Chronic Inflammatory Demyelinating Polyneuropathy Presenting with Recurrent Ataxia, Bulbar Weakness, and Respiratory Dysfunction (P06.135)

Abstract

Objective: To report a case of chronic inflammatory demyelinating polyneuropathy in a patient presenting with recurrent bouts of ataxia, bulbar weakness, and respiratory dysfunction. Background Chronic inflammatory demyelinating polyneuropathy (CIDP) can have a variable presentation leading to delay in diagnosis and treatment. We present a case of a 55-year-old man with recurrent bouts of ataxia and bulbar weakness as his initial presentation of CIDP. Design/Methods: A literature search was performed to look for comparable reports. Results: A 55-year-old diabetic man developed progressive ataxia, fatigue, dysarthria and dysphagia over a six-month period. He then presented to the hospital with acute bilateral upper extremity weakness and respiratory distress requiring intubation. He was found to be areflexic except at the biceps tendon and had difficulty weaning the ventilator. An MRI of the brain as well as initial CSF studies were normal. The patient was presumed to have a variant of Guillain-Barre syndrome and was treated empirically with plasmapheresis. He improved and was extubated. He went on to develop neck extensor weakness, followed by two further episodes of ataxia and respiratory failure at 6 and 9 months thereafter. Each time, he was treated with plasmapheresis and improved. Repeat CSF analysis at 9 months showed 1 RBC, 2 WBCs, 92 glucose, and 60 protein. Complete metabolic, autoimmune, infectious, and paraneoplastic workup was negative. Anti-GQ1b antibodies were negative. Acetylcholine receptor and anti-musk antibodies were negative. Nerve conduction study showed mild to moderate, motor and sensory, mixed axonal and demyelinating polyneuropathy. EMG showed evidence of denervation/reinnervation changes. Single fiber EMG demonstrated mildly increased jitter, though not in the neuromuscular junction disorder range. The patient was started on prednisone and is currently doing well. Conclusions: Chronic inflammatory demyelinating polyneuropathy can have a variable presentation and should be considered in patients with recurrent, unexplained ataxia and bulbar weakness. Disclosure: Dr. Malka has nothing to disclose. Dr. Muntean has nothing to disclose.