A 49-year-old man presented with a 15-month history of binocular diplopia and oscillopsia and a 6-month history of headaches, memory loss, anorexia, myalgias, and unintentional 105-pound weight loss without diarrhea. Neuro-ophthalmic assessment revealed episodic bursts of opsoclonus, intermittent jerk nystagmus, saccadic pursuit, and slowed and dysmetric saccades, and he was unable to maintain fixation (video 1). Anti-DPPX antibodies were positive in the CSF. He was treated with IV methylprednisolone, plasmapheresis, and rituximab, and his ocular symptoms resolved several months after treatment. Opsoclonus is an ocular manifestation of anti-DPPX encephalitis, which may also cause nystagmus, skew deviation, and saccadic pursuit due to DPPX expression in the cerebellum.1,2
Voluntary eye movements were slow and limited in all directions of gaze. The patient had saccadic pursuit, dysmetric saccades, and was unable to maintain fixation. He had episodic bursts of opsoclonus with jerk nystagmus. Six months after treatment, his eye movements were normal apart from a small amplitude gaze-evoked nystagmus.Download Supplementary Video 1 (13.3MB, mp4) via http://dx.doi.org/10.1212/007463_Video_1
Footnotes
Teaching slides links.lww.com/WNL/A875
Author contributions
J.A. Micieli: study conception and design, manuscript preparation. N.J. Newman: study conception and design, final approval of manuscript. C.S. Kase: study conception and design, final approval of manuscript. V. Biousse: study conception and design, final approval of manuscript.
Study funding
This study was supported in part by an unrestricted departmental grant (Department of Ophthalmology) from Research to Prevent Blindness, Inc., New York, and by NIH/NEI core grant P30-EY006360 (Department of Ophthalmology).
Disclosure
The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.
References
- 1.Tobin WO, Lennon VA, Komorowski L, et al. DPPX potassium channel antibody: frequency, clinical accompaniments, and outcomes in 20 patients. Neurology 2014;83:1797–1803. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Balint B, Jarius S, Nagel S, et al. Progressive encephalomyelitis with rigidity and myoclonus: a new variant with DPPX antibodies. Neurology 2014;82:1521–1528. [DOI] [PubMed] [Google Scholar]
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Supplementary Materials
Voluntary eye movements were slow and limited in all directions of gaze. The patient had saccadic pursuit, dysmetric saccades, and was unable to maintain fixation. He had episodic bursts of opsoclonus with jerk nystagmus. Six months after treatment, his eye movements were normal apart from a small amplitude gaze-evoked nystagmus.Download Supplementary Video 1 (13.3MB, mp4) via http://dx.doi.org/10.1212/007463_Video_1