Cardiovascular Journal of Africa: Vol 28 No 6 (November/December 2017)

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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 28, No 6, November/December 2017 e4 AFRICA
Case Report
Miller–Fisher syndrome after coronary artery bypass
surgery
Mustafa Aldag, Sebnem Albeyoglu, Ufuk Ciloglu, Hakan Kutlu, Levent Ceylan
Abstract
Miller–Fisher syndrome (MFS) is an uncommon neurological
disorder that is considered a variant of the Guillain–Barre
syndrome (GBS). It is clinically defined by a triad of symp-
toms, namely ataxia, areflexia and ophthalmoplegia. These
acute inflammatory polyradiculopathic syndromes can be
triggered by viral infections, major surgery, pregnancy or
vaccination. While the overall incidence of GBS is 1.2–2.3 per
100 000 per year, MFS is a relatively rare disorder. Only six
cases of GBS after cardiac surgery have been reported, and to
our knowledge, we describe the first case of MFS after coro-
nary artery bypass surgery. Although cardiac surgery with
cardiopulmonary bypass may increase the incidence of MFS
and GBS, the pathological mechanism is unclear. Cardiac
surgery may be a trigger for the immune-mediated response
and may cause devastating complications. It is also important
to be alert to de novo autoimmune and unexpected neurologi-
cal disorders such as MFS after coronary bypass surgery.
Keywords: Miller–Fisher syndrome, Guillain–Barre syndrome,
coronary artery bypass grafting, cardiopulmonary bypass
Submitted 1/10/16, accepted 13/7/17
Cardiovasc J Afr 2017; 28: e4–e5
DOI: 10.5830/CVJA-2017-033
Miller–Fisher syndrome (MFS) is an uncommon neurological
disorder that is considered a variant of Guillain–Barre syndrome
(GBS).1 MFS is clinically defined by a triad of symptoms, namely
ataxia, areflexia and ophthalmoplegia. Both syndromes usually
occur after viral infections, mostly by Campylobacter jenuni,
cytomegalovirus, and Epstein–Barr and influenza viruses. These
acute inflammatory polyradiculopathic syndromes can also be
triggered by major surgery, pregnancy or vaccination. 2 While the
overall incidence of GBS is 1.2–2.3 per 100 000 persons per year,3
MFS is a relatively rare disorder, accounting for approximately
5% of patients with GBS.4
GBS is rare among post-surgical inflammatory neuropathies.
Only six cases of GBS after cardiac surgery have been reported,5
and to our knowledge, we have described the first case of MFS
after coronary artery bypass surgery.
Case report
A 50-year-old man was admitted to the emergency department
with a four-hour history of angina pectoris. Anterior ST-segment
elevation myocardial infarction was confirmed and the patient
underwent coronary angiography.
Triple-vessel coronary artery disease was demonstrated on
catheterisation, requiring urgent coronary bypass surgery. A
successful emergency coronary artery bypass procedure (left
internal thoracic artery to the left anterior descending artery,
and saphenous vein graft to the circumflex and diagonal arteries)
was performed using cardiopulmonary bypass (CPB) with 30°C
hypothermia.
Alhough the pre- and intra-operative periods remained
uneventful, the patient noticed ataxia, left-sided ptosis, weakness
and paresthaseia of his legs, which progressed rapidly on the fifth
postoperative day. Ataxia was prominent in the lower extremities
during standing and walking. There was no history of viral
infection, fever or other neurological diseases.
On neurogical examination, unilateral ptosis, gait ataxia
and areflexia were noted. After neurology consultation, cranial
computerised tomography (CT) revealed nothing unusual. Brain
CT and cerebrospinal fluid (CSF) analysis yielded normal
results.
CSF viral serology and gram stain culture were negative.
Additional laboratory work-up, including tests for connective
tissue disorders, anti-thyroid peroxidase and anti-thyroglobulin
antibodies were within normal limits. Electromyography and
brain magnetic resonance imaging (MRI) were performed and
a possible diagnosis of Miller–Fisher syndrome was considered.
Urgent plasmapheresis treatment was planned but within 24
hours the patient had serious dysphagia and rapidly developed
dyspnoea. After elective intubation, the patient was transfered to
the neurology intensive care unit. Treatment with plasmapheres
and intravenous immunoglobulin (0.4 g/kg/daily) was started
immediately. Although inotropic support and medications were
given to the patient, cardiopulmonary arrest occured on the
ninth postoperative day and he died inauspiciously.
Department of Cardiovascular Surgery, Siyami Ersek
Thoracic and Cardiovascular Surgery Training and
Research Hospital, Istanbul, Turkey
Mustafa Aldag, MD,
Sebnem Albeyoglu, MD
Ufuk Ciloglu, MD
Hakan Kutlu, MD
Levent Ceylan, MD