The uveo-meningeal syndromes

The uveo-meningeal syndromes are a group of disorders that share involvement of the uvea, retina, and meninges.

We review the clinical manifestations of uveitis and describe the infectious, inflammatory, and neoplastic conditions associated with the uveo-meningeal syndrome.

Inflammatory or autoimmune diseases are probably the most common clinically recognized causes of true uveo-meningeal syndromes. These entities often cause inflammation of various tissues in the body, including ocular structures and the meninges (eg, Wegener granulomatosis, sarcoidosis, Behcet disease, Vogt-Koyanagi-Harada syndrome, and acute posterior multifocal placoid pigment epitheliopathy). The association of an infectious uveitis with an acute or chronic meningoencephalitis is unusual but occasionally the eye examination may suggest an infectious etiology or even a specific organism responsible for a meningeal syndrome. One should consider the diagnosis of primary ocular-CNS lymphoma in patients 40 years of age or older with bilateral uveitis, especially with prominent vitritis, that fails to respond to treatment or who has associated neurologic findings. A paraneoplastic disorder has been described in patients who have combined optic neuritis and retinitis defined serologically by the presence of a paraneoplastic IgG autoantibody CRMP-5-IgG. These patients may have an inflammatory vitritis and may have signs of cerebrospinal fluid inflammation.