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Anti GQ1b antibody disorders

The anti-GQ1b antibody is often associated with acute paresis of the ocular muscles. Thus it is an autoimmune disorder that causes eye muscle weakness.

The antibody has been found in:

Acute ophthalmoplegia without ataxia (AO)

AO is a rare disorder considered to be a variant of GB (see below). The most common manifestation of AO is external ophthalmoplegia with bilateral abduction deficits followed by involvement of the 3rd nerve and internal opthalmoplegia (pupillary paralysis). It should be distinguished from other causes of eye muscle weakness including cerebrovascular disease, tumors, infections, diabetes, multiple sclerosis, and myasthenia gravis. Detection of the anti-GQ1b antibody is useful in that it makes the other possibilities much less likely.

A movie of a patient who has opthalmoplegia (although not this particular syndrome) is found here. Note that while the head moves, the eyes are locked in place.

AO is treated with plasmaphereis and IVIG.

Guillain-Barre syndrome (GB)

This is a rare ascending neuropathy characterized by weakness and areflexia.

Miller-Fisher Syndrome (MFS)

This is a rare disorder related to Guillain Barre, characterized by a combination of ataxia, weakness or paralysis of the eye movements, and peripheral neuropathy with areflexia. Most patients present with diplopia and eventually develop complete paralysis of their eyes. Internal ophthalmoplegia (pupillary paralysis) occurs in about 40% of patients. Antibodies to the ganglioside GQ1b are often associated with Miller Fisher syndrome. This condition should be considered when there is a combination of diplopia, ataxia, and loss of deep tendon reflexes. The ataxia is probably due to loss of sensory input to the cerebellum. Mean time of recovery is at 10 weeks.

 

References:

Lee SH and others. Acute ophthalmoplegia (without ataxia) associated with anti-GQ1b antibody. Neurology 2008;71;426-429

Copyright October 6, 2013 , Timothy C. Hain, M.D. All rights reserved. Last saved on October 6, 2013