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Washington University Experience | PERIPHERAL NEUROPATHY | 7B CIDP | 9A0 Case 9 History
Case 9 History ---- The patient is a 74 year old female who presents with distal paresthesias, progressive weakness, falls, difficulty swallowing and respiratory failure. In late spring 2014, she developed numbness and tingling in her feet, and fingers that progressed over a week to her elbows and knees. She was feeling weaker and began having falls during early May. In addition, she started having difficulty going up stairs. In July 2014, she noted difficulty swallowing, felt more short of breath and walked shorter distances. In July 2014 she developed profound weakness that progressed over the course of 24 hours, greater in her lower extremities than upper extremities. She had an aspiration event and was intubated but was extubated after 1 day. She started to receive IVIg (one of 5 planned doses at 0.4 g/kg) but developed fever and that treatment was discontinued. Late in July 2014 the patient had a hypoxic arrest on arrival and was resuscitated after at least 5 minutes. The etiology was felt to be severe mucus plugging. EMG-NCS showed sural sparing and a primary demyelinating neuropathy with some axonal features and "myopathic" changes on EMG felt to likely represent changes associated with distal axon loss/demyelinating disease. A repeat study in August 2014 showed an acquired primary demyelinating peripheral neuropathy. She had several complications during the July hospitalization but ultimately was discharged to a skilled nursing facility in mid-August. She was readmitted in September for profoundly worsened weakness and sensory loss thought to be due to subacute CIDP, for which she received plasma exchange and IVIg. The hospital course was complicated by multiple transfers to the ICU for respiratory failure, thought to be secondary to mucous plugging and later secondary to a pulmonary embolus; recurrent urinary tract infections; left vocal cord paralysis; and bilateral pleural effusions. Operative procedure: Left deltoid muscle biopsy and left sural nerve biopsy.
