Letters to the Editor  |   February 2008
Miller Fisher Variant of Guillain-Barré Syndrome: A Report of Case
Author Affiliations
  • Matthew P. Kozminski, DO
    Department of Medicine and Neurology Clinic, Madigan Army Medical Center, Tacoma, Wash
Article Information
Neuromusculoskeletal Disorders / Ophthalmology and Otolaryngology
Letters to the Editor   |   February 2008
Miller Fisher Variant of Guillain-Barré Syndrome: A Report of Case
The Journal of the American Osteopathic Association, February 2008, Vol. 108, 51-52. doi:
The Journal of the American Osteopathic Association, February 2008, Vol. 108, 51-52. doi:
To the Editor: Guillain-Barré syndrome (GBS), also known as acute idiopathic polyneuritis, is a type of neuromuscular paralysis that has several variants.1-3 These variants all share similar patterns of symptoms and patient recovery.1-3 Miller Fisher syndrome (MFS) is a rare variant of GBS, observed in only about 1% to 5% of all cases of GBS in Western countries.1,2 Miller Fisher syndrome occurs in more men than women by a ratio of approximately 2:1.3 The mean age of onset of MFS is 43.6 years,3 though onset has been documented in individuals between the ages of 13 and 78 years.2 
The main difference between MFS and more common variants of GBS is that the first nerve groups to be affected by paralysis in patients with MFS are those in the head, resulting in difficulty controlling eye muscles and balance. Paralysis in other forms of GBS typically begins in the legs.1-3 Moreover, MFS is characterized by a triad of conditions: areflexia, ataxia, and ophthalmoplegia.2 
The present report describes the case of a man with risk factors for stroke. He presented chiefly with complaints of diplopia and gait abnormality. Further evaluation revealed the triad of conditions characteristic of MFS. 
A 70-year-old man with several risk factors for stroke—including a history of myocardial infarction, coronary artery bypass graft surgery, essential hypertension, and hyperlipidemia—visited the emergency department at a military medical facility (Madigan Army Medical Center in Tacoma, Wash). His chief complaints at presentation were persistent diplopia of approximately 7 days' duration and a “wobbly” gait. 
The patient's medical history revealed that he had initially sought emergency care for his symptoms at a different facility 6 days before presentation at the Madigan Center. The other facility discharged the patient to home without admission upon determining that a computed tomographic (CT) scan of his head revealed no intracranial abnormalities. A follow-up outpatient Doppler ultrasonographic examination arranged by the patient's primary care physician showed minimal plaque in the carotid arteries. 
During the emergency department encounter at the Madigan Center, the patient expressed concern that his symptoms had been persistent for 7 days without any improvement. He requested a re-evaluation and second opinion by physicians whom he had not previously seen. 
Examination findings by neurology clinic staff included slight exophoria of the right eye upon leftward gaze during cover-uncover (ie, unilateral) testing, as well as subtle signs of ophthalmoplegia (bilateral hypometric saccades, bilateral saccadic pursuits). The examination found no signs of pupillary defects, eyelid ptosis, or nystagmus. Bilateral hyporeflexia was detected throughout the patient's body. Some terminal dysmetria was observed during finger-to-nose testing. The patient showed no sensory deficits to light touch, pinprick, or proprioception, but he did have a mild vibratory deficit bilaterally from his feet to his mid-shin. The patient demonstrated normal strength bilaterally throughout his body. His gait had a wide base, but his stride and arm-swing movements were normal. 
Further examination of the patient was performed by a staff neuro-ophthalmologist using Maddox rods, a set of parallel glass rods used in testing for heterophoria. This test revealed signs of horizontal and vertical gaze paresis. 
After admission to the hospital, additional tests were performed. These tests were most notable for an analysis of cerebrospinal fluid (CSF) consistent with albuminocytologic dissociation and GQ1b ganglioside immunoglobulin G (IgG) antibody titers of 1:128,000. An inpatient electromyographic/nerve conduction velocity study performed by a staff neurophysiologist elicited motor abnormalities in the facial nerve (cranial nerve VII)4 and abnormalities in the patient's blink response—both of which were believed to be consistent with a diagnosis of MFS. 
Miller Fisher syndrome is an uncommon variant of GBS that can be diagnosed by tests for its characteristic triad of conditions: areflexia, ataxia, and ophthalmoplegia.2 
In cases of MFS, ataxia is primarily noted during the patient's gait, typically in the trunk and with lesser involvement of the limbs.2 A patient's motor strength characteristically is spared in MFS.2 Anti-GQ1b antibodies, activated by certain strains of Campylobacter jejuni, have a relatively high specificity and sensitivity for MFS.2 Dense concentrations of GQ1b ganglioside are found in the oculomotor nerve (cranial nerve III), trochlear nerve (cranial nerve IV), and abducens nerve (cranial nerve VI) of patients with MFS, which may explain the relationship between anti-GQ1b antibodies and ophthalmoplegia.3,5 Titers of anti-GQ1b antibodies in CSF that are greater than 1:40 are specific for MFS.3 
Onset of recovery from ataxia and ophthalmoplegia in patients ranges from approximately 2 weeks to 2 months,4 with a mean recovery onset at 10 weeks.3 Most patients will fully recover from ataxia and ophthalmoplegia, as well as areflexia, within 6 months.2 In some cases of MFS, post-gadolinium magnetic resonance imaging might show enhancement of cranial nerves.6 
The present case demonstrates that physicians need to be aware of symptoms and examination findings consistent with MFS so as not to confuse them with those of such ischemic events as stroke and transient ischemic attack, particularly in patients older than 40 years. 
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