What is APMPPE eye disease?
What is APMPPE eye disease?
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare eye disorder of unknown (idiopathic) cause. The disorder is characterized by the impairment of central vision in one eye (unilateral) but, within a few days, the second eye may also become affected (bilateral).
What causes APMPPE?
The exact etiology of APMPPE is unknown, however some believe that it is secondary to a delayed-type hypersensitivity-induced occlusive vasculitis. Gass originally posited that inflammation of the outer retina and RPE causes the APMPPE phenotype.
What is retinal pigment Epitheliopathy?
Epitheliopathy or acute posterior multifocal placoid pigment epitheliopathy (APMPPE) refers to an acquired inflammatory illness affecting the retinal pigment epithelium. It can affect one or both eyes, and is characterized by multiple yellowish white or light-colored lesions that form in the retina.
What is relentless Placoid Chorioretinitis?
Relentless placoid chorioretinitis (RPC) is characterized by retinal lesions similar in clinical and angiographic appearance to acute posterior multifocal pigment placoid epitheliopathy (APMPPE) and serpiginous choroidopathy but differing in its widespread distribution, the numerous lesions typically seen, and the …
How long does Mewds last?
MEWDS is a self limited disease with excellent visual recovery within 2-10 weeks. However residual symptoms including photopsia may persist for months.
What is Pachychoroid pigment Epitheliopathy?
Pachychoroid Pigment Epitheliopathy PPE is thought to represent a form fruste or precursor of CSCR, as it has features of retinal pigment epithelium disturbances similar to CSCR but without clinical or imaging evidence of acute or chronic subretinal fluid.
What causes retinal pigmentation?
What causes RP? RP is an inherited disorder that results from harmful changes in any one of more than 50 genes. These genes carry the instructions for making proteins that are needed in cells within the retina, called photoreceptors.
What is Serpiginous Choroiditis?
Serpiginous Choroiditis is a rare recurrent eye disorder characterized by irregularly shaped (serpiginous) lesions involving two layers of the eye surface (the retinal pigment epithelium and the choriocapillaris). No symptoms are apparent unless a specific area of the retina (macula) is damaged.
How common is MEWDS?
The typical patient with MEWDS is a healthy middle aged female age 15-50. There is a gender disparity as women are affected with MEWDS four times more often than men. Roughly 30% of patients have experienced an associated viral prodrome.
How do you treat MEWDS?
Since MEWDS is a self-limited disease, with almost all patients regaining good visual acuity within 3-9 weeks, no treatment is recommended. Photopsias and scotomata gradually resolve and the lesions will disappear and may be replaced by mild pigment mottling or chorioretinal scarring.
What is Pachychoroid disease?
Abstract. Pachychoroid is a relatively novel concept describing a phenotype characterized by attenuation of the choriocapillaris overlying dilated choroidal veins, and associated with progressive retinal pigment epithelium dysfunction and neovascularization.
What is pattern dystrophy?
Pattern dystrophies are a group of autosomal dominant macular diseases characterized by various patterns of pigment deposition within the macula. The primary layer of the retina effected is the retinal pigment epithelium (RPE) which is responsible for removing and recycling waste within the retina.
Are there any CNS disorders associated with APMPPE?
Other associated CNS disorders that are sometimes associated with APMPPE include optic neuritis, peripheral vestibular disorders, meningoencephalitis, seizures, venous sinus thrombosis, intracerebral hemorrhage, and CVA/TIA . Anterior segment exam is usually normal, though anterior uveitis can be present.
When was acute posterior placoid pigment epithelium ( APMPPE ) first described?
In 1968, Gass first described acute posterior multifocal placoid pigment epithelium (APMPPE) by presenting the clinical and fluorescein angiography findings in three young females with bilateral central vision loss .
What causes hypofluorescence in a placoid angiogram?
Indocyanine Green (ICG) Angiogram: Active placoid lesions manifest as early and late hypofluorescence, which is thought to occur due to poor perfusion of the choriocapillaris. Normalization of the ICG signal may occur as the lesions resolve .
When to consider relentless placoid choroiditis ( RPC )?
Relentless placoid choroiditis: a diagnosis of RPC should be considered with persistent APMPPE that extends beyond 6 months. Placoid lesions in RPC are generally more numerous and peripheral, even anterior to the equator, when compared with APMPPE .
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