Winning Abstracts from the 2006 National Medical Student Abstract Competition: A Delayed Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy.
Author: Rachelle Huang, Michigan State University, 2007
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an underdiagnosed neuropathy. CIDP has a chronic presentation similar to Guillain-Barre. CIDP can be easily misdiagnosed due to its protean presentations. Corticosteroids have been a mainstay treatment of CIDP. Recently, intravenous immunoglobulin (IV Ig) has been used to treat CIDP.
A 56 year old Caucasian man was brought to the emergency room because of bilateral proximal leg weakness. In two weeks, the weakness had increased to the extent that he could not even lift his legs off of his bed. Complaining of baseline weakness since the 1980s, he had lost his job as a machinist due to this weakness. After that initial episode, symptoms eventually improved after he was treated with steroids for two years. He continued to have periods of improvement after he received single doses of steroids before he returned to baseline weakness. Subsequently, he suffered from herniated discs (lumbar 3-5) in the 1990s, and after disectomy developed right foot drop and permanent weakness. His neurological deficits were attributed to the disectomy and occupational exposures. At this presentation, his weakness was worse than baseline. The patient was given Decadron 8mg on his second day of admission and throughout his hospitalization. Electromyography revealed chronic reinervation and denervation. A lumbar puncture showed an absolute increased and relative increased protein from a previous lumbar puncture. Based on the objective findings and chronic history of waxing and waning weakness, CIDP was diagnosed. Since CIDP is an autoimmune demyelination disorder, the patient was started on IV Ig 2g/kg. His symptoms improved dramatically. His legs “felt warmer” by the second day of IV Ig. Previously areflexic, a patellar reflex of 1/5 was present on the third day. By the fourth day, the patient was lifting his legs off the bed, able to stand, and walk around his room. One week after the cessation of IV Ig, the patient was walking upstairs.
CIDP can often be misdiagnosed due to the broad differential diagnosis of weakness. In this patient, his occupational hazards, and weakness following disc surgery confused the diagnosis. His intermittent treatment with steroids temporarily ameliorated his symptoms. Currently, a combination therapy of IV Ig and Decadron decreased his symptoms although long term effects of this regimen remain unknown. This combination bolus helped achieve a dramatic improvement.
A detailed history is important in chronic neurological issues to correctly diagnose CIDP. Symptoms of the disease may not fit the criteria of CIDP initially, but repeated evaluations and a careful examination of history can make the correct diagnosis of CIDP and allow initiation of appropriate treatment.
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