MCQ – 88987

Correct Answer � B Explanation

Answer: B  low cerebrospinal fluid glucose.

Samples of CSF fluid in the VKH syndrome demonstrate elevated levels of protein as well as lymphocytosis.

The clinical features of VKH disease will vary depending on stage of the disease. The four stages of VKH are the prodromal stage, uveitic stage, chronic stage and chronic recurrent stage.

The prodromal stage symptoms will resemble a viral illness. Headaches, fever, orbital pain, nausea, dizziness and light sensitivity are present. The symptoms will last around 3-5 days. Within the first couple of days the patient will begin to complain about blurred vision, photophobia, hyperemia of the conjunctiva, and ocular pain.

The uveitic stage presents with blurred visual acuity in both eyes (though one eye may be affected first, 94% will involve the second eye within two weeks). The first sign of posterior uveitis include a thickening of the posterior choroid manifested by elevation of the peripapillary retino-choroid layer, hyperemia and edema of the optic disk, and circumscribed retinal edema; accompanied by multiple serous retinal detachments. Eventually, the inflammation becomes more diffuse affecting the anterior chamber, presenting itself as a panuveitis.

The chronic or convalescent stage will take place weeks after the uveitic stage. It is characterized by the development of vitiligo, poliosis and depigmentation of the choroid. Sugiura’s sign (perilimbal vitiligo) is the earliest depigmentation to occur,  presenting itself one month after the uveitic stage. Choroidal depigmentation occurs several months after the uveitic stage, leading to a pale disc with a bright red-orange choroid known as a “sunset-glow fundus.” This phase may last several months.

The recurrent stage consists of a panuveitis with acute exacerbations of anterior uveitis. Recurrent posterior uveitis with exudative retinal detachment is uncommon. Iris nodules may appear in this stage. It is during this phase of the illness that most of the vision threatening complications will develop (cataracts, glaucoma, subretinal neovascularization, etc.).

Systemic Associations

• Auditory Signs: They consist of sensorineural hearing loss with tinnitus and vertigo (usually present at the onset of the disease).

• Neurological Signs: May include fever, headache, neck stiffness, nausea and vomiting.

• Dermal Signs: Vitiligo can occur on the face, hands, shoulders and lower back about 2-3 months after the onset of VKH.

• Other Signs: Poliosis and alopecia are often present.