title: Idiopathic multifocal choroiditis/punctate inner choroidopathy with acute photoreceptor loss or dysfunction out of proportion to clinically visible lesions.
abstract: OBJECTIVE
  To report acute/subacute vision loss and paracentral scotomata in patients with idiopathic multifocal choroiditis/punctate inner choroidopathy due to large zones of acute photoreceptor attenuation surrounding the chorioretinal lesions.
  
  
  METHODS
  Multimodal imaging case series.
  
  
  RESULTS
  Six women and 2 men were included (mean age, 31.5 ± 5.8 years). Vision ranged from 20/20-1 to hand motion (mean, 20/364). Spectral domain optical coherence tomography demonstrated extensive attenuation of the external limiting membrane, ellipsoid and interdigitation zones, adjacent to the visible multifocal choroiditis/punctate inner choroidopathy lesions. The corresponding areas were hyperautofluorescent on fundus autofluorescence and were associated with corresponding visual field defects. Full-field electroretinogram (available in three cases) showed markedly decreased cone/rod response, and multifocal electroretinogram revealed reduced amplitudes and increased implicit times in two cases. Three patients received no treatment, the remaining were treated with oral corticosteroids (n = 4), oral acyclovir/valacyclovir (n = 2), intravitreal/posterior subtenon triamcinolone acetate (n = 3), and anti-vascular endothelial growth factor (n = 2). Visual recovery occurred in only three cases of whom two were treated. Varying morphological recovery was found in six cases, associated with decrease in hyperautofluorescence on fundus autofluorescence.
  
  
  CONCLUSIONS
  Multifocal choroiditis/punctate inner choroidopathy can present with transient or permanent central photoreceptor attenuation/loss. This presentation is likely a variant of multifocal choroiditis/punctate inner choroidopathy with chorioretinal atrophy. Associated changes are best evaluated using multimodal imaging.
authors.affiliation: *Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois; †Department of Ophthalmology, Medical University Vienna, Vienna, Austria; ‡Department of Ophthalmology, New York University School of Medicine, New York, New York; §Vitreous-Retina-Macula Consultants of New York, New York, New York; ¶Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia College of Physicians and Surgeons, New York, New York; **Department of Ophthalmology, Casey Eye Institute, Oregon Health and Science University, Portland, Oregon; and ††National Eye Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: Marion R
authors.initials: MR
authors.lastname: Munk
authors.forename: Jesse J
authors.initials: JJ
authors.lastname: Jung
authors.forename: Kristin
authors.initials: K
authors.lastname: Biggee
authors.forename: William R
authors.initials: WR
authors.lastname: Tucker
authors.forename: H Nida
authors.initials: HN
authors.lastname: Sen
authors.forename: Ursula
authors.initials: U
authors.lastname: Schmidt-Erfurth
authors.forename: Amani A
authors.initials: AA
authors.lastname: Fawzi
authors.forename: Lee M
authors.initials: LM
authors.lastname: Jampol
mesh_terms: D000208:Acute Disease; D000328:Adult; D002833:Choroiditis / Q000150:complications / Q000175:diagnosis*; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D008297:Male; D000080364:Multifocal Choroiditis; D064847:Multimodal Imaging; D020419:Photoreceptor Cells, Vertebrate / Q000473:pathology*; D012164:Retinal Diseases / Q000150:complications / Q000175:diagnosis*; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D014786:Vision Disorders / Q000175:diagnosis* / Q000209:etiology; D014792:Visual Acuity / Q000502:physiology; D014794:Visual Fields; D055815:Young Adult