NEUROLOGY2015641365 385..385 NEUROIMAGES Pseudo-Foster-Kennedy syndrome with optic nerve compression by the gyrus rectus A 21-year-old woman presented with headaches and left eye visual loss. Examination revealed acuity 20/20 OD and finger counting OS, a left afferent pupillary defect, papilledema OD, and optic atrophy OS. Left atrophy was unexplained until orbital MRI revealed left nerve compression by the gyrus rectus (figure, A), displaced by an intraventricular central neurocytoma (figure, B). Foster-Kennedy syndrome is characterized by optic atrophy on one side due to direct optic nerve mass lesion compression with contralateral papilledema. This case is termed pseudo-Foster-Kennedy with indirect compressive optic neuropathy due to brain displacement from a tumor distant from the optic nerve.1 Ninad Desai, MBBS, Raymund L. Yong, MD, Amish Doshi, MD, Janet C. Rucker, MD From NYU Langone Medical Center (N.D., J.C.R.); and Mount Sinai Medical Center (R.L.Y., A.D.), New York, NY. Author contributions: Ninad Desai: manuscript writing and editing. Raymund Yong: conception of idea, manuscript writing and editing. Amish Doshi: manuscript writing and editing. Janet Rucker: conception of idea, manuscript writing and editing. Study funding: No targeted funding reported. Disclosure: The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures. Correspondence to Dr. Rucker: janet.rucker@nyumc.edu 1. Klingele TG, Gado MH, Burde RM, Coxe WS. Compression of the anterior visual system by the gyrus rectus. J Neurosurg 1981;55:272–275. Figure Optic nerve compression by gyrus rectus and intraventricular mass causing displacement of gyrus rectus Coronal T2-weighted orbital and brain MRI reveals left lateral ventriculomegaly with downward displacement of the gyrus rectus, resulting in left optic nerve compression (A), due to a left intraventricular mass (B). © 2015 American Academy of Neurology 385 ª 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. http://neurology.org/lookup/doi/10.1212/WNL.0000000000001791 mailto:janet.rucker@nyumc.edu DOI 10.1212/WNL.0000000000001791 2015;85;385 Neurology Ninad Desai, Raymund L. Yong, Amish Doshi, et al. Pseudo-Foster-Kennedy syndrome with optic nerve compression by the gyrus rectus This information is current as of July 27, 2015 Services Updated Information & http://n.neurology.org/content/85/4/385.full including high resolution figures, can be found at: References http://n.neurology.org/content/85/4/385.full#ref-list-1 This article cites 1 articles, 0 of which you can access for free at: Subspecialty Collections http://n.neurology.org/cgi/collection/primary_brain_tumor Primary brain tumor http://n.neurology.org/cgi/collection/optic_nerve Optic nerve following collection(s): This article, along with others on similar topics, appears in the Errata /content/85/14/1263.full.pdf or: page nextAn erratum has been published regarding this article. Please see Permissions & Licensing http://www.neurology.org/about/about_the_journal#permissions its entirety can be found online at: Information about reproducing this article in parts (figures,tables) or in Reprints http://n.neurology.org/subscribers/advertise Information about ordering reprints can be found online: rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X. 1951, it is now a weekly with 48 issues per year. Copyright © 2015 American Academy of Neurology. All ® is the official journal of the American Academy of Neurology. Published continuously sinceNeurology http://n.neurology.org/content/85/4/385.full http://n.neurology.org/content/85/4/385.full#ref-list-1 http://n.neurology.org/cgi/collection/optic_nerve http://n.neurology.org/cgi/collection/primary_brain_tumor http://www.neurology.org/about/about_the_journal#permissions http://n.neurology.org/subscribers/advertise an initial brainstem attack sharing chronic lympho- cytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) fea- tures. After steroid weaning, MOG-seropositive lon- gitudinally extensive transverse myelitis (LETM) involving the conus appeared, but in absence of brainstem lesions.1 A diagnosis of CLIPPERS is dif- ficult in this clinical picture. Brainstem punctate and curvilinear enhancements, a characteristic radiologic finding of CLIPPERS, may conceal several diseases such as glioma, primary CNS lymphoma, lymphomatoid granulomatosis, primary CNS vasculitis, and multiple sclerosis. Except for gli- oma, all of these diseases initially respond to high doses of steroids, and some could have a relapsing-remitting course in absence of immunosuppressive therapy. However, unlike CLIPPERS, the brainstem is not sys- tematically affected at each relapse and lesion distribu- tion does not remain concentrated in the pons.2–4 The Symmonds et al. case highlights a possible pathophysiologic connection between CLIPPERS and demyelinating diseases. Recently, a postmortem analysis performed in a patient sharing all CLIPPERS features revealed the classical perivascular lymphohis- tiocytic infiltrates but also perivenular demyelinating lesions (as seen in acute disseminated encephalomye- litis).5 Since perivenular demyelinating lesions were found in only one CLIPPERS patient, it is unlikely that CLIPPERS is a primary demyelinating disease. However, as suggested by the authors, CLIPPERS may induce immunization against MOG antigen. Author Response: Mkael Symmonds, M. Isabel Leite, Ursula G. Schulz, Oxford, UK: We agree with the important points that Drs. Taieb and Lab- auge raised in response to our recent report.1 Our patient presented with clinical and radiologic features consistent with CLIPPERS, even though the subse- quent episode of LETM involving the conus had not been previously reported as part of this disease spec- trum. While CLIPPERS can relapse, typically with recurrence of brainstem inflammatory features, this does not form key diagnostic criteria and the diagno- sis could be consistent with a single episode. As discussed, the differential diagnosis of CLIPPERS is wide and many of these alternatives will clearly define themselves in time. The histo- pathologic findings of demyelination in an isolated case at postmortem provides interesting additional support for the hypothesis that CLIPPERS may not be a distinct entity, but rather have a range of underlying etiologies. Our case raises the possibility that anti-MOG antibodies may be causal in some CLIPPERS cases, although we cannot exclude the possibility that CLIPPERS itself causes immuniza- tion against MOG epitopes. © 2015 American Academy of Neurology 1. Symmonds M, Waters PJ, Küker W, et al. Anti-MOG anti- bodies with longitudinally extensive transverse myelitis pre- ceded by CLIPPERS. Neurology 2015;84:1177–1179. 2. Jones JL, Dean AF, Antoun N, et al. “Radiologically com- patible CLIPPERS” may conceal a number of pathologies. Brain 2011;134:e187. 3. Limousin N, Praline J, Motica O, et al. Brain biopsy is required in steroid-resistant patients with chronic lympho- cytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurooncol 2012; 107:223–224. 4. Ferreira RM, Machado G, Souza AS, et al. CLIPPERS-like MRI findings in a patient with multiple sclerosis. J Neurol Sci 2013;327:61–62. 5. Moreira I, Cruto C, Correia C, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): postmortem findings. J Neuropathol Exp Neurol 2015;74:186–190. CORRECTION Pseudo-Foster-Kennedy syndrome with optic nerve compression by the gyrus rectus In the NeuroImage “Pseudo-Foster-Kennedy syndrome with optic nerve compression by the gyrus rectus” by N. Desai et al. (Neurology® 2015;85:385), there is an error in the correspondence address. The note at the bottom should read “Corre- spondence to Dr. Rucker: janet.rucker@nyumc.org.” The authors regret the error. Author disclosures are available upon request (journal@neurology.org). Neurology 85 October 6, 2015 1263 ª 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. mailto:janet.rucker@nyumc.org mailto:journal@neurology.org