Article, Neurology

Takotsubo cardiomyopathy associated with Miller-Fisher syndrome

American Journal of Emergency Medicine 35 (2017) 1012-1030

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Takotsubo cardiomyopathy associated with Miller-Fisher syndrome

Miller-Fisher syndrome is a rare variant of Guillain-Barre syndrome and has the following triad, ataxia, areflexia and ophthalmoplegia [1]. It is only observed in only 1%-5% of cases of Guillain-Barre Syndrome [2, 3]. Miller-Fisher syndrome is a relatively uncommon condition in which the body’s immune system attacks part of the peripheral nervous system [1]. It can affect the nerves that control muscle movement, pain, temperature sensation and can be potentially fatal if it affects the respiratory muscles [2]. Although Guillain-Barre Syndrome has rarely been associated with Takotsubo cardiomyopathy, a transient cardiac syndrome that mimics acute coronary syndrome, there have been no re- ports of Miller-Fisher syndrome association with stress cardiomyopa- thy. Here, we present a case of a 51 year old female who developed Takotsubo cardiomyopathy in association with Miller-Fisher syndrome. 51-year-old female presented with progressive paresthesia of lower extremities, Double Vision, and trouble walking. Vitals were remarkable for tachycardia and tachypnea. Physical exam demonstrated areflexia, and ptosis. She had respiratory distress and was intubated. Her initial electrocardiogram showed nonspecific ST segment changes and her Tro- ponin T was elevated to 0.41 ng/mL, which peaked at 0.66 ng/mL. Patient was also started on norepinephrine due to hypotension. Repeat electro- cardiogram in the morning showed nonspecific ST-T segment changes with T wave flattening. A 2D echocardiogram showed a depressed left ventricular ejection fraction to 35% with severely hypokinetic anterior wall and left ventricular apex was severely hypokinetic. An electromyog- raphy nerve conduction study showed severely decreased conduction ve- locity and prolonged distal latency in all nerves consistent with Demyelinating disease. She was treated with 5 days of intravenous immu- noglobulin therapy to which she showed significant improvement in strength in her lower extremities. Repeated echocardiogram showed an improved left ventricular ejection fraction of 55% and no left ventricular wall motion abnormalities. She was weaned off pressor requirement,

and transitioned to Carvedilol and Lisinopril.

Takotsubo cardiomyopathy is a rare complication of Miller-Fisher syndrome and literature review did not reveal any cases. Miller-Fisher syndrome is an autoimmune process that affects the peripheral nervous system causing autonomic dysfunction which may involve the heart [2, 3]. Due to the significant autonomic dysfunction seen in Miller-Fisher syndrome, it could lead to arrhythmias, blood pressure changes, acute coronary syndrome and myocarditis, Takotsubo cardiomyopathy can be difficult to distinguish. The criteria to diagnose Takotsubo cardiomy- opathy include a transient hypokinesis, akinesis, or dyskinesis of the left ventricle wall with or without apical involvement in the absence of ob- structive coronary artery disease, and is often related to a stressful trig- ger [4,5]. Dysregulation of autonomic tone with excessive sympathetic activation in Miller-Fisher syndrome with elevated catecholamine levels is one hypothesis [5]. The pathophysiology of Takotsubo cardio- myopathy seems to be from sympathetic excitation of brain triggering Catecholamine release causing hyperdynamic basal contraction, and

apical systolic dysfunction [4-6]. The treatment of Takotsubo cardiomy- opathy is supportive with beta-blockers and angiotensin-converting enzyme inhibitor is recommended until left ventricle ejection fraction improvement. Takotsubo cardiomyopathy is a rare complication of Miller-Fisher syndrome and must be distinguished from autonomic dysfunction as both diagnoses have different approaches to treatment.


No funding was involved in the production of this manuscript.

Dalvir Gill Department of Internal Medicine, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA

Corresponding author at: 60 Presidential Plaza, Apartment 1104,

Syracuse 13202, NY, USA.

E-mail address: [email protected]

Kan Liu

Department of Cardiology, SUNY Upstate Medical University, 750 East

Adams Street, Syracuse, NY 13210, USA E-mail address: [email protected]


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  2. Iga K, Himura Y, Izumi C, Miyamoto T, Kijima K, Gen H, et al. Reversible left ventricular dysfunction associated with Guillain-Barre syndrome–an expression of catechol- amine cardiotoxicity? Jpn Circ J 1995;59:236-40.
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  4. Bybee KA, Prasad A. Stress-related cardiomyopathy syndromes. Circulation 2008;118:


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  5. Lee VH, Connolly HM, Fulgham JR, Manno EM, Brown Jr RD, Wijdicks EF. Tako-tsubo cardiomyopathy in Aneurysmal subarachnoid hemorrhage: an underappreciated ven- tricular dysfunction. J Neurosurg 2006;105:264-70.

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