Abstract
Mills' syndrome is an extremely rare, slowly progressive, unilateral ascending or descending clinical syndrome of upper motor neuron–predominant hemiparesis. In this article, we describe a case of a middle-aged woman (initial presentation and three years follow-up) who presented with progressive ascending hemiparesis with clinically isolated upper motor neuron signs and normal sensory examination. The patient received monthly intravenous immunoglobulin (IVIG) for three years with no progression of her weakness. To our best knowledge, the response of Mills' syndrome to such an IVIG program has not been reported in the literature so far. We aim to document the clinical response to IVIG in such rare syndrome.