Educate the medical community about the atypical presentation of the MFS

A rare clinical variant of Guillain-Barre syndrome, known as Miller Fisher syndrome (MFS), is an acute immune-mediated demyelinating polyneuropathy classically presenting as a triad of ataxia, areflexia, and ophthalmoplegia. This disorder is typically associated with multiple cranial nerve dysfunction and occasionally with distal paresthesia and motor weakness. MFS is thought to be preceded by an upper respiratory tract or gastrointestinal infection resulting in molecular mimicry and an aberrant autoimmune response specially against gangliosides. Diagnosis of MFS is made based of clinical presentation, cerebral spinal fluid (CSF) analysis, and electromyography and serological evaluation. Understanding the clinical signs and progression of this disease can aid in the early recognition and management of these patients.

72-year-old Caucasian male initially presenting with diplopia and hypertensive urgency was found to have unilateral pupil-involving third-nerve palsy with right eye ptosis. Clinical course included rapid development of ophthalmoplegia, progressive bilateral facial diplegia, ascending weakness, areflexia, and small and large fiber sensory loss. CSF studies showed albuminocytological dissociation with electrodiagnostic evidence of severe sensory-motor polyneuropathy with diffuse axonal loss and serologic studies remarkable for elevated IgG levels to ganglioside-monosialic acid. MRI brain revealed bilateral enhancement of the facial nerve without spinal cord changes. Clinical course was complicated by radiculopathy with severe neuropathic pain in bilateral groin uncontrolled with medical management and respiratory failure requiring mechanical intubation. Patient was immediately transferred to neuro-critical care unit where patient demised was not prevented even after immediate IVIG infusion. 

Monocular isolated cranial neuropathy is a common complaint in Neurology and include an array of diagnosis. However, clinical evolution and physical exam findings are crucial in the diagnosis of MFS. Knowledge of clinical disease progression despite the atypical symptomatic presentation can guarantee the rapid diagnosis, treatment, and management of this condition.