Three of these cases, 1 with low-positive ANA and anti-Ro, 1 with anti-sulfo-glucuronyl paraglo-boside IgG, anti-myelin-associated glycoprotein IgG, and the final patient with a monoclonal gammopathy, responded to immunotherapy.6,8,14 One patient had apparent stabilization of symptoms,6 1 had partial response of weakness and sensory impairment but thereafter had worsening respiratory symptoms,8 and in the last patient, intravenous immunoglobulin relieved her facial numbness and improved the blink reflex, but motor symptoms worsened. Here, CALR is linked to monoclonal gammopathy.