Neuromuscular junction dysfunction in Miller Fisher syndrome.
The Miller Fisher syndrome (MFS) is a variant of Guillain-Barre syndrome with the clinical triad of areflexia, ataxia, and ophthalmoparesis. The classic pathologic mechanism of disease is considered to be peripheral nerve demyelination. We present a patient with binocular diplopia and a diagnosis of myasthenia gravis from 15 years prior. Electrophysiologic studies revealed a decremental response on repetitive nerve stimulation, suggesting recurrent myasthenia. However, pupillary light-near dissociation and areflexia were present and positive anti-GQ1b antibodies confirmed MFS. This patient highlights a developing recognition of impaired neuromuscular transmission in MFS. His presentation is discussed in the context of the animal and human literature on neuromuscular junction abnormalities in MFS.
Silverstein
M P
MP
Rush University Medical Center, Chicago, IL 60612, USA.
Zimnowodzki
S
S
Rucker
J C
JC
D000328:Adult; D001323:Autoantibodies / Q000097:blood; D004172:Diplopia / Q000175:diagnosis; D004594:Electrophysiology; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D008297:Male; D019846:Miller Fisher Syndrome / Q000175:diagnosis* / Q000276:immunology; D009157:Myasthenia Gravis / Q000175:diagnosis; D020511:Neuromuscular Junction Diseases / Q000175:diagnosis* / Q000276:immunology
D001323:Autoantibodies; D005732:Gangliosides; C039129:GQ1b ganglioside