SURVEY OF OPHTHALMOLOGY VOLUME 57 � NUMBER 6 � NOVEMBER–DECEMBER 2012

CLINICAL CHALLENGESPETER SAVINO AND HELEN DANESH-MEYER, EDITORS

Blindness, Weakness, and TinglingDamien Biotti, MD,1 Catherine Vignal, MD,2 Tarek Sharshar, MD,3 Oliver Gout, MD,1

Allison N. McCoy, MD, PhD,4 and Neil R. Miller, MD4

1Department of Neurology; 2Department of Ophthalmology, Fondation Ophtalmologique A. De Rothschild, Paris, France;3Department of Intensive Care Medicine, AP-HP, Raymond Poincare - University Hospital - Garches, France; UniversityVersailles Saint-Quentin en Yvelines, Garches, France; and 4Wilmer Eye Institute, Johns Hopkins University, Baltimore,Maryland, USA

� 201All rig

(In keeping with the format of a clinical pathologic conference,the abstract and key words appear at the end of the article.)

Case Report

A previously healthy 50-year-oldman suffered acutebilateral loss of vision over 24 hours associated withtingling of his hands and feet. The problem began 10days before with a nonfebrile cough symptomaticallytreated over a week. Then dysesthesia of the feet andhands developed. For the last 24 hours beforeadmission, painless bilateral visual acuity loss rapidlyworsened. Neurological examination revealed nomotor deficits and normal flexor plantar response.All tendon reflexes were abolished. His vision washandmotionsOU.Pupilswerepoorly reactive to light,with no relative afferent pupillary defect (RAPD).Extraocular movements were intact, and the rest ofthe eye examination was normal.

What are the differential diagnostic considerations ofacute, bilateral, severe painless visual loss?

Comments

COMMENTS BY NEIL R. MILLER, MD, AND

ALLISON N. MCCOY, MD, PHD

The differential diagnosis for acute, bilateralsevere painless visual loss is extensive and includes

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infectious, inflammatory, ischemic, hereditary, toxic,and nutritional optic neuropathies, as well as retinaland central nervous system disease.

The patient’s antecedent cough suggests the possi-bility of a postinfectious or autoimmune optic neuritis.Such cases are often characterized by painless loss ofvision over days to weeks and may be accompanied byother neurological symptoms, although peripheralneuropathy andareflexia arenot characteristic.Wearenot told of a history of travel, tick bites, or exposure tocats, although the cough could also suggest aninfectious optic neuritis, possibly caused by cat scratchdisease, syphilis, tuberculosis, Lyme disease, Herpeszoster, varicella, or rubella. As the patient was pre-viously healthy, human immunodeficiency virus (HIV)and fungal infections are less likely. Infectious opticneuritis may be unilateral or bilateral, painless orpainful, and the optic disk at presentation may benormal, swollen, or pale.

NMO is an important cause of acute, bilateral,severe vision loss, and must be considered here. Thepatient’s sensory dysfunction could be causedby transverse myelitis, one of the diagnostic criteriafor neuromyelitis optica (NMO).36

0039-6257/$ - see front matterdoi:10.1016/j.survophthal.2011.10.002

566 Surv Ophthalmol 57 (6) November--December 2012 BIOTTI ET AL

Leber hereditary optic neuropathy (LHON) is inthe differential in a male patient with acute,bilateral, severe, and painless vision loss, as about50% of patients with LHON experience bilateralsimultaneous visual loss. LHON may be isolated orassociated with neurological deficits (Leber ‘‘plus’’),rarely including a demyelinating polyneuropathy.10

The risk factors triggering visual loss in patients withLHON remain unclear, though antecedent infec-tion may be one.38 Patients with LHON may presentwith normal disks or mild swelling with peripapillarytelangiectasia.

Toxic and nutritional optic neuropathies alsocause bilateral, severe visual loss, with normal fundiand no RAPD. Although the visual loss in theseconditions is characteristically chronic, it also canbe subacute. We are not given a history of gastricbypass surgery, alcoholism, or anorexia, thoughapparently healthy patients can have unsuspectedfolate or vitamin B12 deficiencies as well as toxicexposures.

Posterior ischemic optic neuropathy (PION) maycause acute, bilateral vision loss with normal fundi;it is more commonly asymmetric or unilateral,however, and is always associated with an RAPDunless there is pre-existing optic nerve (or a bilateralPION) or major retinal dysfunction in the contra-lateral eye. PION typically occurs in the samesettings of anterior ischemic optic neuropathy,including underlying systemic non-inflammatoryvascular diseases, in the perioperative setting, andin patients with one of the arteritides, most oftengiant cell arteritis. This patient has no history orevidence of underlying vascular disease and has notundergone any recent surgery.

Infiltrative optic neuropathy, whether from sar-coidosis, lymphoma, or solid tumors also may causean acute bilateral and often severe visual loss. Sucha presentation may be the first sign of cancer orinflammatory disease in an otherwise healthypatient.

Pituitary apoplexy can cause an acute opticneuropathy, resulting in bilateral, severe and pain-less visual loss with normal fundi. The visual lossfrom pituitary apoplexy is often associated withheadache, nausea, vomiting, altered mental status,and/or signs of adrenal insufficiency, none of whichare present here.

Bilateral, painless visual loss in an older man isunlikely to represent typical optic neuritis, whichtends to occur unilaterally in young women, withover 90% having pain behind the eye or on eyemovement. Although optic neuritis patients mayhave a history of neurologic dysfunction, most donot have neurological symptoms at the time ofvision loss.

Case Report (Continued)

During the first 48 hours of hospitalization motorsymptoms appeared and worsened to quadriplegia,facial diplegia, bulbar palsy, and respiratory distressrequiring ventilatory assistance. The patient wasadmitted to the neurological intensive care unit.

What diagnostic testing would you perform?

Comments (Continued)

The patient’s rapid development of quadriplegia,areflexia, and respiratory failure points to a di-agnosis of Guillain-Barre Syndrome (GBS). Bothunilateral and bilateral optic neuritis have beenreported in both GBS and its variant, Fishersyndrome.4,5 Accordingly, the patient should un-dergo magnetic resonance imaging (MRI) with andwithout gadolinium of the brain and spine todetermine if there are white matter or enhancinglesions or if a space-occupying lesion is present. Alumbar puncture with an assessment of openingpressure and cerebrospinal fluid sent for biochem-istry, microbiology, and cytology, should be per-formed. This may reveal cytoalbumin dissociationin cases of GBS or multiple sclerosis, oligoclonalbands in multiple sclerosis,13 or a causative organ-ism in meningitis. Nerve conduction studies canhelp to define the nature of the polyneuropathy.12

If the MRI, lumbar puncture, and nerve conduc-tion studies are consistent with GBS, it would thenbe helpful to identify the specific GBS subtypethrough serological testing for anti-GQ1b, anti-GM1, and other ganglioside antibodies.26 Routineblood tests for creatine kinase, biochemistry, andimmunoglobulin levels would exclude other causesof weakness and establish the safety of treatmentwith intravenous immunoglobulins (IVIg).29 Sero-logical testing for autoimmune, nutritional, in-flammatory and infectious diseases, includingLyme, syphilis, Mycoplasma, Campylobacter, rubella,varicella zoster virus (VZV), Epstein-Barr virus(EBV), as well as NMO IgG may be appropriate.Although LHON can produce rapid, progressive,bilateral loss of vision in young men, and althoughrare patients with LHON develop neurologicmanifestations, the rapidity and severity of visualloss, as well as the specific neurologic deficits in thispatient, would seem to preclude this diagnosis.

As the patient’s vision loss is not associated withan RAPD or any abnormal findings on the ophthal-mic examination, a visual evoked potential (VEP)may be useful in confirming bilateral visual pathwaydisease. If the VEP were normal, a full-field electro-retinogram would be appropriate to determine ifthe patient has occult retinal disease.

Fig. 1. Coronal gadolinium-enhanced T1-weighted MRimage of the optic nerves (arrows), with fat saturation. Atadmission and at the end of the first month, gadoliniumenhancement of both optic nerves was seen (A). At the50th day, contrast enhancement decreased but was stillpresent (B). At the 100th day, the gadolinium enhance-ment had disappeared.

BLINDNESS, WEAKNESS, AND TINGLING 567

Case Report (Continued)

Ancillary tests performed at admission and thenin the intensive care unit were consistent with thediagnosis of GBS according to clinical, biological,and electrophysiological criteria. The following testswere all normal or negative: anti-nuclear antibodies,anti-extractable nuclear antigen, anti-dsDNA, anti-neutrophil cytoplasmic antibody, anti-aquaporine 4,sedimentation rate, C-reactive protein and fibrino-gen, vitamins B9 and B12, TSH, T3, T4, anti-thyroglobulin, anti-thyroperoxydase and blood testsfor Lyme disease, viral hepatitis, Mycoplasma pneumo-niae, Campylobacter jejuni, cytomegalovirus (CMV),VZV, and HIV. Tests for rubella, EBV, and measleswere positive for IgG antibodies and negative forIgM antibodies, consistent with past infections. Allthese serologic tests were repeated 3 weeks laterwithout seroconversion. Anti-GM1 were weaklypositive (1/400), and other gangliosides antibodieswere negative including anti-GQ1b. Primary muta-tions for LHON were not present. The cerebrospi-nal fluid (CSF) analysis showed no cells, normalglucose level, elevated protein level (0.69 g/l)(cytoalbumin dissociation), and the absence ofoligoclonal bands. CSF bacterial cultures and poly-merase chain reactions for EBV, HSV and VZV werenegative.

Brain and spinal MRI were unremarkable. Orbitalimages revealed T2-weighted hyperintensities of theorbital portions of both optic nerves with gadoli-nium enhancement (Fig. 1A). Visual evoked poten-tial showed a major latency prolongation bilaterally,and the electroretinogram was normal. Retinalnerve fiber layer (RNFL) measured by opticalcoherence tomography (OCT) was normal(Fig. 2A). Muscle biopsy did not show evidence ofmitochondrial disorder, and computed tomographyscan of chest, abdomen, and pelvis were normal.

What is your final diagnosis? And what kind oftreatments would you consider?

Comments (Continued)

The most probable diagnosis is postinfectious/autoimmune bilateral retrobulbar optic neuritis inthe setting of GBS. It is intriguing that the patientdeveloped both central and peripheral nervoussystem involvement, most likely due to molecularmimicry between antigens present in the pathogen,central nervous system, and peripheral nervoussystem.37,38 In this case, multiple cultures werenegative and the identity of the inciting antigen isunknown; optic neuritis associated with GBS hasbeen documented following infection with EBV,2

measles,32 Mycoplasma pneumoniae,11,27 and otherwell-known pathogens, however.

The cornerstone of treatment for GBS is support-ive, managing its potentially fatal complicationssuch as respiratory failure and autonomic dysfunc-tion. To hasten recovery, the patient should receiveIVIg (0.4 g/kg/day for 5 days) or four to six plasmaexchange treatments, the only treatments proven tobe effective for GBS.16 A randomized multicentertrial found that combined treatment with plasmaexchange followed by IVIg had no definite advan-tage compared with either treatment alone.28 Iftreatment were ineffective, we would consider anadditional course of IVIg.9,35 We would also consideremerging therapies such as mycophenolate mofetil,interferon-b1a, and brain-derived nerve growthfactor for which there is level II evidence of efficacyin GBS.16,34

For the patient’s bilateral optic neuritis, we wouldtreat with high-dose corticosteroids, as the opticneuritis associated with GBS is often steroid-responsive. Although some investigators have pos-tulated that steroids can have harmful effects onrecovering peripheral nerves by inhibiting macro-phages, there is no evidence in the literature tosuggest a beneficial or harmful effect of corticoste-roids in GBS.18

The prognosis in GBS is highly variable, rangingfrom complete recovery to death, with 20% ofseverely affected patients still unable to walk after 6

Fig. 2. On the left side retinal photography of the right eye with OCT values; on the right side OCT and retinalphotography of the left eye. A: Examination at day 30. B: Examination at day 50. C: Examination at day 100.

568 Surv Ophthalmol 57 (6) November--December 2012 BIOTTI ET AL

months.17 Even years after the onset of GBS,patients may remain disabled by neurologicaldysfunction, pain, or severe fatigue.33 Age greaterthan 40 confers a worse prognosis. The visualprognosis in patients with optic neuritis associatedwith GBS is equally variable, with some, generallyyounger, patients recovering 20/20 vision andothers with minimal improvement or blind-ness.2,4,8,11,14,15,19,21--24,27,32

Case Report (Concluded)

Clinical features and ancillary tests were consis-tent with the diagnosis of Guillain-Barre syndromeassociated with bilateral optic neuritis. The patientwas treated with intravenous immunoglobulin for 5days (0.4 g/kg/day) associated with high doseparenteral steroids (methylprednisolone 1 g/dayfor 5 days). The patient was extubated 3 weeks later.As sensory and motor symptoms began to improve,however, visual acuity worsened to light perceptionOU. Both optic disks still appeared normal(Fig. 2A), but enhancement of the optic nervespersisted on the orbital MRI (Fig. 1A). Parenteralcorticosteroids were repeated twice during the

following month (methylprednisolone 1 g/day for5 days); the second course of steroids was immedi-ately followed by plasmapheresis (performed every 2days, five times). Fifty days into the clinical coursebilateral optic disk pallor appeared (Fig. 2B). Visionremained light perception, but gadolinium en-hancement of the optic nerves decreased (Fig. 1B).

Three months after the onset of the disease, themotor symptoms related to GBS all resolved. Visualacuity was slightly improved to count fingers at 1foot OU. Fundi and RNFL-OCT showed completeatrophy (Fig. 2C). On orbital-MRI, gadoliniumenhancement of the optic nerves vanished(Fig. 1C). Two months later, visual acuity stabilizedat 20/100 OD and counting fingers at 6 feet OS.

Discussion

The association of optic neuritis and GBS relatedto central and to peripheral nervous system in-volvement is unexpected and rare. We establishedthe diagnosis of GBS on typical clinical features,cytoalbumin dissociation in CSF, and the presenceof established electrophysiological criteria.1,3,12 Inmost of the published case reports, the optic

TABLE 1

Cases Collected in the Literature Since 1970

Author (Year)

Patient(Age [y],

Sex)Side ofthe ON

Visual Acuityat the firstevaluation

Changes inVisual acuity

Chronology GBS-ON Brain MRI CSF Etiology Treatments

Mouren et al.(1971)22

41, F Bilateral No lightperception

At 6 months:1/30 OU

GBS then ONafter 5 days

NA CAD

Nikoskelainenet al. (1972)24

62, M Right Hand motionperception OD

Day 12: 20/20 OD

ON thenmyelitis andGBS after2 weeks

NA CAD Dexamethasonefor 3 weeks

ACTH treatmentfor 4 weeks

De Margerieet al. (1973)8

20, F Left 20/60 At 1 month:normal

GBS then ONafter 3 months

NA CAD Steroids ?

Behan et al.(1976)2

17, F Bilateral 20/70 OU(with discswelling)

At 3 months :20/25 OU

GBS then ONafter 2 weeks

NA CAD Prednisolone

Nadkarni et al.(1993)23

28, F Bilateral No lightperception

Progressiveimprovementafter steroids.

After ‘‘severalmonths’’:‘‘discernshapes andidentify faces’’

GBS then ONafter 3 weeks

Normal atadmission.

Multiple T2lesions withgadoliniumenhancementconcurrent ofvisual loss

CADCSF B- ata ission,p sitivew en visualsy ptomso ured

PossibleMycoplasmapneumoniae

Plasmaexchange MP

Hendersonet al. (1998)15

22, M Bilateral Hand motionperceptionDisc swelling

Day 24: 20/40OD, 20/30 OS

GBS then ONafter 11 days

Normal Abs ce ofC D ata ission.

CAD (Day 4)CSF Bþ: CSFa serum

Mycoplasmapneumoniae

IV-Ig twiceMP 1g for 3 days

Pfausler et al.(2002)27

53, M Bilateral No lightperception

Day 7: Lightperception OU

At 2 months:‘‘normalvision’’ OS and‘‘grosslyimpairedvision’’ OD

Synchronous Normal CAD Mycoplasmapneumoniae

Plasmapheresisfour times

Steroids(80 mg/d)

Hawley et al.(2003)14

57, M Left 20/20 OD,20/40 OS

‘‘Progressiveimprovement’’

GBS then ONafter 1 month

Brain: normalMedullar: T2signal at C3--4level

Nor al Mycoplasmapneumoniae

doxycyclinemonohydrate

(Continued on next page)

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TABLE 1Continued

Author (Year)

Patient(Age [y],

Sex)Side ofthe ON

Visual Acuityat the firstevaluation

Changes inVisual acuity

Chronology GBS-ON Brain MRI CSF Etiology Treatments

Ginestal et al.(2004)11

69, M Bilateral 20/70 OD20/200 OSWith discswelling

Day 8: 20/40 ODand 20/70 OS

Day 44: 20/30

ON then GBSafter 10 days

Normal CADþCSF-OBþ: samethan in serum

Mycoplasmapneumoniae

Steroids 60 mg/day for 3 daysthen IV-Ig

Igarashi et al.(2005)19

68, M Bilateral No lightperception

At 1 month:‘‘Gradualrecovery’’

Synchronous Normal CADþCSF-OB-

CMV IV-IgMP1g for 3 days

Luke et al.(2007)21

55, F Left 20/16 OD!20/200 OSwith left discswollen

At 7 weeks:20/25 OS

At 11 weeks:20/20 OS

GBS then ONafter 6 weeks

Normal CADþCSF-OB-

MP IV-Ig 3 times

An et al. (2008)1 49, M Right Light perception Just aftertreatment: cancount fingersat 30 cm OU

Day 35: 20/120 OU

Synchronous Focal area of highsignal intensityin the leftinternalcapsule

CADþCSF-OB-

EBVreinfection

MP 1g/d for3 days

IV-Ig

Tomiyasuet al. (2009)32

28, F Bilateral Countingfingers OU

At 1 month:20/40 OD, 20/16 OS

GBS then ONafter 10 days

T2 temporaland frontalhyperintensitieswithgadoliniumenhancement

CADþCSF-OB-

Measles MP for 3 days

CAD 5 cytoalbumin dissociation; CSF 5 cerebrospinal fluid; CSF-OB 5 CSF oligoclonal bands; F 5 female; GBS 5 Guillain-Barre syndrome; IV-Ig 5 intravenousimmunoglobulin; M 5 male; ON 5 optic neuritis; OD 5 right eye; OS 5 left eye; OU 5 both eyes; y 5 years.

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neuropathy is considered to be the result of aninflammatory process—but few had exhaustive testsfor differential diagnoses. In our patient, a compres-sive cause has been ruled out by the MRI. Therewere no clinical indications of a toxic or nutritionalmechanism (nonsmoker, nondrinker, no vitamindeficiency found, no weight loss, no drug intake). Abilateral severe optic neuropathy can be observed inLHON and has been described in association withdemyelinating polyneuropathy or with central de-myelination10,25 and is sometimes precipitated by aninfection or metabolism stress.38 No familial historyof LHON was found, and primary mutations wereabsent. The last main differential diagnosis wasretrobulbar ischemic optic neuropathy. We consid-ered this diagnosis improbable because of theabsence of initial symptomatic hypotension andbecause of the inflammation seen on MRI.

Finally, themajor infectious or autoimmune causes,including neuromyelitis optica, were excluded. Fre-quently when GBS and optic neuritis (ON) areassociated (Table 1), no infectious cause is identi-fied.2,8,21,22,24,30 The table presents similar casescollected in the literature since 1970, but not all thecases had undergone extensive investigations. Insomecases, an infectious agentwas found(Mycoplasmapneumoniae, measles, EBV or CMV infection) andconsidered as the origin of both the ON andGBS.1,11,15,19,23,27,32 We tested our patient for theseconditions twice, and there was no seroconversion.

The pathophysiological mechanisms of theseunexpected associations remain unknown. Theoptic nerves are not normally affected duringGuillain-Barre syndrome. Indeed, the optic nervesare an extension of the central nervous system andas such have central myelin. Rarely, otherwise typicalGBS may affect the central nervous system.7,14,23

Two presentations seem to be distinguishable.Some patients have simultaneous optic nerve andperipheral nervous system involvement;1,19,27 inothers, the symptoms are sequential, beginning withON11,24 or with GBS.2,8,14,15,21--23,32 The average timebetween the onset of symptoms was 24.1 days—12days from ON to GBS, and 27.9 days from GBS toON. In the first presentation a single triggeringagent causes a common immune response (molec-ular mimicry) against an antigen shared by both theperipheral nervous system and the optic nerves, andin the other an attack against one or the otherstructure would initiate an immune response thatuncovers a particular shared antigen, leading toinvolvement of the second. Although the motorsymptoms of GBS dramatically improved, visualacuity remained poor. In other reported cases,whatever the treatment received, the visual progno-sis remained unpredictable (Table 1).

Also, we have deliberately excluded the caseswhere GQ1b antibodies were detected. Thesegangliosides are found in both the peripheralnervous system (with a particular tropism for theoculomotor nerves) and the central nervous system,including the optic nerves.6,20,31 In such cases, thepathophysiological mechanisms that cause the ONmay differ from those observed in GQ1b-negativepatients.

Disclosure

The authors reported no proprietary or commer-cial interest in any product mentioned or conceptdiscussed in this article.

References

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Reprint address: Damien Biotti, MD, Department of Neurology,Fondation Ophtalmologique A. De Rothschild, Rue Manin, 75019Paris, France. e-mail: dbiotti@hotmail.com.

Abstract. A previously healthy 50-year-old man complained of acute bilateral loss of vision over 24hours associated with tingling of his hands and feet. During the first 48 hours of hospitalization, heevolved quadriplegia, facial diplegia, bulbar palsy, and respiratory distress requiring ventilatorassistance. At the same time vision deteriorated to hand motions. Lumbar puncture, electromyography,and clinical findings were all consistent with Guillain-Barre syndrome. The diagnosis of concomitantbilateral optic neuritis was made. Tests for immunological, inflammatory, infectious, or genetic diseaseswere unremarkable. Within weeks, repeated treatments with intravenous immunoglobulin, plasma-pheresis, and high-dose parenteral corticosteroids led to resolution of motor findings, although visualacuity improved only to 20/100 OD and count fingers at 6 feet OS. Guillain-Barre syndrome--associatedoptic neuritis is rare and may be associated with an underlyingMycoplasma pneumoniae infection. (SurvOphthalmol 57:565--572, 2012. � 2012 Elsevier Inc. All rights reserved.)

Key words. acute demyelinating polyradiculoneuropathy � Guillain-Barre syndrome � opticneuropathy � visual loss