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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

www.cvja.co.za

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,

mustafa.aldag@saglik.gov.tr

Sebnem Albeyoglu, MD

Ufuk Ciloglu, MD

Hakan Kutlu, MD

Levent Ceylan, MD