Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature.
OBJECTIVE
The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options.


METHODS
Retrospective, noncomparative case series.


METHODS
Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature.


METHODS
A review of nine patients with AMPPE and CNS involvement was performed. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment.


RESULTS
Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis.


CONCLUSIONS
Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. Neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.
O'Halloran
H S
HS
Department of Ophthalmology, University of Kentucky, Lexington, KY 40536, USA. hohall@pop.uky.edu
Berger
J R
JR
Lee
W B
WB
Robertson
D M
DM
Giovannini
J A
JA
Krohel
G B
GB
Meckler
R J
RJ
Selhorst
J B
JB
Lee
A G
AG
Nicolle
D A
DA
O'Day
J
J
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D001927:Brain Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D002533:Cerebral Angiography; D005260:Female; D005451:Fluorescein Angiography; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D012164:Retinal Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013129:Spinal Puncture; D014792:Visual Acuity
D005938:Glucocorticoids