Isolated ophthalmoplegia in a patient with Miller-Fisher syndrome

Case presentation: A 34-year-old male patient was admitted to the hospital due to sudden onset diplopia. The initial neurological examination shown bilateral ophthalmoplegia suggestive of III nerve palsy, associated with mydriasis, dysarthria, and paresthesia on the left side of the face. The first impression of the attending neurologist was a brain stem syndrome with internuclear ophthalmoplegia due to the sudden onset of symptoms. Gradually, during the first 10 days of hospitalization the patient developed complete paralysis of the extrinsic ocular muscles and started with bilateral facial palsy and worsening of the dysartria, the reflexes were also widespread decreased. There was no significant ataxia on examination. The investigation progressed with a lumbar puncture and electroneuromyography that shown elevated protein with normal cellular counts on the cerebrospinal fluid and alterations suggestive of Miller Fisher syndrome on the electroneuromyography. Treatment with Intravenous Immunoglobulin was administered with some improvement of the facial palsy at discharge from hospital. Physical examination at discharge showed mild left facial paresis, mild dysarthria, and he continued with the bilateral ophthalmoplegia, worst on his left side. At follow-up revision by phone the patient stated that he has improved considerably, but remaining with the ophthalmoparesis. Discussion: The Miller-Fisher syndrome (MFS) is usually a rare syndrome characterized by the classical triad: ophthalmoplegia, ataxia and areflexia. It is considered a variant of the Guillain-Barré syndrome, with incidence worldwide around 1-2 cases each 100.000 people. Typically it presents with the acute or subacute onset of the triad, starting with the ophthalmoplegia, diplopia, following with ataxia and generalized areflexia. The diagnoses is usually made by the presence of elevated proteins in the cerebrospinal fluid and electroneuromyography typical findings, and we can also identify the presence of anti-GQ1b IgG antibodies on the cerebrospinal fluid. At the present case we do not have this test available what the public system. The GQ1b antibody is thought to have a direct effect on the neuromuscular junctions of the ocular muscles. The GQ1b ganglioside is also an important component of oculomotor nerve myelin. In the afore mentioned case it is remarkable the unusual presentation of hyper acute symptoms with the association with marked mydriasis at the onset. There was no significant ataxia or gait abnormalities at the onset. Furthermore, it is uncommon for the patient with MFS to present with an hyperacute onset associated with mydriasis and paresthesias. Final comments: The case report related here, shows a rare presentation of the MFS. It elucidates a rare presentation of a variant of GBS, that is an important cause of diplopia and ophthalmoplegia caused by a demyelinating peripheral nerve autoimmune desease that could affect the young population.