Poster 324: Miller Fisher Syndrome—Response to Intravenous Immunoglobulin: A Case Report

Patients or Programs: An 8-year-old girl with progressive extremity weakness. Program Description: This patient was a previously healthy girl who developed progressive symmetric weakness in bilateral upper and lower extremities 10 days after an upper respiratory infection. Weakness was most noted in her proximal muscles. Pain in her low back and legs, urinary incontinence, and hypertension were also noted. Cranial nerves 2-12 were intact. The patient's functional vital capacity was within normal limits. Her electromyography, nerve conduction study, and lumbar puncture were consistent with Guillain-Barre Syndrome (GBS). Cytomegalovirus titers were indicative of a recent infection. The patient received a one time, 2 g/kg, dose of intravenous immunoglobulin (IVIG) at the acute hospital. The patient was transferred for acute inpatient rehabilitation. Setting: Inpatient rehabilitation facility. Results: Two weeks after initial onset of symptoms, the patient developed progressive limb weakness, increasing bilateral facial weakness, decreasing forced vital capacity, and increasing ophthalmoplegia. The patient was transferred to the intensive care unit where she underwent a 5 day course of IVIG. Significant gains in strength, respiratory status, and ophthalmoplegia resolution was observed after 2 days of treatment. Her progressive weakness duration was 5 days. Discussion: Miller Fisher syndrome, which consists of external ophthalmoplegia, ataxia, and muscle weakness is a rare variant of GBS reported in children. Treatment involves either plasmapheresis or intravenous immunoglobulin given over a 5-day course. This treatment regimen has been shown to decrease the deficits caused by this disease process. Studies indicate that relapse and disease severity are higher in patients treated with a shorter duration of IVIG. Conclusions: Close clinical monitoring is needed for patients with GBS on rehabilitation units. Patients that continue to deteriorate should be considered for further treatment with IVIG or plasmapheresis.