Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

Abstract

Objective: to identify the clinical and diagnostic investigations that may help supporting a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the EFNS/PNS electrodiagnostic criteria. Methods: we retrospectively reviewed the data from patients with a clinical diagnosis of CIDP included in a national database. Results: we included 535 patients with a diagnosis of CIDP. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in 3, while two had CISP). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies not fulfilling the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients raising to 54 (80.6%) if we also included a history of a relapsing course as a possible supportive criteria. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% versus 85.9%) Conclusions: Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.

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

“…EFNS/PNS criteria have the best combination of sensitivity and specificity but about one-fourth of patients may not satisfy electrodiagnostic criteria (EFNS/PNS sensitivity = 73%). Thus, clinical and supportive criteria play a major role in clinical practice in diagnosing CIDP when EFNS/PNS electrophysiological criteria are not satisfied [9]. Accordingly, we wondered if the inclusion of DML latency prolongation of median nerve could improve the diagnostic accuracy especially in the subset of patients not fulfilling the EFNS/PNS electrophysiological criteria.…”

“…EFNS/PNS criteria have the best combination of sensitivity and specificity but about one-fourth of patients may not satisfy electrodiagnostic criteria (EFNS/PNS sensitivity = 73%). Thus, clinical and supportive criteria play a major role in clinical practice in diagnosing CIDP when EFNS/PNS electrophysiological criteria are not satisfied [ 9 ].…”

“…The reasons why patients who did not meet the criteria were equally diagnosed as CIDP was discussed in a recent paper. To note these patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria [ 9 ], and we compared demographic features among these classes.…”

Could posturography be a useful outcome measure for chronic inflammatory demyelinating polyneuropathy?

“…CIDP is treated with immune modulating or immune suppressive treatments with the goal of improving muscle strength and sensation as well as improving daily function. Although between 80% and 90% of patients respond to first‐line treatments, both CIDP mis‐diagnosis (representing more than 47% of those that carry a diagnosis of CIDP) and drug free remission in properly diagnosed patients (representing about 31% of CIDP patients) are common 6‐15 . In both of these groups, immunotherapy and treatment decisions are heavily influenced by subjective perception of benefit.…”

“…Although between 80% and 90% of patients respond to first-line treatments, both CIDP mis-diagnosis (representing more than 47% of those that carry a diagnosis of CIDP) and drug free remission in properly diagnosed patients (representing about 31% of CIDP patients) are common. [6][7][8][9][10][11][12][13][14][15] In both of these groups, immunotherapy and treatment decisions are heavily influenced by subjective perception of benefit. This practice can lead to months or years of immunotherapy administration even without any objective evidence of benefit, but with an accumulation of the costs, risks, and burdens of the treatment.…”