Motor conduction studies in Miller Fisher syndrome with severe tetraparesis

Published on Mar 1, 2002in Muscle & Nerve2.505
· DOI :10.1002/MUS.10055
Masahisa Katsuno49
Estimated H-index: 49
 (Nagoya University), Tetsuo Ando10
Estimated H-index: 10
 + 2 AuthorsGen Sobue84
Estimated H-index: 84
 (Nagoya University)
Sources
Abstract
Some patients with Miller Fisher syndrome (MFS) have a severe tetraparesis such as that observed in Guillain–Barre syndrome (GBS). To determine whether pathophysiologic differences exist between the tetraparesis in MFS and that in GBS, we compared clinical and motor conduction findings in 4 MFS patients who developed severe tetraparesis with those in 5 MFS patients without tetraparesis, and 14 GBS patients. MFS patients with or without tetraplegia had normal motor conduction velocities, distal motor latencies, compound muscle action potential (CMAP) amplitudes, and F-wave latencies. CMAP amplitude tended to be lower in tetraparetic MFS patients than in MFS patients without tetraparesis, but not significantly. F-wave occurrence was slightly reduced in 1 MFS patient with tetraparesis and 1 MFS patient without tetraparesis. Motor conduction parameters were abnormal in 13 of 14 patients with GBS, and showed demyelinating features in 10. Our results suggest that the pathophysiology of tetraparesis in MFS differs from that in GBS. © 2002 Wiley Periodicals, Inc. Muscle Nerve 25: 000–000, 2002
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A 29 year old woman developed diplopia and ataxic gait. Neurological examination showed total ophthalmoplegia, cerebellar ataxia, and areflexia. Moreover, there was muscle weakness in all four limbs. An overlap of Fisher's and Guillain-Barre syndromes was dignosed. On day 5 she suddenly developed involuntary flinging movements that affected the face and four limbs. Surface EMG showed 1.5-2 Hz rhythmic grouping discharges. The involuntary movements were considered ballism. This is the first repor...
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A Fisher syndrome (FS) patient with antibody to tetrasyaloganglioside GQ1b (GQ1b) developed late limb weakness. Serial motor conduction velocities (MCVs) showed a marked reduction of distal compound muscle action potential (CMAP) amplitudes, worse at 2–3 weeks, followed by a dramatic increase in week 5. Motor conduction velocities were always in the normal range, distal motor latencies changed only slightly, and conduction block in intermediate nerve segments was absent. These electrophysiologic...
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Guillain–Barre syndrome (GBS) is recognized clinically by the presence of acute, rapidly progressive weakness, areflexia, and albuminocytological dissociation in cerebrospinal fluid. Although GBS was initially considered to be primarily an acute inflammatory demyelinating polyneuropathy (AIDP), several other subtypes have been recognized: acute motor axonal neuropathy (AMAN), acute motor-sensory axonal neuropathy (AMSAN), and Fisher syndrome (FS). Because each of these subtypes may have an indep...
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Guillain-Barre syndrome has been considered to be primarily an acute inflammatory demyelinating polyneuropathy (AIDP). Our experience with Guillain-Barre syndrome in northern China differs from the traditional concept. Electrophysiologically and pathologically, most of our patients have motor axonal degeneration with minimal cellular inflammation, which we have termed ‘acute motor axonal neuropathy’ (AMAN). The current studies were undertaken to characterize prospectively the clinical, electroph...
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Serum IgG autoantibodies to GQ1b ganglioside are associated with the acute phase of the Miller-Fisher syndrome (MFS). We investigated the effects of three anti-GQ1b-positive MFS sera in the mouse phrenic-nerve/diaphragm preparation. Miniature endplate potential frequencies increased eight-fold within 25 min, declined rapidly, and ceased altogether after 3 h, when nerve stimulation no longer evoked a response. One MFS convalescent serum (anti-GQ1b negative) and sera from healthy controls and from...
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To determine the significance of serum anti-GQ 1b IgG antibody, we studied the disease spectrum associated with this antibody and GQ 1b epitope in the human nervous system. We examined sera from 19 patients with typical Miller Fisher syndrome (MFS), five patients with acute postinfectious ophthalmoplegia without ataxia (atypical MFS), six patients with Guillain-Barre syndrome (GBS) with ophthalmoplegia (GBS-OP[+]), and 23 patients with GBS without ophthalmoplegia (GBS-OP[-]). We also examined se...
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Jan 7, 2015·Future Neurology
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