The Characteristics and Prognosis of Miller Fisher Syndrome

Jae Woo Jung; Jong Heon Lee; Jae Ho Jung

doi:10.3341/jkos.2017.58.2.197

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Jung, Lee, and Jung: The Characteristics and Prognosis of Miller Fisher Syndrome

Original Article

J Korean Ophthalmol Soc 2017;58(2):197-202.

Published online: 8 September 2017

DOI: https://doi.org/10.3341/jkos.2017.58.2.197

The Characteristics and Prognosis of Miller Fisher Syndrome

Jae Woo Jung, M.D., Jong Heon Lee, M.D., Jae Ho Jung, M.D., PhD

Department of Ophthalmology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea

Address reprint requests to Jae Ho Jung, MD, PhD Department of Ophthalmology, Pusan National University Yangsan Hospital, # 20 Geumo-ro, Mulgeum-eup, Yangsan 50612, Korea Tel: 82-55-360-2591, Fax: 82-55-360-2161 E-mail: jungjaeho@pusan.ac.kr

Received 29 September 20165 December 2016 Accepted 16 January 2017

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

In the present study, the clinical characteristics and prognosis of patients clinically diagnosed with classic Miller Fisher syndrome were evaluated.

Methods

We retrospectively investigated the clinical and laboratory findings as well as treatment outcomes using the medical records of patients diagnosed with Miller Fisher syndrome. Symptom triad including acute ophthalmoplegia, ataxia, and areflexia were evaluated.

Results

This study included 10 patients. Nine patients had antecedent infectious illness which took an average of 11 ± 9.7 days for onset of diplopia from antecedent infectious systemic illness. Seven patients showed bilateral paralytic strabismus. Specifically, 5 patients showed the involvement of vertical and horizontal extraocular muscles. Pupil impairment and blephar-optosis were observed in 4 patients, limb weakness in 3 patients, dysarthria in 3 patients and facial palsy in 1 patient. Two pa-tients showed contrast enhancement of the abducens nerve on brain magnetic resonance imaging (MRI) and 2 patients showed albumin-cell dissociation on cerebrospinal fluid (CSF) analysis. Eight patients had anti-GQ1b antibodies in their blood serum analysis. Six patients were treated with intravenous immunoglobulins and the other patients were observed with regular fol-low-ups. The duration of diplopia was 2.9 ± 1.2 months in the treatment group and 3.1 ± 1.7 months in the control group ( p > 0.05). The duration of ataxia was 1 ± 0.4 months in the treatment group and 1 ± 0.9 months in the control group ( p > 0.05).

Conclusions

Miller Fisher syndrome should be considered in patients with antecedent infection; acute ophthalmoplegia, ataxia and areflexia as well as anti-GQ1b antibody can be helpful for diagnosis. Final outcomes in the treated group were not sig-nificantly different from the control group and all patients showed good final outcomes.

Keywords: Anti-GQ1b, Bickerstaff’, s brain stem encephalitis, Miller Fisher syndrome, Ophthalmoplegia

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Figure 1.

Case 7. Contrast enhancement of the left sixth cra-nial nerve in orbit magnetic resonance image (white arrow).

Table 1.

Clinical and laboratory findings in 20 eyes of 10 patients with miller fisher syndrome and serum anti-GQ1b antibody positive

No	Age	Sex	Laterality	Alignment	Gaze limitation	Ptosis	Pupil	Anti-GQ1b^*	Neurologic abnormality	Radiologic finding	CSF findings^†
1	57	M	B	XT, RHOT	R Fixed L Vertical and adduction	R	B	-	Bulbar palsy, limb weakens	-	+
2	34	M	B	XT, RHOT	R adduction L horizontal	-	B	+++	-	-	-
3	14	M	B	ET	Bilateral abduction	-	-	++	-	Bilateral 6th nerve enhancement	-
4	8	F	B	XT	Bilateral adduction	-	-	+	-	-	-
5	17	F	R	RXT, RHOT	R fixed	R	R	+	Facial weakness, dysarthria, limb weakness	-	-
6	53	M	B	XT	Bilateral adduction	-	-	+	-	-	-
7	57	F	R	ET	L abduction	-	-	+	-	Left 6th nerve enhancement	-
8	51	M	B	XT, RHOT	Bilateral fixed	B	-	+++	-	-	+
9	13	F	R	XT, LHOT	L fixed	-	R	++	-	-	-
10	7	M	B	XT	Bilateral adduction	R	-	-	Bulbar palsy, limb weakness	-	-

CSF = cerebrospinal fluid; B = both; R = right; L = left; XT = exotropia; RHOT = right hypotropia; ET = esotropia; RXT = right exo-tropia; LHOT = left hypotropia.

^* Anti-gangliosideQ1b (anti-GQ1b) antibody reference range: negative (-), positive (+~+++); dissociation.

^† CSF finding: Albuminocytological

Table 2.

Comparison between intra-venous immunoglobulin treatment group and observation group

	Treatment group (months)	Observation group (months)	Total (months)	p-value^* in comparison of treatment vs. Control group
Loss of diplopia at Primary gaze	2.9 ± 1.2	3.1 ± 1.7	3.0 ± 1.3	0.42
Loss of ataxia	1 ± 0.4	1 ± 0.9	1 ± 0.6	0.75

Values are presented as mean ± SD unless otherwise indicated.

^* p-value: Mann-Whitney test.

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