Copyright 2011 The Korean Society of Neuro-Ophthalmology http://neuro-ophthalmology.co.kr 129
Opsoclonus Associated with Epstein Barr Virus Infection
Seong-Hae Jeong, MD1, Hyun-Jung Kim, MD1, Ji Eun Oh, MD1, Ae Young Lee, MD1, Jae-Moon Kim, MD1, Ji Soo Kim, MD2
Department of Neurology1, Chungnam University School of Medicine, Chungnam National University Hospital, Daejeon; Department of Neurology2, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Bundang, Korea
Opsoclonus consists of involuntary, arrhythmic, chaotic, multidirectional saccades, with horizontal, vertical, and torsional compo- nent. One patient with Epstein-Barr virus infection displayed opsoclonus, and complained severe oscillopsia and imbalance. Ops- oclonus can manifest as a sign of viral infection in a patient who shows deficits of brainstem and cerebellum.
Keywords: Opsoclonus; Epstein-Barr virus
INTRODUCTION
Opsoclonus is an ocular dyskinesia consisting of involuntary, arrhyth- mic, chaotic, multidirectional saccades without intersaccadic intervals.1 This saccadic oscillation can occur in many clinical settings such as in- fections, neoplasms, metabolic disease and so on.2 We report opsoclonus that showed a benign prognosis in a patient with Epstein-Barr virus in- fection.
CASE REPORT
A 19-year-old soldier presented with dizziness and imbalance for one week. Initial vital signs were blood pressure 121/75 mmHg, heart rate 64/
min, respiration rate 22/min and body temperature 36˚C. The patient de- nied preceding upper respiratory tract infection or diarrhea. Past medi- cal and family histories were unremarkable. The patient showed unpre- dictable multidirectional conjugate saccades which is consistent with op- soclonus. He also showed saccadic hypermetria. Downbeat nystagmus developed after horizontal head oscillation (perverted head-shaking nys- tagmus) and during straight head hanging. He had truncal ataxia and limb dysmetria without myoclonus. Deep tendon reflexes were normal.
Examination of the CSF showed 10 WBCs/mm3, 0 RBC/mm3, protein
of 34.2 mg/dL, glucose of 69 mg/dL (serum glucose at 80 mg/dL), nega- tive oligoclonal bands, and normal IgG index. He also showed positive polymerase chain reaction to Epstein-Barr virus (EBV) in the serum. Tu- mor markers and anti-aquaphorin (neuromyelitis optica) IgG antibody were negative. Brain MRI and NCS were normal. One month later, the opsoclonus and cerebellar ataxia began to improve without specific treatments.
DISCUSSION
Opsoclonus is a rapid abnormal ocular movement occurred irregu- larly and intermittently in all directions when the eyes were in the prima- ry position.3 It is usually present during fixation, smooth pursuit, conver- gence, and persists during sleep or eyelid closure. Because of its large am- plitude and high frequency (10-25 Hz), it frequently causes visual blur and oscillopsia (an illusion of movement of the seen world).1,4 Opsoclo- nus differs from nystagmus in that the phase that takes the eye off the tar- get is always a saccade, not a slow eye movement. In contrast to ocular flutter, which consists of back-to-back saccades that are confined to the horizontal plane, opsoclonus is multidirectional.1 There is increasing evi- dences, however, that both humoral and cell mediated immune mecha- nisms are involved. The exact immunopathogenesis is of opsoclonus is
Correspondence to: Seong-Hae Jeong, MD
Department of Neurology, Chungnam National University Hospital, 33 Munhwa-ro, Jung-gu, Daejeon 301-721, Korea Tel: +82-42-280-8057, Fax: +82-42-252-8654, E-mail: [email protected]
Received: Nov. 10, 2011 / Accepted: Nov. 10, 2011
CASE REPORT
ISSN: 2234-0971 대한안신경의학회지: 제1권 제2호
Clin Neuroophthalmol 1(2):129-130, December 2011
Jeong SH, et al. • Opsoclonus Associated with Epstein Barr Virus Infection
Clin Neuroophthalmol 1(2):129-130, December 2011 130 http://neuro-ophthalmology.co.kr
uncertain.4 Although it has been suggested that opsoclonus results from dysfunction of inhibitory saccadic burst neurons from dysfunction of inhibitory saccadic burst neurons, the pathophysiological mechanisms underlying the disorder are yet to be fully elucidated.5,6 Opsoclonus can occur in many clinical settings. Most patients conform to four clinical settings: parainfectious brainstem encephalitis, paraneoplastic syn- dromes, metabolic-toxic states, or without evident cause. Although the pathophysiology of opsoclonus is uncertain, humoral and cell mediated immune mechanisms have been implicated in paraneoplastic and idio- pathic opsoclonus. In paraneoplastic opsoclonus, small cell lung, breast and ovarian cancer are most commonly encountered in adults, whereas more than half of cases of are associated with neuroblastoma in chil- dren.1 Diseases that have recently been reported to cause opsoclonus in- clude infections such as West Nile virus, streptococcal infection, varicel- la-zoster, coxsackie B, entrovirus and Lyme disease.5 Our patients showed findings suggestive of recent EBV infections. As there is no cross- reactivity between EBV and other viruses, positive PCR or anti-EBV an- tibody indicates either recent infection or reactivation of EBV. Neurolog- ical complications do occur in some patients with EBV infection. The clinical spectrum is wide, including meningitis, meningoencephalitis and various neuromuscular complications.7
Our patient with ataxia associated with EBV infection showed ocular manifestations suggestive of disturbed central nervous system, especial- ly, posterior cranial fossa. In view of the self-limited course of this condi- tion, serum markers of EBV infection in previous healthy young patient with opsoclonus and ataxia should be considered.
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