Acute-Onset CIDP

Smitha recently presented a great case of what at first looked like Guillain-Barré syndrome but turned out to be CIDP. There was some discussion of AIDP vs. SIDP vs. CIDP.

I’ve been reading the recent Continuum on peripheral neuropathy and came across a term I hadn’t heard before: Acute-onset CIDP, or A-CIDP. They applied this term to patients who reach plateau within 4 weeks, but then relapse more than once or continue to progress or require maintenance immunomodulatory therapy.

The Continuum article referenced a paper that looked at the differences between AIDP and A-CIDP. The bottom line was that sensory abnormalities were more common in the latter, whereas electrophysiology didn’t distinguish them.

AIDP vs. A-CIDP Table

The paper didn’t address the question of whether A-CIDP was particularly likely to be associated with an underlying systemic pathology. Perhaps we can get some input on that from our neuromuscular colleagues.

What? You thought all we’re going to talk about is stroke? This is a full-service residency blog!

About Justin A. Sattin

I'm a vascular neurologist and residency program director. I created this blog in order to share some thoughts with my resident and other colleagues, and to foster my own learning as well.
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2 Responses to Acute-Onset CIDP

  1. Elie says:

    The patient didn’t plateau within 4 weeks. He presented at least 4 weeks after the onset of symptoms and he was still worsening. The progression was halted by IVIG then he relapsed. So I think he rather has classical CIDP than A-CIDP. Do we need to take into consideration that A-CIDP exists when counseling a patient about AIDP?

    • Justin A. Sattin says:

      Thanks for the clarification. Let’s see if we get some neuromuscular input about this A-CIDP entity. One thing a mentor taught me back in the day was to beware people who make up new acronyms. The question is whether A-CIDP is something that the neuromuscular community has embraced, vs. something an investigator put out there but no one else uses.

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