Associates' Presentations
Intro | Prev | Next | Last  

Guillain-Barre Syndrome And The Miller Fisher Variant

Christopher P. Sobczak, M.D., Safwan Jaradeh, M.D. Medical College of Wisconsin, Milwaukee, WI

Case: Patient is a 52 year old white male who presented to our institution with a five day history of increasing fatigue associated with arm weakness, perioral numbness and progressive ptosis. On the day of admission he needed to hold his eyelids up manually, and he developed ataxia. He denied recent upper respiratory symptoms, diarrhea, and he did not receive the flu shot this year. His past history is significant for Type II diabetes mellitus, hypertension, obesity, low back pain, and two cerebral vascular accidents ten years ago without residual. His exam revealed a BP 198/98, and he was afebrile. His voice was hoarse and right extraocular movements were limited and sluggish compared to the left Reflexes were absent. A tensilon test was negative. Lumbar puncture revealed no RBC's, one WBC, glucose of 124, and an elevated protein of 90. EMG confirmed an acute demylinating process. Stool was negative for enteropathogens and Botulism toxin. Urine was negative for heavy metals and porphyrins. A GQ1b ganglioside autoantibody was present in his serum. This confirmed our diagnosis of Miller Fisher Syndrome.

Discussion: Miller Fisher syndrome is a variant of Guillain-Barre Syndrome involving the triad: ataxia, ophthalmoplegia, and areflexia. The pathophysiology of the syndrome can be explained through GQ1b ganglioside autoantibodies, which are seen in 96 percent of cases of Miller Fisher Syndrome. These autoantibodies recognize epitopes expressed specifically in the nodal regions of oculomotor nerves and cerebellar neurons. Organisms such as Mycoplasma Pneumoniae and Campylobacter jejuni contain cell walls that are capable of inducing the production of these antoantibodies. Treatment is thus involved in immune modulation, through the use of IVIg, plasma exchange, and steroids. Our patient required intubation and wan treated with all three modalities over a three-month hospitalization. He eventually recovered to be discharged to long term rehabilitation.

Intro | Prev | Next | Last