Opsoclonus in Uremia With Resolution After Hemodialysis

A 78-year-old man was evaluated for altered mentation in the setting of significant uremia. On examination, he was found to be encephalopathic with generalized myoclonus and spontaneous opsoclonus. He had no known risk factors for the development of opsoclonus and upon undergoing hemodialysis, experienced near resolution of his eye movement abnormalities, thus highlighting a possible link between the uremic state and opsoclonus.

Photo and Video Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Opsoclonus in Uremia With Resolution After Hemodialysis Abhinav R. Changa, MD, Janet C. Rucker, MD, Patrick S. Drummond, MD Abstract: A 78-year-old man was evaluated for altered mentation in the setting of significant uremia. On examination, he was found to be encephalopathic with generalized myoclonus and spontaneous opsoclonus. He had no known risk factors for the development of opsoclonus and upon undergoing hemodialysis, experienced near resolution of his eye movement abnormalities, thus highlighting a possible link between the uremic state and opsoclonus. Journal of Neuro-Ophthalmology 2022;42:e448–e449 doi: 10.1097/WNO.0000000000001327 © 2021 by North American Neuro-Ophthalmology Society A 78-year-old man with Parkinson disease was admitted to the hospital for an acute myocardial infarction. He underwent percutaneous coronary intervention with placement of a drug-eluting stent. He was titrated off inotropic support and transferred to a rehabilitation facility. However, his course was then complicated by development of hospital- Departments of Neurology (ARC, JCR, PSD) and Ophthalmology (JCR), NYU Langone Health, New York, New York; and Department of Neurology, Fresco Institute for Parkinson’s and Movement Disorders (PSD); NYU Langone Health, New York, New York. The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Web site (www. jneuro-ophthalmology.com). A. R. Changa: conceptualization and designing of the work; formulation of videos; acquisition, analysis, and interpretation of data for the work; Treating physician; drafting of the manuscript and revising it critically; final approval of the version to be published. J. C. Rucker: conceptualization and designing of the work; evaluation of videos; acquisition, analysis, and interpretation of data for the work; drafting of the manuscript and revising it critically; final approval of the version to be published. P. S. Drummond: conceptualization and designing of the work; evaluation of videos; acquisition, analysis, and interpretation of data for the work; treating physician; drafting of the manuscript and revising it critically; final approval of the version to be published. Address correspondence to Abhinav R. Changa, MD, NYU Neurology, 222 East 41st Street, 9th Floor, New York, NY 10017; E-mail: [e-mail] e448 acquired pneumonia, cardiorenal syndrome, and worsening confusion and disorientation. A noncontrast head computed tomography showed mild parenchymal volume loss, but no acute structural abnormality. At the time of his initial evaluation, his blood urea nitrogen (BUN) level was 100 mg/dL. On neurologic examination, he was encephalopathic and in moderate distress, reporting a subjective perception of environmental movement. There were spontaneous bursts of arrhythmic, omnidirectional, conjugate saccades without an intersaccadic interval, consistent with opsoclonus (see Supplemental Digital Content, Video 1, http://links.lww.com/WNO/A486) (1); There was multifocal appendicular and axial myoclonus that was most pronounced with action, a right-hand rest tremor, and bilateral upper extremity cogwheel rigidity intermixed with paratonia. Given the presence of acute uremic encephalopathy, he underwent urgent hemodialysis which successfully lowered his BUN to 31 mg/dL. On repeat examination 24 hours after hemodialysis, there was resolution of encephalopathy, opsoclonus (see Supplemental Digital Content, Video 2, http://links.lww.com/WNO/A487), and myoclonus. The remaining findings on ocular motor examination included saccadic pursuit and square wave jerks. These were attributed to the patient’s underlying premorbid Parkinson disease. Opsoclonus is a rare pathologic eye movement characterized by omnidirectional spontaneous conjugate saccades without an intersaccadic interval. These high-frequency eye movements appear chaotic and typically result in the patient experiencing oscillopsia. The current theory regarding the pathophysiology of opsoclonus, as supported by evidence from a biologically plausible neuromimetic ocular motor model, proposes that inherent dysfunction within the cellular membranes of burst neurons in the brainstem makes them prone to oscillation when an imbalance occurs between burst neuron excitability and external inhibition (2). Although opsoclonus can present in isolation, it often presents with myoclonus. In adults, opsoclonus–myoclonus syndrome (OMS) can result from a multitude of etiologies including: infectious or parainfectious brainstem encephalitidies, toxic-metabolic states, or paraneoplastic effects from Changa et al: J Neuro-Ophthalmol 2022; 42: e448-e449 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay small cell lung cancer, ovarian carcinoma, or breast carcinoma. At times the cause is uncertain and is termed idiopathic OMS. Levetiracetam, gabapentin, topiramate, propranolol, and clonazepam have all been used as agents for symptomatic management although the literature is not robust (3). Management of postinfectious and autoimmune etiologies of OMS includes immunosuppressive therapies such as steroids, intravenous immunoglobulin, or plasma exchange. In paraneoplastic OMS, tumor-directed treatment is the primary modality with the aforementioned immunosuppressive therapies used as an adjunct (4). However, the illustrative case demonstrates opsoclonus in an atypical presentation. Opsoclonus and oscillopsia arose in the setting of uremia and resolved after hemodialysis without the use of any symptomatic or immunosuppressive therapies. Although uremia-related opsoclonus is not widely reported, Shaikh proposed that metabolic states such as hyperammonemia and uremia can alter the extracellular environment of neurons within the saccade generating circuit, altering the burst neuron membrane and making these neurons hyperexcitable, symptomatically manifesting as opsoclonus (5). Given that symptom onset and resolution were temporally tightly linked with the development and resolution of uremia respectively, this case demonstrates a link between opsoclonus, uremia, and the Changa et al: J Neuro-Ophthalmol 2022; 42: e448-e449 effect of correcting metabolic abnormalities. Uremia-related opsoclonus has been alluded to in the literature; however, specific cases highlighting this entity are limited. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: A. R. Changa, J. C. Rucker, and P. S. Drummond; b. Acquisition of data: A. R. Changa, J. C. Rucker, and Patrick S. Drummond; c. Analysis and interpretation of data: A. R. Changa, J. C. Rucker, and P. S. Drummond. Category 2: a. Drafting the manuscript: A. R. Changa, J. C. Rucker, and P. S. Drummond; b. Revising it for intellectual content: A. R. Changa, J. C. Rucker, and P. S. Drummond. Category 3: a. Final approval of the completed manuscript: A. R. Changa, J. C. Rucker, and P. S. Drummond. REFERENCES 1. Leigh RJ, Zee DS. The Neurology of Eye Movements, 5th edition. Oxford, New York: Oxford University Press, 2015. 2. Shaikh AG, Ramat S, Optican LM, Miura K, Leigh RJ, Zee DS. Saccadic burst cell membrane dysfunction is responsible for saccadic oscillations. J Neuroophthalmol. 2008;28:329–336. 3. Lemos J, Eggenberger E. Saccadic intrusions: review and update. Curr Opin Neurol. 2013;26:59–66. 4. Gordon L, Dinkin M. Paraneoplastic syndromes in neuroophthalmology. J Neuroophthalmol. 2019;25:1401–1421. 5. Shaikh AG. Saccadic oscillations—membrane, model, and medicine. Expert Rev Ophthalmol. 2012;7:481–486. e449 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.