Case report
MRI findings of optic pathway involvement in Miller Fisher syndrome in 3 pediatric patients and a review of the literature

https://doi.org/10.1016/j.jocn.2016.12.049Get rights and content

Highlights

  • Miller Fisher syndrome (MFS) is a rare variant of Guillain–Barré syndrome (GBS).

  • Cranial nerve involvement is not infrequent in MFS.

  • Optic nerves/pathways can show abnormal enhancement.

  • Imaging findings do not always correlate with clinical cranial nerve involvement.

Abstract

Background: Miller Fisher syndrome (MFS) is a rare demyelinating condition which may have involvement of cranial nerves. There are a few case reports of optic pathway involvement in children. We describe 3 patients with optic pathway enhancement in pediatric patients with MFS. Case series: We retrospectively reviewed brain imaging findings in 17 pediatric patients with of Guillain–Barré syndrome (GBS) meeting Brighton criteria who had brain MRIs performed during their acute illness. Cranial nerve enhancement was seen in 6/17 patients and optic nerve/chiasm enhancement was seen in 3 patients. Conclusion: Cranial nerve enhancement and optic pathway in particular, can be seen in patients with MFS. Imaging findings do not always correlate with clinical manifestations of cranial nerve involvement.

Introduction

Miller Fisher syndrome (MFS) is an acute demyelinating condition that is a rare variant of Guillain–Barré syndrome (GBS). MFS typically presents clinically as a triad of ophthalmoplegia, ataxia, and areflexia. MFS occurs more commonly in adults, and is uncommon in children. Cranial nerve involvement, especially bilateral weakness of facial muscles, is a feature that strongly supports the diagnosis [1]. On imaging, cranial nerve enhancement has been described, with the third and sixth cranial nerves being most frequently involved, but the fifth, seventh, eleventh, and twelfth cranial nerves can also be involved. There are only a few case reports of optic nerve involvement in MFS. We retrospectively reviewed the imaging findings in pediatric patients with GBS at our institution to look for abnormal imaging findings involving the optic pathway. We report 3 pediatric female patients ranging from 2 to 17 years of age, who all had abnormal findings on magnetic resonance imaging (MRI) involving the intracranial optic nerves/chiasm, and review the literature on cranial nerve involvement in MFS.

Section snippets

Methods

We reviewed our institutional database under institutional review board (IRB) approval for pediatric patients (age < 18 years) with a clinical diagnosis of GBS meeting Brighton criteria who had brain MRIs performed during their acute illness [2]. An experienced neuroradiologist with over 10 years of clinical experience reviewed all MRI scans. The current study was approved by our Institutional Review Board and patient consent was waived.

Results

Seventeen children with a clinical diagnosis of GBS had brain MRI scans performed as part of their diagnostic workup. Six out of 17 patients had abnormal cranial nerve enhancement, 3 out of which showed enhancement involving the intracranial optic nerves/chiasm. All three patients had findings involving multiple cranial nerves as described below.

Case 1

A 2-year-old female was admitted following 3-week history of progressive gait imbalance and pain in the lower extremities. She also had fevers, fatigue and a recent upper respiratory tract infection. On admission she was hypertensive, with blood pressure of 124/61 mmHg, (>95th percentile for age). On neurological examination, she had markedly depressed lower extremity reflexes and hesitancy with ambulating. She had periorbital edema and mild drooping of eyelids bilaterally. There was normal

Case 2

An 11-year-old female presented after 2 weeks of malaise, ankle pain, bilateral leg weakness, gait unsteadiness on her feet and urinary incontinence. Her symptoms developed following a presumed viral gastrointestinal illness with diarrhea. On neurological examination, she had bradyteleokinesia and past pointing on finger to nose test. She subsequently developed areflexia and gait ataxia. As an inpatient she developed significant autonomic instability, including episodes of vomiting, urinary

Case 3

A 17-year-old female presented with headaches, nausea, vomiting, malaise and weakness, tongue swelling, paresthesias in her perioral area and bilaterally in her lower limbs. She had high fever with headache 1 week preceding this episode, possibly due to an antecedent infection. On neurologic examination, V1 and V2 testing was normal, but she was unable to sense both light touch and pinprick in the V3 region bilaterally. She also had facial diplegia. Her symptoms developed further and on exam she

Discussion

MFS was described by Fisher in 1956 characterized by total external ophthalmoplegia, severe ataxia and areflexia [3]. Today, MFS is known as a variant of GBS, compromising for 3–7.7% of GBS cases [4], [5], with an annual population incidence of one per one million. Adults are much more likely to be affected, with children making up only 14% of MFS incidence. The average age of onset of MFS is 43.6 years (ranging from 14 months to 80 years old) with males twice as likely to be affected [6].

MFS

Conclusion

The 3 cases presented demonstrate that optic nerves and tracts may show enhancement on Gadolinium- contrast enhanced MRI in MFS patients. This is a rare finding and further confirms CNS involvement in MFS, as the optic tracts are an extension of the diencephalon.

Funding

None.

Conflict of interest

None.

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