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6. Visual Loss Following Immunization Against Human Papilloma Virus

6. Visual Loss Following Immunization Against Human Papilloma Virus

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Journal of Child Neurology
http://jcn.sagepub.com A 16-Year-Old Girl With Bilateral Visual Loss and Left Hemiparesis Following an Immunization Against Human Papilloma Virus
Francis J. DiMario, Jr, Mirna Hajjar and Thomas Ciesielski J Child Neurol 2010; 25; 321 DOI: 10.1177/0883073809349322 The online version of this article can be found at: http://jcn.sagepub.com/cgi/content/abstract/25/3/321

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Journal of Child Neurology
http://jcn.sagepub.com A 16-Year-Old Girl With Bilateral Visual Loss and Left Hemiparesis Following an Immunization Against Human Papilloma Virus
Francis J. DiMario, Jr, Mirna Hajjar and Thomas Ciesielski J Child Neurol 2010; 25; 321 DOI: 10.1177/0883073809349322 The online version of this article can be found at: http://jcn.sagepub.com/cgi/content/abstract/25/3/321

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http://jcn.sagepub.com
Journal of Child Neurology
DOI: 10.1177/08830738093493222010; 25; 321
J Child Neurol 
Francis J. DiMario, Jr, Mirna Hajjar and Thomas Ciesielski
Against Human Papilloma VirusA 16-Year-Old Girl With Bilateral Visual Loss and Left Hemiparesis Following an Immunization
http://jcn.sagepub.com/cgi/content/abstract/25/3/321
 
The online version of this article can be found at:
 
Published by:
http://www.sagepublications.com
 
can be found at:
Journal of Child Neurology 
Additional services and information for
 
 
Citations
 at Open University Library on February 26, 2010http://jcn.sagepub.comDownloaded from 
 
Original Article
 A 16-Year-Old Girl With Bilateral VisualLoss and Left Hemiparesis Following anImmunization Against Human Papilloma Virus
Francis J. DiMario, Jr, MD, Mirna Hajjar, MD, and Thomas Ciesielski, MD
 We report the course of a 16-year-old girl who presentedwith near complete visual loss associated with chiasmalneuritis and a biopsy proven tumefactive demyelina-ting lesion on magnetic resonance imaging (MRI) inassociation with a recent immunization against humanpapilloma virus.
Keywords:
demyelination;tumefactive;blindness;chiasm;HPV 
 V 
isual loss is a symptom that can occur from lesionsanywhere along the visual pathways. Binocular visual loss can be further localized depending onthesizeandlocationofthescotomaidentifiedonexamina-tion. Lesions affecting the chiasm, in particular, may pro-duce bitemporal visual field loss and the additionalinvolvement of the optic nerves and/or retrochiasmatic visual pathways will induce more complete degrees of blindness.Thereareamultitudeofetiologiesthatproducethis latter pattern of visual loss; however, the pace of pro-gression, the anatomic localization of the process, and theprecipitating circumstances will aid in pathophysiologicclassification as compressive or noncompressive. Non-compressive etiologies involving the chiasm include pro-cesses within the spectrum from acute to a more chronictemporal course. This slower time course is characteristicof infiltrative lesions, granulomatous diseases, axonal die-back phenomenon secondary to multiple sclerosis,
1
andLeber hereditary optic neuropathy. Acute noncompressivelesions of the optic chiasm have been described in infec-tious settings with Lyme disease,
2
Epstein-Barr virus,
3,4
 varicella zoster virus
5
and mumps,
6
systemic lupus erythe-matosus,
7,8
and demyelinating processes (eg, neuromyeli-tis optica and multiple sclerosis). Tumefactivedemyelinating lesions are those defined as large (>2 cm)lesions with a surrounding zone of edema with or withoutaccompanying mass effect. There have been a few casereports and case series in the literature where tumefactivelesionshavebeendescribedinthecontextofbothmultiplesclerosis and acute demyelinating encephalomyelitis.However, chiasmal neuritis as part of acute demyelinatingencephalomyelitis hasnotto ourknowledgebeen reportedin the medical literature. Although chiasmal neuritis gen-erally tends to have a more favorable outcome with even-tual return of vision over time, when it is caused by neuromyelitis optica and Leber hereditary optic neuropa-thy the outcomes have been poorer with sustained visualloss. We report the case of a 16-year-old girl who sufferedan acute and sustained onset of bilateral visual loss andtransient left hemiparesis following an immunizationagainst human papilloma virus, who was found to haveboth a tumefactive demyelinating lesion and chiasmalneuritis as part of a presentation of acute demyelinatingencephalomyelitis.
Case Report
 A 16-year-old previously healthy girl presented to theemergency room with an acute onset of visual loss over48 hours. Initially, there was visual loss noted in the righteye accompanied by a left side headache. These symptoms
Received August 11, 2009. Received revised August 26, 2009. Acceptedfor publication August 26, 2009.From the University of Connecticut School of Medicine, Farmington,Connecticut, and the Departments of Pediatrics
1
at ConnecticutChildren’s Medical Center, Hartford, Connecticut (FJD); and theDepartments of Neurology (MH) and Pathology (TC) at HartfordHospital, Hartford, Connecticut.The authors have no conflicts of interest to disclose with regard to thisarticle. Address correspondence to: Francis J. DiMario Jr, Division of ChildNeurology, Connecticut Children’s Medical Center, 282 Washington St,Hartford, CT 06106; e-mail: fdimari@ccmckids.org.DiMario FJ Jr, Hajjar M, Ciesielski T. A 16-year-old girl with bilateral visual loss and left hemiparesis following an immunization against humanpapilloma virus.
J Child Neurol.
2010;25:321-327.
 Journal of Child Neurology 
 Volume 25 Number 3March 2010 321-327
#
2010 The Author(s)10.1177/0883073809349322http://jcn.sagepub.com
321
 at Open University Library on February 26, 2010http://jcn.sagepub.comDownloaded from 
 
worsened over the next 24 hours to include visual lossinvolving the left eye with a more diffuse headache. Whenevaluated in the emergency room at 48 hours after onset,hervitalsignswerebloodpressure116/65,pulse68/minute,respirations 14/minute, and temperature 98
C,
6
with acompletely normal general physical examination and nosigns of systemic illness. Her examination disclosed a visualacuity of only counting fingers bilaterally with mild left sideweaknessaccompaniedbysensorylosstopinprickintheleftarm. There was a left afferent pupillary defect and normalfundoscopic examination. Her visual ability deterioratedfurther to inconsistently identifying light and movementfrom the left eye only. She complained of no other symp-toms and denied antecedent trauma or prodromal illness.She had, however, received her second vaccination againsthuman papilloma virus 10 days prior to her presentation.There was no family history of demyelinating disease, col-lagen-vascular disease, or rheumatological disorders.Magnetic resonance imaging (MRI) of the brainshowed swollen enhancement within the chiasm extend-ing into both retrobulbar optic nerves and a right occipito-parietal lobe mass (later disclosed as tumefactivedemyelination) with a large zone of surrounding vasogenicedema (Figure 1). Complete spine MRI was normal.Biopsy of the hemispheric mass was performed and histol-ogy revealed demyelination (Figures 2 and 3). Subsequentcultures for aerobic and anaerobic bacteria, fungus, acidfast bacilli, and examination for parasites were negativeas were serum immunoglobulin G and immunoglobulinM titers for
Toxoplasma gondii
. The erythrocyte sedimen-tationratewas16andthewhitebloodcellcountwas6900with 89
%
granulocytes and 9
%
lymphocytes. The patientreceived a 5-day course of high-dose intravenous steroids(1 g methylprednisolone/d divided each 6 hours), followedby 5 double-volume plasma exchanges with no visualimprovement. Other diagnostic testing included neuro-myelitis optica (neuromyelitis optica-immunoglobulinG), antinuclear antigen, Sjo ¨ gren syndrome (SS-A, SS-B),rheumatoid factor, angiotensin-converting enzyme, whichwere all normal or negative. Cerebrospinal fluid analysiswas declined by the patient’s family at this time. The fam-ily refused further therapy with chemotherapy and modu-lating agents. Three months after the onset, the patientstill had not regained any visual function, her weaknessand sensory deficit recovered completely however. repeat MRI of the brain revealed resolution of the hemi-spheric and chiasmal lesions (Figure 4). At 6 months herfunduscopicexaminationdisclosedbilateralopticdiscpal-lor. A repeat MRI at this time demonstrated no newlesions and continued resolution of the previously identi-fied chiasmal enhancement and tumefactive lesion. Againcomplete spine MRI was normal. Cerebrospinal fluid wasobtained6monthsafterinitialsymptomonset.Cerebrosp-inal fluid cytology was negative for malignancy; additionalstudies revealed a clear and colorless fluid with 3 whiteblood cells and 0 red blood cells per microliter, glucose61 mg/dL and simultaneous serum glucose of 91 mg/dL,protein 30 mg/dL, lactate 1.5 mmol/L, and negative oligo-clonal immunoglobulin G bands. At 18 months after theonset of her symptoms, her examination remained stablewith no further neurological complaints, persistent pro-found visual impairment, inconsistently identifying lightand movement from the left eye only. An MRI of the brainwas performed and was unchanged compared with theprevious one done at 6 months (Figure 5).
Discussion
 After a first demyelinating event, the 3 main diagnosticconsiderations are multiple sclerosis, acute demyelinatingencephalomyelitis, and neuromyelitis optica. The clinicalsymptoms and signs, radiological findings, laboratorresults, and clinical course help determine the likely diag-nosis, with time often being the most crucial component.On clinical grounds, our patient had bifocal chiasm andright parieto-occipital lesions occurring 10 days after ahumanpapillomavirusvaccination,witharapidprogressionover 2 days. There were no further demyelinating eventswithin the next 18 months. In the context of prior vaccina-tion in a 16-year-old girl, acute demyelinating encephalo-myelitis is likely to explain the multifocal deficits. Thisdemyelinating event could also represent the index attack of neuromyelitis optica known to cause severe bilateral visuallossduetoopticneuritiswithpoorrecovery.Herneu-romyelitis optica-immunoglobulin G antibody was negative.However, this test has a sensitivity of approximately 70
%
.Permanentvisuallossisnotspecifictoneuromyelitisoptica.Prior studies of optic neuritis from all etiologies encoun-tered in childhood have shown good recovery is usual andthat poor visual recovery occurred in less then 20
%
of chil-dren.
9,10
The monophasic course of our patient’s demyeli-nating event and the lack of ‘‘dissemination in time,’’ makea diagnosis of clinically definite multiple sclerosis unlikely. Atthistime,adivergenceofopinionisnotablefromauthorsof various case series attempting to identify predictive fac-tors of clinically definite multiple sclerosis in children whopresent with a first central nervous system demyelinatingevent or initial bilateral optic neuritis.
11,12
In this instance, there was no supportive clinical orlaboratory evidence for systemic lupus erythematosus,Sjo ¨ gren syndrome, or Lyme infection. Other viral studieswere unrevealing or negative. Bilateral visual loss due toinvolvement of the optic chiasm, as revealed on MRI inour patient, is a rarely described feature of demyelinatingentities. The few reported cases of acute chiasmal neuritisin the post-MRI era have occurred in the context of infec-tions with Lyme disease,
2
 varicella zoster virus,
5
post-infectious Epstein-Barr virus
3,4
and mumps,
6
systemiclupus erythematosus,
7,8
neuromyelitis optica,
13,14
and
322
Journal of Child Neurology
/ Vol. 25, No. 3, March 2010322
 at Open University Library on February 26, 2010http://jcn.sagepub.comDownloaded from 

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