title: Limb motor nerve dysfunction in Miller Fisher syndrome.
abstract: Typical Miller Fisher syndrome (MFS) lacks limb muscle weakness, but some patients may unpredictably progress to severe Guillain-Barré syndrome. The compound muscle action potential (CMAP) scan is a recently developed non-invasive, painless, and reproducible method for detecting early changes in motor nerve excitability. This technique was used to monitor subclinical limb motor nerve dysfunction during disease course in typical MFS. Three Miller Fisher patients with preserved limb muscle strength and normal routine nerve conduction studies were included. Frequent serial CMAP scanning of the median nerve was performed during acute phase and follow-up and was related to clinical course and outcome. All patients showed an abnormal increase in the range of stimulus intensities at the day of hospital admission, indicating reduced motor nerve excitability already at the earliest stage of disease. Median nerve dysfunction progressed in parallel or even before clinical deterioration, and improved with clinical recovery. Our study shows that typical MFS is a more general neuropathy, affecting peripheral motor nerves even in patients with preserved limb strength and conduction velocity. CMAP scanning is a sensitive technique for early detection of subclinical motor nerve dysfunction and for monitoring disease activity in immune-mediated neuropathies.
authors.affiliation: Department of Clinical Neurophysiology, Erasmus MC, University Medical Center, 3000 CA Rotterdam, The Netherlands. j.drenthen@erasmusmc.nl
authors.forename: Judith
authors.initials: J
authors.lastname: Drenthen
authors.forename: Ellen M
authors.initials: EM
authors.lastname: Maathuis
authors.forename: Gerhard H
authors.initials: GH
authors.lastname: Visser
authors.forename: Pieter A
authors.initials: PA
authors.lastname: van Doorn
authors.forename: Joleen H
authors.initials: JH
authors.lastname: Blok
authors.forename: Bart C
authors.initials: BC
authors.lastname: Jacobs
mesh_terms: D000328:Adult; D000368:Aged; D004185:Disability Evaluation; D018450:Disease Progression; D004558:Electric Stimulation; D004576:Electromyography; D019054:Evoked Potentials, Motor / Q000502:physiology; D005121:Extremities / Q000294:innervation / Q000503:physiopathology*; D005260:Female; D020275:Guillain-Barre Syndrome / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D019846:Miller Fisher Syndrome / Q000150:complications*; D009431:Neural Conduction / Q000502:physiology*; D010523:Peripheral Nervous System Diseases / Q000209:etiology*; D011446:Prospective Studies; D013997:Time Factors