The LaryngoscopeVC 2012 The American Laryngological,Rhinological and Otological Society, Inc.

Case Report

Miller Fisher Variant of Guillain-Barr�e Syndrome Masquerading asAcute Sphenoid Sinusitis With Orbital Apex Syndrome

Amit Chaudhary, BA; Tekchand Ramchand, BA; Larry P. Frohman, MD; James K. Liu, MD;

Jean Anderson Eloy, MD, FACS

Miller Fisher syndrome (MFS), a variant of Guillain-Barr�e syndrome, is a rare disorder typically characterized by a triadof ataxia, areflexia, and ophthalmoplegia, which may have a highly variable clinical presentation. We report a case of MFS in a45-year-old female presenting with sphenoid sinusitis and sixth nerve palsy. She underwent endoscopic sphenoid sinusotomywithout improvement, had postoperative deterioration, was diagnosed with MFS, and was treated with intravenous immuno-globulin with complete response. Because of the potential severity of Guillain-Barr�e syndrome, great vigilance should betaken when examining sixth nerve palsies to prevent misdiagnosis and delay in treatment of the MFS variant of this disease.

Key Words: Miller Fisher, Miller Fisher syndrome, Guillain-Barr�e syndrome, Miller Fisher variant of Guillain-Barr�esyndrome, orbital apex syndrome, acute sphenoid sinusitis, cavernous sinus thrombosis, acute viral sinusitis,ophthalmoplegia, intracranial complication of sinusitis.

Laryngoscope, 122:970–972, 2012

INTRODUCTIONGuillain-Barr�e syndrome (GBS) is a relatively

uncommon disease, with an incidence rate of approxi-mately 1 to 2 cases per 100,000 people annually.1

Currently, GBS has six known subtypes, resulting invariable clinical presentations. Symptoms range fromcranial nerve involvement, to neuropathy,2 areflexia,progressive paralysis, or death.3 Emergent treatment isparamount to prevent permanent morbidity or death.We present a unique case in which a patient with GBS,specifically Miller Fisher syndrome (MFS), presentedwith symptoms suggestive of sphenoiditis.

CASE REPORTA 45-year-old female was urgently transferred to

our tertiary care center with a 2-day history of progres-sive diplopia, ataxia, left-sided headache, and leftsphenoid sinus opacification. She reported upper respira-tory tract infection (URI) symptoms for the past 7 daysthat were treated with amoxicillin without improvement.Initial physical examination revealed a left abducensnerve palsy, unsteady gait, general weakness, slurredspeech, and decreased hand sensation. Nasal endoscopyshowed mild congestion. Computed tomography scan-ning of the orbits and paranasal sinuses revealedopacification of the left sphenoid sinus (Fig. 1). Gadolin-ium-enhanced magnetic resonance imaging showedopacification of the left sphenoid sinus with unremarkableorbits, cavernous sinus, and brain parenchyma (Fig. 2).The diagnosis of isolated left acute sphenoid sinusitis withpossible cavernous sinus involvement was entertained,and the patient underwent an uneventful left sphenoidsinusotomy. Intraoperatively, turbid serous fluid wasdrained from the left sphenoid sinus and a biopsy of thesphenoid mucosa was taken. Histopathological analysisshowed fragments of inflamed respiratory mucosa withoutorganisms. The patient was subsequently started on van-comycin and ceftriaxone.

Postoperatively, her neurological symptoms rapidlyworsened. Within 7 hours of the surgery, she progressedto bilateral opthalmoplegia with ptosis, and mild facialdroop. Her speech was fluent, but mildly dysarthric. Shedeveloped sensory loss, motor weakness, and severe limbataxia. Emergent neuro-ophthalmic and neurologic

From the Department of Otolaryngology–Head and Neck Surgery(A.C., T.R., J.A.E.), Department of Ophthalmology and Visual Sciences (L.P.F.),Department of Neurology and Neurosciences (L.P.F.), Department ofNeurological Surgery (J.K.L., J.A.E.), and the Center for Skull Base andPituitary Surgery, Neurological Institute of New Jersey (J.K.L., J.A.E.),University of Medicine and Dentistry of New Jersey–New Jersey MedicalSchool, Newark, New Jersey, U.S.A.

Editor’s Note: This Manuscript was accepted for publicationJanuary 20, 2012.

Presented at the Triological Society Combined Sections Meeting,Miami, Florida, U.S.A., January 26–28, 2012.

The authors have no funding, financial relationships, or conflictsof interest to disclose.

Send correspondence to Jean Anderson Eloy, MD, AssistantProfessor and Vice Chairman, Director of Rhinology and Sinus Surgery,Department of Otolaryngology– Head and Neck Surgery, UMDNJ–NewJersey Medical School, 90 Bergen St., Suite 8100, Newark, NJ 07103.E-mail: jean.anderson.eloy@gmail.com

DOI: 10.1002/lary.23248

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consultations were obtained. Complete areflexia, pro-gressive bulbar palsy with headaches, decreasedrespiratory vital capacity, and difficulty swallowing werenoted. The triad of areflexia, ataxia, and ophthalmople-gia allowed the clinical diagnosis of MFS. Lumbarpuncture was remarkable only for increased cerebrospi-nal fluid glucose; the protein was 18, there were 6 whitecells (79% lymphocytes). She was transferred to the in-tensive care unit for further monitoring and treatmentwith intravenous immunoglobulin therapy. Four dayslater, her clinical status was significantly improved. Herpupils were normal and eye movements just revealedresidual left abducens paresis. Motor and sensationreturned to normal, except for a lack of vibration sensebelow the sternum. Coordination was normal in alllimbs, but Romberg test remained positive. She had acomplete recovery without permanent neurologic seque-lae and was transferred to a rehab facility 1 week later.The anti-Gq1b antibody was positive. She remainedasymptomatic at her 6-month follow-up without anydiplopia and a patent left sphenoidotomy on nasal endos-copy (Fig. 3).

DISCUSSIONGBS is an autoimmune disorder with different

clinical subtypes. These subtypes include MFS, acuteinflammatory demyelinating polyradiculoneuropathy,acute motor axonal neuropathy, acute motor sensoryaxonal neuropathy, and acute panautonomic neuropa-thy.4 Because GBS is an autoimmune disorder, amajority of cases are associated with an antecedentinfection. In fact, Govoni and Granieri5 reported that inpopulations studied in Minnesota and Italy, antecedentinfections prior to the presentation of GBS symptomswere present in 65% and 59% of patients respectively.Furthermore, most of these antecedent infections wereURIs. Our present case also suffered from a URI for 7days prior to admission.

MFS represents only 5% of GBS cases, and usuallypresents with the clinical triad of opthalmoplegia,ataxia, and areflexia,6 all of which our patient demon-strated. Abducens nerves are usually affected bilaterallyin MFS.4 However, some researchers have previouslylinked isolated sixth nerve palsy, as suffered by ourpatient, to MFS. In 2006, Tatsumoto et al.7 examined

Fig. 1. Axial (A) and coronal (B) computed tomography scans of the orbits and paranasal sinuses show complete opacification of the leftsphenoid sinus.

Fig. 2. (A) Axial T1-weighted magnetic resonance imaging of the orbits and paranasal sinuses with gadolinium enhancement show markedenhancement of the left sphenoid sinus mucosal lining with hypointense sphenoid content. (B) Coronal T2-weighted magnetic resonanceimaging of the orbits and paranasal sinuses show a hyperintense left sphenoid content consistent with fluid collection.

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100 cases of isolated sixth nerve palsy. Of the 100 cases,63 had symptoms of an antecedent infection, 27 hadabsent or reduced tendon reflexes, and 25 had positiveserum anti-GQ1b antibodies, suggesting GBS. In fact,the authors concluded that in certain cases, isolatedsixth nerve palsy could be labeled as a regional variantof GBS, or a form of MFS. In 2007, Smith et al.8 pre-sented a case of a 32-year-old man with MFS, whopresented only with left unilateral abducens nerveinvolvement.

Nonetheless, it is exceedingly more common for anisolated unilateral sixth nerve palsy to be found in thecontext of sphenoid sinusitis than in MFS. Ada et al.9

reported a case in which a 12-year-old female presentedwith isolated sphenoid sinusitis, which resulted in uni-lateral sixth nerve palsy. The sixth nerve palsyimproved with antibiotic therapy and endoscopic sphe-noidotomy. Shukla et al.10 reported a similar case inwhich a 36-year-old male presented with acute sphenoi-ditis, who later developed diplopia as a result of isolatedsixth nerve palsy. The ipsilateral optic, oculomotor, andtrigeminal nerves subsequently became involved as thecondition progressed. The patient made a full recoveryafter emergent endoscopic surgical drainage and antibi-otic therapy.

Although unilateral sphenoid sinusitis with associ-ated abducens nerve palsy is not uncommon, given thepossibility of a catastrophic missed diagnosis of MFSvariant of GBS, otolaryngologists should be aware ofthis entity and keep a high level of suspicion in patientspresenting with isolated sphenoid sinusitis with abdu-cens nerve palsy, and inquire about ataxia beyond whatwould be allowed for by diplopia, as well as testing forareflexia. The laterality of the sixth nerve palsy does notrule out either MFS or abducens nerve palsy from sphe-noid sinusitis. If a patient’s clinical course is rapidlydeteriorating after adequate sphenoid drainage andbroad-spectrum antibiotics, and no purulence is found

intraoperatively in the opacified sinus cavity, emergentreevaluation is warranted and other potential diagnoses(such as MFS) entertained and investigated.

CONCLUSIONMFS, a variant of GBS, is a rare disorder with a

highly variable clinical presentation. One such presenta-tion is unilateral abducens nerve palsy, which maymimic sphenoid sinusitis with cavernous sinus involve-ment and abducens nerve palsy. Although abducensnerve palsy secondary to sphenoid sinusitis is usuallysuccessfully treated with urgent sphenoidotomy withappropriate antibiotics, this management strategy isineffective in GBS, and may cause significant delayin treatment with potential devastating outcomes.Otolaryngologists should maintain a high level of suspi-cion in patients presenting with unilateral sixth nervepalsy in the setting of an opacified sphenoid sinus asthis may mask a potentially more devastating condition.

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1978;19:249–260.2. Pascuzzi RM, Fleck JD. Acute peripheral neuropathy in adults. Guillain-

Barre syndrome and related disorders. Neurol Clin 1997;15:529–547.3. Vedanarayanan VV, Chaudhry V. Guillian Barre syndrome—recent advan-

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as a regional variant of Guillain-Barre syndrome. J Neurol Sci 2006;243:35–38.

8. Smith J, Clarke L, Severn P, Boyce R. Unilateral external ophthalmoplegiain Miller Fisher syndrome: case report. BMC Ophthalmol 2007;7:7.

9. Ada M, Kaytaz A, Tuskan K, Guvenc MG, Selcuk H. Isolated sphenoidsinusitis presenting with unilateral VIth nerve palsy. Int J PediatrOtorhinolaryngol 2004;68:507–510.

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Fig. 3. Six-month, 30�, postoperative nasal endoscopic examination shows a patent left sphenoidotomy opening.

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