Abstract
IntroductionElectrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging. Thus, with the aim ofproviding some practical advice in electrophysiological approach to a patient with suspected CIDP, we analyzed electrophysiological data from 499 patients enrolled inthe Italian CIDP Database.MethodsWe calculated the rate of each demyelinating feature, the rate of demyelinating features per nerve, the diagnostic rate for upper andlower limb nerves, and, using a ROC curve analysis, the diagnostic accuracy of each couple of nerves and each demyelinating feature, for every CIDP subtype.Moreover, we compared the electrophysiological data of definite and probable CIDP patients with those of possible and not-fulfilling CIDP patients, and by a logisticregression analysis, we estimated the odds ratio (OR) to make an electrophysiological diagnosis of definite or probable CIDP.ResultsThe ulnar nerve had the highestrate of demyelinating features and, when tested bilaterally, had the highest diagnostic accuracy except for DADS in which peroneal nerves were the most informative.In possible and not-fulfilling CIDP patients, a lower number of nerves and proximal temporal dispersion (TD) measurements had been performed compared to definiteand probable CIDP patients. Importantly, OR for each tested motor nerve and each TD measurement was 1.59 and 1.33, respectively.ConclusionOur findingsdemonstrated that the diagnosis of CIDP may be missed due to inadequate or incomplete electrophysiological examination or interpretation. At the same time, thesedata taken together could be useful to draw a thoughtful electrophysiological approach to patients suspected of CIDP.
Highlights
Electrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging
We divided our dataset according to electrophysiological data: 352/499 patients (70.6%) were classified as definite CIDP, 10/499 (2%) as probable CIDP, 57/499 (11.4%) as possible CIDP, and 80/499 (16%) as CIDP not fulfilling the EFNS/PNS electrophysiological criteria [6]
For patients that did not meet EFNS/PNS electrophysiological criteria for demyelination, the diagnosis of CIDP was discussed among the neurophysiologist panel, the database coordinator center, and the physician who had enrolled the patient
Summary
Electrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic and disabling disease with a range prevalence of 0.8–8.9 cases per 100,000 and incidence of 0.2–1.6/ 100,000 [1]. It is the most frequent acquired immunemediated chronic neuropathy and has a broad spectrum of Extended author information available on the last page of the article possible clinical presentation including typical and atypical forms [2,3,4]. Diagnosis of CIDP is made by demonstrating peripheral nerve demyelination, commonly by electrophysiological testing.
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