Bickerstaff's brainstem encephalitis, Miller Fisher syndrome and Guillain-Barre syndrome overlap in an asthma patient with negative anti-ganglioside antibodies.
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Guillain-Barre syndrome and anti-ganglioside antibodies: a clinician-scientist's journey.
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Guillain-Barre syndrome (GBS) is the most frequent cause of acute flaccid paralysis. Having seen my first GBS patient in 1989, I have since then dedicated my time in research towards understanding the pathogenesis of GBS. Along with several colleagues, we identified IgG autoantibodies against ganglioside GM1 in two patients with GBS subsequent to Campylobacter jejuni enteritis. We proceeded to demonstrate molecular mimicry between GM1 and bacterial lipo-oligosaccharide of C. jejuni isolated from a patient with GBS. Our group then established a disease model for GBS by sensitization with GM1 or GM1-like lipo-oligosaccharide. With this, a new paradigm that carbohydrate mimicry can cause autoimmune disorders was demonstrated, making GBS the first proof of molecular mimicry in autoimmune disease. Patients with Fisher syndrome, characterized by ophthalmoplegia and ataxia, can develop the disease after an infection by C. jejuni. We showed that the genetic polymorphism of C. jejuni sialyltransferase, an enzyme essential to the biosynthesis of ganglioside-like lipo-oligosaccharides determines whether patients develop GBS or Fisher syndrome. This introduces another paradigm that microbial genetic polymorphism can determine the clinical phenotype of human autoimmune diseases. Similarities between the clinical presentation of Fisher syndrome and Bickerstaff brainstem encephalitis have caused debate as to whether they are in fact the same disease. We demonstrated that IgG anti-GQ1b antibodies were common to both, suggesting that they are part of the same disease spectrum. We followed this work by clarifying the nosological relationship between the various clinical presentations within the anti-GQ1b antibody syndrome. In this review, I wanted to share my journey from being a clinician to a clinician-scientist in the hopes of inspiring younger clinicians to follow a similar path. (+info)
Demyelinating features in sensory nerve conduction in Fisher syndrome.
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OBJECTIVE: A significant number of patients with Fisher syndrome (FS) exhibit sensory symptoms in addition to the classical triad of opthalmoplegia, ataxia and areflexia. Previous studies have shown the amplitudes of sensory nerve action potentials (SNAPs) to decrease in patients with FS, thus implying the presence of an axonal pathology in the sensory nerves. METHODS: We included ten consecutive patients with FS who were divided into the following two groups: those with hypesthesia (group H) and those without hypesthesia (group NS). The parameters obtained from nerve conduction studies (amplitudes of compound muscle action potentials, motor conduction velocities, amplitudes/duration of SNAPs and sensory conduction velocities) were retrospectively compared between the two groups. In addition, follow-up sensory nerve conduction studies were conducted in one representative patient from each group. RESULTS: Of the 10 patients with FS, four (40%) showed hypesthesia and eight (80%) showed distal paresthesia. The amplitudes of the SNAPs of both the median and sural nerves were lower in group H than in group NS. Moreover, the duration of the sural SNAPs was longer in group H than in group NS. Desynchronization of SNAPs in the acute phase was observed during follow-up in both patients who underwent follow-up studies. CONCLUSION: The prolonged duration of SNAPs in group H and the desynchronization of SNAPs in the two patients who underwent follow-up studies suggest the presence of a concomitant demyelinating process in the sensory nerves. (+info)
Miller Fisher syndrome associated with influenza A infection.
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A 36-year-old, previously healthy man presented with Miller Fisher syndrome (MFS) five days after he was diagnosed with an influenza A infection by a rapid antigen test. He had not received any recent vaccinations. He had no loss of consciousness. Bilateral ophthalmoplegia, blepharoptosis, areflexia, and ataxic gait were noted. One week after treatment with intravenous immunoglobulin, his ophthalmoplegia, blepharoptosis, and ataxic gait had gradually improved, and his deep tendon reflexes returned. Anti-GQ1b IgG antibodies were detected in his serum. There has been no previous report of postinfectious MFS following confirmed an influenza A infection in an adult. (+info)
Fisher syndrome with taste impairment.
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A 60-year-old woman was admitted to our hospital with a two day history of truncal ataxia and diplopia. Three days after admission, complete paresis of eye movements, left ptosis, taste impairment and absence of deep tendon reflexes appeared. The patient displayed normal facial movements; however, she reported decreased sensations of sweet and salty tastes. Anti-GQ1b antibodies were detected in the serum, and Fisher syndrome was therefore diagnosed. Intravenous immunoglobulin was administered starting five days after admission, with limitations of eye movements, areflexia and taste impairment showing improvements by 12 days after onset. Taste disturbance is rare in patients with Fisher syndrome. In this case, we hypothesize that autoantibodies may have targeted antigens in the chorda tympani, glossopharyngeal nerve or taste buds. (+info)
Miller Fisher syndrome mimicking ocular myasthenia gravis.
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