Miller Fisher syndrome (MFS) was first recognized by James Collier in 1932 as a clinical triad of ataxia, areflexia, and ophthalmoplegia. Later, it was described in 1956 by Charles Miller Fisher as a possible variant of Guillain-Barré syndrome (GBS). Here, we write a case of a patient with atypical presentation of this clinical triad as the patient presented with double vision initially due to unilateral ocular involvement that progressed to bilateral ophthalmoplegia. He developed weakness of the lower extremities and areflexia subsequently. A diagnosis of MFS was made due to the clinical presentation and the presence of albuminocytologic dissociation in the cerebrospinal fluid (CSF) along with normal results of brain imaging and blood workup. The patient received intravenous immune globulin (IVIG), and his symptoms improved. The initial diagnosis of MFS is based on the clinical presentation and is confirmed by cerebral spinal fluid analysis and clinical neurophysiology studies. This case which emphasizes the knowledge of a rare syndrome can help narrow down the differentials to act promptly and appropriately manage such patients.
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