Total ocular akinesis: Miller Fisher or Guillain-Barré syndrome?

Abstract

Total, bilateral ophthalmoplegia is very rare. More than 50% of cases are Miller Fisher (MFS) and Guillain-Barré (GBS) syndromes. There is a correlation of MFS with anti-GQ1b antibodies. High levels of GQ1b gangliosides are found in myelin sheathes of cranial nerves supplying the extraocular muscles. This may explain the association of anti-GQ1b antibodies with ophthalmoplegia. Anti-GQ1b were also found in cases of GBS accompanied by ophthalmoplegia, atypical MFS (MFS without ataxia), MFS/GBS overlap syndromes and Bickerstaff brainstem encephalitis. This has led some authors to classify them as 'anti-GQ1b syndromes'. In this article we describe a diagnostically difficult case of a patient with a very rare, total bilateral paralysis of all ocular muscles, accompanied by bilateral ptosis, diminished tendon reflexes of upper extremities, paresis and hypoesthesia of the left upper extremity.