) tuberculosis may represent a significant diagnostic challenge. Refinements in polymerase chain reaction techniques and neuroimaging have strengthened the battery of tests used to diagnose CNS and ocular tuberculosis, yet in many cases, the diagnosis remains one of exclusion; it may ultimately be determined through exacerbation by anti-inflammatory therapy with subsequent improvement by antitubercular medication treatment. Because of emerging drug resistance, at least a two-drug regimen is required for therapeutic testing and treatment of isolated ocular tuberculosis. If pulmonary or miliary disease coexists, a 6-month, four-drug regimen with isoniazid, rifampin, pyrazinamide, and ethambutol is required for treatment. Tubercular meningitis is treated with the same four-drug regimen for at least 9 to 12 months. Burden of therapeutic compliance rests on the treating physician and public health sector. Best compliance is realized with directly observed therapy.

Lacrimal gland involvement in Kikuchi-Fujimoto disease.

PURPOSE. A 32-year-old Saudi female presented with typical Kikuchi-Fujimoto disease, i.e., fever, cervical lymphadenitis and leukopenia, but there was also painful upper eyelid swelling with pain on upgaze. METHODS. A connective tissue disease and lymphoma workup were unremarkable, as were antibody titers to Apifia felis and Bartonella henselae. RESULTS. Orbital computed tomography showed significant lacrimal gland enlargement. Cervical node biopsy revealed necrotizing lymphadenitis. CONCLUSION. Concomitant lacrimal gland inflammation and cervical lymphadenopathy may be a benign self-limited disease.