the optic nerve (see Chapter 4, Fig 4-20). Optic nerve dysfunction usually results in disturbance of vision (blurred, dim, or dark vision). Signs of optic nerve involvement include visual field defects and decreased Snellen visual acuity, color vision, and/or contrast sensitivity. A relative afferent pupil defect (RAPD) will be present if optic nerve involvement is asymmetric. The optic disc may appear normal or may appear edematous or atrophic. Most patients with compressive optic neuropathy have evidence of a restrictive myopathy with or without proptosis.

Diagnosis of thyroid eye disease

The diagnosis of TED begins with clinical suspicion. The thyroid function may be normal or abnormal, but the pathognomonic combination of signs such as eyelid retraction and multiple enlarged extraocular muscles secures the diagnosis. In contrast with inflammatory myositis, imaging studies of the orbit will demonstrate sparing of the insertions of the muscles into the globe (tendons). The differential diagnosis of proptosis is extensive and includes orbital inflammatory disease, orbital masses, and carotid cavernous fistula. In the setting of eyelid retraction, other conditions to be considered include dorsal midbrain syndrome and aberrant regeneration of the oculomotor nerve.

Treatment of thyroid eye disease

A full discussion of treatment may be found in BCSC Section 7, Orbit, Eyelids, and Lacrimal System. Systemically, any abnormalities in thyroid function should be addressed. Any patient with TED who will be treated with radioactive iodine should receive concurrent corticosteroid therapy to decrease exacerbations of eye disease. Smoking cessation programs should be instituted, as smoking is a risk factor for TED progression.

Ocular and orbital therapy should be tailored to the signs and symptoms. The presence of optic nerve dysfunction requires prompt therapeutic intervention, which may include high-dose oral or pulsed intravenous corticosteroids followed by radiation therapy or surgical decompression.

See BCSC Section 7, Orbit, Eyelids, and Lacrimal System, for a more extensive discussion of TED; and Section 1, Update on General Medicine, for further coverage of thyroid abnormalities.

Bradley EA, Gower EW, Bradley DJ, et al. Orbital radiation for Graves ophthalmopathy: a report from the American Academy of Ophthalmology. Ophthalmology. 2008;115(12):398–409.

Phillips ME, Marzban MM, Kathuria SS. Treatment of thyroid eye disease. Curr Treat Options Neurol. 2010;12(1):64–69.

Sarcoidosis

Sarcoidosis is a multisystem, noncaseating granulomatous inflammation commonly diagnosed in young adults, aged 20–40 years. The annual incidence for blacks is about 3 times that for whites, and the disease is more common in women than in men. The lungs are involved most frequently, but the eyes, liver, lymph nodes, skin, and musculoskeletal system are commonly affected. Neurologic manifestations occur in 5%–15% of patients and include meningitis, hydrocephalus, parenchymal involvement (most commonly hypothalamic), encephalopathy, seizures, dural venous thrombosis, vasculitis, and peripheral neuropathy. However, 30%–60% of cases may be discovered incidentally on a routine chest radiograph.

Gullapalli D, Phillips LH II. Neurologic manifestations of sarcoidosis. Neurol Clin. 2002;20(1):59–83.

Koczman JJ, Rouleau J, Gaunt M, Kardon RH, Wall M, Lee AG. Neuro-ophthalmic sarcoidosis: the University of Iowa Experience. Semin Ophthalmol. 2008;23(3):157–168.