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Review
. 2020 Dec 5;143(11):3214-3224.
doi: 10.1093/brain/awaa265.

Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy

Filip Eftimov  1 , Ilse M Lucke  1 , Luis A Querol  2   3 , Yusuf A Rajabally  4 , Camiel Verhamme  1
Affiliations
Review

Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy

Filip Eftimov et al. Brain. .

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consists of a spectrum of autoimmune diseases of the peripheral nerves, causing weakness and sensory symptoms. Diagnosis often is challenging, because of the heterogeneous presentation and both mis- and underdiagnosis are common. Nerve conduction study (NCS) abnormalities suggestive of demyelination are mandatory to fulfil the diagnostic criteria. On the one hand, performance and interpretation of NCS can be difficult and none of these demyelinating findings are specific for CIDP. On the other hand, not all patients will be detected despite the relatively high sensitivity of NCS abnormalities. The electrodiagnostic criteria can be supplemented with additional diagnostic tests such as CSF examination, MRI, nerve biopsy, and somatosensory evoked potentials. However, the evidence for each of these additional diagnostic tests is limited. Studies are often small without the use of a clinically relevant control group. None of the findings are specific for CIDP, meaning that the results of the diagnostic tests should be carefully interpreted. In this update we will discuss the pitfalls in diagnosing CIDP and the value of newly introduced diagnostic tests such as nerve ultrasound and testing for autoantibodies, which are not yet part of the guidelines.

Keywords: CIDP; diagnostic accuracy; diagnostic pitfalls; misdiagnosis; underdiagnosis.

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Figures

Figure 1
Figure 1
A conceptual framework for a diagnostic work-up in chronic autoimmune neuropathies. A conceptual framework for a diagnostic work-up in chronic auto-immune neuropathies, assuming future emphasis on immunological tests with high specificity to show evidence for autoimmunity. Combinations and number of tests required for diagnosis depend on specificity of clinical phenotypes, of immunological tests and of supportive tests, such as nerve conduction studies, imaging, CSF examination, pathology and response to treatment. NCS currently have the best diagnostic accuracy.
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