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The aim of the present study was to determine the severity and distribution of assessed muscle weakness and to relate muscle performance to measures of function and quality of life in long-term chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Fourteen patients with 8.7 years (3.3–11.5) of confirmed CIDP consecutively referred to the referral center for CIDP patients at Aarhus University Hospital, Denmark, during the period 1992–2002 were compared with matched healthy controls. The main outcome parameter was muscle performance assessed with isokinetic dynamometry. Overall disability sum score (ODSS), neurological symptom score (NSS), neuropathy impairment score (NIS), health-related quality-of-life survey (SF-36), nerve conduction studies, physical fitness, hand and walking performance, and quantitative sensory testing were secondary variables. The mean (95% CI) isokinetic strength of all measured muscles was reduced by 19.4% (5.9–32.8%) (p < 0.01). In the legs, distal weakness was predominant, strength at ankle being 37.0% (14.7–59.2%) reduced. Isokinetic strength was closely related to manual muscle strength, ODSS, NIS, walking performance, and physical components of SF-36. In conclusion, isokinetic strength relates to measures of function, impairments, gait performance, and physical components of health-related quality of life in long-term CIDP. Furthermore, a detailed characterization of severity and distribution of weakness has been provided using this technique. [ABSTRACT FROM AUTHOR]

Introduction: The effect of long-lasting immune-modulating therapy was studied in patients with chronic inflammatory demyelinating polyneuropathy (CIDP)., Methods: A population-based, cross-sectional study of treated patients referred to the Danish health-care system between 1985 and 2006., Results: The 51 participating patients had a median disease duration of 16 (interquartile range, 14-21) years. Twenty-seven patients (53%) had discontinued therapy and 46 walked independently. Disability and isokinetic strength were impaired by 17% and 20%, respectively, as compared with matched control subjects. For a few patients long-term CIDP was associated with severe morbidity (6%) and even mortality (1%). Prolongation of time until start of therapy was associated with an increased burden of long-term disability., Discussion: Long-term prognosis in treated CIDP is characterized by limited disability in the majority of patients. Disability is related to delay of therapy. Therefore, more attention should be given to early treatment start in CIDP., (© 2019 Wiley Periodicals, Inc.)