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(Stroke. 1996;27:996-1001.)
© 1996 American Heart Association, Inc.

Articles

Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Sinan Çomu, MD; Thierry Verstraeten, MD; Jeffrey S. Rinkoff, MD Neil A. Busis, MD

From the Division of Child Neurology (S.Ç.) and Departments of Ophthalmology (J.S.R.) and Neurology (N.A.B.), University of Pittsburgh School of Medicine, and Retina Service (T.V.), Allegheny General Hospital, Pittsburgh, Pa.

Correspondence to Sinan Çomu, MD, Children's Hospital of Pittsburgh, Division of Child Neurology, 3705 Fifth Ave, Pittsburgh, PA 15213-2583.

Background Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a chorioretinal disease that causes acute visual symptoms with characteristic fundus findings. Although this entity has been associated with a variety of neurological complications, it has received little attention in the neurological literature. We wanted to emphasize the spectrum of neurological involvement, in particular the occurrence and management of strokes in patients with APMPPE.

Case Descriptions We report three patients with APMPPE and neurological disease. All three presented with marked visual disturbances and headaches. One patient developed recurrent strokes involving different vascular territories of the brain and required immunosuppressive treatment for presumed cerebral vasculitis. The other two patients had cerebrospinal fluid pleocytosis and persistent headaches but recovered spontaneously. The review of the literature demonstrates a particular pattern of neurological complications in a subgroup of patients with APMPPE.

Conclusions APMPPE should be considered among the causes of stroke and aseptic meningitis in young adults. The diagnosis is critically dependent on a thorough ophthalmologic examination. Severe neurological complications are difficult to predict at the onset of the ophthalmologic disease. The patients should be monitored closely. If investigations suggest cerebral vasculitis, immunosuppressive treatment may be helpful to prevent recurrences.

Key Words: cerebral infarction • meningitis • ocular disease • vasculitis • vision disorders

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