Published in:
Open Access
01-04-2021 | Guillain-Barré Syndrome | Original Communication
Underdiagnosis and diagnostic delay in chronic inflammatory demyelinating polyneuropathy
Authors:
Umair J. Chaudhary, Yusuf A. Rajabally
Published in:
Journal of Neurology
|
Issue 4/2021
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Abstract
Background
The frequency and causes of underdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) are uncertain. We aimed to assess the frequency and electroclinical features of pre-referral CIDP underdiagnosis and the duration of delay prior to diagnosis and treatment initiation in a tertiary specialist clinic.
Methods
We retrospectively investigated 60 consecutive patients attending our Inflammatory Neuropathy Service, between 2015 and 2019, with a final diagnosis of treatment-responsive definite/probable CIDP. We reviewed the clinical and electrophysiological data in light of European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines and determined the frequency, causes and delay in diagnosis of CIDP.
Results
An initial alternative diagnosis to that of CIDP had been made in 68.3% (41/60) of patients. The commonest alternative diagnosis was of Guillain–Barré syndrome (GBS) in 23.3% (14/60) patients. Non-GBS underdiagnoses (27/60; 45%) mainly consisted of genetic neuropathy (8/27; 29.6%), diabetic neuropathy (5/27; 18.5%) and chronic idiopathic axonal polyneuropathy (4/27; 14.8%). Non-GBS underdiagnoses were predominantly due to non-recognition of proximal weakness (70.4%), multifocal deficits (18.5%) or proprioceptive loss (7.4%). Electrophysiological misinterpretation was contributory to pre-referral non-GBS underdiagnoses of CIDP in 85% of patients. Mean diagnostic delay in patients with non-GBS underdiagnoses of CIDP was of 21.3 months (range 2–132 months).
Conclusion
Underdiagnosis of CIDP is frequent and may lead to significant diagnostic and treatment delay. We suggest that lack of comprehensive and precise attention to typical electroclinical features of CIDP and its diagnostic criteria at the time of initial evaluation are equally contributory to underdiagnoses.