Early electrophysiological findings in Fisher-Bickerstaff syndrome

Abstract

Introduction: The term Fisher-Bickerstaff syndrome (FBS) has been proposed to describe the clinical spectrum encompassing Miller-Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis. The pathophysiology of FBS and the nature of the underlying neuropathy (demyelinating or axonal) are still subject to debate. This study describes the main findings of an early neurophysiological study on 12 patients diagnosed with FBS.

Patients and methods: Retrospective evaluation of clinical characteristics and electrophysiological findings of 12 patients with FBS seen in our neurology department within 10 days of disease onset. Follow-up electrophysiological studies were also evaluated, where available.

Results: The most frequent electrophysiological finding, present in 5 (42%) patients, was reduced sensory nerve action potential (SNAP) amplitude in one or more nerves. Abnormalities were rarely found in motor neurography, with no signs of demyelination. The cranial nerve exam revealed abnormalities in 3 patients (facial neurography and/or blink reflex test). Three patients showed resolution of SNAP amplitude reduction in serial neurophysiological studies, suggesting the presence of reversible sensory nerve conduction block. Results from cranial MRI scans were normal in all patients.

Conclusion: An electrophysiological pattern of sensory axonal neuropathy, with no associated signs of demyelination, is an early finding of FBS. Early neurophysiological evaluation and follow-up are essential for diagnosing patients with FBS.

Keywords: Anti-GQ1b syndrome; Bloqueo de conducción sensitivo reversible; Electromiograma; Electromyography; Fisher-Bickerstaff syndrome; Guillain-Barré syndrome; Miller-Fisher syndrome; Nerve conduction study; Reversible sensory nerve conduction block; Síndrome anti-GQ-1b; Síndrome de Fisher-Bickerstaff; Síndrome de Guillain-Barré; Síndrome de Miller-Fisher.