The neurophysiological lesson from the Italian CIDP database

Abstract

Introduction Electrophysiological 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. Methods We 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. Results The 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. Conclusion Our 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.

Motor Nerve Conduction Block Estimation in Demyelinating Neuropathies by Deconvolution

“…Discriminating among TD and CB is fundamental to refine the clinical picture and to design properly the treatment. Moreover, an estimation insensitive to TD could allow to redefine the diagnostic standards for the assessment of CB [ 27 , 30 ], which are still largely conservative in order to face the possible bias affecting amplitude and area approaches [ 9 , 18 ]. A more accurate and robust estimation of CB could also allow for tracking the patient’s response to a treatment or for better stratification of different patients.…”

“…A retrospective study was performed on CIDP and MMN patients (followed from a clinical, electrophysiological and therapeutic point of view by D.C., following the normal clinical practice carried out at “I.R.C.C.S. Istituti Clinici Scientifici, S. Maugeri Foundation, Pavia, Italy”), which are part of a more relevant case series contained in the Italian CIDP Database and Italian MMN Database [ 7 , 27 ]. Diagnostic criteria were defined in [ 40 ] for CIDP and in [ 41 ] for MMN.…”

“…Thus, with the intent of extending the promising results shown in [ 19 ] to neuropathic patients with abnormal TD of the APs, in this paper SAW has been included in the deconvolution kernel. The method was then tested on experimental signals from both healthy subjects and pathological patients, with either Chronic Inflammatory Demyelinating Polyneuropathy (CIDP [ 1 , 7 , 26 , 27 , 28 , 29 , 30 ]) or Multifocal Motor Neuropathy (MMN [ 26 , 28 , 29 , 31 , 32 ]). Our results are expected to contribute to the discussion on guidelines [ 7 , 27 , 30 ] and the proposal of innovative methods for the assessment of CB [ 1 , 31 ].…”

“…The method was then tested on experimental signals from both healthy subjects and pathological patients, with either Chronic Inflammatory Demyelinating Polyneuropathy (CIDP [ 1 , 7 , 26 , 27 , 28 , 29 , 30 ]) or Multifocal Motor Neuropathy (MMN [ 26 , 28 , 29 , 31 , 32 ]). Our results are expected to contribute to the discussion on guidelines [ 7 , 27 , 30 ] and the proposal of innovative methods for the assessment of CB [ 1 , 31 ].…”

Prolonged distal motor latency of median nerve does not improve diagnostic accuracy for CIDP

“…Diagnosis of CIDP relies on clinical signs and symptoms confirmed by electrophysiological test demonstrating peripheral nerve demyelination, according to the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) criteria. Supportive criteria can assist the diagnosis [ 4 , 5 ].…”