ENOMOTO Tsuyoshi

Hoshino Sachiko

Kusunoki Susumu

Ohkoshi Norio

Shiraiwa Nobuko

TAMAOKA Akira

Umesawa Mitsumasa

Neurology International

Tsukuba Repository  (Tulips-R)

                             Neurology International 2017; volume 9:7312Miller Fisher syndrome withsinus arrestNobuko Shiraiwa,1,2 MitsumasaUmesawa,2,3 Sachiko Hoshino,2Tsuyoshi Enomoto,4 Susumu Kusunoki,5Akira Tamaoka,6 Norio Ohkoshi11Course of Neurology, Department ofHealth, Faculty of Health Sciences,Tsukuba University of Technology;2Department of Neurology, TsukubaMemorial Hospital; 3Department ofPublic Health, School of Medicine,Dokkyo Medical University;4Department of Cardiology, TsukubaMemorial Hospital; 5Department ofNeurology, Faculty of Medicine, KindaiUniversity; 6Department of Neurology,Graduate School of ComprehensiveHuman Science, University of Tsukuba,Tsukuba, JapanAbstractDysautonomia in Guillain-Barre syn-drome (GBS) rarely causes serious cardio-vascular complications, such as sinus arrest.Miller Fisher syndrome (MFS) is recog-nized as a variant of GBS. There have beenfew reports regarding the associationbetween MFS and dysautonomia. Wedescribe a case of a 68-year-old man withophthalmoplegia, bulbar palsy, truncal atax-ia, and areflexia. He was diagnosed withMFS because he exhibited the classicalclinical triad and had elevated serum anti-GQ1b immunoglobulin G levels. A magnet-ic resonance imaging scan of his head wasnormal. His 24-hour Holter recordingshowed sinus arrest. He was treated withintravenous immunoglobulin, whereuponhis symptoms gradually improved. Thisincluded the sinus arrest, which was consid-ered a symptom of dysautonomia in MFS.Therefore, clinicians should be mindful ofdysautonomia not only in GBS patients, butalso in cases of MFS.IntroductionDysautonomia is present in approxi-mately two-third of patients with Guillain-Barre syndrome (GBS).1 It results in a widerange of symptoms, including various car-diac arrhythmias. Although dysautonomia is usually ofminor clinical importance, it may cause life-threatening cardiovascular complications.Serious arrhythmias have been found in 7-34% of GBS patients. Recent studies indi-cate that these complications are not limitedto severely disabled patients who requiremechanical ventilation, but may also affectthose with milder forms of the condition,who do not require artificial ventilation, andare able to walk more than 5 meters.2,3Miller Fisher syndrome (MFS) is recog-nized as a variant of GBS. It is a relativelyrare condition that is characterized by theacute onset of ophthalmoplegia, ataxia, andareflexia or hyporeflexia.4,5 An associationbetween MFS and autonomic dysfunctionhas been previously reported, with cardio-vascular complications being rare.6,7 Thepresent case report describes sinus arrest ina patient with MFS.Case ReportA 68-year-old man had an acute onsetof truncal ataxia, dysarthria, and diplopia inMarch 2009. He developed dysphagia thenext day and underwent a magnetic reso-nance imaging (MRI) scan, which was nor-mal. He presented to our neurology outpa-tient department with truncal ataxia, opthal-moplegia, and areflexia five days later.Upon admission, he had a normal bloodpressure of 136/96 mmHg, a regular heartrate of 98 beats per minute (bpm), and aperipheral capillary oxygen saturation(SpO2) of 97% in room air. Neurologicalexamination revealed mydriasis, absentpupillary light reflex bilaterally, total oph-thalmoplegia with both eyes fixed centrally,bulbar palsy with nasal voice, dysphagia,absent pharyngeal reflex, and absent deeptendon reflexes. There was no limb weak-ness. Finger-to nose and heel-to-shin testsshowed no dysmetria or decomposition.Romberg’s test and Mann’s test were bothpositive. He was unable to walk unaideddue to the truncal ataxia. There were no sen-sory abnormalities, orthostatic hypotension,and dysfunction of the bladder and bowel.His blood count and serum biochemicalparameters were normal. Antinuclear, anti-deoxyribonucleic acid (DNA), and anti-acetylcholine receptor (Ach R) antibodieswere all negative. Vitamin B1 levels werenormal (46.3 ng/mL). Cerebrospinal fluidanalysis was normal with a cell count of1/µL (mononuclear leukocytes), proteinlevel of 33 mg/dl, glucose level of 66 mg/dl(serum glucose 97 mg/dl), and an IgG indexof 0.52. However, he tested positive forserum anti-GQ1b IgG, an autoantigenicantiganglioside antibody. His repeated headMRI and chest X-rays were normal.Given his presentation with acute onsettotal ophthalmoplegia, ataxia, and areflexia,he was diagnosed with MFS. He had aFunctional Systems Score (FSS) of 3 uponadmission. Treatment with intravenousimmunoglobulin (IVIG; 0.4 g/Kg bodyweight/day for 5 days) was initiated on daysix. He underwent 24-hour Holter recordingon day seven because he had exhibitedbradycardia (heart rate under 40 bpm), sinusarrest with the P wave falling off, and a2.94-second duration of the greatest R-Rinterval (Figure 1). There were five pausesdaily. The 24-hour Holter recording alsodisplayed atrioventricular dissociation withjunctional escape beats. After commencingIVIG therapy, his bulbar palsy improvedfirst, followed by the internal ophthalmo-plegia and truncal ataxia. In addition, arepeat 24-hour Holter recording on day 19showed that the sinus arrest had resolved.He was discharged with an FSS of 2 on day32. Approximately two months after theonset of symptoms, the diplopia secondaryto the ophthalmoplegia had resolved.DiscussionThe present report described a classiccase of MFS defined by the acute onset ofophthalmoplegia, ataxia, and areflexia, withpositive detection of serum anti-GQ1bIgGantibody. The patient showed sinus arrestearly after the onset of his symptoms. Thesinus arrest, together with the other symp-toms, improved soon after he had beenCorrespondence: Nobuko Shiraiwa, Course ofNeurology, Department of Health, Faculty ofHealth Sciences, Tsukuba University ofTechnology, 4-12-7 Kasuga, Tsukuba, Ibaraki305-8521, Japan.E-mail: shiraiwa@xa2.so-net.ne.jpKey words: Miller Fisher syndrome, Guillain-Barre syndrome, Dysautonomia, Sinus arrest.Conflicts of interest: the authors declare nopotential conflicts of interest.Acknowledgments: we would like to thankEditage (www.editage.jp) for English lan-guage editing.Received for publication: 14 July 2017.Accepted for publication: 23 July 2017.This work is licensed under a CreativeCommons Attribution NonCommercial 4.0License (CC BY-NC 4.0).©Copyright N. Shiraiwa, et al., 2017Licensee PAGEPress, ItalyNeurology International 2017; 9:7312doi:10.4081/ni.2017.7312[page 36]                                                        [Neurology International 2017; 9:7312]Nn commrcialuse nlycommenced IVIG therapy. There was noneed for cardiac pacemaker insertion. Similarly to GBS, cardiovascular com-plications are rarely reported in MFS andare considered to be a complication ofdysautonomia.6,7 Kuzumoto et al. showedan association between autonomic testabnormality and anti-GQ1b antibody inMFS.8 Recent studies on GBS indicate thatthese complications are not only limited toseverely disabled patients who requiremechanical ventilation, but also affect thosewith milder forms of the condition, who donot require artificial ventilation and are ableto walk more than 5 meters.2,3 The degree ofmotor impairment could not predict theoccurrence of serious bradyarrythmias,including sinus arrest. Early recognition ofsuch life-threatening events is important sothat appropriate preventive measures, suchas the insertion of a cardiac pacemaker, canbe promptly taken. ConclusionsThis report has shown that rare life-threatening cardiovascular complicationsmay occur not only in GBS, but also inMFS. References1. Hartung HP, Pollard JD, Harvey GK, etal. Immunopathogenesis and treatmentof the Guillain-Barre syndrome-Part I.Muscle Nerve 1995;18:137-53.2. Flachenecker P, Lem K, Mullges W, etal. Detection of serious bradyarrhyth-mias in Guillain-Barre syndrome: sensi-tivity and specificity of the 24-hourheart rate power spectrum. Clin AutonRes 2000;10:185-91. 3. Flachenecker P, Mullges W, Wermuth P,et al. Eyeball pressure testing in theevaluation of serious bradyarrhythmiasin Guillain-Barre syndrome. Neurology1996;47:102-8.4. Fisher M. An unusual variant of acuteidiopathic polyneuritis (syndrome ofophthalmoplegia, ataxia and areflexia).New Engl J Med 1956;255:55-65. 5. Wakerley BR, Uncini A, Yuki N.Guillain-Barre and Miller Fisher syn-drome-new diagnostic classification.Nat Rev Neurol 2014;10:537-44.6. Kordouni M, Jibrini M, Siddiqui MA.Long-term transvenous temporary pac-ing with active fixation bipolar lead inthe management of severe autonomicdysfunction in Miller Fisher syndrome:a case report. Int J Cardiol2007;117:e10-2. 7. Osada O, Iwasaki A, Kusunoki S.Fisher syndrome with 2:1 atrioventricu-lar block supposed by brain’s regulatingfailure of autonomic nervous system. Acase report. Neurol Med 2016;84:316-21.8. Kuzumoto Y, Shioyama M, Kihara M,Kusunoki S. Abnormal sudomotor axonreflex and antiganglioside antibodies.Muscle Nerve 2006;33:828-9.                                                                                                                      Case Report                                      [Neurology International 2017; 9:7312]                                                       [page 37]Figure 1. Holter recording on day seven displaying sinus arrest with P wave (arrow)falling off, and 2.94 seconds at the greatest R-R interval. There were five pauses daily. Non comercial use oly

Miller Fisher syndrome with sinus arrest

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Miller Fisher syndrome with sinus arrest

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