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Neurology International 2017; volume 9:7312

34% of GBS patients. Recent studies indi-

cate that these complications are not limited
Miller Fisher syndrome with
Correspondence: Nobuko Shiraiwa, Course of
sinus arrest to severely disabled patients who require Neurology, Department of Health, Faculty of
mechanical ventilation, but may also affect Health Sciences, Tsukuba University of
Nobuko Shiraiwa,1,2 Mitsumasa those with milder forms of the condition, Technology, 4-12-7 Kasuga, Tsukuba, Ibaraki
Umesawa,2,3 Sachiko Hoshino,2 who do not require artificial ventilation, and 305-8521, Japan.
Tsuyoshi Enomoto,4 Susumu Kusunoki,5 are able to walk more than 5 meters.2,3 E-mail:
Akira Tamaoka,6 Norio Ohkoshi1 Miller Fisher syndrome (MFS) is recog-
Key words: Miller Fisher syndrome, Guillain-
1Course of Neurology, Department of nized as a variant of GBS. It is a relatively
Barre syndrome, Dysautonomia, Sinus arrest.
Health, Faculty of Health Sciences, rare condition that is characterized by the
Tsukuba University of Technology; acute onset of ophthalmoplegia, ataxia, and Conflicts of interest: the authors declare no
2Department of Neurology, Tsukuba
areflexia or hyporeflexia.4,5 An association potential conflicts of interest.
between MFS and autonomic dysfunction
Memorial Hospital; 3Department of
has been previously reported, with cardio- Acknowledgments: we would like to thank
Public Health, School of Medicine, vascular complications being rare.6,7 The Editage ( for English lan-
Dokkyo Medical University; present case report describes sinus arrest in guage editing.
4Department of Cardiology, Tsukuba
a patient with MFS.
Memorial Hospital; 5Department of Received for publication: 14 July 2017.
Neurology, Faculty of Medicine, Kindai Accepted for publication: 23 July 2017.
University; 6Department of Neurology,
Graduate School of Comprehensive Case Report This work is licensed under a Creative
Commons Attribution NonCommercial 4.0
Human Science, University of Tsukuba, License (CC BY-NC 4.0).
A 68-year-old man had an acute onset
Tsukuba, Japan

of truncal ataxia, dysarthria, and diplopia in
©Copyright N. Shiraiwa, et al., 2017
March 2009. He developed dysphagia the

Licensee PAGEPress, Italy
next day and underwent a magnetic reso- Neurology International 2017; 9:7312
nance imaging (MRI) scan, which was nor-
mal. He presented to our neurology outpa-

tient department with truncal ataxia, opthal-
Dysautonomia in Guillain-Barre syn- us
moplegia, and areflexia five days later.
drome (GBS) rarely causes serious cardio-
Upon admission, he had a normal blood Functional Systems Score (FSS) of 3 upon
vascular complications, such as sinus arrest.
pressure of 136/96 mmHg, a regular heart admission. Treatment with intravenous
Miller Fisher syndrome (MFS) is recog-
rate of 98 beats per minute (bpm), and a immunoglobulin (IVIG; 0.4 g/Kg body
nized as a variant of GBS. There have been
peripheral capillary oxygen saturation weight/day for 5 days) was initiated on day

few reports regarding the association

(SpO2) of 97% in room air. Neurological six. He underwent 24-hour Holter recording
between MFS and dysautonomia. We

examination revealed mydriasis, absent on day seven because he had exhibited

describe a case of a 68-year-old man with
pupillary light reflex bilaterally, total oph- bradycardia (heart rate under 40 bpm), sinus
ophthalmoplegia, bulbar palsy, truncal atax-

thalmoplegia with both eyes fixed centrally, arrest with the P wave falling off, and a
ia, and areflexia. He was diagnosed with
bulbar palsy with nasal voice, dysphagia, 2.94-second duration of the greatest R-R
MFS because he exhibited the classical

absent pharyngeal reflex, and absent deep interval (Figure 1). There were five pauses
clinical triad and had elevated serum anti-
tendon reflexes. There was no limb weak- daily. The 24-hour Holter recording also
GQ1b immunoglobulin G levels. A magnet-

ness. Finger-to nose and heel-to-shin tests displayed atrioventricular dissociation with
ic resonance imaging scan of his head was
showed no dysmetria or decomposition. junctional escape beats. After commencing
normal. His 24-hour Holter recording
Romberg’s test and Mann’s test were both IVIG therapy, his bulbar palsy improved
showed sinus arrest. He was treated with

positive. He was unable to walk unaided first, followed by the internal ophthalmo-
intravenous immunoglobulin, whereupon
due to the truncal ataxia. There were no sen- plegia and truncal ataxia. In addition, a
his symptoms gradually improved. This
sory abnormalities, orthostatic hypotension,

included the sinus arrest, which was consid- repeat 24-hour Holter recording on day 19
and dysfunction of the bladder and bowel. showed that the sinus arrest had resolved.
ered a symptom of dysautonomia in MFS.
His blood count and serum biochemical He was discharged with an FSS of 2 on day
Therefore, clinicians should be mindful of
parameters were normal. Antinuclear, anti- 32. Approximately two months after the
dysautonomia not only in GBS patients, but
deoxyribonucleic acid (DNA), and anti- onset of symptoms, the diplopia secondary
also in cases of MFS.
acetylcholine receptor (Ach R) antibodies to the ophthalmoplegia had resolved.
were all negative. Vitamin B1 levels were
normal (46.3 ng/mL). Cerebrospinal fluid
Introduction analysis was normal with a cell count of
1/µL (mononuclear leukocytes), protein Discussion
Dysautonomia is present in approxi- level of 33 mg/dl, glucose level of 66 mg/dl
mately two-third of patients with Guillain- (serum glucose 97 mg/dl), and an IgG index The present report described a classic
Barre syndrome (GBS).1 It results in a wide of 0.52. However, he tested positive for case of MFS defined by the acute onset of
range of symptoms, including various car- serum anti-GQ1b IgG, an autoantigenic ophthalmoplegia, ataxia, and areflexia, with
diac arrhythmias. antiganglioside antibody. His repeated head positive detection of serum anti-GQ1bIgG
Although dysautonomia is usually of MRI and chest X-rays were normal. antibody. The patient showed sinus arrest
minor clinical importance, it may cause life- Given his presentation with acute onset early after the onset of his symptoms. The
threatening cardiovascular complications. total ophthalmoplegia, ataxia, and areflexia, sinus arrest, together with the other symp-
Serious arrhythmias have been found in 7- he was diagnosed with MFS. He had a toms, improved soon after he had been

[page 36] [Neurology International 2017; 9:7312]

Case Report

commenced IVIG therapy. There was no

need for cardiac pacemaker insertion.
Similarly to GBS, cardiovascular com-
plications are rarely reported in MFS and
are considered to be a complication of
dysautonomia.6,7 Kuzumoto et al. showed
an association between autonomic test
abnormality and anti-GQ1b antibody in
MFS.8 Recent studies on GBS indicate that
these complications are not only limited to
severely disabled patients who require
mechanical ventilation, but also affect those
with milder forms of the condition, who do
not require artificial ventilation and are able
to walk more than 5 meters.2,3 The degree of
motor impairment could not predict the
occurrence of serious bradyarrythmias,
including sinus arrest. Early recognition of
such life-threatening events is important so
that appropriate preventive measures, such
as the insertion of a cardiac pacemaker, can
be promptly taken.

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.

This report has shown that rare life-

threatening cardiovascular complications heart rate power spectrum. Clin Auton Long-term transvenous temporary pac-
may occur not only in GBS, but also in
Res 2000;10:185-91. ing with active fixation bipolar lead in
3. Flachenecker P, Mullges W, Wermuth P, the management of severe autonomic
et al. Eyeball pressure testing in the dysfunction in Miller Fisher syndrome:
evaluation of serious bradyarrhythmias a case report. Int J Cardiol

in Guillain-Barre syndrome. Neurology 2007;117:e10-2.

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Muscle Nerve 1995;18:137-53. 5. Wakerley BR, Uncini A, Yuki N. 21.

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al. Detection of serious bradyarrhyth- drome-new diagnostic classification. Kusunoki S. Abnormal sudomotor axon
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