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Chronic Inflammatory Demyelinating
Polyradiculoneuropathy 101—Pitfalls and
Pearls of Diagnosis and Treatment
Said R Beydoun, 1 Thomas H Brannagan III, 2 Peter Donofrio, 3 Carol Lee Koski 4 and Eric Lancaster 5
1. Neuromuscular Division, Keck Medical Center of University of Southern California, US; 2. Department of Neurology, Neurological Institute,
Columbia University, New York, New York, US; 3. Vanderbilt University Medical Center, Nashville, Tennessee, US; 4. GBS/CIDP Foundation
International, Narberth, Pennsylvania, US; 5. The University of Pennsylvania, Philadelphia, Pennsylvania, US.
C hronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which is caused by demyelination of the peripheral nerves, is
characterized by progressive weakness and impaired sensory function in the arms and legs. CIDP is a treatable condition in which early
diagnosis is crucial to limit chronic disability. CIDP can mimic other neuropathies and it is important to identify these in order to ensure
prompt treatment. Patients with other causes of neuropathy should be suspected of having CIDP if there is rapid progress or proximal weakness.
Intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange are first-line therapies. The IVIG CIDP Efficacy (ICE) trial, the largest trial
reported of any CIDP treatment, demonstrated that IVIG therapy reduced disability and functional impairment, as well as improved quality of life.
Autoantibodies against membrane proteins of the peripheral nerve axons or the myelin sheath have been reported recently, and an improved
understanding of antibody responses in CIDP may enable the development of future targeted therapeutic interventions.
Keywords Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP),
differential diagnosis, intravenous immunoglobulin (IVIG), corticosteroids,
Disclosure: Said R Beydoun has received research grants from CSL Behring and is a
consultant/speaker for Grifols and Baxalta. Thomas H Brannagan II has served as a
consultant/speaker for Grifols, a consultant for Shire and has received clinical trial
support from Novartis. Peter Donofrio has served on advisory boards for CSL Behring,
UCB Pharma and Grifols. Carol Lee Koski has served as a speaker for Grifols and CSL
and as a Medical Advisor for GBS CIDP FI. She is also a Member of Safety committee
for CSL on SubQ trial CIDP. Eric Lancaster has received grant support and course
teaching from Grifols, Inc. He has carried out consulting for Amgen, Jaansen,
Medimmune, and has been involved in writing expert reports and testifying for the
Vaccine Injury Compensation program.
Authorship: All named authors meet the International Committee
of Medical Journal Editors (ICMJE) criteria for authorship of this
manuscript, take responsibility for the integrity of the work as a whole,
and have given final approval to the version to be published.
Open Access: This article is published under the Creative Commons Attribution
Noncommercial License, which permits any noncommercial use, distribution,
adaptation, and reproduction provided the original author(s) and source are given
Received: November 10, 2016
Accepted: January 05, 2017
Citation: US Neurology, 2017;13(1):18–25
Corresponding Author: Said R Beydoun, USC Health Care Consultation Center II,
1520 San San Pablo Street, Suite 3000, Los Angeles, California, US 90033.
Support: The publication of this article was supported by Grifols. The views and
opinions expressed in the article are those of the authors and not necessarily those of
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is
an autoimmune demyelinating polyradiculoneuropathy characterized by
chronically progressive weakness and impaired sensory function in the
lower and upper extremities. 1 Symptoms, which are progressive over at
least 8 weeks, may include weakness of the arms and legs (both proximal
and distal), loss of vibration and joint position sense, poor balance,
numbness, paresthesias, and loss of deep tendon reflexes (areflexia).
Cranial nerves (other than cranial nerve V or VII) and autonomic functions
are generally preserved. 2 The phenotype of symmetrical proximal and
distal motor and sensory symptoms and signs define typical CIDP. Atypical
CIDP includes other clinical presentations, such as asymmetric, multifocal
motor and sensory symptoms, distal sensory, or predominantly motor or
sensory types. Moreover, up to 16% of patients with CIDP may demonstrate
acute-onset CIDP, which is characterized by a rapidly progressive onset
within 8 weeks. 3,4
The exact mechanisms that underlie the development of CIDP have not
been elucidated fully, although evidence suggests likely contributions by
both cellular and humoral factors. It is twice as common in men, with
increasing frequency after age 60, although it can occur at any age. 5 The
incidence and prevalence of CIDP have been estimated at 1.6/100,000/year
to 8.9/100,000, respectively. 6 There are many phenotypic variants of CIDP
(Table 1), which suggests that the disorder may not be a discrete entity, but a
spectrum of conditions. 7 Elevated levels of cerebrospinal fluid (CSF) protein
are present in the majority of patients although normal CSF results do not
exclude the diagnosis of CIDP. 7,8 Currently, there are no well-established
biomarkers, although autoantibodies to contactin-1 and neurofascin-155
define CIDP subsets of patients with specific clinical features. 9
Diagnosis Early diagnosis is vital for this treatable condition in order to limit disability
as a result of secondary axonal damage. Initial diagnostic criteria, including
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