Primary Uveal Lymphoma is an exceptionally rare type of intraocular lymphoma involving the choroid, iris, or ciliary body. PVRL is the most common intraocular lymphoma, and also constitutes the majority of neoplastic uveitic masquerading syndromes, representing 75% of the malignant UMS cases documented in the reports of Grange and Rothova (30/40). Primary Intraocular Lymphoma (PIL), otherwise known as primary vitreoretinal lymphoma (PVRL) is a subset of Primary CNS Lymphoma (PCNSL) where ocular symptoms represent the initial clinical findings. Herein we provide an overview of the conditions that can mimic uveitis, with a focus on clinical presentation, incidence, and clinical strategies for appropriate and timely diagnosis.Įtiology Neoplastic Masquerade Syndromes Intraocular lymphomas Masquerade syndromes can mimic more common ocular inflammatory conditions such as sarcoidosis, toxoplasmosis, syphilis, tuberculosis, intermediate uveitis, acute retinal necrosis, birdshot chorioretinitis and idiopathic retinal vasculitis. Awareness of the clinical conditions and presentations that mimic uveitis, and timely diagnosis of the underlying pathology, is essential to preserve visual acuity, and, in cases of neoplastic masqueraders, can be life-saving. While rare, Uveitis Masquerade Syndromes represent an important clinical entity. Similarly Grange reported a UMS prevalence of 2.5%, as 21/853 patients reporting to the National Eye Institute with uveitis between 20 were diagnosed with a neoplastic masquerade syndrome. In 2001 Rothova found that UMS was diagnosed in 5% of patients within a tertiary uveitis clinic (40/828 patients), and furthermore 48% of those UMS cases were found to have intraocular malignancy (19/40 patients). However, there have been two large retrospective studies within the literature that reported frequency of masquerading syndromes within large uveitis centers. The relative paucity of cases, in addition to common misdiagnosis and a dearth of published reports on UMS cases, has made it challenging to accurately report epidemiology and clinical characteristics of UMS. 1.2.2 Non-Malignant Masquerade Syndromes.National Institute of Neurological Disorders and Stroke. Sleep disorders in children with Tourette syndrome. ![]() Philadelphia, Pa.: Saunders Elsevier: 2012. In: Swaiman's Pediatric Neurology: Principles and Practice. Journal of Obsessive-Compulsive and Related Disorders. Pharmacological treatment of Tourette syndrome. Long-term outcomes of globus pallidus internus deep brain stimulation in patients with Tourette syndrome. In: Ferri's Practical Guide: Fast Facts for Patient Care.
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