Vertical gaze palsy and saccadic intrusions due to anti-Ri from head and neck carcinoma

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Identifier Anti_Ri_Midbrain_Syndrome
Title Vertical gaze palsy and saccadic intrusions due to anti-Ri from head and neck carcinoma
Subject OMS Mesencephalon, Square Wave Jerks, Upgaze Palsy, Downgaze Palsy, Abnormal Range, Skew, Vertical Gaze Palsy
Creator Peggy Lazerow, MD., Department of Neurology The Johns Hopkins School of Medicine; Sara Hardy, MD, Department of Neurology, Johns Hopkins School of Medicine; Daniel R. Gold, DO, Departments of Neurology, Ophthalmology, Neurosurgery, Otolaryngology - Head & Neck Surgery, The Johns Hopkins School of Medicine
Description A 55-yo- woman was admitted for imbalance and double vision. Three weeks prior to presentation she first noticed swelling on the right side of her face and neck. CT of the head and neck showed right-sided cervical adenopathy and enlarged left retropharyngeal node. Ultrasound- guided biopsy of the neck mass showed a poorly differentiated, non-small cell carcinoma with focal squamous features. ; MRI brain was initially normal, but T2 changes within the midbrain were seen as her condition worsened. Work-up demonstrated lymphocytic pleocytosis on lumbar puncture and serum paraneoplastic panel was positive for anti-Ri antibody (ANNA-2). Over weeks, she developed complete ophthalmoplegia, dysphagia and respiratory failure. ; On exam, she could not make upward or downward saccades or smooth pursuit movements. However, there was mild preservation of the vertical VOR (i.e., vertical defects were overcome partially), suggestive of at least some degree of "supranuclear" involvement. There was also a left hypertropia, and because she was unable to generate vertical refixation saccades with cover-uncover testing, her misalignment could not be accurately measured. This could have been related to partial 3rd(s) nerve palsy on the basis of nuclear or fascicular damage (although no clear ptosis, mydriasis, adduction paresis, and vertical VOR wouldn't be expected to overcome vertical motility deficits related to a 3rd); however, involvement of the otolith-ocular motor pathways in or around the interstitial nucleus of Cajal (INC - either left sided or left>right sided) causing a skew deviation seemed the most likely explanation. In her case, vertical gaze palsy was probably due to a combination of deficits involving the fascicles of the 3rd nerve, INC, and riMLF. ; The saccadic intrusions seen in this video are most consistent with square wave jerks, although occasionally there appeared to be back-to-back horizontal saccades with no intersaccadic interval, suggestive of ocular flutter. Eye movement recordings could not be completed. Of note, anti-Ri has been associated with ocular flutter and opsoclonus.
Contributor Peggy Lazerow, MD Department of Neurology; The Johns Hopkins School of Medicine Sara Hardy, MD; Department of Neurology; The Johns Hopkins School of Medicine; Daniel R. Gold, D.O.; Departments of Neurology, Ophthalmology, Neurosurgery, Otolaryngology - Head & Neck Surgery; The Johns Hopkins School of Medicine
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Type Image/MovingImage
Format video/mp4
Rights Management Copyright 2017. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E, SLC, UT 84112-5890
Collection Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
Language eng
ARK ark:/87278/s6gt9c6s
Setname ehsl_novel_gold
Date Created 2017-05-31
Date Modified 2018-01-22
ID 1251073
Reference URL https://collections.lib.utah.edu/ark:/87278/s6gt9c6s
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