Miller Fisher syndrome with early intracranial hypertension and delayed bilateral simultaneous facial nerve palsy: a case report

Abstract

Miller Fisher syndrome (MFS), a variant of Guillain–Barré syndrome, is characterized by ataxia, areflexia and ophthalmoplegia. This case report describes a 40-year old male that presented with a 3-day history of unsteady walking and numbness on both hands, and a 2-day history of seeing double images and unclear articulation. Lumbar puncture revealed an opening pressure of 260 mm H 2 O. Plasma serology was positive for anti-ganglioside M1-immunoglobulin M (anti-GM1-IgM) antibodies and negative for anti-ganglioside Q1b (anti-GQ1b) antibodies. The patient was diagnosed with MFS based on the clinical course and neurophysiological findings. On the 4th day of treatment with intravenous immunoglobulin (IVIG), his ataxia and unsteady walking improved, but his bilateral eyeballs were fixed, and over the next few days he developed bilateral peripheral facial paralysis. After 5 days of IVIG treatment, methylprednisolone treatment was offered and the patient's symptoms gradually improved. Early intracranial hypertension and delayed facial nerve palsy may be atypical presentations of MFS. Anti-GM1-IgM antibodies may be the causative antibodies for MFS. If the IVIG therapy does not stop the progression of the disease, the addition of corticosteroid therapy may be effective. However, the relationship between IgM type, anti-GM1 antibody and MFS remains unclear and requires further research.