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ANTI-GQ1B IGG ANTIBODY SYNDROME: MILLER FISHER SYNDROME AND BICKERSTAFF’S BRAINSTEM ENCEPHALITIS SUPERPOSITION
Last modified: 2014-04-03
Abstract
Introduction
Miller Fisher syndrome (MFS) is clinically characterized by acute external ophthalmoplegia, ataxia, and arreflexia; while impaired consciousness, external ophthalmoplegia and ataxia constitute Bickerstaff’s brainstem encephalitis (BBE). Anti-ganglioside antibodies, anti-GQ1b, are associated with MFS, Guillain-Barré, acute isolated ophthalmoplegia and cranial polyneuropathy.
Objective
To present a pediatric patient with sequential development of MFS and BBE with positive serum anti-GQ1b IgG antibody.
Case description and methods:
A 3 year-old previously healthy boy, presented with ataxia, ophtalmoparesia, arreflexia, lower limb weakness and swallowing difficulties. With SMF diagnosis, he was treated with intravenous immunoglobulin showing marked improvement. A week later he presented with apathy, mutism, orolingual automatisms and impaired consciousness associated with pyramidalism. He was given high dose steroid treatment, after a BBE diagnosis was made, followed by complete recovery.
Results
Brain MRI showed midbrain and cranial nerve enhancement. Albuminocytologic dissociation in CSF was present. Nerve conduction study: motor and sensory polyradiculneuropathy, with facial involvement and blink reflex absence. Anti-GQ1b IgG antibody was positive.
Conclusions
Sequential development of MFS and BBE occurred in a pediatric patient with positive serum anti-GQ1b IgG antibodies. Immunotherapy helped recovery.
Serum IgG antibodies to GQ1b can cause different clinical phenotypes, with overlapping between them, MFS and BBE being considered part of a continuous clinical spectrum.
Miller Fisher syndrome (MFS) is clinically characterized by acute external ophthalmoplegia, ataxia, and arreflexia; while impaired consciousness, external ophthalmoplegia and ataxia constitute Bickerstaff’s brainstem encephalitis (BBE). Anti-ganglioside antibodies, anti-GQ1b, are associated with MFS, Guillain-Barré, acute isolated ophthalmoplegia and cranial polyneuropathy.
Objective
To present a pediatric patient with sequential development of MFS and BBE with positive serum anti-GQ1b IgG antibody.
Case description and methods:
A 3 year-old previously healthy boy, presented with ataxia, ophtalmoparesia, arreflexia, lower limb weakness and swallowing difficulties. With SMF diagnosis, he was treated with intravenous immunoglobulin showing marked improvement. A week later he presented with apathy, mutism, orolingual automatisms and impaired consciousness associated with pyramidalism. He was given high dose steroid treatment, after a BBE diagnosis was made, followed by complete recovery.
Results
Brain MRI showed midbrain and cranial nerve enhancement. Albuminocytologic dissociation in CSF was present. Nerve conduction study: motor and sensory polyradiculneuropathy, with facial involvement and blink reflex absence. Anti-GQ1b IgG antibody was positive.
Conclusions
Sequential development of MFS and BBE occurred in a pediatric patient with positive serum anti-GQ1b IgG antibodies. Immunotherapy helped recovery.
Serum IgG antibodies to GQ1b can cause different clinical phenotypes, with overlapping between them, MFS and BBE being considered part of a continuous clinical spectrum.
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