Underdiagnosis and diagnostic delay in chronic inflammatory demyelinating polyneuropathy
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ORIGINAL COMMUNICATION
Underdiagnosis and diagnostic delay in chronic inflammatory demyelinating polyneuropathy Umair J. Chaudhary1 · Yusuf A. Rajabally1,2 Received: 20 July 2020 / Revised: 8 October 2020 / Accepted: 20 October 2020 © The Author(s) 2020
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. Keywords Chronic inflammatory demyelinating polyneuropathy · Diagnostic delay · Guillain–barre syndrome · Underdiagnosis
Introduction Chronic inflammatory demyelinating polyneuropathy (CIDP) is a progressive motor and/or sensory neurological condition affecting peripheral nerves. The prevalence of CIDP ranges between 1.6 and 7/100,000 population and may vary based on the diagnostic criteria used [1, 2]. CIDP can have multiple relapses causing significant disability in
* Yusuf A. Rajabally [email protected] 1
Inflammatory Neuropathy Clinic, Department of Neurology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK
Aston Medical School, Aston University, Birmingham, UK
2
affected population if left untreated. Hence, timely and correct diagnosis and treatment is of paramount importance. The issue of overdiagnosis of CIDP i.e., patients receiving an erroneous diagnosi
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