Neonatal Opsoclonus

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Identifier 166-3
Title Neonatal Opsoclonus
Ocular Movements Opsoclonus; Upbeat Nystagmus; Lid Nystagmus
Creator Shirley H. Wray, M.D., Ph.D., FRCP, Professor of Neurology Harvard Medical School, Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Contributor Primary Shirley H. Wray, MD, PhD, FRCP, Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Subject Neonatal Opsoclonus; Upbeat Nystagmus; Lid Nystagmus; Saccadic Oscillations
Presenting Symptom Jiggling Eyes
History This child was one of the first cases of opsoclonus that I saw with Dr. Cogan in the early 1970's. The baby is a unique case in that in addition to neonatal opsoclonus with the characteristic multidirectional conjugate back-to-back saccades, periods of large amplitude upbeat nystagmus also occurred. The child had no developmental delay and was neurologically intact. He made a complete spontaneous recovery regaining normal eye movements over a period of two months. Differential Diagnosis: 1. Benign encephalitis 2. Parainfectious opsoclonus 3. Neuroblastoma Opsoclonus in Childhood: The term "opsoclonus" coined by Orzechowski in 1913, denotes a striking ocular motility disorder characterized by involuntary, irregular, chaotic bursts of conjugate multidirectional, high amplitude saccades without an intersaccadic interval. When purely horizontal, such oscillations are called ocular flutter. Opsoclonus in children may occur as part of a "benign" brainstem encephalitis. In affected children or adults, vertigo and trunkal ataxia following a prodrome of malaise and fever is the characteristic onset. Cerebellar and long tract signs accompanying shivering movements of the head and body occur along with the constant changing, often forceful myoclonic jerking of the extremities and trunk (polymyoclonia), there may also be shock-like torsions of the head and neck as well as opsoclonus. Spinal fluid protein and lymphocyte count may be elevated. The term myoclonic encephalopathy or dancing eyes and dancing feet have been given to opsoclonus with myoclonus. Myoclonic encephalitis in childhood usually recovers over a period of weeks to months, but the clinical course may be protracted and recovery incomplete. Recent findings of small neuroblastomas or ganglio- neuroblastomas in children with the chronic form of myoclonic encephalopathy have led some investigators to suggest that myoclonic encephalopathy may reflect the presence of an indolent neural crest tumor that was previously impossible to identify without high-resolution CT scanning or MR imaging. Parainfectious Brainstem Encephalopathy: Although Cogan and I saw cases of myoclonic encephalopathy, we tended to see more children with either a mild benign form of neonatal opsoclonus without myoclonus or preschool children with parainfectious opsoclonus. Parainfectious opsoclonus may be due to an infection with: 1. Enterovirus 2. Coxsackie B3 3. Epstein-Barr virus where the presence of a strong intrathecal immune response is associated with high EBV viral titers. Only the results of the EBV serology give a beyond-doubt diagnosis. Opsoclonus has less commonly been attributed to exposure to toxins or drugs, systemic disease, trauma, meningitis, hydrocephalus, and intracranial tumors. For a complete overview of opsoclonus in childhood, I recommend you review all the cases in this collection. ID 166-4 Neonatal Opsoclonus ID 166-6 Parainfectious Opsoclonus ID 166-12 Opsoclonus in the Dark ID 936-1 Neonatatal Opsoclonus ID 936-8 Paraneoplastic Opsoclonus Downbeat Nystagmus ID 166-12 is shown courtesy of Dr. John Leigh. It is a striking illustration of opsoclonus in the dark. Dr. Leigh made a film of the rapid multidirectional saccades by placing a light diode on the surface of the eyeball in a patient with opsoclonus. ID 936-8 is a very instructive case, previously published in the New England Journal of Medicine in 1995. The child presented with paraneoplastic opsoclonus due to an occult neuroblastoma. How to investigate a child with opsoclonus is fully outlined in ID936-8. Interested readers are referred to Pediatric Neuro-Ophthalmology. Editors: Brodsky MC, Baker RS, Hamed LM. Spinger-Verlag, New York, Inc. 1996.
Clinical This baby, who is in excellent general health and stable sitting upright, has two distinctive eye movement abnormalities. • Neonatal opsoclonus characterized by multidirectional saccadic eye movements and • Periods of large amplitude upbeat nystagmus. In this child, opsoclonus and upbeat nystagmus occurred as a transient phenomenon in an otherwise normal infant. (11)
Etiology Neonatal opsoclonus
Disease/Diagnosis Neonatal Opsoclonus; Downbeat Nystagmus
Treatment None
References 1. Averbuch-Heller L, Remler BF. Opsoclonus. Semin Neurol 1996;16:21-26. 2. Cogan DG. Ocular dysmetria: flutter like oscillations of the eyes and opsoclonus. Arch Ophthalmol 1954;51:318-335. 3. Dyken P, Kolar O. Dancing eye dancing feet: Infantile polymyoclonia. Brain 1968; 91:305-320. 4. Hankey GJ, Sadka M. Ocular flutter postural body tremulousness and CSF pleocytosis: a rare postinfectious syndrome. J Neurol Neurosurg Psychiatry 1987;50:1235-1236. 5. Hoyt CS, Mousel DK, Weber AA. Transient supranuclear disorders of gaze in healthy neonates. Am J Ophthalmol 1980;89:708-711. 6. Hoyt CS, Gelbart SS. Vertical nystagmus in infants with congenital ocular abnormalities. Ophthalmic Pediatr Genet 1984;4:155-162. 7. Kinsbourne M. Myoclonic encephalopathy of infants. J Neurol Neurosurg Psychiatry 1962;25:2712-276. 8. Kuban KC, Ephros MA, Freeman RL, Laffell LB, Bresnan MJ. Syndrome of opsoclonus-myoclonus caused by Coxsackie B3 infection. Ann Neurol 1983;13:69-71. 9. Leigh RJ, Zee DS. Diagnosis of Nystagmus and Saccadic Intrusion. Chp 10:475-558. In: The Neurology of Eye Movements, Fourth Edition. Oxford University Press, NY. 2006. 10. Mitchell WG, Snodgress SR. Opsoclonus-ataxia due to childhood neural crest tumors: a chronic neurologic syndrome. J Child Neurol 1990;5:153-158. 11. Morad Y, Benyamini OG, Avni I. Benign opsoclonus in preterm infants. Pediatr Neurol 2004;31:275-278. 12. Nellhaus G. Abnormal head movements of young children. Dev Med Child Neurol 1983;25:384-389. 13. Orzechowski K, Walichiewicz T. Przypadek operowanej torbieli srodpajeczy mozdika (operated cyst of the cerebellum) Licowski Tygodnik Lekurski 1913;18:219-227. 14. Pranzatelli MR. The neurobiology of the opsoclonus-myoclonus syndrome. Clin Neuropharmacol 1992;15:186-228. 15. Pranzatelli MR, Tate ED, Kinsbourne M. Caviness VC, Mishra B. Forty-one year follow-up of childhood-onset opsoclonus-myoclonus-ataxia: cerebellar atrophy, multiphasic relapses and response to IVIG. Mov Disord 2002;17:1387-1390. 16. Pranzatelli MR, Travelstead AL, Tate ED, Allison TJ, Verhulst SJ. CSF B-cell expansion in opsoclonus-myoclonus syndrome: a biomarker of disease activity. Mov Disord 2004:19:770-777. 17. Pranzatelli MR, Tate ED, Travelstead AL, Longee D. Immunologic and clinical responses to rituximab in a child with opsoclonus-myoclonus syndrome. Pediatrics 2005;115:115-119. 18. Rivner MH, Jay WM,Green JB, Dyken PR. Opsoclonus in hemophilus influenza meningitis. Neurology 1982;32:661-663. 19. Shawkat FS, Harris CM, Wilson J, Taylor DSI. Eye movements in children with opsoclonus. Neuropaediatrics 1993;24:218-223. 20. Wiest G, Safoschnik G, Schnaberth G, Mueller C. Ocular flutter and truncal ataxia may be associated with enterovirus infection. J Neurology 1997, 244:288-292. 21. Wong AM, Musallam S, Tomlinson RD, Shannon P, Sharpe JA. Opsoclonus in three dimensions: oculographic, neuropathologic and modeling correlates. J Neurol Sci 2001;189:71-81.
Relation is Part of 166-4, 166-6, 166-12, 936-1, 936-8
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1972
Type Image/MovingImage
Format video/mp4
Source 16 mm Film
Rights Management Copyright 2002. For further information regarding the rights to this collection, please visit:
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
Language eng
ARK ark:/87278/s61c4tfw
Setname ehsl_novel_shw
Date Created 2008-04-15
Date Modified 2017-11-27
ID 188615
Reference URL
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