Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune inflammatory polyneuropathy characterized by a progressive or relapsing course. It has been reported that approximately 16% of CIDP patients may present with an acute clinical onset much like Guillain-Barré syndrome (GBS) but with a subsequent chronic progression. These patients are classified as acute-onset CIDP (A-CIDP). In CIDP, bulbar and ventilator involvement is uncommon. We report a case of a 56-year-old man in previous good health who developed progressive paresthesia and weakness in the limbs within 2 weeks. The diagnosis of A-CIDP was made. The patient's course fluctuated greatly over 5 months and he developed oropharyngeal weakness and ventilatory insufficiency that led to a tracheostomy. He slowly improved after IVIG treatment in combination with rituximab but required mechanical ventilatory support for 3 months. One month later he was able to perform all activities of daily living independently.
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