Kohortenanalyse von Patienten mit chronischer Immunneuropathie an der Klinik für Neurologie
Doctoral thesis
Date of Examination:2025-12-02
Date of issue:2025-11-12
Advisor:Prof. Dr. Jens Schmidt
Referee:Prof. Dr. Jens Schmidt
Referee:Prof. Dr. David Liebetanz
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Abstract
English
Chronic immunoneuropathies, particularly Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP), are rare disorders characterized by potentially severe neurological impairment. Given the risk of irreversible long-term consequences, early and accurate diagnosis as well as timely initiation of appropriate therapy are crucial. This study aimed to (1) characterize a comparatively large cohort of patients, (2) identify parameters predictive of disease progression, (3) evaluate the correlation and predictive value of standardized clinical scales (INCAT, MRC, and ROD-B/ROD-S) with electrophysiological parameters, and (4) determine whether disease characteristics differ between male and female patients, considering previously reported epidemiological distinctions. A total of 172 patients treated for suspected or confirmed immunoneuropathies at the Department of Neurology, University Medical Center Göttingen (UMG), between 1996 and 2020 were retrospectively analyzed. Data extracted from medical records included diagnostic results such as clinical scales and electrophysiological findings, as well as therapeutic interventions. The cohort was first analyzed descriptively (Part A). To model disease progression (Part B), mixed logistic regression was applied, while mixed linear regression was used to assess the predictive value of the three clinical scales (Part C). Finally, disease characteristics of male and female patients (Part D) were compared using independent t-tests and Mann–Whitney U tests. Our findings provide a comprehensive characterization of 172 patients, 71% of whom were male, and 77% of whom were diagnosed with CIDP. Female sex was significantly associated with earlier disease progression. Additionally, progression correlated significantly with changes in demyelinating parameters on electrophysiological testing. The three clinical scales (INCAT, MRC, and ROD-B) were highly correlated, indicating that they reflect comparable levels of functional impairment. Moreover, MRC and INCAT scores demonstrated significant correlations with demyelinating electrophysiological parameters. In summary, this study offers a detailed characterization of a large cohort of patients with chronic immunoneuropathies, consistent with findings from previous studies. Our results support the use of the three clinical scales (INCAT, MRC, ROD-B) as reliable tools for objectively assessing clinical status, complementary to electrophysiological measures. Although sex-specific differences in disease progression were observed, prospective studies with standardized assessments are needed to validate potential clinical and therapeutic implications. The data collected herein serves as an important foundation for improving future studies on chronic immunoneuropathies.
Keywords: Neurology; CIDP; chronic immunoneuropathies; characterization; retrospective analysis; sex-specific differences; INCAT; MRC; ROD-B; progression
