Pecularities of Atypical Forms of Chronic Inflammatory Demyelinating Polyneuropathies
摘要
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an acquired, immune-mediated neuropathy characterized by chronic, progressive, or relapsing motor and sensory dysfunction caused by peripheral nerve demyelination. The mechanism of nerves damage is presumed to be immune mediated. Despite advances in diagnosis, CIDP remains a diagnostic challenge and is sometimes confirmed only by the patient's response to specific immunomodulatory therapies. Atypical forms account for approximately 50% of all CIDP cases and are more frequently identified with the aid of supplementary diagnostic methods. The aim of this study was to establish clinical and laboratory criteria for the diagnosis of atypical CIDP forms. Two patient groups were analyzed: 30 with typical CIDP and 30 with atypical CIDP. All participants underwent nerve conduction studies, blood biochemical testing, serum protein electrophoresis, and immunofixation. Functional disability was assessed using the Overall Neuropathy Limitations Scale (ONLS). In typical CIDP, the degree of functional disability correlated directly with the severity of upper limb muscle weakness. In contrast, patients with atypical CIDP demonstrated predominant impairment of proprioception and deep sensory modalities, with relative preservation of muscle strength. Nerve conduction studies are not considered a gold standard for diagnosing atypical sensory CIDP. However, predictive equations derived from multiple regression analysis may help forecast the progression of disability in CIDP patients.