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Background and purpose: The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria. Methods: The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed. Results: In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty‐seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill 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 rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. 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% vs. 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 in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non‐diagnostic nerve conduction studies. [ABSTRACT FROM AUTHOR]

Background and purpose: The role of lifestyle and dietary habits and antecedent events has not been clearly identified in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: Information was collected about modifiable environmental factors and antecedent infections and vaccinations in patients with CIDP included in an Italian CIDP Database. Only patients who reported not having changed their diet or the lifestyle habits investigated in the study after the appearance of CIDP were included. The partners of patients with CIDP were chosen as controls. Gender‐matched analysis was performed with randomly selected controls with a 1:1 ratio of patients and controls. Results: Dietary and lifestyle data of 323 patients and 266 controls were available. A total of 195 cases and 195 sex‐matched controls were used in the analysis. Patients eating rice at least three times per week or eating fish at least once per week appeared to be at decreased risk of acquiring CIDP. Data on antecedent events were collected in 411 patients. Antecedent events within 1–42 days before CIDP onset were reported by 15.5% of the patients, including infections in 12% and vaccinations in 1.5%. Patients with CIDP and antecedent infections more often had an acute onset of CIDP and cranial nerve involvement than those without these antecedent events. Conclusions: The results of this preliminary study seem to indicate that some dietary habits may influence the risk of CIDP and that antecedent infections may have an impact on the onset and clinical presentation of the disease. [ABSTRACT FROM AUTHOR]

Background and purpose: There are other options open to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are non-responders to conventional treatment, including immunosuppressive and immunomodulatory agents (IA). The aim of this study was to assess whether the use of IA is able to increase the number of responders. Methods: Clinical and electrophysiological data of patients with refractory CIDP, followed at 10 Italian centres, were collected, and the clinical outcome (Rankin Scale) and drug side effects (SE) for the different therapies were analysed. Results: A total of 110 patients were included. These patients underwent 158 different therapeutic procedures with IA. Seventy-seven patients were treated with azathioprine, 18 rituximab, 13 cyclophosphamide, 12 mycophenolate mofetil, 12 cyclosporine, 12 methotrexate, 11 interferon-alpha and three interferon beta-1a. The percentage of patients who responded to azathioprine (27%) was comparable to the percentage of responders to other therapies, after the exclusion of interferon beta-1a that was not effective in any of the three patients treated. The percentage of SE ranges from 8% (methotrexate) to 50% (cyclosporine). Conclusions: One-fourth of patients, refractory to conventional treatment, showed an improvement in their disability with IA. Methotrexate had the lowest SE; cyclosporine was associated with severe SE and often led to drug discontinuation. [ABSTRACT FROM AUTHOR]

Background and purpose: The guidelines for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) therapy suggest to use immunoglobulins (IVIg) and steroid as first-line therapies. Patients who do not respond to one of the two drugs should be switched to the other drug. We collected therapeutic outcome data in patients followed at 11 centres in order to document the clinical practice in Italy. Methods: Clinical and electrophysiological data of patients with CIDP were entered into a central database. The clinical outcome (Rankin Scale) and drug side effects (SE) for first- and second-line therapies were recorded. Results: A total of 267 patients were included. The percentage of responders (R) to first-line therapy [steroid or IVIg or plasma exchange (PE)] was 69%; this number increased to 81% when patients who switched to different therapies were included. Overall, the percentage of R to IVIg was similar to R to steroids ( P = 0.07) and higher than R to PE ( P < 0.001). Of the main therapies, PE frequently caused SE (19%), followed by steroids (12.5%) and IVIg (4%). Conclusions: Switching between traditional therapies increases the number of responder patients. IVIg was confirmed to be a therapy with low SE. [ABSTRACT FROM AUTHOR]

The article discusses self-reports and behavior in social psychology. A variable that motivates a person to resolve cognitive inconsistencies, including attitude-behavior inconsistencies, is suggested in a theory of objective self-awareness. The theory implies that a number of motivational effects can follow when self-focused attention is induced, no matter how that state of attention comes about. A self-focused person will be motivated to bring these potentially disparate elements into alignment, and given that the behavioral element is relatively fixed, this consistency can be brought about most easily by altering the self-report. Based on a theory of objective self-awareness it was proposed that test invalidity results when a person's attention is not adequately focused on his test-taking behavior. The central theoretical idea is that a person's self-reports will be more introspective and accurate when environmental stimuli lead him to focus attention on himself. This introspectiveness or self-evaluation will produce answers consistent with past behavior and predictive of future behavior. In the three experiments a variety of self-report forms proved to be more valid, using actual behavior as a criterion, when subjects made their self-reports while confronted with their mirror images.

We describe a 65-year-old smoker male followed for five years for a pure motor demyelinating peripheral neuropathy. The patient had a monthly motor relapse with severe weakness restricting him to a wheelchair, so he needed monthly high dose IVIg. On EMG the MCV were very slowed (30 m/sec) without evidence of conduction blocks while SCV were in the normal range. CSF disclosed a high protein level. Laboratory findings did not reveal any other abnormality except for the presence of monoclonal gammopathy IgMk and high titer anti GD1a serum IgM antibodies (1:5000). In March 2003 he had the most severe relapse with flaccid tetraplegia and respiratory failure so severe that he required ventilatory support. A total body CT scan revealed a nodular lung lesion with diffuse lymphangiitis. Biopsy disclosed a lung adenocarcinoma with a severe infiltration of CD8 cells. Surgical eradication of the tumor caused the last severe relapse. At the moment the patient is relapse-free and no more treatment was administered. The clinical course of the motor demyelinating relapsing neuropathy suggests a possible paraneoplastic pathogenesis of the neurological illness also supported by the severe inflammatory infiltration of the tumor. [ABSTRACT FROM AUTHOR]