The Uveo-Meningeal Syndromes

Abstract

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.

Basilar leptomeningitis in Vogt-Koyanagi-Harada disease

“…1 Although the targets of inflammation, melanin-containing cells, 1,2 are predominantly located over the ventral medulla, 3 our patient demonstrated a ponto-mesencephalic and cerebellar predilection of inflammation. …”

“…There was cerebrospinal lymphocytic pleocytosis. Following IV Solu-Medrol, his fundus was significantly improved.Vogt-Koyanagi-Harada disease is a uveomeningeal syndrome of panuveitis with bullous serous retinal detachment, meningitis, and dysacusis.1 Although the targets of inflammation, melanin-containing cells, 1,2 are predominantly located over the ventral medulla, 3 our patient demonstrated a ponto-mesencephalic and cerebellar predilection of inflammation. …”

Neuro-Ophthalmic Presentation of Neuro-Sweet Disease

“…Autoimmune processes commonly associated with uveo-meningeal syndrome include microscopic polyangiitis (MPA), sarcoidosis, Behçet's disease, Vogt-KoyanagiHarada (VKH) disease, and APMPPE. 10 Several cases of SS in literature describe simultaneous occurrence of intraocular and central nervous system (CNS) inflammation, which suggests that SS may be a cause of uveo-meningeal syndrome. 8 We believe that our patient most likely had uveo-meningitis at presentation.…”

Vogt-Koyanagi-Harada syndrome: Experience in a Belgian university hospital and review of the literature

“…Behçet's disease or sarcoidosis), as well as infections or malignancy. [9] Early (< 15 days) and aggressive treatment with high dose corticosteroids (1-2 mg oral prednisolone per kg per day +/-intravenous pulses of 1 g methylprednisolone during the first 3 days) is the first-line treatment in the acute uveitic phase, in order to prevent recurrent uveitis and to avoid evolution to a chronic phase. Intravitreal injections can be applied to treat intra-or subretinal fluid.…”