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
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
. 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. A 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).
After a first demyelinating event, the 3 main diagnosticconsiderations are multiple sclerosis, acute demyelinatingencephalomyelitis, and neuromyelitis optica. The clinicalsymptoms and signs, radiological findings, laboratory results, 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
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.
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,
varicella zoster virus,
post-infectious Epstein-Barr virus
Journal of Child Neurology
/ Vol. 25, No. 3, March 2010322